1-Romanowska

Organization of patient
management and epidemiology of
fungal infections in CF in Poland
Ewa Romanowska, Hanna Dmeńska, Magdalena Kołodziejczyk, Alicja
Pawińska, Katarzyna Dzierżanowska-Fangrat
The Children’s Memorial Health Institute
Warsaw, Poland
Cys$c fibrosis pa$ent’s registry •  1661 registred (but the number is probably higher) •  1552 living pa$ents 1017 children (0-­‐18 years) 65,5% 535 adults (>18 years) 34,5%
Polish Cystic Fibrosis Registry – (data not published) by Pogorzelski, X.2012
•  23 ins$tu$ons specializing in CF pa$ents management •  4 ins$tu$ons > 100 pa$ents with CF •  Time of diagnosis (age) 0 – 43 yo •  Diagnosis within first year of life – 731 pa$ents (51%) •  Diagnosis not connected with rou$ne screening for CF at birth: 4,5 yo Polish CF Registry 2012
393 Adult pa3ents included 166 IMiDZ 324 41 Gdańsk 130 48 KPAD Poznań 123 12 PMD Poznań 72 72 Karpacz 71 24 Łódź 18 Bydgoszcz 63 61 IGiChP 52 52 INSTITUTION Rabka Nr of registred pa3ents 18 INSTITUTION Nr of registred pa3ents Katowice GCZDiM 45 CZD 44 Zabrze 41 Lublin 22 Rzeszów 21 Białystok KChD 19 Koszalin 16 Kraków USD 11 Szczecin SPWSZ 10 Katowice WPChP 8 Białystok KChPiG 8 Kraków WSSD 7 Szczecin SPSK1 6 Szczecin Zdroje 5 Adult pa3ents included 2 15 20 6 7 10 5 4 4 2 8 1 0 0 Number of pts diagnosed in subsequent years (Poland) year
number
year
number
2011
68
2000
65
2010
80
1999
64
2009
98
1998
64
2008
95
1997
63
2007
77
1996
56
2006
62
1995
58
2005
56
1994
46
2004
76
1993
34
2003
66
1992
25
2002
77
1991
24
2001
69
1990
26
Prevalence of CF (with reference to registry) YEAR No of births in Number of Poland registered CF cases (in tousands) frequency 2008 414,5 95 1 : 4363 2009 417,6 98 1 : 4261 2010 413,3 80 1 : 5166 2011 391,0 68 1 : 5750 Polish Cystic Fibrosis Registry – data not published A. Pogorzelski, X.2012
GENDER •  popula$on of Poland F: 19 883 870
M: 18 654 577 1 : 0,94 age F M F:M ra3o 0 – 3 77 72 1 : 0,94 0 – 18 517 507 1 : 0,98 0 – 61 762 790 1 : 1,04 > 18 248 287 1 : 1,16 90 CF distribution in relation to age in Poland
80 70 60 50 40 30 20 10 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 Polish Cystic Fibrosis Registry – not pulished data by.A. Pogorzelski, X.2012
Percentage of adult pa3ents Country
%
Poland
34,5
France
47
Ireland
54
Belgium
46
Slovakia
46
Canada
59
Australia
47
Germany
56
USA
47
The age of pa3ents with CF Country
mean
median
Poland
14,75
France
16,8
15
Ireland
18
17
Australia
18,7
17
Germany
19,6
18
Polish Cystic Fibrosis Registry – data not published A. Pogorzelski, X.2012
Pa3ents > 40 yo Country
%
Poland
0,97
(n=15)
France
4,4
Great Britain
7
Lung transplanta$ons
(data till end of 2013)
Ins3tu3on No of Ltx
CF patients
Rabka IMiDz IGiChP Karpacz 17 5 3 1 Łódź Poznań 1 1 Gdańsk 1 Total 29 Poland 2013: 19 lung
transplantations
-not only in pts with CF
29 Polish CF pts after lung
transplantation (3 Ltx performed in
Poland)
23 Polish CF living pts after
lung transplantation
In 1995, the Children’s Health Ins3tute received the status of a research and development unit as well as the name “The Children’s Memorial Health Ins3tute” Organiza$on of pa$ent management and epidemiology of fungal infec$ons in CF in CMHI •125 pa$ents • Consulta$on and Pulmonary Clinic: 75 pa$ents (60%) • Gastroenterology and Hepatology Department: 47 pa$ents (38%) •Pediatrics, Metabolic and Nutri$on Department: 18 pa$ents (15%) -­‐ Surgery Unit, Intensive Care Unit: 5 pa$ents (4%) 15 pa$ents (15%) under care of more than one unit. CMHI January 2010 – May 2014
Pa$ents with CF •  65 Boys (52%) •  60 Girls (48%) Age: 15 months – 23 years 93 pa$ents (75%) ≤ 18 yo 32 pa$ents (25%) ≥18 yo CMHI January 2010 – May 2014
CF distribution in relation to age in CMHI
Number of patients
12
10
8
6
4
2
0
1
3
5
7
9
11 13 15 17 19 21 23
Age of patