and Journal on Developmental Disabilities

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and Journal on Developmental Disabilities
Journal on Developmental Disabilities 2012 vol.18 n.2 Le journal sur les handicaps du développement
La publications officielle de l’association ontarienne
sur les handicaps du développement
The official publication of the
Ontario Association on Developmental Disabilities
Special joint issue: International Journal of Down Syndrome
(formerly DSQ) and Journal on Developmental Disabilities
vol.18, n.2
2012
Editorial Policy
Journal on Developmental Disabilities / Le journal sur les handicaps du développement
published by the Ontario Association on Developmental Disabilities, Toronto, ON
ISSN: 118-9136
© Ontario Association on Developmental Disabilities
The Journal on Developmental Disabilities is published three times yearly by the Ontario Association
on Developmental Disabilities (OADD). The Association has established the following objective for
the journal:
To foster and promote thoughtful and critical dialogue about relevant issues in the field of developmental
disabilities, including those broader social issues that impact on persons with developmental disabilities.
Content
Each issue features a selection of research, conceptual, informational, and editorial papers. An
issue may have a central theme. Reviews of relevant books, movies, websites, software, and other
resources are welcomed, as are letters to the editor. In this way we strive to collectively enrich
our understanding of issues, encourage stimulating debate among those working in the field, and
improve services.
Down Syndrome International is a supporter of the International Journal
of Developmental Disabilities (formerly Down Syndrome Quarterly)
Language
Submissions are invited in either French or English and, if accepted, will be printed in the
language of submission with an abstract in the other language. Submissions must be free of any
gender and ethic bias. People-first language (e.g., persons with developmental disabilities) must
be used throughout the manuscript (including figures and tables). Submissions must also use the
non-possessive form in eponyms (e.g., Down Syndrome not Down’s Syndrome).
Editorial Process
Each submission will undergo a peer review by two or more reviewers with relevant expertise.
The authors of the manuscripts will not be identified to the reviewers. The reviewers will
recommend for or against publication and provide their reasons. Reviewers are asked to judge the
manuscript on several criteria including its contribution to increasing our knowledge and clarity
of communication. The reviewers are asked to write in a positive and constructive manner to help
the authors improve their work, if necessary. Reviewers are asked to complete the reviews within
two weeks using electronic communication.
Final Decision
The Editor-in-Chief, or guest editor, reserves the final decision regarding publication of a
submission. To promote the publication of theme issues, the Editor reserves the right to decide in
which issue to publish the article if it has been accepted.
Copyright
Design: Ted Myerscough
Layout: Leah Gryfe
Cover Image by Angela Gillis
Marker on paper
Angela Gillis attends the Manuela Dalla-Nora Program at Tycos Centre through Vita Community Living
Services/Mens Sana. She has graced the program with her unique quirky style and her love of the arts
for 25 years. Angie loves to spend hours on her artwork and is meticulous in the placement of lines and
selection of colour that she uses, and always includes her signature within the art itself. Her art reflects her
life in that moment.
Manuscripts should not be submitted if previously published elsewhere or under review unless
explicitly requested by the Editor. Once published, the article becomes the property of OADD. If
an author wishes to re-publish materials originally published in the Journal on Developmental
Disabilities, written permission is required from OADD. To re-publish previously published
material in this journal, written permission must be obtained from the person or organization
holding the copyright to the original material.
The opinions expressed in this publication are those of the individual authors and do not necessarily
reflect the views of the Ontario Association on Developmental Disabilities / L’association ontarienne
sur les handicaps du développement.
The Journal on Developmental Disabilities greatly
appreciates the support shown by the following
in sponsoring this issue:
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living
huronia
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June 14, 2012
Chief Editorial Board
Journal On Developmental Disabilities
Ontario Association on Developmental Disabilities
2 Surrey Place
Toronto, ON M5S 2C2
Dear Members of Chief Editorial Board,
We are proud to collaborate with the Journal on Developmental Disabilities on the first joint issue of
JoDD and the new International Journal of Down syndrome (formerly Down Syndrome Quarterly).
The Down Syndrome Quarterly journal was first published in March of 1996, by then Editor in Chief,
Samuel J. Thios Ph.D. with the support of Denison University, The Nisonger Center and Ohio State
University, and ran for seven years, until 2002. The Down Syndrome Research Foundation was honoured
when asked to take over publication of the journal in 2005, and in 2006 we produced the next issue, a
special issue covering topics from the World Down Syndrome Congress which was held in Vancouver in
August of 2006. Since then Down Syndrome Research Foundation has published 12 issues with the most
recent being the double issue in 2011.
Our thanks to the Editorial Board and Staff of JoDD, and to DSQ Co-Editors Dr. Jake Burack and Dr.
Roy Brown for their efforts in assisting with this joint publication.
Yours sincerely,
Dawn A. McKenna
Executive Director
Langdon Down Centre 2A Langdon Park Teddington TW11 9PS United Kingdom The B
Board
off D
Directors
The oard o
irectors OADD OADD
2 urrey Place
Place 2 SSurrey
Toronto, ON Toronto, ON
M5S C2 M5S 22C2
CANADA CANADA
[email protected]
[email protected] 17 July 2012 To our colleagues and friends at the Journal on Developmental Disabilities, Down Syndrome International is very grateful to the Journal on Developmental Disabilities for co-­‐sponsoring this special issue of the International Journal of Down Syndrome. For us it is the first issue under the Journal's new name, formerly Down Syndrome Quarterly. We appreciate the co-­‐operation as well as your enthusiastic support and interest in Down syndrome and your recognition of the need to make clear research and applied implications arising from this important area. Knowledge transfer, particularly in developing countries, is one of our missions, as well as passing on critical research to all families with children with Down syndrome who are increasingly being accepted into the world community. This joint issue will be made available to all participants at the World Down Syndrome Congress to be held in Cape Town, South Africa from 15-­‐17 August 2012. Thank you for all your interest, support and practical help in making this happen! Yours Sincerely PENNY ROBERTSON O.A.M CHAIR OF TRUSTEES – DOWN SYNDROME INTERNATIONAL Reg Charity No. 1091843 T: +44 (0)20 8614 5124 Reg Company No. 4327941 E: [email protected]
[email protected]­‐int.org W: www.ds-int.org
www.ds-­‐int.org Table of Contents
Introduction to International Journal of Down Syndrome,
Volume 1, Issue 1 and
Journal on Developmental Disabilities, Volume 18, Issue 2
Roy I. Brown, and Jacob A. (Jake) Burack . . . . . . . . . . . . . . . . . vii
Volume 16, Number 3, 2010
Articles
Education and Cognition
Chief Editorial Board
Maire Percy,
Ann Fudge Schormans,
Lynn Martin,
Jonathan Weiss
Assistant Editors
Carly McMorris,
Melody Ashworth
Media Editor
Esther Ignagni
Editor Emeritus
Ivan Brown
OADD Board
Representative
Joe Persaud
Developing Numeracy in Young Adults with
Down Syndrome: A Preliminary Investigation of
Specific Teaching Strategies
Lorraine Gaunt, Karen B. Moni, and Anne Jobling . . . . . . . . . . 8
The Impact of Verbal and Nonverbal Development
on Executive Function in Down Syndrome and
Williams Syndrome
Oriane Landry, Natalie Russo, Tammy Dawkins,
Philip D. Zelazo, and Jacob A. Burack . . . . . . . . . . . . . . . . . . . . 24
Families
A Study of Nigerian Families Who Have a Family
Member with Down Syndrome
Paul M. Ajuwon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 34
Relations of Parental Perceptions
to the Behavioural Characteristics of Adolescents
with Down Syndrome
Meghan M. Burke, Marisa H. Fisher,
and Robert M. Hodapp . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 48
The Middle Years and Beyond: Transitions and
Families of Adults
with Down Syndrome
Nancy S. Jokinen, Matthew P. Janicki,
Mary Hogan, and Lawrence T. Force . . . . . . . . . . . . . . . . . . . . . 57
Health
Production Manager
Jennifer Shaw
Design
Ted Myerscough
Production and Layout
Leah Gryfe
High Plasma Homocysteine and Low Serum Folate Levels
Induced by Antiepileptic Drugs in Down Syndrome
Antonio Siniscalchi, Giovambattista De Sarro,
Simona Loizzo, and Luca Gallelli . . . . . . . . . . . . . . . . . . . . . . . . 68
Puberty, Menstruation and Pain in Australian and
Irish Females with Down Syndrome
Margaret R. Kyrkou, Rebecca O’Rourke,
Joan P. Murphy, Verity M. Bottroff, Dennis S. Smith,
Hilary M. C. V. Hoey, and Roy I. Brown . . . . . . . . . . . . . . . . . . 75
Book Review
book review:
Encore Edie by Annabel Lyon
Susan Petko . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76
Editorial Board
Roy I. Brown, PhD
University of Victoria
University of New England, Australia
Jacob A. Burack, PhD
McGill University
Management Co-ordinator
Dawn McKenna
Down Syndrome Research Foundation
Burnaby, British Columbia
Associate Editors
Education
Genetics
Language/Literacy
Medicine/Neurology
Perception/Cognition
Social Development
Monica Cuskelly, PhD
University of Queensland
Cheryl Wellington, PhD
University of British Columbia
Elizabeth Kay-Raining Bird, PhDIra Lott, MD
Dalhousie University
University of California, Irvine
Matthew Heath, PhDDeborah Fidler, PhD
University of Western Ontario
Colorado State University
Editorial Board
Armando Bertone, PhD
McGill University
Anne Jobling, PhD
University of Queensland
David W. Evans, PhD
Bucknell University
Joanne Nye, PhD
University of Bristol
Rhonda Faragher, PhD
Australian Catholic University
Natalie Russo, PhD
Syracuse University
Tara Flanagan, PhD
McGill University
Louis Schmidt, PhD
McMaster University
Jesus Florez, PhD
University of Cantabria
Naznin Virji-Babul, PhD
University of British Columbia
Robert Haslam, MD
Emeritus, University of Toronto
Daniel Weeks, PhD
University of Lethbridge
Robert M. Hodapp, PhD
Vanderbilt University
Jennifer Wishart, PhD
University of Edinburgh
Grace Iarocci, PhD
Simon Fraser University
Volume 18, Number 2, 2012
Authors
Roy I. Brown,
Jacob A. (Jake) Burack
Introduction to
International Journal of Down Syndrome,
Volume 1, Issue 1
and
Journal on Developmental Disabilities,
Volume 18, Issue 2
We are very pleased to have edited this collaborative issue
between the International Journal of Down Syndrome (IJDS),
formerly Down Syndrome Quarterly, and the Journal on
Developmental Disabilities (JoDD), that is being published
to mark the 11th World Down Syndrome Congress in Cape
Town, South Africa on August 15–17, 2012. This special issue
includes original research reports and research reviews
from basic science to professional practice by scholars from
around the world.
The articles, which cover the lifespan, have been grouped
into sections on education and cognition, family life, and
health. We hope that they, and this special issue, will contribute meaningfully to the literature on Down syndrome in
particular and developmental disabilities in general, and will
be a memorable and informative souvenir from the World
Congress for parents and professionals alike.
Correspondence
[email protected]
We would like to thank the chief editors of JoDD (listed in
the Table of Contents) for their generous support, advice,
and practical help in developing this joint project. Jennifer
Shaw and Leah Gryfe worked with us patiently and supportively in producing the special issue. We also wish to
acknowledge the assistance of Vanessa dos Santos, National
Executive Director, Down Syndrome South Africa for arranging distribution of the special issue to the participants of the
World Congress and the support of other trustees of Down
Syndrome International (DSI), including Penny Robertson,
Chair of Trustees, and Balbir Singh, Past President.
Best wishes to all the attendees for a successful and informative congress.
Co-Editors
Roy I. Brown, PhD
University of Victoria, Canada
University of New England, Australia
Jacob A. (Jake) Burack, PhD
McGill University, Canada
NOTE: A fully edited version of this article can be found
on the OADD website at http://www.oadd.org/index.
php?page=753 after August 15, 2012.
Developing Numeracy in Young Adults
with Down Syndrome:
A Preliminary Investigation of
Specific Teaching Strategies
Volume 18, Number 2, 2012
Authors
Lorraine Gaunt,
Karen B. Moni,
Anne Jobling
School of Education
The University of
Queensland,
Brisbane St Lucia, QLD,
Australia
Abstract
This article reports the implementation of a specific teaching
program (STP) to improve the numeracy skills (place value) of
a cohort of young adults with Down syndrome. The STP was
based on a range of teaching strategies that research had shown
were effective across various groups who have difficulties with
numeracy learning.
The participants were selected from a post school literacy program at the University of Queensland (Moni & Jobling, 2000).
The twelve participants in the program were assessed using
the Booker Profiles of Mathematics (Booker, 1995) and divided
into three groups based on these profiles. The group chosen to
participate in the STP showed the prerequisite skills in number
recognition and counting needed to learn place value but had
no prior knowledge of the concept. The other groups participated in a series of game sessions and did not receive the STP.
The data comprised pre and post testing, field notes, classroom
observations and family histories. The five participants in the
STP showed improvements in their skill acquisition, including
developing some understanding of place value.
Correspondence
[email protected]
Our findings demonstrate that individuals with Down syndrome can learn basic numeracy concepts but due to the small
sample size more research is required.
There is a commonly held educational perception that not
everyone can learn numeracy skills. Elliot and Garnett (1994)
expressed concern about the acceptance of this perception in
society, especially among parents, as this negativity would
never be tolerated about literacy; nevertheless, this attitude
has persisted in texts for special educators (Massey, Noll &
Stephenson, 1994; Rosenberg, Westling & McLeskey, 2011)
where it has been suggested that numeracy for some students,
including those with intellectual impairment, should be confined to developing functional skills for everyday life.
These attitudes persist due to the limited knowledge both
about the numeracy abilities of adults with Down syndrome
and how these abilities can be developed over time. In addition, there is limited information on the effect of specific
teaching strategies on this development (Leonard-Giesen,
2009). Other research has suggested that the development
of mathematical understanding relies on effective pedagogy based on knowledge of how students develop an understanding of mathematical concepts rather than on traditional teaching
which is often mostly teacher centred (Lowrie,
Bobis, & Mulligan, 2008). With this orientation
towards how students learn, the characteristics
of learners with Down syndrome, such as the
degree of intellectual impairment, can be taken
into account (Selikowski, 1997). Students with
Down syndrome benefit from being taught
abstract mathematical concepts with practice
and variation using concrete materials over
time (Buckley, 2007).
Added to their intellectual impairment, learners
with Down syndrome may also have sensory
impairments (vision and hearing) that could
contribute to their generalized language learning delay and affect their numeracy learning
(Hammond & Millis, 1996; Lorenz, 1998; Tiens,
1999). Correlations between language learning
delays and numerical ability have been found
by Nye, Clibbens and Bird (1995) while Buckley
(2007) and Horstmeier (2004) stated that generally their numeracy skills were approximately
two years behind their literacy skills.
The Development of Research in
Aspects of Numeracy and Down
Syndrome
There have been limited studies of numerical abilities of individuals with Down syndrome. Studies by Brown and Deloache (1978);
Cornwall (1974); Gelman (1982) and Gelman
and Cohen (1988) suggested that children with
Down syndrome showed no understanding of
the principles of counting, and merely repeated
what they had learned by rote. However, studies by Caycho, Gunn and Siegal (1991), Nye,
Clibbens and Bird (1995), Porter (1999) and
Nye, Fluck and Buckley (2001), have suggested
that children with Down syndrome can learn
to count, but their ability to learn these skills
is related to their language development skills.
Bird and Buckley (2002) also suggested that
students with Down syndrome still failed to
reach a competent level of numeracy skills even
though they had reached a competent level in
their literacy skills.
Other researchers have found that children
with Down syndrome are capable of learning
v . 18 n .2
9
Developing Numeracy in Young Adults with Down Syndrome
to complete addition tasks by using the strategy counting all (Buckley & Sachs, 1987; Irwin,
1991). Faragher and Brown (2005) found that
adults with Down syndrome learned numeracy
skills effectively when they were taught within
the context of the everyday situations in which
the skills were used. Turner, Alborz and Gayle
(2008) discussed factors that may influence the
development of literacy and numeracy skills in
young people with Down syndrome from primary to secondary and post school settings and
found that mainstream schooling had a positive
effect on academic attainment of these young
people. Thus it may be, as some researchers
have found, that deficits in numeracy “more
likely reflects a lack of teaching rather than a
lack of ability” (Tiens, 1999, p. 5). Porter (1999)
shared this view with Bird and Buckley (1994)
arguing that “…with good teaching there is no
reason to predict a level that cannot be surpassed…” (p. 65). A challenge also came from
Shepperdson (1994) who concluded from their
study of the reading and numeracy abilities of
teenagers and young adults with Down syndrome who were born in the sixties and seventies, that “…if they are taught, individuals can
learn.” (p. 101).
This article reports on the development, implementation and evaluation of a STP designed to
develop the numeracy skills, specifically the concept of place value, of a group of young adults
with Down syndrome. The STP was designed to
meet their identified learning needs in numeracy by using the strategies of explicit teaching,
repeated practice and the use of concrete materials. Games sessions were used to help the participants practise what they had learnt.
Aims
The aims of this study were to explore the current body of knowledge and research about the
numerical abilities of individuals with Down
syndrome and to develop, implement and
evaluate a program to teach a basic number
concept, place value, to a group of young adults
with Down syndrome. The study drew on
three bodies of knowledge: the development
of numeracy skills in typically developing children; knowledge of effective teaching strategies for students with learning difficulties; and
knowledge of teaching and learning for individuals with Down syndrome.
10
Gaunt et al.
This study addressed three questions:
1. What are the numeracy abilities of a group
of young adults with Down syndrome?
2. What kind of specific teaching program would
meet the needs of the individual students?
3. How effective were the strategies of explicit
teaching, repeated practice and use of concrete materials and games in teaching the
targeted students?
Materials and Methods
The key concept of understanding of place value
in the numeracy development for the partici­
pants was investigated. This study used the
four specific teaching strategies of explicit
teaching, repeated practice and use of concrete
materials and games in teaching the targeted
participants. The four strategies were embedded in the STP.
A single subject case study design was used to
investigate the effectiveness of explicit teaching of place value concepts to a group of young
adults with Down syndrome. The purpose of
single subject research is to discover the effects
of some type of intervention on an individual
(McCormick, 1995). This design model can
demonstrate the effectiveness of an intervention
better than many statistical approaches because
the subject’s achievements can be directly
attributed to the intervention. In this study,
each of the participants formed an individual
case study (Gay, 1996). As only five participants
were selected and targeted for the STP, it was
decided that the most useful information would
be achieved following this case study model
as this would involve in depth analysis of the
achievements of each student.
To select participants for the study and to
develop the STP at an appropriate level within
the numerical development of the participants,
the Booker Profiles of Mathematics (Booker,
1995) were administered as a pre test to the
full cohort of students attending a post school
literacy programs for young adults (Moni &
Jobling, 2000). Individual profiles of each student were developed from the Booker Profiles
of Mathematics (Booker, 1995) and these were
used to match the learner’s profiles to the STP.
During both assessment and teaching sessions,
qualitative data in the form of observational
notes were also collected. These data were used
to enhance the data collected from the Booker
Profiles of Mathematics (Booker, 1995).
Participants
The twelve participants (4 females and 8 males)
for this study were young adults who have Down
syndrome (aged 17 years, 2 months to 21 years,
2 months) and had completed or were completing their last year of secondary schooling.
Procedure
The procedure for this study is presented in
two parts. The first part comprises the assessment to place participants into different groups
for intervention, and the second part describes
the development and implementation of the
STP and its related teaching strategies.
Assessment and Group Selection of
Participants
Whilst most teachers may not have access to
these assessments, in designing any intervention, teachers need to recognise the limitations
demonstrated by individuals with Down syndrome in their receptive and expressive language skills. In this research study, these skills
were formally assessed to guide the development of the STP.
Assessment Tools
Two standardised assessment tools were used:
(1) the Peabody Picture Vocabulary Test – Third
Edition (PPVT-III, Dunn, Dunn, & Williams,
1997), and (2) the Expressive Vocabulary Test
(EVT, Williams, 1997).
The PPVT-III (Dunn et al., 1997) measures
receptive vocabulary attainment, and is a
screen­ing test of verbal ability. The age equivalent score for receptive language was used in
this study because an understanding of the
participant’s receptive language is essential for
JoDD
teachers to know when planning the language
content of a teaching project.
The EVT (Williams, 1997) measures expressive
vocabulary knowledge. The age equivalent
score for expressive language was used in this
study because the participant’s responses to
questions rely on their expressive language and
it is important to understand an individual’s
strengths and weaknesses in this area before a
teaching program can be implemented.
As well as investigating the participants’ language abilities, it was necessary to understand
the numerical abilities of the participants before
developing the STP. A number of standardized
tests are available which give age equivalent or
grade level equivalent scores but as Westwood
(1997) argues:
…standardized tests are of rather limited value
for programming purposes since they do not
yield a comprehensive picture of a student’s
broad range of knowledge and skills. (p. 170)
It was, therefore, decided to use a tool in which
individual performance is presented as a profile of numeracy skills rather than a normative
score as this provides a broader range of information related to the participants’ numeracy
knowledge and skills. The Booker Profiles of
Mathematics (Booker, 1995) is an extensive
individual assessment tool and has two separate sub-tests, one for numeration and one for
computation. The numeration test compiles a
profile for each aspect of numeration: the use
of materials, language and symbols and being
able to move between them; the understanding of place value, sequencing, comparison and
counting. It contains subsets for single digit
numbers, two digit numbers and up to seven
digit numbers. Only the first two sections of the
numeration test were used in this study: single
digit numbers and two digit numbers.
Booker (1995) claims that “these tests not only
profile an individual’s understanding and skill
but also provide the basis for developing an
individually tailored program to consolidate
this knowledge as a foundation for using and
applying mathematics.” (p. i of manual). It was
on the basis of this profile that the participants
were separated into groups for this study.
v . 18 n .2
11
Developing Numeracy in Young Adults with Down Syndrome
Lastly, the personal background data, including family and educational history as well as
major health problems were collected using the
files kept on the students who had participated in the DSRP longitudinal study (Jobling &
Cuskelly, 1998).
Results of Assessments
As can be seen from Table 1, the PPVT-III scores
ranged from 4 years, 2 months to 11 years,
3 months and the EVT from 3 years, 10 months
to 6 years, 8 months. These results align with
previous research that demonstrates that receptive language is more developed than expressive
language among persons with Down syndrome
(Horstmeier, 2004) and support the correlation
between language development and numerical
attainment reported by Rahman (2005). In the
context of developing the STP, this meant that
the researcher needed to be mindful of the language used in explanations of numeracy concepts and needed to check for understanding
of the participants as they may not necessarily
have had the means to ask for assistance if they
did not understand a concept.
Table 2 shows the data from the Booker Profiles
of Mathematics (Booker, 1995). The different
sections of the test are shown across the table
and the total possible score for each section is
given in brackets. Group A is represented by
the first 5 students listed, Group B by the next
three students italicized and Group C the last
four students shaded darkest.
The table shows the number of correct responses each student achieved in each section. The
first six sections of the test deal with the ability to recognise numbers and manipulate them
using language (saying, reading and writing
numbers in words), symbols (numbers) and
materials (base 10 materials and paddle pop
sticks to represent numbers). The other sections of the test investigate students’ abilities to
understand the concept of place value, sequencing numbers, comparing the size of numbers,
counting (both forwards and backwards and by
ones, twos, tens, etc.) and the ability of students
to round numbers to the nearest 10, 100, etc.
Participants were divided into three groups on
the basis of results from The Booker Profiles of
Mathematics (Booker, 1995). Those selected for
Group A had good counting and number rec-
12
Gaunt et al.
Table 1. Participant Summary
EVT
Range
PPVTIII
Mean
PPVTIII
Range
17y 2m
to
20y 9m
7y 5m
5y 8m
to
8y 7m
5y 6m
3y 10m
to
6y
20y 2m
18y11m
to
21y 2m
8y 9m
6y 11m
to
11y 3m
6y
5y 10m
to
6y 8m
19y 3m
18y 9m
to
19y 11m
6y
4y 2m
to
8y 7m
4y 9m
4y 1m
to
5y 10m
Group
Participants CA Mean
A
Julie
David
Tom
Edward
Kylie
19y 2m
B
Nancy
Peter
Jim
C
John
Kelly
Jack
Pat
CA Range
ognition skills. It can be seen from the scores
in the counting column reported in Table 2 that
most participants in Group A demonstrated
an ability to count. The questions the participants got incorrect were counting in tens and
counting backwards. One participant scored
one and another scored zero; however, both
participants demonstrated their ability to count
in other questions on the test and it was considered appropriate to include these two students in the target Group. Those selected in
this group had mastered counting skills but
had no understanding of place value as can be
seen from Table 2, where all the participants
in Group A, except Tom, scored zero on place
value. Tom was able to answer some questions
on place value. However, he made no distinction
between the ones and the tens in the materials
he used although he managed to arrive at the
correct answer in some of the simple questions.
Hence, the target Group had the prerequisite
skills to learn about place value, but no existing
concept of place value.
The participants who were selected to participate
in Group B had both good counting and number
recognition skills as well as the beginnings of an
understanding of the concept of place value (refer
to Table 2). Their scores across the first eight
sections of the test were higher than those of
Group A. However, they were unable to apply
this knowledge to skills such as counting by tens
beyond 100 or increasing or decreasing a given
number by ten. This is evidenced in their similar
EVT Mean
scores to Group A on the comparison and counting sections of the test. These participants were
not selected as the target Group for the STP as
they had some concept of place value.
The participants in Group C did not have a
strong knowledge of counting nor number recognition (refer to Table 2). Many of the members
of this group did not score in many sections of
the test. They would sometimes miss numbers
in the count string or not show one-to-one correspondence when counting. It was determined
that these participants did not have the necessary prerequisite skills to progress onto learning about place value concepts and thus were
also not selected as the target Group for this
study.
Development and Implementation of the
Specific Teaching Project (STP)
Development of the STP
The STP to teach place value was developed
using the participants’ skill levels and was
based on assessment results to find the participant’s level of learning. (see Tables 1 and 2).
From the literature, explicit teaching strategies
(Maccini, Mulcahy, & Wilson, 2007; Mercer,
Jordan & Miller, 1996; Munro, 2000; Smith &
Gellar, 2004) were employed with repeated
practice (Impecoven-Lind & Foegen, 2010;
Pagliano & Gillies, 2009, Westwood, 1997;
JoDD
v . 18 n .2
3
3
2
3
5
3
2
1
1
0
1
Edward
Julie
Kylie
Tom
Nancy
Peter
Jim
Kelly
Jack
John
Patrick
1
0
0
0
3
4
4
2
1
2
1
2
0
1
0
0
4
4
3
2
3
1
1
1
3
0
0
0
4
4
4
4
1
2
2
2
The number in brackets shows the total number of questions in each section.
3
David
Name
2
0
0
1
1
4
4
4
4
2
2
1
2
1
1
1
2
4
4
4
3
2
1
2
Materials to Language to Materials to Symbols to Symbols to Language to
Language Materials
Symbols
Materials Language
Symbols
(5)1
(4)
(4)
(4)
(4)
(4)
Table 2. Individual Test Results for Booker Profiles of Mathematics
0
0
0
1
4
0
4
3
0
0
0
0
Place
Value
(4)
3
2
0
0
7
6
8
5
6
1
3
3
Sequence
(10)
1
0
0
0
1
1
0
1
1
0
0
1
Com­parison
(3)
0
0
0
0
2
3
2
2
0
2
2
1
Counting
(4)
0
0
0
0
0
0
0
0
0
0
0
0
Rounding
(1)
Developing Numeracy in Young Adults with Down Syndrome
13
14
Gaunt et al.
van Kraayenoord & Elkins, 2009). The use of
concrete materials (Booker, 2000a; Booker &
Windsor, 2010; Booker, Bond, Sparrow & Swan,
2010; Mercer, Jordan & Miller, 1996; Westwood,
1997), and the use of games (Booker, 2000b;
Booker et al. 2010; Westwood, 1997; Westwood,
2004) were employed in the STP. To ensure
participants were given the best opportunities
to learn, short instructional periods in a familiar setting (Ashman & Merrotsy, 2009) were
considered appropriate. The sequential stages
of the STP and the teaching and game sessions
are outlined below in Table 3. There were nine
weekly sessions of specific place value teaching for Group A and nine games sessions for
all participants. After nine weeks, this was followed by the post test again using the Booker
Profiles of Mathematics (1995).
Table 3. Stages of the STP
Stage of
Project
Pre
Tests
Table 4 shows an outline of the concepts taught
each week, the resources and strategies used
in each explicit teaching session. The program
focussed on repetitive and concrete teaching
methods. During each teaching session, the participants received explicit instruction on place
value concepts. They used concrete materials
(base ten blocks), various worksheets from Silbey
(1989) and received individual tutoring in learning these concepts. Sessions were developed
sequentially, reflecting the considered opinions
of researchers that the nature of learning numeracy is sequential (Booker et al. 2010; Munro, 2000;
van Kraayenoord & Elkins, 2009).
Games Sessions
All participants (Table 1) took part in a games
session on Friday morning. Booker (2000b) suggested that games are an effective teaching tool
and can both enhance the learning that has pre-
All participants were assessed
using the Booker Profiles of
Math­e­matics prior to being
organised into groups for the
STP
Teaching There were nine teaching
Sessions: sessions in all.
Group A
Each session was 70 minutes in
Only
total, 40 minutes before morning
tea and 30 minutes after.
Participants in Group A were
divided into two smaller groups:
three attended Wednesday
and two attended Thursday
mornings.
Implementation of the STP
Each morning the teaching session was divided into two by a morning tea break. The time
frame was designed to take into account difficulties with sustained attention as identified
by Rosenberg, Westling and McLeskey (2011)
and Ashman and Merrotsy (2009). In addition,
it was assumed the learners would have better concentration in the morning. In the first
40 minute session of the morning new material
was introduced (Goodman, 1996) and then consolidated in the second 30 minute session.
Description
Games
Sessions
There were nine game sessions
in all.
All five participants in Group
A participated in the games
session for the first 50 minutes
on Friday mornings.
After morning tea, the other two
Groups, B and C, each attended
a 50-minute games session.
Post
Tests
All participants in Groups A, B
and C were assessed using the
Booker Profiles of Mathematics
one week after the teaching
sessions were complete.
viously been taught in the classroom and be
used to introduce new concepts to be learnt.
Moreover, as stated by Booker (2000b) “they
contribute to the development of knowledge
while having a positive influence on the affective or emotional component of learning situations.” (p. 1). Thus Groups A, B and C attended
the games sessions to ascertain if there were
any benefits to their numeracy ability as measured by the Booker Profiles of Mathematics.
JoDD
15
Developing Numeracy in Young Adults with Down Syndrome
Table 4. Development and Sequence of Strategies
Week Concepts Taught
1
Recognizing numbers 1–20
in words and numbers.
Recognizing the date in
words and numbers
2
Recognizing numbers from
1–100 using materials
Games
Strategies
Number recognition games: •Teacher directed
Number play
discussion of each concept
to engage students prior
knowledge
Counting games
e.g., Snakes and ladders
3
Repeat week 2
Bingo: One more/less
4
Counting Board: counting
in 10s starting from any
number
Bingo: Ten more
5
Review of counting in tens
from any number.
Computer games
Finding ten more or less
than a number
6
Review of previous
concepts
Ten less bingo
Ordering numbers
Introduction of hundreds
Using MAB and dice, make
largest number: throw dice
three times then using ones,
tens and hundreds make
largest number possible.
8
Three digit numbers
Card Game: make largest
number from three chosen
cards; extra points for
stating ten more than
number.
9
Review of two and three
digit numbers.
Assessment
Ideas for the games were developed from The
Maths Game, Booker (2000b).
During each games session, the participants
played games specifically designed to reinforce the concepts taught to Group A during
the previous lessons that week. For example,
when participants were learning to increase or
decrease numbers by ten, the game played was
10 more bingo. When a number was called out,
the participants had to find the number on their
v . 18 n .2
•Explicit teaching of
concepts followed by
teacher directed activities
followed by independent
practice
•Regular review activities
throughout the program
Introduction of decreasing
numbers by ten
7
•Repetitive activities: e.g.,
the date was written on
the board each session and
discussion about different
ways of writing the date
and what the numbers
meant was the first
activity of each lesson.
•Games sessions linked to
previous content taught
card, which was ten more than the number
called out. If the concept of the game was too
difficult for participants in Group B or C, it was
modified. For example, when playing 10 more
bingo, Group C had difficulty and the game was
changed to 1 more bingo. Prizes were occasionally given to the winners but mostly the participants enjoyed playing the game without requiring extrinsic rewards.
16
Gaunt et al.
of Numeration, one half of the Booker Profiles
of Mathematics (Booker 1995). The other half of
the assessment instrument is the Booker Profiles
of Computation. This section of the test was
not administered as this research concentrated
on the numerical concept of place value, a fundamental skill to all other aspects of numeracy
(Booker et al., 2010; van Kraayenoord & Elkins,
2009; Zevenbergen, Dole & Wright, 2004).
In each classroom session of the STP, anecdotal
observational notes were recorded by the first
author to determine the individual learning
characteristics of the Group A participants.
Results
The results are presented first as a group analysis and second as selected individual case studies due to the small number of participants in
each group.
The comparison of pre and post test means
(Table 5) show that some individuals in all
Groups showed improvement in different sections of the test (improved scores are italicized
and in larger font). The target group (Group A)
showed improvements across all areas of the
test except section two (language to materials)
where the scores remained the same, and section 11 (rounding) which was not covered in the
STP. Groups B and C, which only received the
games sessions, did show some improvements
in test scores, but these improvements were
more varied and not as consistent as Group A.
Group Results
The results of pre and post test scores on the
Booker Profiles of Mathematics (Booker 1995)
are presented in Table 5. The table lists all of the
Assessment Tasks (left hand side) participants
completed both before and after the intervention
of the STP and Games sessions. The number in
the brackets indicates the maximum possible
score for that section. Full details of each task
can be found in Booker (1995). For example, the
assessment task on place value, which is the focus
of this study, explores participants’ understanding of ones, tens, hundreds, etc. in different
numbers. The eleven sections of the assessment
tasks administered comprise the Booker Profiles
The results for Group A showed an improvement
across all areas of the assessment tasks that were
covered in the STP except for section two (language to materials) where the average score was
maintained but not improved. Improvement was
shown in the place value section where the mean
Table 5. Mean Score Per participant On Assessment Tasks
Group A
Assessment Tasks
Pre Test
Group B
Post Test
Pre Test
Group C
Post Test
Pre Test
Post Test
1. Materials to Language (5)
2.60
3.20
3.30
5.00
0.75
2.00
2. Language to Materials (4)
1.60
1.60
3.60
3.60
0.25
0.75
3. Materials to Symbols (4)
1.60
3.20
3.60
3.00
0.25
0.75
4. Symbols to Materials (4)
2.00
2.60
4.00
4.00
0.75
1.00
5. Symbols to Language (4)
2.40
2.80
3.00
4.00
0.75
0.75
6. Language to Symbols (4)
2.00
2.80
3.30
3.30
1.25
1.25
7. Place Value (4)
0.40
2.00
2.60
3.60
0.25
0.00
8. Sequence (10)
3.60
4.00
7.30
7.60
1.50
2.75
9. Comparison (3)
0.40
0.60
0.60
0.60
0.25
0.25
10.Counting (4)
1.00
1.40
2.30
2.30
0.00
0.50
11.Rounding (1)
0.00
0.00
0.00
0.00
0.00
0.00
The number in brackets shows the total number of questions in each section.
JoDD
improved from a score of 0.40/4.00 to 2.00/4.00.
Other improvements include section one , materials to language from 2.60 to 3.20; section three,
materials to symbols from 1.60 to 3.20 and section six language to symbols where students
average scores improved from 2.00 to 2.80.
The place value scores also improved for
Group B from 2.60/4.00 to 3.60/4.00. This group
also showed improvements in three other areas.
In section one (materials to language) there was
improvement from 3.30 to 5.00, in section five
(symbols to language) from 3.00 to 4.00 and
in section eight (sequencing) from 7.30 to 7.60.
Although it appears that Group B scored better
in each section than the target group (Group A),
it needs to be remembered that Group B began
this project with higher scores across the board
(see Table 4 and Table 2) and participants
already had the beginning of an understanding
of place value.
Comparing the improved scores of Group B
with those of Group C, it is evident that Group C
scores were not as high as Group B; however,
they still showed some improvements across
more areas than Group B. Group C improved
in six sections of the test. Section one (materials
to language), average sores improved from 0.75
to 2.00, in section two and three (language to
materials and materials to symbols), from 0.25
to 0.75, section four (symbols to materials) from
0.75 to 1.00, section eight (sequencing) from 1.50
to 2.75 and section 10 (counting) from 0.00 to
0.50. Group C was the only group that did not
show any improvements in place value.
Individual Case Study Profiles
Table 6 showed the individual scores of
Group A participants. Sections where the individuals have improved have been italicized in
larger numbers. To highlight the results, three
individual case study profiles have been included. The three profiles have been chosen to highlight some of the particular achievements of the
students in the STP.
Tom
The reason Tom was chosen to be profiled was
because he was confident and correct in class
but did not perform as well on the assessment
v . 18 n .2
17
Developing Numeracy in Young Adults with Down Syndrome
for the study as would be expected from his
achievement in class.
Background Information and Classroom
Observations
Tom was 19 years, one month old and his
receptive language was an age equivalency of
7 years, 3 months while his expressive language
was an age equivalency of 6 years. Tom always
worked eagerly in class completing classroom
activities keenly and successfully. He showed a
good understanding of what he was doing and
would frequently complete exercises quickly
and correctly. However, when it came to assessment tasks on the post test, which he had previously completed successfully in class, he was
unable to recall his newly learnt skills.
Skills Achieved
As can be seen in Table 6, Tom did show some
improvement in sequencing (section eight),
where he scored five in the pre test and six
in the post test. There was also improvement
in section six, language to symbols, where Tom
scored three in the pre test and four (100%) in
the post test. In section seven (place value) Tom
scored two on the pre test and three on the post
test. In some of the 29 questions relating to place
value and the ability to recognise and manipulate numbers on the post test (the first seven
sections of the test), Tom showed an awareness of the concept even though the answer
was incorrect e.g., when shown the number 47
constructed from the base ten materials, Tom said
“four tens and seven ones…makes eleven,” and
when making 87 from the base ten materials, he
made the number correctly and then said that it
was 15 (the sum of eight and seven).
Edward
Edward was chosen to profile as he showed the
most improvement of all of the participants in
Group A.
Background information and Classroom
Observations
Edward was aged 20 years and his receptive
language was an age equivalency of 8 years,
1 month while his expressive language was an
age equivalency of 5 years, 11 months. Edward
2
3
3
3
5
2
3
3
3
(post)
Edward (pre)
(post)
Julie (pre)
(post)
Kylie (pre)
(post)
Tom (pre)
(post)
1
2
4
1
2
2
1
1
1
2
1
2
3
3
4
1
4
1
4
1
3
3
2
1
4
2
2
2
2
2
The number in brackets shows the total number of questions in each section.
3
David (pre)
Name
4
4
4
4
2
2
3
2
1
1
4
3
2
2
3
2
4
1
2
2
Materials to Language to Materials to Symbols to Symbols to Language to
materials
language
Symbols
Materials Language
Symbols
(4)
(5)2
(4)
(4)
(4)
(4)
Table 6. Group A Pre Test and Post Test scores
3
2
3
0
0
0
4
0
0
0
Place
Value
(4)
6
5
6
6
3
1
4
3
2
3
Sequence
(10)
1
1
0
1
0
0
1
0
1
1
Comparison
(3)
2
2
2
0
1
2
1
2
1
1
Counting
(4)
0
0
0
0
0
0
0
0
0
0
Rounding
(1)
18
Gaunt et al.
JoDD
always worked quietly and was very keen to
please. He always tried to complete tasks, even
new ones. He would frequently repeat mistakes
and would write an incorrect answer rather
than leave it blank if he didn’t understand but
did not get upset about having his work corrected. He was very pleased with himself when
he learnt new skills.
Skills Achieved
Table 6 revealed that Edward showed improvement in areas involving place value. In section three (materials to symbols) Edward’s score
improved from one on the pre test to four (100%)
on the post test. Sections six and seven also
showed improvement with his score improving from one to four (100%) on section six and
from zero to three (maximum score was four) on
section seven. He also showed some improvement in sections five (symbols to language)
and eight (sequence) where his score improved
from two out of four to three and from three out
of ten to four, respectively. There was a slight
improvement in section nine (comparison)
where Edward scored one correct response in
the post test after he scored zero on the pre test.
In section 10 (counting, which includes counting backwards), Edward’s score improved from
zero on the pre test to one out of five on the post
test. In the 29 questions involving place value
concepts (the first seven sections of the assessments), Edward achieved nine correct responses
on the pre test and 20 correct responses on the
post test. His comparison skills in recognizing
greatest and least numbers, section nine of the
assessment, also changed slightly on the posttest. Edward achieved one out of three correct
on the post-test where he had not achieved any
correct responses in the pre test.
Kylie
Kylie showed good improvements in some
areas tested and was a very keen and interested
student.
Background Information and Classroom
Observations
Kylie was aged 20 years, 9 months and her
receptive language age equivalency was 5 years,
8 months while her expressive language age
equivalency was 5 years, 11 months. Typically,
v . 18 n .2
19
Developing Numeracy in Young Adults with Down Syndrome
Kylie was pleased to participate in class activities
particularly if she understood the work. If the
work was difficult, she would often work very
slowly and wait for the answers to be put on the
board, or try and copy from other students. She
was happy to ask for assistance most times and
would work very hard once she understood the
concept and was a keen games participant who
was not really interested in prizes.
Skills Achieved
As can be seen in Table 6, Kylie improved in
the place value skills of manipulating the base ten
materials to read and construct numbers (sections one to four), during the nine-week implementation of the STP. Her scores improved from
five out of nineteen on the pre test to twelve correct on the post test. She also applied her knowledge of place value to develop an understanding
of the number of ones and tens in a number
(section seven). Kylie achieved three out of four
correct responses on the post test where she had
failed to score any correct responses in the pre
test. There was also improvement in section 10,
counting, where Kylie’s score improved from
zero out of five to two out of five. During the
pre test, Kylie answered 12 out of 29 questions
involving the concept of place value correctly
(sections one to seven). One of these was a picture of 37 using three bundles of ten paddle pop
sticks and seven individual sticks. She achieved
the correct answer by counting each stick separately not by counting the three bundles of tens
as tens. During the post-test, Kylie answered 20
of the 29 questions correctly (including determining the number 37 by counting the tens and
ones). She had much greater success when she
manipulated the base ten materials rather than
reading materials already constructed for her.
Prior to the implementation of the STP, Kylie
showed no signs of any knowledge of place
value but after the program, she showed an
awareness of ones and tens.
Discussion
The results demonstrate that these individuals
with Down syndrome can learn the numeracy
skill of place value with a program based on specific teaching, repeated practice, the use of concrete materials and games. This has been demonstrated through the improvement in performance
20
Gaunt et al.
across almost all areas of the assessment tasks of
students in Group A. Although Groups B and C
also showed improvements, they were less consistent and less widespread than Group A. A discussion of the results follows.
The concept of place value was the main focus of
the STP and the increase in results for Group A
participants support the value of the STP and
demonstrate that individuals with Down syndrome, are able to learn basic numeracy skills if
they are taught appropriately. Neither Group B
nor C showed as comprehensive a pattern of
improvement as Group A which received both
the STP and the games sessions.
The lack of improvement of Group A participants in section two of the test (language to
materials) could be explained due to the short
teaching period of the STP. To be able to manipulate the materials following a verbal prompt is
a more abstract skill and hence more difficult
than to start with the materials that you can
then manipulate. Further instruction here was
required.
Group B did show some improvements in some
areas of the assessment; however, Group B
started at a higher level of understanding than
Groups A or C and already had a basic understanding of place value. The participation of
Group B in the games sessions could have triggered links with previous knowledge in each
area. Previous research has shown that linking
new mathematical concepts with known concepts allows for a deeper understanding of new
knowledge (Booker et al., 2010; Van de Walle,
2007). As these participants had completed
schooling, participation in the games sessions
would have assisted this group in remembering
knowledge that they may have forgotten when
they completed the pre-test and the games
could also have linked with their prior knowledge and developed their understanding further. If Group B had participated in the STP as
well as the games sessions, a greater improvement in results would have been expected.
Group C had lower scores across the board on
the pre test and did not demonstrate an understanding in many areas of fundamental numeracy skill. For example, they did not display
knowledge of a stable counting string and had
difficulty manipulating materials and symbols
to represent numbers. Hence, Group C had lim-
ited existing knowledge from which to develop
further skills. The games developed for the STP
frequently had to be adjusted for the level of
learning required by this group. For example,
when playing 10 more or less bingo, the game
was adjusted for Group C to be 1 more bingo.
Hence although some improvements were
shown by participants in Group C which can
be attributed to the games sessions they participated in, improvements were not expected
to be as great as for the other two Groups. A
targeted intervention program for this group of
students could have shown a greater improvement in numeracy skills across the areas tested.
Group C was the only group that did not show
improvements in place value from the pre test
to the post test (the average score decreased
from 0.25 to 0). This group were not specifically taught the skill of place value, only receiving the games session. Participants did not have
the known concepts to link new mathematical
concepts and develop a deeper understanding
of new knowledge (Booker et al., 2010, Van de
Walle, 2007). Hence, games alone could not be
relied upon to teach new concepts. Games can
enhance students learning but individuals with
Down syndrome benefit from explicit teaching
of numeracy concepts.
The results demonstrated that the games sessions
did assist the participants in the development of
some numeracy skills but that this improvement
in understanding was not as great as for those
students who had received a targeted, direct
teaching program to develop those skills. Games
can enhance numeracy development, but specific
teaching of concepts is still required.
Conclusion
The data question the perceptions that numeracy education for individuals with Down syndrome should be confined to functional skills
and concurs with findings of Bird and Buckley
(1994) and Bochner, Outhred and Pieterse (2001)
that numeracy achievements can be achieved
with appropriate teaching.
As the results have demonstrated, improvement was shown by participants in all three
groups but particularly participants in the target Group A, who received the STP and the
games sessions. Although the improvements
JoDD
21
Developing Numeracy in Young Adults with Down Syndrome
were sometimes variable, the results show the
importance of individualized targeted instruction. Furthermore, the results display the benefits of direct instruction with repeated practice
and the use of concrete materials in conjunction
with targeted and relevant games in the teaching of basic numerical skills.
There were two limitations in this study. First,
the instruction provided through the STP was
for a limited time, and second, there were a
small number of participants in this study.
Both of these issues could be addressed by further replication with greater sample sizes. Even
though there were limitations, this study has
demonstrated that with specifically directed
teaching strategies, individuals with Down
syndrome are able to show improvements
in numeracy skill (place value) acquisition, an
awareness of place value, an improvement in the
individual’s ability to complete tasks based on
place value and increased ability to manipulate
numbers and materials to solve problems.
This article presented the results of the STP
that used teaching strategies considered to be
effective in improving the numeracy abilities
of individuals with Down syndrome. From the
presented individual case studies, the additional information gathered from personal background data and classroom observation complemented the assessment results and illustrated the progress of the individual participants
in Group A. These data have shown that even
with such a short program of instruction, the
strategies of explicit teaching, repeated practice
and the use of concrete materials and games
were effective in improving the numeracy skills
of the participants in the target skill of “place
value.” As Shepperdson (1994) stated, “… if
they are taught, individuals can learn.” (p. 101).
Acknowledgments
The authors would like to acknowledge the
participation and co-operation of the young
people with Down syndrome who attended
the LATCH-ON program and their families.
Without your continued support and participation in research, progress in the understanding
of the educational needs of people with Down
syndrome would not continue.
v . 18 n .2
Key Messages From This Article
People with Down syndrome: You should never
allow people to tell that you are not capable of
learning because with the right help you can
achieve many things.
Professionals: Explicit teaching strategies combined with the use of games can enhance the
learning of place value concepts.
To help young people with Down syndrome
learn place value concepts, teachers must:
• Determine their current level of understanding
• Target instruction to individual needs
• Directly teach concepts and strategies
Policymakers: Policy to ensure that young people with Down syndrome continue to receive
access to quality academic education throughout school and beyond is necessary to promote
the idea of lifelong learning.
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Note: At time of first publication of this
article, minor changes were pending. A fully
edited version of this article can be found on
the OADD website at http://www.oadd.org/
index.php?page=754 after August 15, 2012.
The Impact of Verbal and Nonverbal
Development on Executive Function in
Down Syndrome and Williams Syndrome
Volume 18, Number 2, 2012
Authors
Oriane Landry,1
Natalie Russo,2
Tammy Dawkins,3
Philip D. Zelazo,4
Jacob A. Burack3
Psychology Department,
Dalhousie University,
Life Sciences Centre,
Halifax, NS
1
Syracuse University,
Department of
Psychology,
Syracuse, NY
Abstract
Using Vygotsky’s verbal mediation framework, we hypothesised that verbal development would be more strongly associated with executive function than non-verbal development
in populations with developmental disabilities with distinct
verbal/non-verbal profiles. We used correlational analyses to
explore the developmental relationship between verbal and
non-verbal development and the executive function components of cognitive flexibility and working memory among persons with Down syndrome and those with Williams syndrome.
We found that verbal development was uniquely correlated
with cognitive flexibility and working memory in both groups.
We conclude that verbal development is a better predictor of
both cognitive flexibility and working memory independent of
non-verbal development in persons with Down syndrome and
Williams syndrome.
2
McGill University,
Department of
Educational Psychology,
Montreal, QC
3
University of Minnesota,
Institute of Child
Development,
Minneapolis, MN
4
Correspondence
[email protected]
Keywords
Down syndrome,
Williams syndrome,
cognitive flexibility,
working memory,
verbal development
Language development is considered by many to provide
the building blocks upon which other cognitive skills can
grow (Luria, 1961; Luria & Wertsch, 1981; Vygotsky, 1962).
According to Vygotsky and Luria, verbalization serves to
focus attention and mediate the voluntary control of behaviour, which can be operationalized by tasks measuring
executive functions (EF). In typical development, support for
the developmental relationship between language and EF is
found in both correlational analyses and experimental studies (Joseph et al., 2005; Kirkham et al., 2003). Disentangling
the roles of different aspects of cognition in the development
of EF is difficult with typically developing children, because
by definition, verbal IQ and non-verbal IQ develop in parallel; that is how we define typical. However, populations
such as persons with Down syndrome (DS) and those with
Williams syndrome (WS) provide particularly compelling
opportunities to examine the relationship between language
development and EF because they present opposing profiles
of verbal and non-verbal IQ, despite relatively similar full
scale IQs. This will allow us to tease apart the relative contribution of verbal and non-verbal skill development in the
development of EF. Accordingly, we compared the relationship among the EF tasks of Dimensional Change Card Sort
(DCCS; Frye et al., 1995) and Self-Ordered Pointing (SOP;
Archibald & Kerns, 1999; Petrides & Milner, 1982), and measures of verbal and non-verbal development between persons with DS and WS.
The Role of Language in
Executive Function
Evidence from preschool aged children suggests an important role of language in the
development of EF, although the co-contribution of non-verbal development has not been
fully addressed. For example, the DCCS (Frye
et al., 1995) is a sorting task in which children
are asked to sort first by one dimension, either
colour or shape, and then to change strategies
and sort by the other dimension using the same
set of cards. The key feature of a task such as
this is that the rules are mutually exclusive and
incompatible; on any given trial, the test card
matches one target card on colour, and the
other target card on shape. Typical 3-year-olds
can successfully sort by one dimension (preswitch) but perseverate on the initial sorting
rule when the sorting rule is changed (postswitch), regardless of which rule is presented
first. However, typical 4-year-olds can switch
rule sets and typical 5-year-olds can switch
rapidly between rules (Frye et al., 1995; Zelazo
& Frye, 1997). The ability to switch between
incompatible rule sets is referred to as cognitive flexibility or set-shifting; it involves reasoning according to two contradicting pairs of
rules, forcing the participants to, at first, think
according to a certain rule, but to switch their
mind set in order to follow a different rule if
they want to succeed on a subsequent part
of the task (Frye et al., 1995). The failure to
switch mental sets leads to perseverative errors
(Zelazo & Müller, 2002).
Performance on the DCCS appears to be verbally mediated as labelling cards according to
the relevant dimension improves performance
among typically developing children. The
majority of 3-year-olds fail the post-switch trials (Zelazo et al., 2003), but when children are
prompted to label the cards themselves, the
proportion of children sorting correctly on postswitch cards increases (Kirkham et al., 2003).
Labelling the relevant dimension also improves
cognitive flexibility on a separate task for preschool children, the Flexible Item Selection Task
(Jacques & Zelazo, 2001). Vygotsky and Luria
would argue that verbal representation of the
relevant dimension focuses the child’s attention
on the relevant attribute, guiding them to represent the problem in a new way.
v . 18 n .2
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Development & Executive Function
Another EF task that appears to be verbally
mediated is the Self-Ordered Pointing task
(SOP; Petrides & Milner, 1982), a working
memory task originally developed for use
with adult neurological patients and modified for use with children. This task is thought
to assess the capacity to initiate a sequence
of responses, retain the responses, and monitor the consequences of behaviour (Petrides &
Milner, 1982). The task involves participants`
self-directed selection of items in an array such
that all items are selected once and only once.
Hongwanishkul, Happaney, Lee, and Zelazo
(2005) adapted the task to make it appropriate for preschool children and found that performance improved with age between 3- and
5-year-olds. The 3-year-olds were able to successfully perform the task with an average of
4.5 items, while the 5-year-olds could successfully perform the task with an average of 6.5
items. Thus, working memory span appears to
increase throughout childhood, with significant improvement during the preschool years.
The SOP is assumed to be verbally mediated
because the task is considerably easier when the
objects can be verbalized. Joseph et al. (2005)
presented a group of typically developing children (mean age 8 years) with two versions of
a SOP task, one containing concrete namable
objects, and a second containing abstract nonnamable objects. They found that the typically
developing children committed fewer errors
on the version with the concrete objects. This
supports the verbal mediation model, which
suggests that performance is facilitated by an
internal dialogue.
We hypothesized that this internal dialogue
would facilitate performance on EF tasks among
individuals with DS and WS, resulting in EF
performance commensurate with language
development more so than non-verbal skills.
Among persons with DS, language skills are an
area of relative weakness, however Pennington
et al. (2003) reported that children with DS did
not differ in their performance on EF tasks relative to typically developing children with similar vocabulary scores. Thus, EF skills are on par
with vocabulary development in DS.
Among persons with WS, language skills are an
area of relative strength. Hoffman et al. (2003)
concluded that persons with WS have intact
26
Landry et al.
executive processes because they use similar
methods as verbal mental age matched typically
developing persons to solve puzzles. Hoffman
et al further suggested that visual-spatial difficulties may impede performance among persons with WS on EF tasks that are primarily
visual-spatial in presentation. Vicari et al. (2001)
tested 12 low functioning children with WS
on the Tower of London, an EF task of visualspatial planning, and found performance to
be poorer than that of MA matched typically
developing children, however details about verbal versus performance IQ of the participants
were not provided. While poor visual-spatial
skills may put individuals with WS at a disadvantage when tested with visual-spatial based
materials, in accordance with the verbal mediation theory (Luria, 1961; Luria & Wertsch, 1981;
Vygotsky, 1962), we would expect that among
persons with DS and WS, individuals with more
advanced verbal development will also show
more advanced EF skills. While verbal development is not the same as verbal mediation, the
verbal mediation model predicts that verbal
development should be associated with EF abilities more so than non-verbal development.
Current Study
The aim of this study was to explore the differential roles of verbal and non-verbal cognitive development in the acquisition of EF abilities among children with DS and WS. We used
the Peabody Picture Vocabulary Test (Dunn
& Dunn, 1997), a standardized measure of
receptive vocabulary, as our estimate of verbal development and the Leiter International
Performance Scale (Roid & Miller, 1997), a standardized measure of non-verbal intelligence,
as our estimate of non-verbal development. We
administered two tasks of EF, a test of cognitive flexibility and a test of working memory.
In order to measure cognitive flexibility, we
administered the DCCS (Frye et al., 1995) and
to measure working memory, we administered
the SOP (Petrides & Milner, 1982). In accordance with the verbal mediation model, we
predicted that for both the participants with DS
and those with WS that performance on the EF
tasks would be more related to verbal development than to non-verbal development.
Method
Participants
The participants included 11 persons with DS
and 14 persons with WS. Most of the participants
were functioning in the moderate mental retardation range, however one participant with WS
achieved IQ scores in the borderline to normal
range. Nine of the participants with DS were
recruited from a special education school and
two were recruited from a service agency for
persons with intellectual disabilities. The participants with WS were recruited at a National
Williams syndrome conference. The ethics
boards of the university and schools involved
approved the study. The chronological ages (CA)
and mental age (MA) equivalents of the participants are provided in months in Table 1.
Intellectual Development Measures
Verbal development. The Peabody Picture Voc­
ab­u ­lary Test – Third Edition (PPVT; Dunn &
Dunn, 1997) was used to measure verbal development. This test is a measure of one word
receptive language in which respondents are
required to point to a picture from a set of
four that best represents a word spoken by the
experimenter. For example, the child is presented with four pictures (a cat, a spoon, a crib and
a dog) and is asked to “point to the picture of
the spoon.” The PPVT is commonly used with
persons with developmental disabilities and is
especially appropriate for those with particular
difficulties in expressive language. Raw scores
were converted into mental age equivalents.
Non-verbal development. The Leiter Inter­
national Performance Scale-Revised (Leiter-R;
Roid & Miller, 1997) was used to measure
non-verbal development. The Leiter-R is a
brief measure of non-verbal intelligence that
includes 4 subtests of reasoning and visualization appropriate for individuals between the
ages of 2 through 20 years of age. As the test
relies on non-verbal cues to convey the instructions, requires no verbal responses and has no
time limitations, it is especially appropriate for
administration to individuals with developmental disabilities. Raw scores were converted
into mental age equivalents.
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Development & Executive Function
Table 1. Chronological Ages (CA), Verbal Mental Age (VMA) Estimates, and Non-Verbal Mental Age
(NVMA) Estimates of Participants, in Months
Participant Characteristics
CA
VMA
NVMA
DS01
112
50
48
DS02
154
56
54
DS03
141
12
38
DS04
176
45
60
DS05
231
36
48
DS06
228
12
42
DS07
157
55
55
DS08
162
21
47
DS09
214
58
67
DS10
255
64
74
DS11
105
54
NA
MEAN
175.9
42.1
53.3
49.8
19.0
11.2
SD
WS02
120
103
110
WS06
590
153
89
WS07
154
90
94
WS09
102
52
58
WS10
73
53
48
WS11
142
NA
71
WS12
138
58
62
WS13
63
34
NA
WS14
81
58
NA
WS15
NA
122
NA
WS18
102
NA
61
WS21
NA
29
NA
MEAN
156.5
75.2
74.1
SD
155.3
40.4
21.3
v . 18 n .2
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Landry et al.
Executive Function Tasks
Dimensional Change Card Sort. The DCCS (Frye
et al., 1995) is a card-sorting task with three
levels of difficulty. These levels are called preswitch, post-switch, and complex. Participants
were presented with target cards of a red boat
and a blue rabbit that were affixed to two trays.
The cards that the participants were given to
sort included those with a blue boat and those
with a red rabbit. These cards are shown in
Figure 1.
Figure 1. Target (top) and test (bottom) cards
used in the DCCS. Dark shading
represents blue and light shading
represents red. Dark arrow represents a
correct “colour” sort, while the dashed
arrow represents a correct “shape” sort.
In the pre-switch phase, the participants were
introduced to either the colour or the shape
game, counterbalanced across participants. For
example, in the colour game, the participants
were told, “We are going to play the colour game.
In the colour game, all the red ones go here,” as
the experimenter pointed to the tray with the
red boat, “and all the blue ones go here,” as the
experimenter pointed to the tray with the blue
rabbit. The experimenter demonstrated with
one card of each colour, and then the test phase
began. The participants were reminded of the
rules before each card was turned over. This
phase consisted of six cards. Participants who
sorted five of six cards correctly were told that
they would now play a different game and were
administered the post-switch phase. Participants
who were unable to sort five of six cards correct-
ly were told the game was over and proceeded
to the next task. In the post-switch phase, participants who initially played the colour game were
told, “we are not going to play the colour game
any more, now we are going to play the shape
game. In the shape game all the rabbits go here
and all the boats go here.” The experimenter
then administered the shape game in the same
manner as the colour game had been presented
in the pre-switch phase.
The participants who correctly sorted five of six
cards in the post-switch phase progressed to the
complex phase. These participants were shown a
new set of cards in which half of the cards had
black borders, and the remainder were identical
to those used in the pre-and post-switch phases. The new rules were explained and demonstrated to the participant. For example, “if the
card has a black border, then we play the colour
game, and if it has no black border then we play
the shape game. Remember, in the colour game,
all the red ones go here and the blue ones go
here, and in the shape game all the boats go here
and all the rabbits go here.” This phase consisted of 12 cards. Before each card was played, the
participant was reminded of the rule that “if the
card has a black border, then we play the colour
game, and if it has no black border then we play
the shape game”. The rules concerning the black
border were counterbalanced across participants.
Across all the phases, a maximum score of three
points was possible. One point was assigned for
passing each of the pre-switch and post-switch
phases, and one point for correctly sorting 9 of
12 cards during the complex phase.
Self-Ordered Pointing. This task is an adaptation
of Milner and Petrides’ (1982) original task,
simplified for younger children. In this task,
the participants were presented with drawings
of objects arranged in a matrix on a 21.6 cm x
27.9 cm page in a binder. The participants were
instructed to choose a picture on each page,
and when the page was turned to choose a different picture from the same group of objects
that were displayed in different locations. For
each level, this continued until the participant
pointed to every object in the array, so that
three pages were seen for the level with three
objects and nine for the level with nine objects.
An incorrect response was scored when the
participant pointed to an object that had been
pointed to on a previous page in that level. An
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Development & Executive Function
example of a three-object display is presented
in Figure 2. In this scenario, if the participant
pointed to the train on the first page, then
pointing to either the rabbit or the crayons
would be correct on the second page. On the
third page, the remaining item from the rabbit or crayons that was not pointed to on the
second page would be the correct response.
Objects were not repeated across sets.
The task involved nine levels, each increasing
the number of items in the array by one, starting with two items and ending with ten. When
the participants successfully completed a level,
they continued to the next one. If the participants committed an error, they were given a
second chance at that level with a second set of
objects. The task continued until the participant
failed both sets of pictures at a particular level
or passed all nine levels. The score on the task
reflected the highest level that was passed. The
number of items in each level is one more than
the level (e.g., level five contains six items, level
nine contains 10 items).
Procedure
Most participants were able to complete the
cognitive measures in one session and the EF
tasks in a second session on another day. Each
session lasted approximately 45 minutes. On
the first session, participants were administered the PPVT-III (Dunn & Dunn, 1997) and
the Leiter-R (Roid & Miller, 1997) in counterbalanced order. The EF tasks were presented in
counterbalanced order during the second session. A third session was included if necessary.
Results
Not all of the children in each of the groups
completed both the two cognitive measures
and the two EF tasks. One participant with DS
did not complete the Leiter-R. One participant
with WS did not complete the DCCS, four did
not complete the PPVT, and six did not complete the Leiter-R. Sample sizes thus varied
across groups as well as across analyses.
Performance on the DCCS and SOP tasks is
provided in Table 2. The mean DCCS score for
participants with DS suggests many passed preswitch but failed post-switch, while more particv . 18 n .2
Figure 2. Sample three-item array from the
SOP. The child is required to point to
one item in the first array, a different
item in the second array, and the
remaining item in the third array.
30
Landry et al.
Table 2. Performance on Executive Function Tasks for Participants with Down Syndrome
and Williams Syndrome
DCCS
SOP
Sample Size
Mean
Standard
Deviation
Sample Size
Mean
Standard
Deviation
DS
11
1.18
0.75
11
3.36
2.38
WS
13
1.92
0.95
14
2.93
1.59
ipants with WS passed post-switch. On the SOP,
participants with DS had on average a working
memory span of 3–4 items, while participants
with WS had a working memory span of 2–3
items, although the average for both groups is
close to 3. Comparisons were not made between
groups as the groups were not matched for MA.
significance were used to maximize power and
compensate for the small sample sizes of some
of the groups. Chronological age was not correlated with performance on the EF tasks; the
correlation coefficients ranged from .05 to .34
and were influenced by one particularly older
participant with WS.
In an attempt to examine the relationship
between measures of cognitive development
and performance on executive function tasks,
a series of correlational analyses were conducted. Given a priori assumptions that all correlations would be positive, one-tailed tests of
Correlation matrices for mental ages and EF
tasks for each group are presented in Table 3.
Significant correlations were noted for the participants with DS for all measures. In the group
with WS, the PPVT was significantly correlated
with both EF measures, but the Leiter was not
Table 3. Intercorrelations Between Measures of Verbal and Non-Verbal Development and Measures of
Executive Function for Participants with Down Syndrome and Williams Syndrome
LEITER
DCCS
Down Syndrome
PPVT
LEITER
DCCS
SOP
r
.838**
.876**
.693**
n
10
11
11
r
.800**
.663*
n
10
10
r
.799**
n
11
Williams Syndrome
PPVT
LEITER
DCCS
r
.720
.683*
.584*
n
6
10
10
r
.614
.220
n
8
8
r
.286
n
13
* Correlation is significant at the 0.05 level (1-tailed).
** Correlation is significant at the 0.01 level (1-tailed).
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Development & Executive Function
significantly correlated with either EF measure.
Scatterplots showing these relationships are
presented in Figure 3. Next, partial correlations
were used to examine the unique association
between the measures of verbal and non-verbal
development and the measures of EF for participants with DS. The group with WS were
excluded from this analysis because no bivariate correlation was found between Leiter and
the measures of EF. Again, one-tailed tests were
used due to a priori assumptions that all correlations would be positive. When controlling
for performance on the Leiter, there remained
a significant association between the DCCS and
PPVT among participants with DS, r (7) = .75,
p = .01. When controlling for performance on
the PPVT, no association was found between
the DCCS and Leiter among the participants
with DS, r (7) = .15, ns. When controlling for
performance on the Leiter, no association was
found between SOP and PPVT for the participants with DS, r (7) = .40, ns. When controlling
for performance on the PPVT, no association
was found between SOP and Leiter for the participants with DS, r (7) = .16, ns.
Discussion
The relationships among measures of verbal
and non-verbal development and EF components of cognitive flexibility and working memory for persons with DS and WS were examined. In accordance with the verbal mediation
model of Vygotsky and Luria, we expected that
verbal development would be associated with
performance for both executive function tasks
for both groups of participants. This hypothesis
was supported. In both groups, verbal development was significantly correlated with both
measures of EF, but non-verbal development
was also correlated with both measures of EF
among the participants with DS. Partial correlations showed that the association between
cognitive flexibility and verbal development
was robust even when controlling for the nonverbal development, but no partial correlations
with working memory were significant.
These findings can be contrasted to those of
Hongwanishkul et al. (2005) who administered
the same tasks to a group of 98 three to five
year olds. They reported that verbal MA was
significantly correlated with both the DCCS
Verbal Mental Age by DCCS
DS
Verbal Mental Age by SOP
9
WS
2
SOP Score
DCCS
3
1
0
0
20
40
3
0
20
Nonverbal Mental Age by DCCS
DS
WS
2
1
0
0
20
40
60
80
Nonverbal Mental Age (Months)
40
60
80
100
Verbal Mental Age
100
DS
140
WS
6
3
0
0
20
40
60
80
100
Nonverbal Mental Age (Months)
Figure 3. Scatterplots of verbal mental age and non-verbal mental age by DCCS and SOP
scores for participants with Down syndrome and Williams syndrome
v . 18 n .2
120
Nonverbal Mental Age by SOP
9
SOP Score
DCCS
3
WS
6
0
60 80 100 120 140 160 180
Verbal Mental Age
DS
120
32
Landry et al.
(.44) and SOP (.28) tasks after CA was partialled
out, but that after partialling out chronological
age, performance mental age was only correlated with DCCS (.46). The similarity in correlation coefficients between both measures
of intellectual functioning and the DCCS support our contention that verbal and non-verbal
intelligence cannot be easily parsed in typically
developing children.
Likewise, our data suggest a stronger association between EF and verbal relative to non-verbal intelligence. Cognitive flexibility was positively associated with verbal abilities among the
participants with DS even after controlling for
non-verbal abilities, whereas non-verbal abilities were no longer correlated after controlling
for verbal abilities. Among the participants with
WS, verbal but not non-verbal development was
associated with cognitive flexibility. These findings support the special role of verbal development in the development of cognitive flexibility.
Working memory was also associated with the
verbal abilities of both groups. In contrast, nonverbal abilities were only correlated with working memory among the participants with DS,
although neither verbal nor non-verbal abilities were significantly correlated with working
memory after controlling for the other.
The finding that verbal, and not non-verbal,
abilities were associated with performance on
both EF tasks among both the participants with
DS and those with WS is relevant to theories of
verbal mediation, as they display contrasting
patterns of strength and weakness with respect
to verbal and nonverbal development. The finding that performance was associated with verbal
development both for children for whom language is a relative strength (children with WS)
and for who it is a relative weakness (children
with DS) lends support to the notion that cognitive flexibility and working memory are verbally mediated, even among persons with mental retardation of differing aetiologies. Further,
experimental findings of verbally mediated
performance for typically developing children
(Jacques & Zelazo, 2001; Kirkham et al., 2003)
suggest that, despite intellectual impairments,
the developmental principles under operation
for the typically developing children appear to
be applicable to both persons with Down syndrome and those with Williams syndrome.
Further research is needed on the relationship
between verbal and nonverbal development and
EF measures in both typically and atypically
developing groups. The numbers of participant
in the two groups in this study were small and
fell within a restricted mental age range. In addition, the broader variability in CA and MA ranges represented among the participants with WS
than among participants with DS, might be partly responsible for the group differences in the
correlations. Future studies could include more
tasks that are appropriate to assess achievement
from a wider range of mental ages, and could
also be focused on the relationship between language development and additional measures of
cognitive flexibility and working memory. Our
failure to record gender or sociodemographic
data of our participants should also be corrected
in future reports of studies.
Preliminary evidence for the primacy role of
language development in cognitive flexibility
and working memory was provided. However,
we only used one measure of verbal ability,
the PPVT, which is widely used to estimate
children’s language level but is only a measure of children’s receptive vocabulary. Further
research should also include more comprehensive language measures that provide both a
more global assessment of children’s language
development as well as the ability to break
down language development to determine if
any aspects are more or less pertinent to verbally mediating behaviour. The findings reported
by Jacques and Zelazo (2001), Kirkham et al.
(2003), and Zelazo et al. (2003), as well as those
described by Luria (1961), demonstrate that
typically developing children who have not yet
incorporated speech into their problem solving
can learn the strategy from adults. This could
be useful to develop training programs for children with Down syndrome and Williams syndrome, who might have the necessary language
skills but have not spontaneously developed
the verbal mediation strategy.
Key Messages From This Article
Professionals: For practical skills like problem
solving and planning, the language skills of the
client should be considered in setting developmentally appropriate goals. Educators may
want to focus on language skills in early years.
Policymakers: Individuals with developmental
disabilities have a wide range of skills and skill
levels and deserve opportunities to maximize
their skills.
JoDD
33
Development & Executive Function
Acknowledgements
The authors thank the participants in the
study, who include students from Summit
School (Montreal), clients of the West Island
Readaptation Center (Montreal), and attendees
at the annual Williams Syndrome Conference.
We thank Nicki Saros and Rhoda Root for their
help in recruiting the participants with Down
syndrome. The work on this project was funded
an operating grant from the Social Sciences and
Humanities Research Council to Jacob A. Burack.
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NOTE: A fully edited version of this article can
be found on the OADD website at http://www.
oadd.org/index.php?page=755 after August 15,
2012.
A Study of Nigerian Families Who Have
a Family Member with Down Syndrome
Abstract
Volume 18, Number 2, 2012
Author
Paul M. Ajuwon
Department of
Counseling, Leadership,
& Special Education,
Missouri State University,
Springfield, MO
Correspondence
The Family Quality of Life Survey (FQOLS) (Brown et al.,
2006), was administered to a group of 31 families who have
children with Down syndrome enrolled at a community-based
centre in Nigeria. Ideas on how families perceive services for
their children, and the extent to which families are coping, are
discussed. Suggestions are made for effective policy and practice
and for enhanced understanding of disabilities by the society.
This research project focuses on an exploration of quality of
life of families that have children with Down syndrome (DS)
in Nigeria, using the Family Quality of Life Survey (Brown et
al., 2006). Although this instrument has been widely applied
to the measurement of family satisfaction and well-being in
some Western countries, the utilisation of the instrument is
just now beginning to be employed not only as a research
method, but also as an approach to understanding disabilities and family dynamics in a developing country like
Nigeria (Ajuwon & Brown, 2012).
In the study to be reported, the FQOLS was utilized to assess
several domains and dimensions reflecting the major components and characteristics of family quality of life of 31 families who have children at the Down Syndrome Foundation
of Nigeria (DSFN). The DSFN, established in 2001, is a privately-operated community-based centre in Lagos, Nigeria.
It is the only centre of its kind in Lagos for educating learners, most of whom have been diagnosed with DS (see http://
www.dsanigeria.org). This study aims to provide a thematic
analysis of the qualitative comments supporting the quantitatively measured domains and dimensions of the survey
and to explore their relationships to one another.
[email protected]
missouristate.edu
Method
Keywords
Instrumentation
families;
quality of life;
Down syndrome;
satisfaction;
well-being;
Nigeria
The FQOLS is a comprehensive tool for examining family quality of life, and is used to gather both quantitative and qualitative information. The survey tool consists
of a section requesting details about the family, and nine
domains: Health of the Family, Financial Well-Being, Family
Relationships, Support from Others, Support from DisabilityRelated Services, Influence of Values, Careers/Preparing
for Careers, Leisure and Recreation, and Community/Civic
Interaction.
Each domain contains questions related to six
key dimensions: Importance, Opportunities,
Initia­t ive, Attainment, Stability, and Satis­
faction. The questionnaire has now been used in
research in 20 countries. For a detailed description of the components of the survey, see Brown
et al. (2006); Brown, Anand, Fung, Isaacs, and
Baum (2003); Isaacs, Brown, Brown, et al. (2007);
and Werner, Edwards, Baum, Brown, Brown,
and Isaacs (2009).
Procedures and Respondents
The researcher obtained formal approval from
his university in the United States to conduct the
study. On his arrival in Nigeria, the researcher
convened a meeting with the management of
the Down Syndrome Foundation of Nigeria.
Approval was obtained to invite willing parents
to the centre for a meeting at which the nature
and scope of the study were explained, and one
participant from each household was subsequently recruited for the project. These participants represented families with diverse socioeconomic and educational backgrounds. Since
English is the lingua franca in Nigeria, study
participants had no difficulty in understanding
and/or answering the questionnaire items. A
clerical worker at the centre was identified and
trained to assist with the administration of the
instrument. The survey was completed by the
volunteer respondents in small groups at the
centre over a five-day period. From a standpoint
of transportation and organizational efficiency, it
was more effective to have the parents complete
the survey when they brought their children to
the centre in the morning, and/or returned them
home in the afternoon.
A total of 31 out of 45 parents having a family member with DS completed the survey.
Respondents were nearly evenly split between
male and female (15 vs. 16). The majority of
male respondents (8) were the father of the DS
family member. Likewise, most female respondents were the mother of the DS family member.
Seventy-four percent (23) of the 31 respondents
reported belonging to an immediate family with
two parents.
Characteristics of Household Members
and Caregiving Responsibilities
In one family, there was a pair of 8-year-old
twin girls with DS, bringing the total number
of DS family members in the study to 32. More
v . 18 n .2
35
Nigerian Families Who Have a Family Member with Down Syndrome
than half of the DS family members were male
(56%). Their ages ranged from 1 to 40; 56% were
minors under the age of 18. Only one DS family
member was not living at home, specifically, a
24 year-old female.
With regard to caregiving roles, most families
(29 or 94%) included the biological mother of
the family member with DS. A majority of these
mothers (83%) both lived in the home and were
caregivers. They ranged in age from 28 to 72,
with a mean of 46.7. Most families (23 or 74%)
also included the biological father of the DS
family member, 65% of whom both lived in the
home and were caregivers. Their age range was
35 to 69, with a mean of 48.6. Thirty-five percent
of respondents reported the presence of one or
more siblings of the DS family member living
at home and acting as caregivers. These 22 siblings ranged in age from 7 to 35. The mean age
of the 10 male siblings was 21.1, while the mean
age of the 12 female siblings was 23.25.
Involvement in family life appeared to
be distributed across all family members. Respondents were asked which family member(s) was the most involved with the
daily life of the DS family member. Mothers
(alone) were named most frequently (18 or
58%), followed by a combination of parents
and/or other family members (12 or 39%); only
one father (alone) was named. With regard to
the amount of personal responsibility that the
respondent had for the family member with
DS, responses were almost evenly divided into
thirds as follows: 32% (10) indicated they had
either “‘less’ or ‘about the amount of’ responsibility that I like,” 32% (10) indicated they
had “more responsibility than I would like,”
and 36% (11) indicated they had “much more
responsibility than I would like.”
Results
The following results are responses from the
family respondents. As such they are their
perceptions of the quality of life of their family. Others may have differing perceptions, but
since the primary carer is making the responses, they are likely to be views which play an
important role in driving the family behaviours
and actions.
36
Ajuwon
Developmental and Health Issues
Associated with Down Syndrome
As in other countries, the well-being of many
Nigerian children and adults with DS is negatively impacted by multiple developmental and
health issues. Table 1 summarizes the open-ended comments made by the respondents when
asked to describe the physical, mental, or behavioural conditions that they had identified.
Over three-fourths of respondents cited speech
or language difficulties and delays, including
receptive and expressive language, mutism,
and incoherent speech as primary concerns to
providing effective care. Behavioural problems
involving poor coordination, restlessness, inattention, and inability to follow directions were
discussed by 29%, followed by developmental
delays related to physical growth, feeding, toileting, and cognition by 19%. Fewer remarks
were made regarding physical and mental
health problems.
Overall, the open-ended comments from respondents to this last question, together with the
question “Is there anything else you would like
to tell us about your family member(s) with an
intellectual or developmental disability?” yielded a variety of interesting insights. For example,
ten respondents indicated that their DS family member’s well-being was inhibited by poor
social skills and a tendency to social timidity in new situations. These children with DS
often required constant parental supervision to
interact with the wider community. Conversely,
two parents noted that their experiences were
enhanced for themselves and their child with
DS because she/he had a generally happy disposition or a desire to learn new things.
Daily Caregiving Experience with a
Family Member with Down Syndrome
Over a third of respondents (12) identified
caregiver burden as the primary stressor in
their life. The daily burden was physically and
financially overpowering for these caregivers
and their families, because for most families
“home care assistance” was unavailable. This
daily burden resulted in loss of other normative activities for caregivers – from social activities to loss of employment and independence
for mothers, who were most often the primary
caregiver.
Six respondents expressed specific concerns
about the future well-being of their DS family
member. These concerns were rooted in a lack
of services that kept the adult child with the
family and dependent on the family. For parents, the caregiver burden increased with age
because disability-specific services were even
less accessible when their child transitioned to
adulthood. At the same time, individuals with
DS had very limited access to vocational education and self-help training that would promote
even basic independence in adulthood. In one of
the interview sessions, an exasperated mother of
a teenage daughter with DS remarked thus: “I’m
not sure what will become of my daughter now
that she is getting to that age, when other teen-
Table 1. Summary of Qualitative Descriptions of Physical, Mental, or Behavioural Condition
Condition
N
Percent
Speech or language difficulties and delays1
24
78.4
Behavioural problems
9
29.0
6
19.4
4
12.9
2
6.5
2
Developmental delays
3
Physical health problems
Mental health problems
3
4
5
1
2
4
5
Receptive and expressive language, mutism, and incoherent speech
Poor coordination, restlessness, inattention, inability to follow directions
Physical growth, feeding, toileting, cognition
Heart condition, poor vision and/or hearing, chronic digestive problems
Mood or anxiety disorders (including schizophrenia) and anger
JoDD
age girls without disability are doing some type
of work, or engaging in social activities with
boys, and looking ahead to possible relationship
and marriage. Frankly speaking, I do not know
how to handle the situation, and I am not going
to be around forever to look after her!”
A number of caregivers (41.9%) indicated that
personal and family attitudes enhanced their
caregiving experience. These attitudes were
captured in three broad themes: first, caregiving was a family commitment; second, they
accepted the DS family member’s condition and
the primacy of the caregiving role; and third,
their family member with DS brought a special
joy and love to their entire family, in spite of
negative experiences they sometimes encounter
in society.
Health Care Issues for Affected
Families
Adequate health care is a luxury for most
Nigerians, and barriers to health care services
were broadly themed as: affordability, accessibility, and public attitude. It was revealed
by most families (54.8%) during group interviews that affordability was the primary issue.
Factors included the poor state of the Nigerian
economy, low personal and family incomes, the
high cost of good private health care, the high
cost of healthy food, and the need for ongoing
care for some individuals with DS. Respondents
linked the high cost of medical care to potential health risks. Since medical care and prescription drugs were unaffordable for at least
50% of participants, many families relied on
unregulated herbal remedies from traditional
healers for many ailments. These products and
practices come with unknown risks and side
effects. First, traditional healing practices may
not be effective with developmental conditions
like Down syndrome and associated behaviours (although some herbalists are known to
lay claim to such a “remedy”). Second, unregulated herbal medicines may have unknown side
effects, especially when prescribed for special
needs populations.
Financial issues were identified in a broader
context, that is, financial need and long work
hours generally impeded families from pursuing healthy lifestyle choices like regular exer-
v . 18 n .2
37
Nigerian Families Who Have a Family Member with Down Syndrome
cise. Respondents indicated access to basic
health care services was problematic for most
families and most problematic for persons with
a developmental disability. Key inhibitors were
systemic: scarcity of publicly funded health
services, unaffordable private care, absence of
governmental support for low-income families,
and poor economic conditions. Consequently,
public health facilities were under-funded,
poorly equipped, and lacked specialists having disability-specific skills or knowledge. This
point was reinforced by one male participant,
thus: “When our baby son with DS was born in
the public hospital, the doctors and nurses were
not of much help. We spent our meager savings
on searching for a “cure” from traditional healers. Luckily, through the DSFN, the Kanu Heart
Foundation provided us with financial support
to go to India for three months where successful open heart surgery was done. Today, our
baby is doing well physically, although he still
requires help in feeding and other social skills.”
Feeling neglected was a major theme expressed
by respondents. An underfunded public health
care system, along with poor attitudes of health
professionals towards persons with disabilities, little or no early intervention, and poor
dissemination of knowledge to patients were
key issues raised by respondents. One frustrated mother of 8-year-old twin daughters with
DS angrily stated: “When I was carrying the
pregnancy, the hospital staff either did not do
proper pre-natal testing or failed to inform me
of their conditions. If I had been told, I would
have chosen to terminate the pregnancy. Six
months after the twins were delivered, my husband ran away, and I’ve since been left alone
to look after them. But, I owe a lot to the DSFN
where the girls are now enrolled and where I
also work as a teacher.”
Still, there is increasing public awareness of
health care needs and a growing expectation of
access to services in Nigeria. This shift in public attitude and health knowledge is driven by
media access, mainly radio and television, but
also newspapers and magazines. This is leading to increased levels of frustration because
families cannot access modern services they
know will improve their health and wellbeing, because of the high cost of obtaining
such health-related services. This declining
situation has led some observers to believe that
38
Ajuwon
Nigeria may be unable to meet the health-related Millennium Development Goals (MDGs) by
2015, which mandates each country to spend
15 percent of their yearly national budget on
health. Nigeria currently spends less than seven
percent annually (http://www.nigerianbestforum.com/generaltopics/?p=85074).
Respondents also described enhancers and
inhibitors to health care access. Geographic
location, along with personal behaviours and
attitudes, were prominent themes that respondents identified as enhancing health outcomes.
In spite of concerns about transportation and
cost, residents of large urban centres had much
better access to care.
Personal behaviours were the primary means
for most respondents to maintain health. They
indicated that eating well or improving their
diet, getting regular exercise, improving their
knowledge of what is required to maintain
good health, and trying to access appropriate
services were major contributors to well-being.
Personal attitudes strongly linked to positive
health behaviours included having an optimistic outlook on daily life; contentment with life
situation; and a desire for better health. These
attitudes were often integrated into their faith
and daily religious practices.
Financial Well-Being of Families
Three-quarters of participants expressed dissatisfaction with galloping inflation, general
insecurity in their communities, the widespread degradation of the environment occasioned by traffic congestion, toxic wastes, air
pollution, frequent power outages, constant
noise from generating plants, and incessant
flooding of urban centres that lack poor drainage systems. They cited ineffective social support programmes for persons with disabilities
and their families. Almost all respondents commented on lack of access to basic amenities and
infrastructures for good quality of life. It was
obvious that chronic daily struggle to survive
when raising a child with a disability intensified with the poor state of the economy.
Slightly over two-thirds of respondents (67.7%)
indicated a declining financial position. Some
experienced long-term unemployment; many
were underemployed and worked long hours
to keep their jobs. Chronic unemployment was
also a reality for many young adults with no
disability who were graduates of post-secondary institutions and still living at home several
years after graduation.
Three prominent themes emerged that related
to poor future financial outcomes for families.
First, family financial well-being was negatively impacted by inflation that dominates the
economy. Second, aging and retirement issues
were of concern for older caregivers. Many
indicated poor earnings over their life course
would be compounded by poor-quality pensions that are not paid on a regular schedule.
Retirement would mean less income and less
stable income, but not less caregiving responsibility. Third, a majority of respondents (80.6%)
lamented the pervasive corruption at national,
state, and local levels. Such corruption was seen
as contributing immensely to the high levels of
social and economic instability in the country
(Dike, 2008; Sulaiman, 2008).
However, some respondents indicated their
financial situation was improving. A few
reported good financial management and
good-performing investments, while others indicated job promotion or new business
opportunities had enhanced family finances.
Generally, respondents indicated that employed
young adult children with no disability still
living at home enhanced the financial wellbeing of the family. Some indicated membership in cooperative societies enabled them to
access additional resources for meeting needs.
As with many other aspects of family life in
Nigeria, respondents indicated personal effort
and faith, which, along with a positive attitude
that things will get better, was central to current and future well-being.
Building and Maintaining Family
Relationships
Ninety percent of respondents described
strong, positive social and emotional relationships with nuclear and extended family members. These relationships were enhanced in
families that followed traditional, cultural values and actively practised their religious faith.
Caregivers often received important emotional
and instrumental support from adult children,
JoDD
39
Nigerian Families Who Have a Family Member with Down Syndrome
extended family, and their religious community. Satisfaction with family relationships was
multi-dimensional. Some caregivers associated
family satisfaction with personal growth and
the ability to access training and continuing
education. Most suggested satisfaction with
family was related to mature personal attitudes. For older caregivers, family attitudes
that valued the elderly and sought out their
advice on important matters greatly added to
satisfaction in this domain.
On the other hand, satisfaction with family
relationships was inhibited by chronic stressors related to urban life, financial hardship,
and long hours of work that reduced contact
between members. For some caregivers, satisfaction decreased when they had employed
adult children struggling to make ends meet in
their own families and unable to offer support
to their siblings with DS or to aging caregiving
parents. These employed adult children were
employed in jobs that paid considerably less
than their qualifications; yet, a great proportion
of their monthly income was spent on transportation to and from worksites.
Accessing Community Support
Most participants (58%) indicated access to
community supports was largely inhibited by
a lack of economic resources, along with the
time burden of daily household duties and
child care responsibilities. Additional caregiving for the family member with DS exacerbated this social isolation. Some identified work
responsibilities and the lack of encouragement
from others as key factors that limited access
to community supports. Three respondents
mentioned self-imposed isolation because they
feared those outside their religion or immediate family. The general economic turmoil in
the country contributed to social isolation of
some participants. Poverty, lack of job security,
and a daily struggle to survive can create an
environment in which people do not support
others outside their immediate family. This is
especially true in large urban areas, where the
influence of Western society has challenged
complex social relationships present in traditional Nigerian cultural roles and eroded traditional family values.
Satisfaction with community support was
enhanced by individual desire to socialize
and ability to achieve balance between work,
personal and domestic needs. Respondents
globally indicated that receiving emotional
and instrumental support, the presence of religious faith and traditional family values, and
the growing independence of the DS family
member improved satisfaction in this domain.
Caregivers noted that community visibility – engaging in community activities with
their affected child – resulted in improved
social awareness and acceptance of disability. One parent described her experience as …
“Interaction with the community can go a long
way towards showing them that we love our
daughter with a disability. This will be a way to
tell others in a similar situation to accept their
offspring who may have a similar disability.”
In turn, improved community understanding
of disability resulted in greater social acceptance of persons with disabilities and their
families as integral members of the community. In the words of one caregiver … “The best
approach to getting the community informed
about disability is for the families of the disabled to continue to involve their children in
community-based programmes. That way, the
public will gradually become more enlightened
about their abilities and needs.”
Accessing Support Services
Caregivers identified three specific categories
of disability-related support services. These are
summarized thus:
A.Professional services:
1. Speech and language therapy, occupational therapy, and audiology services
2. Behaviour therapy
3. Specialized pediatric services and medical services for persons with disabilities
4. Sexuality training for adolescents and
young adults with DS
B. Community-based services:
1. Vocational training, including adapted
computer programmes
2. Advocacy training for parents
v . 18 n .2
40
Ajuwon
3. Nutritional services/counselling for family
4. Psychological counselling and self-help
skills training for individuals with DS
5. Adapted recreation activities
6. Specialized transportation
C. Home-based services:
1. Specialized health care services
2. Social workers
3. Respite care and related home support
services
The major barriers to support services are summarized as being the following: the high cost
for private school and specialized services and
training; the limited number of governmentsupported schools for the disabled that are not
well-staffed or equipped; and poor economic
conditions that eroded family incomes and
prevented them from purchasing private services. It was noted by a small number of participants (19.3%) that access to these services
was enhanced by: sufficient personal income to
purchase private services; proximity to private
schools and services; and the presence of philanthropists or organizations funding special
education and rehabilitation programmes.
However, for a number of caregivers (12.9%),
access to advanced education or communitybased services decreased even further with
transition to adulthood, and there was no vocational training for the disabled adult population. Service access was further inhibited by
lack of finances for some parents to purchase
their own car and the poor public transportation systems in some neighbourhoods. This situation has forced two parents to resort to using
the “Okada” (the local motor cycle) to bring
their child with DS to and from the Centre
each day. Underlying the access problem was a
lack of national interest or policy to develop a
transportation-friendly programme, especially
in urban centres of the country. As one parent
noted: “The government would be improving
the quality of training if it can set up a special
transportation programme for children and
youth who are disabled. Such a service would
greatly relieve the burden of parents who
spend several hours in the traffic each day as
they bring their child to and from school.”
Influence of Values
Personal and community world views were
strongly influenced by formal religious values,
strong religious faith, and traditional cultural
values. Caregivers noted that traditional superstitions associated with any form of disability
were problematic and must be replaced with a
modern understanding of disability. They identified religious and cultural leaders as the key
to this shift in knowledge and practice. While
many leaders did not promote disability awareness in their everyday dialog and actions, places
of worship among Moslems, Christians, and
traditional believers were viewed as appropriate
venues to educate the broader public on disability-related issues, given the extreme religiosity
of the people. It was suggested that professionally trained special educators and counsellors
should work closely with such religious leaders
to increase their knowledge and awareness, as
well as enhance the integration into religious
activities of members with disabilities.
Indeed, religious values can be seen to have
strong protective effects for individuals and
families, reducing the negative impacts of daily
life stressors and challenges associated with
raising a child with a disability. The influence of
religious beliefs and traditional cultural values
can therefore be translated into greater personal
and family worth and dignity and a sense that
each person is valued for who they are, with
special “compassion and love for the disabled.”
Career Development
Respondents indicated that career development
was influenced by personal and structural factors. Individuals needed an enthusiastic attitude to learn, along with specific educational
goals that focused on completing higher education or vocational training opportunities. One
family succinctly characterized this philosophy
thus: “Education is the passport to prosperity.”
In the majority of cases (52%), of participants
stated that access to scholarships for education
was crucial to success. However, such scholarships are becoming limited, due to reduced
education funds at both federal and state levels. To compound the situation, parents were
frustrated with frequent strikes by lecturers in
government-owned post-secondary institutions
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41
Nigerian Families Who Have a Family Member with Down Syndrome
agitating for better learning climate, as well as
improved salaries. Indeed, such instability in
higher education imposed serious limitations
on opportunities for most students, and this in
turn negatively impacted their career prospects.
In addition to the high cost of education, it was
observed that the curricula in the post-secondary institutions failed to inculcate in students the knowledge and skills needed for selfemployment. The outcome was labour instability with high unemployment rates in general,
and for new graduates in particular. There were
even fewer work opportunities for older workers
and individuals with any identifiable disability.
Some employed family members observed that
their employers made little or no provision for
advanced training on the job. Other inhibitors
to career development included the depressed
economy and the absence of effective policy to
support education, training, and employment.
Leisure and Recreation Pursuits
In the home, joint leisure and recreation for
families with DS focused on watching TV
and movies together, listening to music, and
reading. Other home-based pursuits included
entertaining visitors and cooking. Two-thirds
of respondents commented they lack the funds
to purchase computer and computer games for
their children with ID. In addition, these parents decried their inability to teach basic specialized computer literacy skills. Yet they feel
that access to home-based computers and toys
can supplement the school learning activities of
their children with DS, and promote interaction
with siblings and friends.
Thirty-nine percent of caregivers described the
poor availability and high cost of organized
recreational activities in large urban areas.
Lack of structured activities for individuals
with any form of disability was problematic for
all families and reflected the low value placed
on disability-related services by society and the
government. Low personal and family incomes
and the high cost of public transportation further impeded family participation in many
formal activities. For some families, caregiving burden and general family demands left no
time for leisure and recreation.
Community Interaction
Respondents indicated that much of their community interaction involved faith based activities – participating in choirs, or doing volunteer
activities. They also relied on social interactions
with extended family members. Many families were members of the Centre, and service
clubs like the YWCA. It was noted by some
participants that the Centre periodically organizes community-based events that positively
promote the aims of the centre. Such activities included: walkathons, running, and soccer
matches.
Analysis of Domains and Dimensions
A majority of families (85%) indicated their
community activities were centred on religiousbased events. However, a small percentage
of respondents (19.3%) also spoke of going on
family vacations, visiting friends and extended
family members in the village, shopping, picnics, and outdoor sports as favorite pastimes.
Table 2 provides the means and standard deviations of the Family Quality of Life Survey
measures by the six dimensions (Importance,
Opportunities, Initiative, Attainment, Stability,
and Satisfaction) within each of the nine
domains (Health of the family, Financial wellbeing, Family relationships, Support from others, Support from services, Influence of values,
Careers or preparing for careers, Leisure and
recreation, and Community interaction). In
addition, the last two columns display the mean
and standard deviation for each Domain Score,
that is, the arithmetic mean of the six dimension
measures within each domain.
Most caregivers (81%) expressed an understanding that leisure pursuits contributed to
physical and emotional well-being. They indicated that their leisure choices were based on
activities that helped the family to relax, but
inclusivity was a factor and the activity was
selected to include the member with DS.
On average, families gave the least importance
to the domains of Support from others (mean
response on a scale of 1 to 5=3.55) and Leisure
and recreation (3.81), and the most importance
to financial well-being (4.87) and Health of
family (4.90). The fewest opportunities were
associated with Support from services (1.84)
v . 18 n .2
42
Ajuwon
Table 2. Means and Standard Deviations of FQOLS Responses by Six Dimensions and Nine Domains
Importance
Oppor­
tunities
Initiative
Attainment
Stability
Satisfaction
Domain
Score
Mean SD Mean SD Mean SD Mean SD Mean SD Mean SD Mean SD
Health of
family
4.90 0.40
2.06 0.89
3.61 1.28
3.55 1.03
3.65 0.98
3.32 1.14
3.52 0.56
Financial
well-being
4.87 0.34
2.39 0.84
4.32 0.94
3.16 1.10
3.48 1.24
3.06 1.18
3.55 0.66
Family
relationships
4.65 0.80
4.39 0.62
4.68 0.65
4.48 0.85
4.19 0.91
4.29 1.04
4.45 0.59
Support from
others
3.55 1.21 2.33 0.88
2.68 1.17 2.35 0.76
3.19 0.54
3.00 0.89 2.83 0.59
Support from
services
4.61 0.62
1.84 0.82
3.97 1.20
2.10 0.94
3.10 0.79 2.84 1.10
3.08 0.54
Influence of
values
4.61 0.67
4.13 0.96
4.45 0.77
4.48 0.85
4.23 0.84
4.06 0.96
4.33 0.70
Careers/
preparing
for careers
4.77 0.50
2.63 1.19
4.43 0.82
3.86 1.16
3.48 1.06
3.29 0.94
3.75 0.67
Leisure and
recreation
3.81 0.98
2.90 0.94
3.52 0.93
3.45 0.96
3.55 0.89
3.29 1.04
3.42 0.76
Community/
civic
interaction
3.84 1.00
3.35 0.92
3.35 1.08
3.23 0.92
3.45 0.81
3.52 1.03
3.46 0.77
and Health of family (2.06), while the most
opportunities were associated with Influence
of values (4.13) and Family relationships (4.39).
The least initiative, on average, was taken by
families with respect to Support from others (2.68) and Community or civic interaction
(3.35), and the most initiative was taken with
respect to Influence of values (4.45) and Family
relationships (4.68). Support from services (2.10)
and Support from others (2.35) were rated the
lowest domains for attainment, while Family
relationships (4.48) and Influence of values
(4.48) were rated the highest.
Support from services (3.10) and Support from
others (3.19) were perceived to be the least stable domains, and Family relationships (4.19)
and Influence of values (4.23) were perceived to
be the most stable domains. The least satisfaction was associated with the domains Support
from services (2.84) and Support from others
(3.00), while the most satisfaction was associated with Influence of values (4.06) and Family
relationships (4.29).
The bar chart in Figure 1 graphically displays
the mean and standard deviation for each of
the nine computed Domain Scores in descending order by mean value. As shown, the two
highest means are for Family relationships
(4.45) and Influence of values (4.33), while the
two lowest means are for Support from services
(3.08) and Support from others (2.83).
Discussion
Living with a child with disability in the
Nigerian society can have profound effects
on the entire family dynamics. In this study,
respondents expressed their perspectives as
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43
Nigerian Families Who Have a Family Member with Down Syndrome
5.00
Likert Scale Value
4.00
3.00
4.45
4.33
3.75
3.55
3.52
3.46
3.42
3.08
2.00
2.83
1.00
Influence
of values
Family
relationships
Financial
well-being
Careers
Community
Interaction
Health
of family
Support
from services
Leisure and
recreation
Support
from others
Figure 1. Means and standard deviations of nine FQOLS domain scores
they provide meaningfully for their children
with DS. Table 2 and Figure 1 data are reasonably comparable with other studies (Brown,
2010; Cagron, Schmidt, & Brown, 2011; Clark,
Brown, & Karrapaya, 2012; Werner, Edwards,
Baum, et al., 2009). As Brown (2010) points
out in a comparison of data from different
countries using the same survey in terms of
satisfaction and attainment dimensions, for
example, the Nigerian data (Ajuwon & Brown,
2012) shows the lowest rating in Support from
Others, and Support from Services and, again
in terms of consistency with other countries,
Family Relations and Health of Family were
rated highly, and this is also true of the sub-set
of Down data in the present study. However,
there are differences, and in the present study
values were consistently high on all dimensions
which, as pointed out by Brown (2010), may
have reflected the importance of religious and
spiritual beliefs. Although there were often lev-
v . 18 n .2
els of variation between higher and lower economic countries, similar domain patterns tend
to exist though complicated by family attitudes
and societal values (see Brown, Hong, Shearer,
Wang, & Wang, 2011).
Discussion also needs to reflect on the variations within domains and dimensions within
a country. An average rating below 4 for any
domain or dimension suggests that many of
the families perceived themselves as having
low family scores. Where the scores are below
3, most families fell in this negative range. For
example, all of the dimensions associated with
the domains covering Support from Others and
Support from Services represent aspects that
require particular attention both in planning
and practical support (Brown, Hong, Shearer,
Wang, & Wang, 2011; Wang & Brown, 2009),
and this issue is further illustrated in the qualitative information above.
44
Ajuwon
It appears that only one other FQOL study has
been carried out with families having children
with Down syndrome as a separate group
(Brown, MacAdam-Crisp, Wang, & Iarocci,
2006). Again, the pattern of results appears
reasonably comparable to the present study.
However, it would be interesting to know
whether in the current study Down families
gained higher domain scores in otherwise vulnerable areas in other groups of families where
there are multiple disabilities and/or behavioural challenges. It seems possible that social
attitudes and environmental opportunities may
be important variables in this context.
Some of the qualitative comments point to the
implications of not having in place concrete
special education, health and social policies.
This gap in service has impeded overall development and community integration of their
children, leading to a decline in most family
members’ life satisfaction and well-being. One
participant noted: “This is not the kind of lifestyle I wish for members of my household or
anyone for that matter … Nigeria has abundant
natural resources that can be used to benefit
the masses … yet most of its citizens live in
poverty because of corruption on the part of
some government officials. These officials visit
the advanced countries where they see good
social programmes in place. But they refuse to
see anything good in bringing such practical
social ideas to our own land.”
Nonetheless, a handful of participants took
their son or daughter with DS with them to
places of worship, social gatherings, or to the
market place. However, teasing of children
with DS and accompanying family members
when in the public like the market-place or
even the neighbourhood water bore-hole was
not an isolated event. A number of participants mentioned social isolation because other
people avoided them in these public places. A
few respondents described denial of retail services because they brought their child with DS
to the market-place. Thus, based on such prevailing myths, a vendor of foodstuffs or shoes
may view selling her/his merchandise to a
buyer accompanied by a child with DS as illluck, especially if the transaction was the first
to occur that day. Similarly, a commercial bus
operator may disallow passengers with DS the
opportunity to ride in the bus, because of the
discomfort the presence of the child with DS
might cause the passengers. Even at church,
one mother observed the uneasiness of other
members the first time she went to service with
her daughter with DS. The mother noted: “After
service that day, I met with the Pastor to talk
about my daughter, and the need to include
aspects of the condition in future sermons …
and as time progressed, and my daughter also
began to show improved behaviours, I could
see changes in the attitudes and behaviours of
the people.”
Most Nigerian parents recognized that these
negative attitudes stem from superstitions that
are rooted in traditional beliefs about disability
in some communities (Okoh, 1987), a pressing
issue in a number of other countries (Brown,
2010). These superstitions point towards better
education about disabilities, and also indicate
that positive changes can be made with family
members who are willing to assume advocacy
roles.
From the current FQOLS study, levels of unmet
needs were found in relation to health, financial well-being, disability-specific support,
careers, and community interaction, and this
resulted in caregiver burden for most participants. The daily care situation often prevented family members (especially mothers) from
independently engaging in social activities.
Excessive care burden resulted in long-term
effects of chronic stress on the health status of
other family members. Thus, several respondents were concerned about their own future
well-being and that of their adult family members with DS. But the positive attitudes of some
respondents continue to inspire them to seek
training for their children, in spite of the high
costs of transportation, health care, and special education from privately operated schools.
These families feel that providing meaningful
special education to their children with DS is
a worthwhile investment, because they view
caregiving as a family commitment, and a way
to show special joy and love for their children
with DS.
Some parents of children with DS provide care
well in excess of that reported by other parents.
They spend several hours on basic care of their
children with DS in the evenings and/or week-
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Nigerian Families Who Have a Family Member with Down Syndrome
ends, and unable to access respite care services.
This situation ultimately interferes with the
ability of caregivers to work and contribute to
the household income and/or provide quality
care for other siblings, and to aged grandparents who live with them.
and their children with DS should expand their
focus to include a broader spectrum of the disability groups and their families.
A number of currently-employed respondents
commented that the skyrocketing inflation was
impeding their ability to purchase their own
housing and vehicles. One major concern was
the families’ constant struggle with high tuition
fees for their other children enrolled in privately run tertiary institutions. Furthermore, some
participants were dissatisfied that their trained
family members could not secure employment
after graduation from institutions of higher
learning. These educated older siblings without
disabilities continued to live at home, unable to
augment the household income, further inhibiting the families’ financial capacity to access
quality specialized resources. Clearly, these situations severely limited choices for the families
and their sons or daughters with DS. It is obvious that unless adequate social protections are
established for these families and their children
with disabilities, these families will continue to
experience declining life satisfaction and wellbeing as their children grow up.
In light of the multiple needs expressed by
families interviewed, and who also completed
the Family Quality of Life survey, the following
specific recommendations are made:
Limitations of the Project and
Recommendations for Future Service
One limitation of the current project relates
to the samples selection. As previously noted,
participants in this study were drawn from
the Down Syndrome Foundation of Nigeria, a
small centre in the city of Lagos. It should be
emphasized that study participants consisted
of mothers and fathers who had children with
DS at the Centre; hence, the views expressed on
family quality of life were those of the participants, and not necessarily those of the rest of
the household. As such, the opinions of other
family members should be explored in any
future research.
Although the participants demonstrated sufficient awareness of the impact of DS on the family structure, a larger sample of families from
the rest of the country would have provided
a more representative data. Future research
efforts aimed at exploring Nigerian families
v . 18 n .2
Recommendations
1. The DSFN should establish a variety of
professional services that focus on developing speech and language skills, appropriate behaviours, specialized pediatric and
health care services, and sexuality training
of adolescents with DS. Provisions must also
be made for in-home assistance including
counselling of parents and the siblings of the
child with DS.
2. The need for community-based vocational
services and computer literacy training
should be in the forefront of the Centres’
curriculum planning efforts, especially for
young adults. The focus should be to capitalize on indigenous customs that will be
inclusive of the individuals with DS, and
their families at school, home, and community events. This process should involve the
utilization of local resources and manpower
to sufficiently prepare trainees with DS for
employment and community integration, in
the public or private sector, or even as selfemployed individuals.
3. Although participants in this study are a
small fraction of the total number of parents
of children with disabilities in Nigeria, they
represent an exemplary group of advocates
who are seeking for their children quality
education that will guarantee for them a
functional and independent living. In order
to achieve this, these parents have demanded
respite care to allow them to receive a break
from care-giving. This is justifiable because
raising a child with a disability is challenging, particularly in a society that has no
social safety nets (Brown, Ajuwon, Wang,
& Vahakuopus, 2009). As several parents
have alluded to in this study, there are many
demands placed on families in the process of
adjusting to their children with DS, not the
46
Ajuwon
least of which are: the number of appointments for medical and support services,
challenges of learning how to provide and/
or adapt home supports, and working with
school personnel. These families are often
over-stressed as they try to juggle medical
appointments during work hours and care
for the children with a disability and their
siblings. Parents are tired and depressed, yet
they continue to sacrifice their own health
and well-being to meet care-giving obligations of their children.
4. Respondents commented positively on their
religious values and how they participate
with their daughter or son in activities organized by their worship centres.
In this regard, religious centres should capitalize on this and assume a major role in fostering greater acceptance of and respect for
persons with disabilities and their families.
This view is supported by a number of other
studies that point out that meeting child and
parent needs, particularly spiritual needs,
helps some parents deal with their challenges
(Kober, 2011). There are many ways leaders in
these places of worship can accomplish this.
They can give sermons that highlight the
dignity and worth of people, including children, youth, and adults with disabilities. A
mosque, a church or a shrine can sponsor the
education of a child with DS. They can sponsor educational field-trips or sporting events
like the Special Olympics. They can construct
classrooms at these special schools, or even
fund a child’s medical treatment that is often
beyond the means of the family. The ultimate
goal would be to identify with, and provide
moral support for, programmes appropriate
for the chronological age of the special needs
learners.
5. The themes describing family experiences
that emerged from the participants’ comments in this study indicate a strong need
for the development of government disability policies, as well as greater initiatives to
train family members or care-givers. In this
regard, federal, state, and local authorities
must set aside sufficient funds each year
to implement new programmes. However,
care must be taken to ensure that such programmes are planned and implemented
within the community, so members become
more cognizant of the difficulties that children with disabilities face, and are empowered to challenge the stereotypes held by the
public towards people with disabilities.
In conclusion, these recommendations will provide mutual support and joint action in advocating for the families and their offspring with
DS that are aimed at improving their overall
quality of life.
Acknowledgments
The author is grateful to Professor Roy I. Brown
for his support and invaluable ideas and guidance in expanding the discussion section of this
manuscript.
I would also like to express my gratitude to
Robert Downie and Rebecca Stallings for their
advice and the statistical analyses contained in
this manuscript.
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Note: At time of first publication of this
article, minor changes were pending. A fully
edited version of this article can be found on
the OADD website at http://www.oadd.org/
index.php?page=756 after August 15, 2012.
Relations of Parental Perceptions
to the Behavioural Characteristics of
Adolescents with Down Syndrome
Volume 18, Number 2, 2012
Authors
Meghan M. Burke,1
Marisa H. Fisher,2
Robert M. Hodapp1
Vanderbilt Kennedy
Center,
Nashville, TN
1
University
of Massachusetts,
Boston Center
for Social Development
and Education,
Boston, MA
2
Correspondence
meghan.m.burke
@vanderbilt.edu
Keywords
Down syndrome,
adolescence,
coping,
transition,
maladaptive behaviours
Abstract
This study examined how the behaviours of individuals with
Down syndrome relate to parent functioning during the adolescent years. Measures of personality, intelligence, adaptive,
and maladaptive behaviour were collected for 42 adolescents
with Down syndrome and related to parental depression, ways
of coping, worries about the future, and positive perceptions.
Across the adolescent years, most parents continued to feel
rewarded by their adolescents with Down syndrome; adolescents who displayed more positive personality characteristics had parents who felt more rewarded by their children.
Conversely, those who displayed higher levels of internalizing (but not externalizing) problem behaviours had parents
who were significantly more worried about their adolescents’
futures. Implications are discussed.
Many studies of families of individuals with Down syndrome
(DS) have identified a “Down syndrome advantage” (Hodapp,
Ly, Fidler, & Ricci, 2001; Seltzer & Ryff, 1994). This perspective holds that, compared to families of children with other
intellectual and developmental disabilities, families of individuals with DS cope better. Compared to these other families, then, families of individuals with DS tend to be warmer (Mink, Nihira, & Meyers, 1983), mothers experience less
stress (Hodapp, Ricci, Ly, & Fidler, 2003), fathers report close
relationships (Hornby, 1995), and siblings report increased
amounts of positive benefits (Hodapp & Urbano, 2007).
While the “Down syndrome advantage” does seem to exist,
the reasons for its existence continue to be debated. Some
argue that closer family dynamics may relate to the personality phenotype of the child. Specifically, children with DS
often have fairly low levels of behaviour problems, more positive personalities (Dykens & Kasari, 1997; Kasari, Mundy,
Yirmiya, & Sigman, 1990), and, compared to individuals
with other types of intellectual disabilities, often have less
difficult temperaments (Kasari & Sigman, 1997). Conversely,
advantages for families may be due primarily to demographic characteristics. On average, parents of children with
DS tend to be older (Urbano & Hodapp, 2007), to hold more
senior job positions (Corrice & Glidden, 2009), and to be more
affluent (Grosse, 2010). In some studies, controlling for such
demographic variables reduced the parental rewards of raising children with DS (Corrice & Glidden, 2009).
To date, most studies have been conducted with families of
younger children with DS (Hodapp, 2007), with little focus
49
Adolescents with Down Syndrome
on adolescents. But just as for individuals without disabilities, adolescence is a difficult time
for many individuals with intellectual and
developmental disabilities. Moreover, across
the adolescent years, age may be related to
subtle increases in internalizing behaviours for
many individuals with DS (Dykens & Kasari,
1997; Dykens, Shah, Sagun, Beck, & King, 2002).
Even if, when compared to those without DS,
adolescents with DS increase in internalizing
behaviours across adolescence, we do not yet
know how internalizing behaviours affect others. Do, for example, the increased maladaptive
behaviours of adolescents with DS affect parental reactions and their concerns about their
child’s transition to adulthood? Alternatively,
certain (mostly unchanging) demographic characteristics may continue to explain the “Down
syndrome advantage” across adolescence for
individuals with DS.
This study related parent perceptions to the
adolescents’ personality, behaviours, and IQ.
Specifically, this study cross-sectionally examined: (1) the personality, demographic, and
behavioural characteristics of adolescents with
DS; (2) parental coping styles, feelings of rewards,
positive perceptions, and worries for the future;
and, (3) the connections among the adolescent’s
characteristics and parental responses, positive
perceptions, and transition concerns.
Method
Participants
This study included 42 adolescents with DS
(26 M; 16 F) and their parents (3 M; 39 F).
Most participants were White (90.2%), and
the remaining participants were AfricanAmerican. Adolescents averaged 15.12 years of
age (SD = 2.82) with a range from 11–21 years.
All adolescents were in the mild to moderate
range of intellectual disability, with a mean IQ
of 45.93 (SD = 6.77; range from 40 to 61). The
mean standard score for verbal knowledge was
48.93 (SD = 9.38) and for non-verbal knowledge
was 50.05 (SD = 9.78). Of those who were still
in school, most adolescents (74.2%) were educated in a general education setting, while the
remainder attended self-contained special education classes. Over half (62.1%) had repeated
v . 18 n .2
a grade in school and parents reported that
their adolescents had an average of 1.81 friends
(SD = 1.13).
The mean age of the mothers was 48.18
(SD = 5.94) and of fathers was 50.36 (SD = 6.67).
Mothers worked an average of 21.49 hours
(SD = 19.6) outside of the home and fathers
42.31 hours (SD = 15.32). Family size averaged
2.83 (SD = 1.0) children and, in 73.1% of families, the mother was married to the father of the
adolescent.
Measures
Adolescents were administered the Kaufman
Brief Intelligence Test (K-BIT) (Kaufman &
Kaufman, 1990). A psychometric measure for
individuals aged 4–90 years. The K-BIT includes
a composite IQ as well as standard scores for
verbal and for non-verbal knowledge. The
K-BIT has been used with persons with intellectual disabilities (Dykens, Rosner, & Ly, 2001).
Parents also completed two packets of questionnaires, one of adolescent-related measures
and another of parent-related measures.
Adolescent-Related Measures
The Wishart Scale (Wishart & Johnston, 1990)
consists of 23 commonly-mentioned personality
characteristics of individuals with DS. Parents
rated each personality trait on a 5-point Likert
scale, with possible scores ranging from 23 to
115 (higher scores denote individuals who are
more outgoing and sociable). For this sample,
Cronbach’s alpha equaled .832.
Child Behavior Checklist (CBCL) (Achenbach,
1991) includes 112 items concerning maladaptive behaviours, each rated from 0 to 2
(0 = not present; 1 = somewhat or sometimes
true; 2 = very true or often). The CBCL consists of two broad-band factors (Internalizing,
Externalizing), which are composed of eight
narrow-band factors. Higher scores indicate
more maladaptive behaviours. A reliable and
valid measure of maladaptive behaviour in
children and adolescents, the CBCL has been
used with individuals with intellectual disabilities (Dykens & Kasari, 1997).
50
Burke et al.
The Reiss Profiles of Fundamental Goals and
Motivational Sensitivities for Persons with
Mental Retardation (Reiss Profiles) (Reiss
& Havercamp, 1998) assesses motivational
strengths and styles of individuals with intellectual disabilities. Respondents rated the adolescent on 100 statements along a 5-point scale
(1 – strongly disagree, not at all characteristic
of person to 5 – strongly agree, definitely characteristic). Although the Reiss Profiles have 15
factors, our interests were primarily in factors
relating to five positive and social constructs
(independence – desire to make own decisions;
curiosity – desire to learn; help others – desire to
help friends; morality – desire to behave in accordance with a code of conduct; and social contact
– the desire to be in the company of others).
Parent-Related Measures
The Beck Depression Inventory (BDI) (Beck,
Steer, & Brown, 1996) measures symptoms
associated with depression (e.g., sadness, guilt,
self-dislike, crying, insomnia). Respondents
rated the severity in which they experience
each symptom (0 to 3). Ranging from 0 to 63,
scores above 10 fall within the clinical range.
Positive Perceptions in Families Questionnaire
(PPFQ) assesses how rewarded the parent feels
about having a child with a disability. Six statements are rated on a 4-point scale (1 = strongly disagree to 4 = strongly agree). Statements
include: “I enjoy and feel satisfaction in my role
as a parent of a child with a disability”; “Being
a special parent has strengthened my marriage
and our family”; and “I feel like I have my priorities straight because I am a special parent.”
Ranging from 6 to 24, higher scores indicate
that parents feel more rewarded (alpha = .887).
Kansas Inventory of Parental PerceptionsPositive Contributions Section (KIPP-PC)
(Behr, Murphy, & Summers, 1992) assesses
how much the parent feels the adolescent with
a disability positively contributes to the family.
Respondents rated 41 statements on a 4-point
scale (1 = strongly disagree; 4 = strongly agree).
In this study, we used seven (of nine) KIPP-PC
subscales: (1) Learning through experience with
special problems (e.g., “My child is responsible
for my increased awareness of people with special needs”; alpha = .679); (2) Happiness and fulfillment (e.g., “Because of my child, I have many
unexpected pleasures”; alpha = .673); (3) Personal
strength and family closeness (e.g., “Because
of my child, our family has become closer”;
alpha = .856); (4) Personal growth and maturity
(e.g., “My child is the reason why I am a more
responsible person”; alpha = .833); (5) Awareness
of future issues (e.g., “I consider my child to
be what makes me realize the importance of
planning for my family’s future”; alpha = .616);
(6) Expanded social network (e.g., “My child is
why I met some of my best friends”; alpha = .815);
and (7) Career or job growth (e.g., “The presence
of my child is an inspiration to improve my job
skills”; alpha = .613).
COPE Questionnaire (Carver, Scheier, &
Weintraub, 1989) is a 53-item index used to assess
different coping styles. Respondents indicate
how much they use a particular behaviour when
they experience stressful events in their lives
(1 = I usually don’t do this at all to 4 = I usually
do this a lot). Lyne and Roger (2000) identified
three coping factors: (1) Rational-Active Coping
(accept that a stressful situation occurred; “I
try to come up with a strategy about what to
do”); (2) Emotional Coping (express feelings
and seek emotional support; “I try to get emotional support from friends and relatives”); and,
(3) Avoidance-Helplessness Coping (behavioural
disengagement and denial; “I act as though it
hasn’t even happened”). Scores range from 18 to
72 for Rational-Active Coping (alpha = .838), from
8 to 32 for Emotional Coping (.869), and from 11
to 44 for Avoidance-Helplessness Coping (.757).
Transition Daily Rewards and Worries
Questionnaire (TDRWQ) (Glidden & Jobe, 2007)
is a 28-item questionnaire measuring parental
rewards and worries as their adolescents transition to adulthood. Respondents indicated their
level of agreement on a 5-point Likert scale
(1 = strongly disagree to 5 = strongly agree),
with higher scores indicating more rewards and
fewer worries. Questionnaire factors include:
Positive Future Orientation (general feelings
about the child’s future; “I am optimistic about
my child’s future”; alpha = .682); Community
Resources (feelings toward the child’s job
preparation and independent living; “I believe
that there are a lot of resources available in
my child’s community”; .672); and Financial
Independence (perception of the child’s likelihood of becoming financially self-supporting;
“I worry that my child will never be self-supporting”; .704). An additional domain, Family
JoDD
51
Adolescents with Down Syndrome
Relationship with Siblings, was not used in this
study, and a final domain, Family Relations,
was not used because its component items did
not form a single scale (alpha = -.353).
Procedure
Participants were recruited through DS support
groups in Tennessee and Illinois. Interested
parents either brought their adolescent with DS
to the research lab or the researchers traveled
to a location where the assessments could be
completed (e.g., home; hotel conference room).
Prior to the visit, parents were mailed a packet
of adolescent-related measures to complete;
during the visit, a research assistant conducted
the K-BIT with the adolescent, while parents
completed the parent-related measures.
Results
Characteristics of Adolescents
with Down Syndrome
On average, parents rated these adolescents as
having fairly social, outgoing personalities and
relatively low levels of behaviour problems.
Wishart Scale scores averaged 91.20 (SD = 9.98),
significantly above the scale’s mid-point (i.e.,
69), t (40) = 8.47, p < .001. Similarly, most adolescents did not present many behavioural
problems. On CBCL internalizing behaviours,
87.2% of adolescents scored in the normal range
and 12.8% in the clinical range. Among specific
narrow-band factors, thought problems showed
the highest percentage of adolescents (23%) in
the clinical range. Similarly for externalizing
behaviours, 92.3% of adolescents scored in the
normal range and 7.7% scored in the clinical
range. For total behaviour problems as well,
84.6% scored in the normal range and 15.4%
in the clinical range. No significant relations
emerged between the participant’s age or IQ
and personality or maladaptive behaviours.
Characteristics of Parents
While adolescents with DS generally showed
few problems, the reactions and perceptions
of their parents were more mixed. On the
positive side, parents held generally positive
perceptions about raising an adolescent with
v . 18 n .2
Down syndrome. Compared to parents of children with various disabilities who answered
about the positive contributions in the original KIPP-PC study (Behr et al., 1992), parents
of these adolescents with DS scored higher on
all but two of the 7 KIPP-PC sub-scales. Thus,
whereas the original norming group scored
slightly higher on the KIPP-PC’s Happiness
and Fulfillment domain, t(1302) = -3.59,
p < .01, and no group differences emerged on
Personal Growth and Maturity, t(1302) = 0.94,
ns, parents of these adolescents with Down
syndrome scored higher than did the norming group on Learning Through Experience
with Special Problems, t(1302) = 3.33, p < .01;
Personal Strength and Family Closeness,
t(1302) = 1.98, p < .05; Awareness of Future
Issues, t(1302) = 2.19, p < .05; Expanded Social
Network, t(1302) = 4.91, p < .001; and Career or
Job Growth, t(1302) = 6.68, p < .001.
Second, most parents showed high levels of
Rational Coping (considered the best type of
coping) and relatively low levels of Avoidant
Coping. Compared to a mean on the COPE’s
Rational-Coping Domain of 33.29 (SD = 8.74)
for Lyne and Roger’s (2000) community-based
sample, parents of these adolescents with DS
scored 55.75 (SD = 6.57), t(519) = 16.02, p < .001.
Conversely, compared to a community-sample average of 33.70 (SD = 4.74) on the COPE’s
Avoidance-Helplessness Coping domain, parents of these adolescents scored much lower, at
15.78 (SD = 3.78), t(550) = 30.53, p < .001. No differences emerged between the two groups on
the COPE’s Emotional Coping factor, nor were
there significant correlations in the DS group
among the three coping styles. Parents displaying higher levels of Rational-Active Coping felt
more rewarded (PPFQ) and perceived their
child as contributing more positively to their
family within areas such as Learning Through
Experience with Special Problems, Personal
Strength and Family Closeness, and Awareness
of Future Issues (see Table 1).
Third, parents’ depression scores averaged significantly below the cutoff for mild depression,
t(38) = -6.87, p < .001; several parents, however,
experienced higher levels of depressive symptoms. Overall, the sample’s BDI averaged 6.17
(SD = 7.12), with a range of 0 to 35. While most
parents (71.1%) were not depressed, nine (23.7%)
were mild/moderately depressed (scores
52
Burke et al.
Table 1. Means of Parental Perceptions of Rewards and Contributions and Transitions,
and correlations with Depression and Coping Styles
M (SD)
PPFQ (Rewards)
BDI
Rational Active
Coping Style
19.90 (3.62)
-.45**
.34*
Learning Through Experience with Special
Problems
3.53 (0.46)
-.01
.54**
Happiness and Fulfillment
3.10 (0.57)
-.12
.31
Personal Strength and Family Closeness
3.23 (0.58)
-.34*
.49**
Awareness of Future Issues
3.11 (0.64)
-.04
.43**
Personal Growth and Maturity
2.74 (0.68)
-.28
.29
Expanded Social Network
3.00 (0.65)
-.26
.24
Career or Job Growth
2.96 (0.51)
-.18
.40*
Positive Future Orientation
3.08 (0.72)
-.10
-.37*
Financial Independence
2.27 (0.93)
-.22
.04
Community Resources
2.40 (0.71)
.10
-.07
KIPP-PC (Positive Family Contribution)
TDRWQ (Transition Worries/Rewards)
between 10.2 and 15.1) and two (5.2%) showed
moderate-to-severe depressive symptoms
(scores = 20.4 and 35.3). While no significant
relations occurred between parental depression
and parental perceptions of the child’s positive contributions (r’s of BDI to KIPP-PC factors = -.34 to -.01, ns), parental depression scores
were negatively related to parental feelings of
adolescent rewardingness (BDI to PPFQ). See
Table 1.
Finally, parents of adolescents with DS characterized certain transitions as rewards or worries. Parents felt most concerned about their
adolescent’s future financial independence
(2.27; SD = .93) and community resources (2.40;
SD = .71).
Relations Between Parent and
Adolescent Variables
Demographic and functional skills. No significant correlations emerged between the adolescent’s age, gender, or IQ score and parental
depression (BDI), coping styles (3 COPE factors), rewardingness (PPFQ), perceptions of
adolescent’s positive contributions (KIPP-PC),
or transition rewards or worries (TDRWQ).
Adolescent Behaviour Problems. Parents’
depression scores were positively related to
the adolescent’s Internalizing behaviours and,
to a lesser extent, to total behaviour problems
(see Table 2). Parental BDI scores were also
related to the CBCL narrow-band domains of
Thought Problems, r = .59, p < .01, Withdrawn/
Depressed, r = .50, p < .01, Social Problems,
r = .43, p < .01, and Somatic Complaints, r = .37,
p < .05.
Consistent relations also emerged between
the adolescent’s higher amounts of internalizing problems and parental worries about
the adolescent’s future. Specifically, those
parents who judged their adolescents to have
more Internalizing problems rated themselves lower on TDRWQ Positive Future
Orientation. Such parental concerns about the
child’s future were significantly related to all
Internalizing narrow-band domains (r’s from
-.56, with Somatic Complaints, to -.42, with
Thought Problems; p’s < .02). Negative correla-
JoDD
53
Adolescents with Down Syndrome
Table 2. Correlations between the adolescent’s level of behaviour problems and parent depression, feelings of
reward, feelings of adolescent contribution, and transition worries or rewards
Internalizing
Problems
Externalizing
Problems
Total
Problems
BDI
.425**
.130
.437**
PPFQ
.038
-.129
-.116
Learning through experience
.175
.042
.088
Happiness and fulfillment
.018
-.063
-.055
Strength and family closeness
.029
-.015
-.035
Awareness about future issues
.070
.067
.042
Personal growth and maturity
.034
-.005
-.029
Expanded social network
-.117
.017
-.124
Career/job growth
.065
.103
.065
Positive Orientation
-.618**
-.160
-.395*
Community Resources
-.088
.145
-.021
Financial Independence
-.373*
-.297
-.397*
KIPP-PC
TDRWQ
tions were also found between the adolescent
Internalizing domain (and overall problems)
and Financial Independence (see Table 2). No
relations emerged between parental scores
on the TDRWQ and the adolescent’s level of
Externalizing problems.
Personality. Parental perceptions were related to
higher levels of positive—but not of negative—
personality characteristics of the adolescent with
DS. Specifically, the degree to which parents perceived their child as a source of reward (PPFQ)
increased for parents who rated their adolescent
higher on independence, curiosity, morality, and
social contact. Parents reported less depression
when their adolescents were more moral and
independent. Similarly, as Table 3 shows, parents who rated their child higher on many of the
more positive personality characteristics also
viewed their adolescent as contributing more to
their family’s happiness/fulfillment, strength,
learning, closeness, growth, expanded social
network, and even career/job growth (KIPPPC). Few correlations emerged between parental perceptions and any of the negative Reiss
v . 18 n .2
factors; nor were there consistent correlations of
either positive or negative adolescent personality
characteristics and worries or rewards related to
transition. There were also no relations between
parent depression, reward, or family contributions and parents’ ratings of their child on the
Wishart Scales (r’s = -.26 to .30, ns).
Discussion
Although for many decades individuals with
Down syndrome and their families have been
the subject of study, we continue to know little
about how adolescent characteristics relate to
parental reactions. As such, this study provides
additional information about adolescents with
DS, their parents, and the interplay between
characteristics of adolescents with DS and their
parents in relation to coping styles, transition,
and positive effects.
This study produced four main findings. First,
as a group, adolescents with DS remained
friendly and cheerful during their teenage
54
Burke et al.
Table 3. Correlations Between Positive Reiss Personality Factors and Parent Depression,
Feelings of Reward, and Perceptions of the Adolescent’s Positive Contribution to the Family
Independence
Curiosity
Help Others
Morality
Social Contact
BDI
-.381*
-.333*
-.089
-.349*
-.261
PPFQ
.432**
.422**
.256
.406**
.391*
.483**
.366*
.352*
.220
.369*
Happiness/Fulfillment .346*
.440**
.294
.385*
.383*
Strength/Family
Closeness
.551**
.540**
.292
.363*
.397*
Awareness about
Future Issues
.570**
.485**
.397*
.413**
.354*
Growth/Maturity
.408**
.485**
.351*
.417**
.345*
Expanded Social
Network
.315*
.265
.082
.104
.376*
Career/Job Growth
.514**
.406**
.391*
.440**
.410**
KIPP-PC
Learning Through
Experience
years. While adolescence is often a difficult
time for parents and their children, most parents of adolescents with DS continue to view
these children as happy, affectionate, and with
few internalizing or externalizing maladaptive
behaviours. While internalizing problems may
become more common as individuals with DS
get older (Dykens, et al., 2002; Stores, Stores,
Fellows, & Buckley, 1998; Tonge & Einfeld,
2003), these problems were not yet pervasive or
of clinical significance in this sample.
Second, we noted the importance of active,
rational coping for these parents. Even compared to Lyne and Roger’s (2000) community
group, parents of adolescents with DS more
often endorsed a style of coping that was active
and rational, less often one that relied on avoidance. Levels of rational coping, in turn, related
to feelings of reward, both in relation to parenting the adolescent and from the benefits that
parents perceived that child as bringing to the
family. Prior studies have also noted that an
active coping style relates to parents’ feelings
of reward and positive contributions (Mak &
Ho, 2007), and that avoidant coping styles were
not related to positive perceptions. Hastings
and Taunt (2002) suggested that a focus on posi-
tive perceptions as coping resources could be
used as an intervention to help families adapt
to raising a child with disabilities. Those parents with more positive perceptions could also
help parents who are struggling, in that contact
with parents who have a positive attitude may
make other parents’ attitudes more positive,
thus helping them cope more effectively.
Third, we noticed an intriguing pattern of
relations between child negative and positive
behaviours and parental perceptions-reactions.
Thus, even though adolescent levels of internalizing problems were low, did not relate to age,
and few participants showed clinical levels of
CBCL Internalizing problems, such problems
were worrisome to parents. Indeed, adolescent
levels of Internalizing problems were related
to increased parental depression, and greater
worries (versus rewards) concerning their adolescents’ positive future outcomes and financial
independence. Conversely, parents feel that they
and their families benefit when their children
are more independent, social, moral and curious.
Such connections varied slightly with different
measures, but—just as parents are worried about
internalizing or “inwardness” of their children—
JoDD
they seem rewarded when the child shows more
positive personality characteristics.
Fourth, several expected relations did not occur.
In contrast to the large (over 200) cross-sectional sample noted in Dykens et al. (2002), we did
not not find increases in CBCL Internalizing
problems with increased adolescent age. Thus,
even though parents worried about the inwardness of their children, levels did not seem to
increase over age. Most likely such changes
were more subtle and hard to detect with our
smaller sample. In addition, few connections
emerged between CBCL Externalizing problems and parental reactions of any type. From
these data, then, Internalizing problems most
affect parents; indeed, even the hint of a slightly
less sociable, more inward-directed personality
in their children seemed to influence parents.
While it offers many insights, this study nevertheless has certain limitations. Specifically, our
sample was mostly comprised of highly educated, White parents; as such, these results may
not be generalizable to families from different
cultural and racial backgrounds. As a crosssectional examination, we also provided only a
snapshot of families of adolescents with DS; we
thus cannot determine how parental perceptions and coping styles may have changed over
time. Finally, with the exception of the K-BIT
IQ test, parents served as the reporters of the
child’s personality-maladaptive behaviours and
of their own reactions.
Still, this study constitutes a first step in
describing families of adolescents with DS.
Although research is needed using multiple
time points, reporters, and contrast groups, this
study sheds light on both adolescents with DS
and their parents. Ultimately, if our goal is to
support parents to help their adolescents, we
need to understand more fully the characteristics of individuals with DS; of parental coping,
depression, and rewards; and the connections
between offspring and parental characteristics
over the adolescent years.
v . 18 n .2
55
Adolescents with Down Syndrome
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index.php?page=757 after August 15, 2012.
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The Middle Years and Beyond:
Transitions and Families of Adults
with Down Syndrome
Volume 18, Number 2, 2012
Authors
Nancy S. Jokinen,1
Matthew P. Janicki,2
Mary Hogan,3
Lawrence T. Force4
1
University of Northern
British Columbia,
Prince George, BC
2
University of Illinois
at Chicago,
Chicago, IL
3
Eliot, ME
4
Mt. Saint Mary College,
Newburgh, NY
Correspondence
[email protected]
Keywords
aging,
Down syndrome,
families,
intellectual disability,
transition
Abstract
Normally expected transitions connect the various periods of
life. Often these transitions are prompted by life events that
require adaptation to a changed circumstance and may challenge both individual and family quality of life. Such transitions may be planful (proactive) or demand (reactive). Little,
however, has been written about the nature of such transitions
and how they specifically affect older-aged families of adults
with Down syndrome. Such families are often predominate
lifelong carers of adults with Down syndrome. Drawing on
research and experience, the authors examined three transition
points from a family perspective. Each of these points of change
requires that people adapt and may lead to various outcomes,
including at times outcomes that are unexpected, stressful, and
challenging. The three points of transition examined include
moving away from the parental home, changes occurring within a residential service (e.g., staff changes, relocations), and the
reactions to the onset and course of dementia. Vignettes and
quotes illustrate the complexities of these transitions and show
that, even with planful management, often such transitions can
go awry and produce unpredictable outcomes.
Worldwide population profiles indicate that life expectancy
is increasing, particularly in the more affluent nations. This
“longevity shift” has broad implications for the various segments of societies, including older individuals with disabilities, their family members, and the nature of programs within the aging and disability networks. As people continue to
experience an extension in lifespan, theoretical models, practice interventions, and service delivery systems will need to
be cognizant of the intersection between age, disability, and
disease. Nowhere is this more relevant than to the expanding
longevity of adults with Down syndrome (Percy, Summers,
& Lovering, 2011).
Societies have deeply rooted notions about independence,
self-reliance, family responsibilities, and of the role of government policies that may help shape support provided to
individuals within a range of dependent populations, including people with disabilities and older persons, and their families. Among societies, the need for and availability of services and supports varies significantly. While many developed
countries have produced diverse systems of social services in
attempts to meet needs, other countries have few if any formal social services. Many countries and their governments
are also struggling with economic woes while attempting to
meet the demands of an aging population. In the meantime,
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Jokinen et al.
there is a growing recognition of the important
role and place of family caregiving. Indeed,
across the life span, family members predominately provide support for most people (young
or old, with Down syndrome or other disabilities) (Braddock, Hemp, & Rizzolo, 2008). The
individualized nature and context of familial
caregiving (e.g., individual attributes, culture,
location, and policies that support or impede
family care), however, are necessary considerations in designing effective interventions
to support families in their many and varied
roles (Dilworth-Anderson, Hilliard, Williams,
& Palmer, 2011).
Transitions are basically a movement through
various life stages or notable life-course events
that create some level of insecurity and require
individuals to adapt (Golan, 1981, as cited
in Ray, Bernard, & Phillips, 2009, p. 99). With
increasing age, transitions can be expected to
be marked by progressively more complex and
socially significant life events that may include
a change in residence, dealing with acute ill
health or the onset of chronic conditions, and
loss or death of family and friends. Processes
associated with such transitions may be planful (proactive) or demand (reactive). Planful
transitions often have more positive outcomes,
since the stage for change has been set and
the actors are prepared. Such transitions may
include finding a new home, changing work,
being drawn into a new circle of friends, and
taking on new life responsibilities. Demand
transitions may have unpredictable or adverse
outcomes, since the transitional change is often
precipitous or generally unexpected. Such transitions may include being injured or acquiring
an unexpected disease or condition, having to
relocate precipitously or without much warning, and the withdrawal of familiar supports.
All such transitional events impact individuals as well as their families – requiring adjustments to relationships, routines, and long held
roles and responsibilities. Many older-aged
adults and families apparently have a preference to “age in place” -- to maintain their current living arrangements for as long as possible
(Jokinen, 2008). However, as individuals age,
support may be required in order to maintain
current living arrangements and families are
often forced to consider alternative arrangements. While older-aged adults and their fami-
lies are a diverse group with varying needs,
many manage such transition periods without seeking supports from formal services.
However, supports obtained through social
connections, individual self-care habits, and
psycho-social resources can ease some transitions. Individuals and/or their families may,
therefore, only come forward requesting assistance when a situation becomes problematic
(Denton & Kusch, 2006). Despite the growing
body of evidence on family caregiving, however, there is still much to learn about family
dynamics and involvement in various periods
of transition (e.g., who becomes involved, range
of tasks, differing needs) (Gitlin & Wolff, 2011).
With respect to middle-aged and older adults
with Down syndrome, life expectancy has
increased substantially since the mid 1950s.
At birth, the current cohort of middle-aged
and older adults with Down syndrome (those
in their fifties and older) was often not expected to survive much beyond early adulthood
(Bittles & Glassen, 2004). Yet with advances
in social and health care, many adults with
Down syndrome are now living to ages previously unseen (Janicki, Dalton, Henderson, &
Davidson, 1999; Percy et al., 2011). Although
most do not attain the longevity averages for
men and women in the general population,
some middle-aged and older adults with Down
syndrome may outlive their parents, siblings,
or other close relatives. These adults were born
in an era when professionals often suggested
to parents that they institutionalize their sons
or daughters, in part because community supports were limited at the time. Nonetheless,
many parents chose to raise their child with
Down syndrome within the family alongside
their other children. Commonly, in those early
years, families faced and endured negative
public attitudes and opinions about Down syndrome. Seeking out mutual support and solutions to the dilemmas they faced, some of these
families pioneered the first community services
for persons with Down syndrome and other
disabilities. Now, these same families are older-aged and experiencing inevitable age-related
challenges and transitions.
Generally, the literature on transitions and
adults with Down syndrome is somewhat
divided. One focus has been on younger-aged
adults transitioning from educational to adult
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services and employment (see as examples
Bouck, 2012; Pueschel, 2006), and another focus
has been on the health problems or decline
in health of older adults (e.g., Torr, Strydom,
Patti, & Jokinen, 2010), or neuropathologies,
(e.g., Janicki, Zendell, & DeHaven, 2010). Patti,
Amble and Flory (2005) reported that adults
with Down syndrome aged 50 to 59 experienced more life events compared to similarly
aged adults with other intellectual disabilities.
These life events included changes in living
circumstance and/or day activity or program
routines; death of, or separation from, a parent,
friend, or roommate; and the onset of medical problems. Additionally, adults aging with
Down syndrome were reportedly more likely
to be in jeopardy of referral to a nursing home
in later life – which raised the question what
impact such relocations have on the individual (Patti, Amble, & Flory, 2010). Lunsky and
Elserafi (2011) found that specific life events
experienced by persons with intellectual disability were associated with increased hospital emergency department visits in reaction to
a crisis. Furthermore, life events that prompt
transitions likely have adverse impact on adults
with intellectual disability and services may be
able to control some events (e.g., staff changes
and relocations), thereby mitigating emotional
distress (Hulbert-Williams & Hastings, 2008).
Grant, Nolan and Keady (2003) acknowledged
that individuals and families access various
services throughout life and the impact of these
transitions is not well understood. A dominant
theme in the literature on older-aged families
of adults with intellectual disability, including those with Down syndrome, is the need
for parents (and families) to establish future
plans. This is often viewed as a means to ease
transitions and avoid the co-occurrence of two
significant adverse life events (the death of a
parent and need for relocation). While various
planning models have been developed (see as
examples Baxley, Janicki, McCallion, & Zendell,
2005; Bigby, 2004), commonly these planning
processes highlight the needs of the adult who
has a disability and promote a move from the
family home prior to the incapacity of the main
carer, usually the parent. Yet, although there
is an underlying assumption that alternative
living arrangements may be readily available,
parents often lack information about housing
options and few studies have reported fam-
v . 18 n .2
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Transitions and Older-Aged Families
ily perspectives on this type of move (Gilbert,
Lankshear, & Petersen, 2008). The literature also
points to a reluctance for older-aged parents
to preplan for transitions or engage disability
services (Bigby, 2004), yet planning processes
seem to give little attention to the family as a
whole with limited regard for the impact such
plans have on other family members (Jokinen,
2008). Miettinen (2012) suggested that government policies and reduced social service funding have both direct and indirect influences
on family decisions about the future. Limited
access to housing alternatives and the quality
of services provided seem to reinforce and prolong family caregiving potentially to the detriment of parents and their adult offspring.
It is within this context that we assess three
transitions often encountered by older adults
with Down syndrome and their families; some
are typical and some are atypical. First, we
consider the circumstances and perceptions of
relocation when adults with Down syndrome
move from the parental home where they have
co-resided since birth. Next, we consider the
transitions associated with changes in residential services (i.e., staff turnover and/or moving
homes). Lastly, we consider transitions associated with the onset and progression of dementia. For each of these transitions, we highlight
family carer perspectives. The vignettes1 and
quotes used illustrate circumstances suggestive
of strategies that may ease transitions and better support both individuals and their families,
and portray some unexpected outcomes even
with parental or carer involvement.
Moving From the
Parental Home
Parents and their adult sons or daughters with
Down syndrome often wrestle with the decision as to whether to arrange separate living
arrangements or continue co-residing. These
decisions are influenced by a number of factors
1 The first two vignettes are drawn from published
research (Jokinen, 2008) and the author extends
appreciation to the families who participated in that
research and their willingness to share their stories.
The third vignette is drawn from a published article
reflecting family perspectives (Hogan, 2010). All names
used in the vignettes are pseudonyms.
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Jokinen et al.
including familial values and beliefs, finances,
opportunities, parental retirement plans, and
the health status of either the parent or their
offspring. In the following discussion, family
perceptions are highlighted regarding planned
moves, and two examples of how plans may
also change are considered.
Planned Moves
Vignette 1 offers a brief overview of the situation of Larry, a man with Down syndrome in
his 40s (Jokinen, 2008). Larry’s parents made
plans for him to move out of the family home
and were able to successfully realize their plans.
They became actively engaged with a local provider organization so as to better understand
the services available prior to the move and so
that they could take advantage of a residential
Vignette #1:
A siblings’ story (Larry)
Dan and George are middle-aged brothers; both have their own families and
work full time. Their brother, Larry, aged
41, has Down syndrome and requires
significant support for activities of daily
living and lives within a 24/7 residential
service. Prior to and following his move
out of the family home, both parents
became actively involved on the agency’s board of directors and were familiar with the services provided. After the
move, Larry visited with his parents
frequently and was included in family get-togethers. Both parents are now
deceased and the brothers expressed
appreciation for all that their parents had
done in arranging Larry’s living circumstance. They feel they now carry on with
something their parents wanted. While,
admittedly, the brothers do not see each
other often, they involve Larry in family
gatherings and support him as needed
(e.g., with medical appointments). As to
the future, the brothers would like things
to remain as they now are. They are confident in the residential service and deal
with issues as they arise.
option when one became available. An opening
occurred in a supervised apartment living setting and the parents and Larry decided to take
it. Larry moved to his new home, but all was not
as smooth as it appeared on the surface. One
of Larry’s brothers confided that “My mother,
I don’t know, hesitated for a good number of years
because she [was a] typical mother, ‘no one can look
after my son as well as I can’. That was tough for her,
for him to move out on his own. And she was over
there every night of the week to make sure supper was
cooked properly.” The daily visits by Larry’s mother reportedly reduced over time as her comfort
level grew with the staff support provided.
Nonetheless, she and her husband maintained
regular weekly support and visits. Parental support ended only with the deaths of both parents.
In other families, when parents do not initiate
future residential plans for their adult son or
daughter with whom they co-reside, siblings
sometimes influence decisions and planning
(Jokinen, 2008). One sibling, when reflecting on
such a situation within her family, noted, “We
prompted my mom to start considering putting [my
sister with Down syndrome] somewhere because
my mom was getting [old]… she was 70 or something then, and we thought maybe that would be
good and to do it while my mom was still alive so
she could see that it worked.” The sister reported
that after some further family conversations
with her mother, she accepted the idea that her
daughter with Down syndrome move. When
an opportunity for a residential option -- in
this case a small group home -- became available, the sister with Down syndrome, with the
encouragement of her siblings, moved from
her mother’s home. Following the move, however, for unknown reasons the service provider reportedly was not supportive of family
visits and this resulted in some tense relations
between the family and service provider.
The reflections of yet another sibling regarding
her brother’s move away from the parental home
also offers some insights into what might ease
or hamper the transition of moving from the
parental home (Hogan, 2010). The sibling noted
that when her brother moved out of the home,
he left behind a familiar neighbourhood as well
as other residents and business owners with
whom he had interacted over many years as a
part of his daily routines. Consequently, after he
moved into an agency group home, he experi-
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enced a sense of loss and his absence in his old
community was noted. This loss of involvement
in one’s neighbourhood and the inherent social
relationships has generally received little, if any,
attention, in the literature. The sister noted that
once he relocated to the group home, her brother routinely returned home to spend weekends
with his mother. In some respects, continued
family contact is desirable and can ease transition while avoiding a sense of abandonment and
abrupt separation from a valued community.
On the other hand, such visits back to the family home may also inhibit being fully included
in social activities with new housemates, slow
down the development of new friendships, and
hinder establishing an identity within the new
neighbourhood. While routine familial support
may increase, at least during the initial stages of
a move, over time familial contact and support
typically seems to settle into a new or adjusted
pattern (Jokinen, 2008).
An individual’s daily routines and responsibilities also likely differ between a family and new
residential circumstance; whether this creates
additional challenges needs to be considered.
Brown and Brown (2003) referred to everyday
routines that make life enjoyable, and to the
need to balance established routines with new
experiences that may or may not be adopted
into daily life. Routines are an important aspect
of both individual and family life (Jokinen,
2008; Knox & Bigby, 2007) and a fuller understanding of them can better ease transitions
(Zisberg, Young, Schepp, & Zysberg, 2007). One
lesson from these situations is that when necessity dictates a transition from the family home
for an adult with Down syndrome, it should be
individually tailored and designed to accommodate and manage those aspects of daily life
that are important both to the individual making the move as well as the family.
Change in Plans
Despite having initiated plans for alternative
living arrangements, sometimes an unexpected
medical or similar significant crisis experienced
by either the parent or adult with Down syndrome trumps a smooth transition. For example,
a mother in her early 70s made an application
for residential supports on behalf of her 40ishaged daughter Julie who had Down syndrome
(Jokinen, 2008). The mother, being proactive,
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had registered her daughter with a local agency
for residential options offered. After being on
the wait list for almost ten years, an emergency
relocation was required as the mother had been
seriously injured and required hospitalization as
well as an extended period of convalescence. The
mother confided that “…if I hadn’t broken my hip,
more than likely my daughter would still be at home.”
While the emergency relocation turned into a
permanent separate living arrangement for Julie,
the transition was apparently fraught with disappointments, emotional upheaval, and challenges
for both mother and daughter. Following emergency respite services, arrangements were made
for Julie to move temporarily into a basement
bedroom in a house rented by several adults
with intellectual disability supported by a service provider. Julie had to adjust to both unfamiliar staff helping her and housemates whom
she had never met. The mother reported that
Julie’s sleep patterns changed, she was waking
and dressing in the middle of the night, and then
later falling asleep on the living room couch. On
the mother’s first visit to her daughter’s new residence when she was able to walk, she also found
that the home’s housekeeping standards did not
meet her expectations (she found cobwebs, dirty
carpets, soiled bedding, and the like). This issue
was not fully resolved at that particular home,
although housekeeping standards were better,
at the mother’s insistence, in a subsequent home
Julie moved to and lived in. The lesson here is
even though the mother was planful in getting
her daughter on a list for a residence service – in
preparation of the transition to moving from the
parental home – she lost control of the process
when she was injured and her daughter required
emergency relocation. Although emergency
relocations are not reportedly commonplace
(Bigby, 2004; Jokinen, 2008), extensive residential wait lists across many jurisdictions that now
exist may increase such situations and or place
increased demands on other family members.
Furthermore, standards within residential services seem to vary and parents as well as other
family members may need to be prepared to
advocate for reasonable living conditions.
Another example of a family situation offers a
different circumstance that can alter planning
for an alternative living option. Sometimes,
parents and their offspring make decisions
to live separately, only to have a medical crisis experienced by the adult with Down syn-
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Jokinen et al.
drome stymie plans and reinforce continued
co-residency. One mother related that she had
helped their daughter Helen to make plans to
move away from the parental home (Jokinen,
2008). The mother related that: “Well, there was a
time when she wanted to go to an apartment on her
own. And we were all for [it]. And of course that fall,
when this was in the making, she got sick with diabetes. She just, she went into a coma and I got her to
the hospital…So since then she hasn’t wanted to be
anyway else but home.” The mother related that
Helen barely survived the medical crisis and
the aftermath required adjusting to living with
a chronic condition (in this case, brittle diabetes). The plans for Helen to live independently
were abandoned. She continues living with
her aging parents, now in their late 80s, with
nominal services, and the family foresees her
co-residing with her sister and brother-in-law
in the future.
practices within agencies or the results of external forces (Hulbert-Williams & Hastings, 2008).
Vignette 2 illustrates staff turnover and location
moves within residential services as a period of
transition for older-aged family members.
Older parents and their adult children with
Down syndrome who make decisions to coreside for as long as is possible need information and support to ensure that inevitable future
transitions are well managed. The degree to
which families have discussed matters and have
made plans varies (Jokinen, 2008), yet this is a
critical task and warrants serious conversation
within the family. Cultural and ethnic beliefs
also play a role in shaping attitudes towards
aging and disability (Hogg, Lucchino, Wang,
Janicki, & Group, 2000) and can influence an
older-aged family’s caregiving experiences,
expectations, and plans for the future. There is
an urgent need to better understand transitions
as they occur in conjunction with adult sibling
co-resident arrangements. This information
will aid family planning and help discern pros,
cons, and pitfalls of future sibling co-residency
in order to adequately plan for the needs of all
family members (Jokinen, 2008).
Vignette #2:
Even well-intended family
help can go awry (Mark)
Changes Occurring in
Residential Services
Many older-aged families of adults with Down
syndrome have extensive experience with disability services, often spanning decades, and
with the changes that occur within a residential
service that lead to transitions for both the adult
with Down syndrome and the family. Some of
these changes may be initiated by administrative
Changes in Staff
Disability organizations have long struggled
with staff recruitment and retention (Hall &
Hall, 2002). Numerous changes in direct care
staff and their supervisors can disrupt relationships and impact the supports provided
(Jokinen, 2008). In the second vignette, Mark’s
mother remarked that over the years “hundreds
of people” have come and gone with respect to
providing supports for her son. She commented on the staff changes that had occurred and
In one particular service, adults with
intellectual disabilities rented accommodations and the agency provided
support staff. Mark, a middle-age man
with Down syndrome, had been living
in a number of such accommodations
since he left home. Mark’s mother, however, always took note of where Mark
lived and under what conditions. She
noticed that landlords often gave notice
for tenants to move and rental premises
were not always properly maintained.
To overcome these problems, Mark’s
mother purchased a duplex. She lived in
one part of the house and her son along
with three male housemates moved into
the other half with part time support
from residential staff. The men became
well known by neighbours, local businesses and a church community in the
area. With an organizational change,
management decided to move the men
across town to different rental homes.
No discussions were apparently held
with the families and the men lost contact with their neighbourhood friends
and acquaintances.
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the relations with various professionals, volunteers, and students that inevitably ended: “Like
their whole lives are up and down.... different people
working with them coming into their lives for a short
time and going.” Sometimes, she said, people who
moved on would try to keep in touch with Mark,
but eventually these relations always dwindled
away. Likewise, in the third vignette, Peter’s sister noted that her brother would become emotionally attached to “his staff” and generally
they too would become attached to him. Often it
was traumatic when staff turnover occurred and
there was a limited understanding of why they
had left. His family, like others, tried to help
Peter stay connected to some of these people;
however, most times, it was to no avail.
Staff changes impact family-service relations as
well as the continuity and quality of supports.
New staff often has a steep learning curve in
getting to know an individual’s abilities, his or
her history, and the nature of the family relations (Hogan, 2010). One mother noted, “You
know we’ve had 10 or 11 [supervisors] from the
time that house opened... and I said [to the agency]
I don’t know who I’m supposed to call… half the
time I never even met the [supervisor]” (Jokinen,
2008). Such changes in staff can lead to administrative problems, as well as personal difficulties for the person and their family. Often, with
multiple staff turnover, there can be a generalized loss of knowledge about an individual,
such as behavioural and medical history. This
loss of knowledge may well contribute to medical disparities and aggravate health conditions,
and possibly spur the onset of adjustment
problems that are then characterized as “problematic” behaviours. These staffing difficulties
may pose significant challenges for parents and
other relatives involved with the adult with
Down syndrome who face these transitions
with trepidations, having experienced the consequences many times over.
Parents and adult siblings acknowledge staff
turnover as a reality and this may reinforce
the notion that families are the one constant in
their relative’s life. Yet families may also have
ideas to minimize the impact of staff turnover
and should be consulted. For example, when
staff changes are unavoidable, family members
have suggested that agencies keep the number of staff moved around to a minimum and
retain in place at least two or three key person-
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nel who have worked with their relative. In
doing so, familiar staff can ease the transition
for the individual, their family, and the new
staff coming onboard (Jokinen, 2008).
Relocations Within
Residential Services
In the second vignette, Mark’s mother also
raised several concerns about relocations within
residential services. Although the solution to the
situation Mark faced was functional and productive, in the end it was undermined by the
decision of the agency (providing key supports)
to change Mark’s living situation. Relocations
within a residential service occur and are influenced by a number of factors, including whether
the disability organization owns the residential
properties or, as in this vignette, provides support staff for people who are renters. In the latter,
long term security and upkeep of premises may
depend upon landlords concerned with business
opportunities who are not particularly sympathetic to disability or aging issues. Relocation
may also be prompted by a restructuring of the
organization, governmental budgeting vagaries,
legislative initiatives, the dynamics of housemate relations, and changes in health status that
require support beyond the capabilities of the
current site. While some agencies have explicit
policies, others lack guidelines about residential
moves (Webber, Bowers, & McKenzie-Green,
2010). Nonetheless, as for anyone, changing
residences and moving is a major life event and,
when people move into a new home and new
environment, adjustments are required by the
individual, his or her family, staff, and those
others residing at the new location.
In an attempt to reduce the likelihood of
adverse relocations and to provide long term
housing security, some parents have purchased
homes where their sons or daughters and others might live with specialized supports. We
recall another mother who suggested, “It’s a
sure way of having your child not moved every two
or three years… if you want them closer to home,
you can make an effort to find a house that you can
buy in the locality where you want it.” Referring
to her own situation, she said of her daughter:
“She’s seven minutes away from me.” However, the
dual role responsibilities of parent and landlord are likely to be challenging and stressful.
Decisions about who will live there, what the
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Jokinen et al.
home will offer in terms of furnishings and
household goods, upkeep, and negotiations
with a residential services provider to secure
support are a few such details that need to be
worked out (Jokinen, 2008). Further, while the
option of purchasing a separate residence may
be beyond the financial means of many families
(Canada Mortgage and Housing Corporation,
2006, 2010), today’s extensive wait times for residential services means that there is ever more
demand on families to be self-reliant.
Changes Due to Onset and
Progression of Dementia
Worldwide, the impact of age-related decline
and the onset and progression of neuropathologies, such as Alzheimer disease and related
dementias, on individuals and family carers is
well recognized (World Health Organization,
2012). These events perhaps pose the last significant transition event in the lifespan, save for
death. When adults with intellectual disability
and their families are affected by this transition, it becomes more important that families
and other carers are knowledgeable and prepared to deal with the challenges that are to
come (Factor, Heller, & Janicki, 2012; National
Task Group on Intellectual Disabilities and
Dementia Practices, 2012). Many families of
adults with Down syndrome are aware of the
increased risk of Alzheimer disease associated with Down syndrome and, with increased
age, there is a genuine concern about what this
elevated risk poses for the future. In this final
section, we discuss transitions occurring as a
result of the onset and progression of dementia.
Vignette 3, drawn from Hogan (2010), is illustrative of some of the issues faced by families
with respect to this transition.
Parents and other family members face increasing challenges when their relative with intellectual disability evidences cognitive decline
or behavioural change as a result of cognitive
impairment or dementia. In one of few studies
reporting family perspectives, Janicki, Zendell,
and De Haven (2010) found many parents had
a profound commitment and preference to
keep and care for their son or daughter with
Down syndrome at home. Their study noted
that some parents also claimed that there was
nowhere else for their son or daughter affected
by dementia to go, or that the family was resistant to moving him or her.
Decisions in response to progressive decline
experienced by their sons or daughters were
difficult and often considered to be influenced
by a number of factors. Many parents report
that they would seek help from a doctor or
other clinician, look for personal care assistance, and or seek the support of a sibling. They
Vignette #3: Frustrations
with a lack of knowledge
and information (Peter)
Four years after the diagnosis of
Alzheimer disease and 15 months before
our brother Peter’s death, he exhibited
more dramatic changes in behaviour,
personality and level of functioning.
After exploring several nursing homes,
we became convinced that he needed to
stay with people who knew him. Safety
risks for both him and the staff became
a major factor and our only option was
to request a transfer to a 12-person
group home for severely handicapped
and medically fragile people. The transition was very traumatic for our brother who was very confused and further
agitated by the change. We needed to
play a major role in the transition to
make sure that the new staff bonded
with him and to help them understand
his needs. They had never had a resident with Alzheimer disease and, with
little specific training on Alzheimer
disease, needed a great deal of support and encouragement with difficult
behaviours, such as changed sleep patterns. In hindsight, our family realized
that none of us had fully understood
the types of changes that would occur
as Alzheimer disease progressed. We,
as a family, were not mentally or emotionally prepared for what unfolded or
for the rate of decline. Our brother was
49 years old when died of complications
related to aspiration pneumonia seven
months after the transition to the new
group home.
JoDD
suggested specific strategies to cope and manage things as they arose. These included acquiring support from an agency’s treatment team,
use of medications, and guidance from other
carers who have had similar experience. When
it came to finally facing the difficult decision
of whether to seek help to arrange for another
residential option, the parents noted that continued at-home care as dementia progressed
was influenced by various factors. These factors
included the medical needs of their offspring,
the impact caregiving was having on their personal health, barriers in the home environment,
safety issues, the level of support provided by
other family members, and outside services
available to meet needs. A critical aspect in
the decision-making process was where the
adult offspring would most likely go if he or
she moved out of the family home. The carers
speculated, if such a move was made, their son
or daughter would likely go live with another
family member or go to a group home while
some indicated a preference for a long term
care facility (i.e., a dementia care unit or nursing home). However, a prevailing theme was
uncertainty; the parents were unsure where
their son or daughter might end up, and this
uncertainty was a major stressor with which
they had to contend (Janicki et al., 2010).
In the situation of Peter, his sister noted that
while her brother experienced progressive
Alzheimer disease and remained living in his
original group home, most staff at the home
lacked training in understanding the progressive nature of the disease and in managing the
new behaviours and diminishing abilities. Yet,
at the same time, the staff remained committed
to his care and tried hard to meet his needs.
The lack of, and need for, staff training specific
to dementia care is well recognized (National
Task Group on Intellectual Disabilities and
Dementia Practices, 2012; U.S. Department
of Health and Human Services, 2012) as is
often staff’s hesitancy to relinquish supporting the person and have him or her move to
another location (McCarron, McCallion, FaheyMcCarthy, Connaire, & Dunn-Lane, 2010). In
Vignette 3, while additional support staff was
scheduled, the ultimate challenge became the
move to another group home during the later
stages of Alzheimer disease (the home chosen
was not a dementia-capable home, but one that
provided for adults with severe impairments).
While the intention was to have old and new
staff work together for a planned transition, the
v . 18 n .2
65
Transitions and Older-Aged Families
changeover was complicated and difficult for
all concerned. Eventually, the new staff learned
to care for Peter, both physically and emotionally and to communicate with his family – and
the family reciprocated. As the sister reported,
“It was the best we could do.” In circumstances
such as this, education, training, and communication are crucial to dispel misunderstandings
about the move, the disease, its symptoms, and
care requirements.
The lesson taken from these types of transition experiences is that catastrophic and other
changes in function, whether cognitive or
physical, pose special challenges for families.
Dementia poses a realization for many parents
that supportive caregiving will become more,
and not less, pronounced and demanding with
their own advancing age. Both the parents as
they age and their offspring, as dementia takes
its toll, will find continued care more taxing
and force personal and family examination of
transitions and compel conversation on what to
do next. Older age will always necessitate transition planning, but the demands presented by
catastrophic change such as that brought on by
Alzheimer disease may lead to this conversation taking place sooner than anticipated.
Discussion
In this paper, we have attempted to address
some particular lifespan transitions not previously raised and examined. Admittedly, this
examination is cursory and based on summative and anecdotal data, but it does begin to
examine family situations and perspectives
as changes are encountered due to transitions
that evolve organically or forced by crisis. We
observed that this part of the lifespan potentially offers multiple examples of both planful
and demand transitions.
We conclude that planful transitions are always
less stressful and often lead to more positive
and predictable outcomes and remain largely
under parental and family control. Demand
transitions, on the other hand, often lead to
unpredictable outcomes and may result in control being relinquished to others outside the
family. What we observed from our examination is that, even in situations where families
observe natural pathways to decision-making
and attempt to control and produce reliable
outcomes, these may go awry due to factors
66
Jokinen et al.
beyond their control. What we have learned
from this exercise is that it is helpful to examine
such transition situations, evaluate the options
and alternatives, and follow in more detail
those factors that facilitate or impede them.
What can also be gleaned from this exercise is the knowledge that the natural course
of the lifespan can be interrupted by transitions. Sometimes these transitions are positive
events and help lead to advantageous outcomes.
Sometimes these transitions are calumnious
and lead to adverse outcomes. Loss of staff has
the potential to impact transitions and the level
of care; communication or the lack thereof can
interfere with the best made transition plans;
and staff training needs to be examined as a
possible means to ease transitions, especially in
relation to diagnosis of a progressive condition.
Families and those staff and organizations functioning in aid to families will be better equipped
if they understand the dynamics inherent
in these transitions and conjure up ways to
constructively address them. Discussions of
nuanced older age in the arena of intellectual
disabilities are relatively new and, as of yet, a
little explored frontier. With the extended and
more normal longevity of adults with Down
syndrome, any and all inquiries into this new
frontier are most welcome and will be helpful in
aiding families with planful transitions.
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article, minor changes were pending. A fully
edited version of this article can be found on
the OADD website at http://www.oadd.org/
index.php?page=758 after August 15, 2012.
language
specific to
this note
indicated
in blue.
High Plasma Homocysteine and Low
Serum Folate Levels Induced by
Antiepileptic Drugs in Down Syndrome
Volume 18, Number 2, 2012
Authors
Antonio Siniscalchi,1
Giovambattista De Sarro,2
Simona Loizzo,3
Luca Gallelli2
Department of
Neuroscience, Neurology
Division, “Annunziata”
Hospital, Cosenza, Italy
1
Department of Health
Science, School of
Medicine, University
of Catanzaro, Clinical
Pharmacology Unit,
Mater Domini University
Hospital, Catanzaro, Italy
2
Dentistry Division,
“Annunziata” Hospital,
Cosenza, Italy
3
Correspondence
[email protected]
Keywords
hyper-homocysteinemia,
Down syndrome,
epilepsy,
folate,
vitamin B12
Abstract
Clinical and epidemiological studies suggested an association
between hyper-homocysteinemia (Hyper-Hcy) and cerebrovascular disease. Experimental studies showed potential proconvulsant activity of Hcy, with several drugs commonly used
to treat patients affected by neurological disorders also able to
modify plasma Hcy levels. We assessed the effect of long-term
AED treatment on plasma Hcy levels in patients with Down
syndrome (DS) and epilepsy. We also evaluated the relationship between the plasma Hcy levels, and folic acid or vitamin
B12. We enrolled 15 patients in the Down syndrome with epilepsy group (DSEp, 12 men and 3 women, mean age 22 ± 12.5
years old) and 15 patients in the Down syndrome without
epilepsy group (DSControls, 12 men and 3 women, mean age
20 ± 13.7 years old). In the DSEp group the most common
form of epilepsy was simple partial epilepsy, while the most
common AED used was valproic acid. Plasma Hcy levels were
significantly higher (P < 0.01) in the DSEp group compared
with the DSControl group. Significant differences (P < 0.01)
between DSEp and DSControls were also observed in serum
concentrations of folic acid, but not in serum levels of vitamin
B12. In conclusion, our observations suggest that treatment
with AEDs in DSEp patients induces an increase in plasma
Hcy levels and a significant decrease in serum folic acid, therefore supplementation with vitamins may be useful in order to
obtain normal plasma Hcy values and reduce the risk of both
cardiovascular and neurological diseases
Homocysteine (Hcy) is a thiol-containing amino acid that
is formed by the demethylation of methionine, an essential
amino acid derived from the animal proteins introduced
with the diet (Selhub, Jacques, Wilson, Rush, & Rosenberg,
1993; Siniscalchi et al., 2005). It is metabolized through two
major pathways: methylation and trans-sulfuration that are
dependent on several cofactors. In particular, the methylation of Hcy to methionine requires folate and vitamin B12,
whilst the trans-sulfuration to cysteine requires vitamin B6
(Siniscalchi, 2004; Siniscalchi et al., 2005; Siniscalchi, Gallelli,
Mercuri, Fererri Ibbadu, & De Sarro, 2006).
The recycling of folate cofactors is dependent on vitamin
B6 and B2; vitamin B2 is necessary to activate vitamin B6 to
pyridoxal 5-phosphate (PLP) (Siniscalchi, 2004; Siniscalchi et
al., 2005; Siniscalchi et al., 2006).
Effects of Antiepileptic Drugs in Down Syndrome
Clinical and epidemiological studies suggested
an association between hyper-homocysteinemia (Hyper-Hcy), occlusive arterial vascular
diseases (D’Angelo and Selhub, 1997; Perri,
1999; Refsum, Ueland, Nygard, & Vollset, 1998;
Siniscalchi, 2004) and venous thromboembolism (den Heijer et al., 1996; 1998).
Hyper-Hcy represents both a reaction to acute
illness and an important risk factor for cardiovascular mortality in patients with a history of
myocardial infarction, stroke, angina pectoris,
diabetes, or hypertension (Boysen, Brander,
Christensen, Gideon, & Truelsen, 2003; Vollset
et al., 2001).
Experimental studies have shown a potential
proconvulsant activity of Hcy (Siniscalchi 2004)
with several drugs commonly used to treat
patients affected by neurological disorders
also able to modify plasma Hcy levels. Clinical
studies have reported that patients treated with
old and new AEDs showed increased plasma
Hcy levels (Belcastro et al., 2010; Linnebank et
al., 2011; Schwaninger et al. 1999; Siniscalchi et
al., 2005)
Moreover, folate deficiency occurs in some
epileptic patients treated with AEDs such as
valproate (VA) and carbamazepine (CBZ),
and this effect may induce Hyper-Hcy (Kishi,
Fujita, Eguchi, & Ueda, 1997). Therefore, vitamin B plays a role in altering plasma Hcy levels during AEDs treatment (Siniscalchi 2004;
Siniscalchi et al.,2005)
In this paper, we assessed the effect of longterm AEDs treatment on plasma Hcy levels
patients with Down syndrome and epilepsy.
We also evaluated the relationship between
the plasma Hcy levels, and serum levels of folic
acid or vitamin B12.
Patients and Methods
Study Design
We undertook an open label, control-group,
single centre study on patients with Down syndrome and seizures treated with AEDs and
enrolled at the Cosenza Hospital. This study was
approved by the Researchers’ Ethics Committee
and was conducted in accordance with the
v . 18 n .2
69
Declaration of Helsinki and the Guideline for
Good Clinical Practice; all parents of participants provided written informed consent.
Patients eligible for the study were male or
female, aged from 18 years up to 35 years, with
primary epilepsy and prescribed AEDs (DSEp
group).
Patients with the following conditions were
excluded: allergy to AEDs, progressive serious medical conditions (i.e., cancer or AIDS),
renal diseases (serum creatinine concentration more than 1.2 times the upper limit of the
normal range according to the central laboratory definition reference values), liver dysfunctions (serum alanine transaminase or aspartate transaminase concentrations more than
1.5 times the upper limit of the normal range
according to the central laboratory definition
reference values) and drugs known to increase
the plasma Hcy levels. Patients with Down syndrome and without epilepsy were also enrolled
in our study and represented our control-group
(DSControl group).
End Points
The primary end point was the effects of AEDs
on plasma Hcy levels. Moreover, co-endpoints
included the relationship between both the
plasma Hcy levels and the vitamin B12 or folic
acid levels.
Evaluation of Hcy, Vitamin B12 and
Folic Acid Levels
For plasma Hcy levels evaluation, blood samples
collected in heparin were immediately stored
in fresh ice (T = 0°C) and then centrifuged
(T = 18°C, 1500 g/min for 10 min). Plasma was
take through a polyurethane pipette, put in a
polyurethane tube and analyzed with high-performance liquid chromatography (HPLC-UV)
in agreement with literature data (Amarnath,
Amarnath, Amarnath, Valentine, & Valentine,
2003; Kuo, Still, Cale, & McDowell, 1997).
Serum levels of folic acid and vitamin B12
were analyzed by chemiluminescence with the
AxSYM instrument in agreement with literature data (Bamonti et al., 2010).
70
Siniscalchi
Statistical Analysis
ic acid, with a mean duration of treatment of
9.7 ± 3.0 years.
Statistical analysis was performed using SPSS.
We evaluated the effects of drugs on the plasma
concentration of Hcy, vitamin B12 and folic acid,
but it is not easy to define a clinically significant
change and therefore it is not possible to obtain
a power calculation. Consequently, these results
should be considered exploratory. The Student’s
t test was used to evaluate the change between
groups. For all comparisons, differences were
considered significant at p < 0.05.
Results
During the study we enrolled 15 patients in
the DSEp-group (12 men and 3 women, mean
age 22 ± 12.5 years old) and 15 patients in the
DSControl-group (12 men and 3 women, mean
age 20 ± 13.7 years old).
In the DSEp-group the most common form of
epilepsy was simple partial epilepsy (Table 1),
and the most common AED used was valpro-
Plasma Hcy levels were significantly higher
(p < 0.01), in the DSEp group compared with
the DSControl group (Table 3). Significant differences (p < 0.01) between the DSEp group and
DSC group were also observed in the serum
concentrations of folic acid, but not in serum
levels of vitamin B12.
Discussion
In this study we evaluated the effects of AEDs
on plasma Hcy levels and serum vitamine B12
and folic acid levels in patients with Down syndrome (DS) with and without epilepsy.
Table 1. Epilepsy Manifestations in DSEp-Group
Simply Partial Generalized
Epilepsy
Epilepsy
7
Complex
Partial Epilepsy
5
3
Table 2. Antiepileptic Treatment of Patients with Down Syndrome and Epilepsy (DSEp-Group)
Valproic acid
Carbamazepine
Phenobarbital
Treatment
5 mono-therapy
2 poly-therapy
4 mono-therapy
1 poly-therapy
3 mono-therapy
Mean time of
treatment (years)
9.7 ± 3.0
10.1 ± 2.5
17.5 ± 3.2
Table 3. Concentrations of Homocysteine in Plasma, and Vitamin B12 and Folate in Serum.
Values are Expressed as Mean ± Standard Deviation.
Homocysteine
(normal values 5–12
µmol/L)
Vitamin B12
(normal values
145–914 pg/mL)
Folic acid
(normal values 3–20
ng/mL)
DSControl Group
DSEp Group
P Value
8.2+2.0
14.8 ± 1.8
0.000
359.1 ± 35.1
354.6 ± 40.1
0.746
17.9 ± 1.0
9.3 ± 1.5
0.000
JoDD
Effects of Antiepileptic Drugs in Down Syndrome
Our data demonstrate that patients in
DSControl-group show normal levels of folate
and Hcy, while long-term treatment with AEDs
in patients with DSEp may increase the plasma
Hcy levels and this effect is related to a decrease
in serum levels of folic acid. These data are in
agreement with previous studies which demonstrated that patients with epilepsy treated with
AEDs showed low serum folate and elevated
plasma homocysteine (Belcastro et al., 2010;
Linnebank et al., 2011; Schwaninger et al., 1999;
Siniscalchi et al., 2005).
Although the mechanisms by which AEDs
induce a decrease in serum folate levels are
unclear, it has been hypothesized that AEDs
may induce a decrease in intestinal absorption of folate, or an induction of liver enzymes
(Siniscalchi et al., 2005).
The conclusions from previous studies on plasma levels of vitamin B12 in patients with epilepsy are controversial. Researchers have variously described not only a decrease (Refsum et
al., 1998), as well an increase in concentrations of
vitamin B12 (den Heijer, Blom, Gerrits, Rosendaal,
& Haak, 1995), but also reported normal levels of
vitamin B12 (Van Beynum, Smeitink, den Heijer,
te Poele Pothoff, & Blom, 1999).
Recently, Linnebank et al., (2012) in a prospective monocentre study documented that the
mean plasma levels of vitamin B6 were not significantly different between AED-treated, the
untreated patients and the controls.
Thus further studies are needed to better analyze
the relationship between AEDs and vitamins.
Plasma Hcy levels represent both an atherogenic and a thrombogenic risk factor in the development of cerebrovascular manifestations.
This could be important in patients with DS
who have been documented to be more susceptible at the development of cardiovascular
(Irving and Chaudari, 2012; Raina, McGrath, &
Gunn, 2011) and neurological diseases (Lott &
Dierssen 2010).
In patients with epilepsy, chronic treatment
with AEDs induced an increase in plasma Hcy
levels that represents a risk factor for the development of cerebrovascular diseases (Karabiber
et al., 2003).
v . 18 n .2
71
Patients with epilepsy receiving long-term AED
therapy showed an alteration in circulatory
markers of vascular risk that may contribute to
a rapid increase of the atherosclerotic process
(Chuang et al., 2012). With regard to a potential proconvulsant activity of Hcy, it has been
documented that up to 20% of patients with
homozygous cystathionine ß-synthase deficiency manifested seizures, therefore an increase in
plasma Hcy levels (usually 50–200 µmol/L) may
be able to induce epilepsy (Mudd et al., 1985).
In agreement, recent investigations in experimental animal models, showed that systemic
administration of higher doses of Hcy are
able to induce seizures (Marangos et al., 1990;
Mares, Folbergrova, Langmeier, Haugvicova, &
Kubova, 1997). However, at the moment it has
not yet been demonstrated in humans whether
lower levels of Hyper-Hcy (15–20 µmol/L), are
able to induce epilepsy. Different mechanisms
have been proposed to explain the convulsing properties of Hcy. The L-isomer of Hcy
and its oxidized product, homocysteic acid,
acts as an agonist at the N-methyl-D-aspartate
(NMDA)-type glutamate receptor sites (Lipton
et al., 1997; Schwarz, Zhou, Katki, & Rodbard,
1990) and at metabotropic glutamate receptors
(Shi et al., 2003). Another hypothesis suggests
that Hcy could increase the adenosine uptake
able to reduce the seizure threshold (McIlwain
& Poll 1985). Thus Hcy may also increase seizure frequency in patients treated with AEDs.
Therefore, the decrease of plasma Hcy levels
could lead to a decrease of seizure activity.
Clinical studies (Apeland et al., 2002; Siniscalchi
et al., 2005) reported that the supplementation
with folate, vitamin B12 and B6 in patients
with Hyper-Hcy induced by AEDs decreasesd
the plasma Hcy levels and influenced endothelial activation, showing that the B group of
vitamins have a vascular-protective effect in
patients administered AEDs.
Previously it has been suggested that in men
and women treated with AEDs, supplementation with folic acid tablets (1 mg each) or as part
of a multivitamin supplement could be useful
in order to normalize the Hcy plasma levels
(Morrell et al., 2002).
Fillon-Emeryet et al. (2004) showed in adults
with trisomy 21 that Hcy levels were decreased
72
Siniscalchi
by folic acid administration. Therefore, supplementation with this vitamin may also be able
to reduce plasma Hcy levels in patients with DS
and epilepsy treated with AEDs.
However, based on our data showing that
DS patients without epilepsy don’t show an
increase in Hcy plasma levels or a decrease in
folate levels, we hypothesized that folic acid
may be used in patients with DS and epilepsy
treated with AEDs even if a deficit of folate has
not been reported.
In conclusion, our observations suggest that
treatment with AEDs in patients with DS and
epilepsy induces an increase in plasma Hcy
levels and a significant decrease in serum folic
acid. Therefore supplementation with this vitamin may be useful for achieving normal plasma Hcy values and consequently reduce the
potential for the development of both cardiovascular and neurological.
Key Messages From This Article
• Patients with DS show normal levels of Hcy
and folate.
• AEDs treatment induced in patients with DS
and seizures an increase in Hcy plasma levels with a reduction in folate serum levels.
• The increased levels of Hcy could represent
a risk factor for cardiovascular and neurological diseases in patients usually more
susceptible at these manifestations.
• The addition of folate through diet or vitamin supplements could normalize the levels
of Hcy.
Acknowledgements
The authors acknowledge to support the collection of data from this study the Groups of
Progetto DAMA (Disabled Advanced Medical
Assistance) dell’Azienda Ospedaliera di
Cosenza. The authors have no conflicts of interest that are directly relevant to the contents of
this manuscript.
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NOTE: A fully edited version of this article can
be found on the OADD website at http://www.
oadd.org/index.php?page=759 after August 15,
2012.
JoDD
Puberty, Menstruation and Pain
in Australian and Irish Females
with Down Syndrome
Volume 18, Number 2, 2012
Authors
Margaret. R.Kyrkou,¹
Rebecca O’Rourke²,
Joan P. Murphy2
Verity M. Bottroff,¹
Dennis S. Smith,¹
Hilary M. C. V. Hoey,²
Roy Brown1,3
The Flinders University
of South Australia,
Adelaide, SA,
Australia
1
Department of
Paediatrics,
University of Dublin,
Trinity College,
National Children’s
Hospital,
Dublin, Ireland
2
University of
New England,
Armidale, NSW,
Australia
3
Correspondence
[email protected]
flinders.edu.au
Keywords
Down syndrome,
puberty,
menstruation,
menstrual pain,
pain
Abstract
Females with Down syndrome (DS) have generally been considered a homogeneous group, equated with females in the general population in terms of puberty, menstruation and ability
to indicate pain. However, parents who related more closely
to females with DS were reporting otherwise, indicating that
these aspects of development in females with DS are still poorly
understood. Information was sought from parents, and where
parents chose, females with DS in Australia by means of a
questionnaire collecting cross-sectional data. With permission
from the principal researcher, the same questionnaire was used
when interviewing females with DS and their parents (predominantly mothers) in Ireland, the only question changes being
the addition of demographic questions regarding age of parents
at conception, and the female’s karyotype. The results are analysed and discussed with practical recommendations provided.
Note: At time of first publication this article was in press. A
fully edited version of this article can be found on the OADD
website at http://www.oadd.org/index.php?page=760 after
August 15, 2012.
book review:
Encore Edie
by Annabel Lyon
Volume 18, Number 2, 2012
Author
Susan Petko
NOTE: At time of first publication an introduction to the
author was not available. A fully edited version of this article
can be found on the OADD website at http://www.oadd.org/
index.php?page=761 after August 15, 2012
I have just read Encore Edie by Annabel Lyon. This book is
about Edie who is 13. It is about how things are now Edie is
at high school. Edie is a very interesting girl who has a great
love for Shakespeare and her family too. She is having a hard
time making friends and finding her place at school.
The book is also about her and her cousin Merry who has
Down syndrome and how Edie is worried what other kids
will think. I enjoyed reading the book and I liked the storyline and the characters. But I did not like the way Edie
described how Merry looked. Edie said “Merry has something called Down syndrome, which makes her short and
pudgy, with tiny ears and stubby fingers and eyes like pods
and worst of all, it makes her slow.” I’ve had Down syndrome
since I was born and I know people who have Down syndrome and even though we look different we are the same
as anyone. That description was poor but I like the character
of Merry. Merry was 14 year old and to Merry Edie is her
favourite cousin.
Correspondence
[email protected]
I think that the author, Annabel Lyon knows what Down
syndrome is but doesn’t understand or feel what it is like to
have Down syndrome. I think that the way Annabel showed
Merry was positive because Merry did do a lot with the other
characters in the book that she met. I think that the other
characters, like Regan and Sam liked Merry and they treated
her the same as anyone.
+
Edie went to the drama club and came up with the idea of
putting on King Lear as a musical. She was put in charge.
Edie’s parents wanted Edie to put Merry in the play because
they wanted her to have a chance to do something with Edie.
So Edie tried to put Merry in charge of the music and that
didn’t work.
Merry was at all the rehearsals and eventually Edie discovered that Merry knew all the lines for all the characters.
When Edie had an argument with Raj who was playing the
fool, he just wouldn’t do the part the way Edie thought he
should, so she gave the role to Merry. But that did not work
out well.
“I realize now – what it must have looked like
everyone thinks I was making fun of Merry. I
realized the moment they started to Boo, they
thought I was trying to get everyone to laugh
at her, by putting her onstage and making her
the fool. They didn’t realize that she could actually do the part, that it wasn’t just a grotesque
practical joke. That, I realize, is what I have to
explain that if we all just give her a chance, she
can actually do the part.” Edie wasn’t thinking of Merry as someone who has Down syndrome but as person who can actually can play
the part. Someone reading the book would be
impressed by that. Edie did a good job by putting Merry in the play.
There are lot of other interesting things that
happen in the book. The characters in the book
change and grow. In the end of the book Edie
came to realize that she can be friends with
Merry as well as her cousin. It is a good story
and a good book and I would personally recommend this book because of the characters.
You get to know a lot of characters and one
of them especially, who just happens to have
Downs syndrome.
Lyon, A. (2011). Encore Edie. Puffin Books,
192 pp., ISBN 978‑0143177418
v . 18 n .2
77
Encore Edie
Editorial Policy
Journal on Developmental Disabilities / Le journal sur les handicaps du développement
published by the Ontario Association on Developmental Disabilities, Toronto, ON
ISSN: 118-9136
© Ontario Association on Developmental Disabilities
The Journal on Developmental Disabilities is published three times yearly by the Ontario Association
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Design: Ted Myerscough
Layout: Leah Gryfe
Cover Image by Angela Gillis
Marker on paper
Angela Gillis attends the Manuela Dalla-Nora Program at Tycos Centre through Vita Community Living
Services/Mens Sana. She has graced the program with her unique quirky style and her love of the arts
for 25 years. Angie loves to spend hours on her artwork and is meticulous in the placement of lines and
selection of colour that she uses, and always includes her signature within the art itself. Her art reflects her
life in that moment.
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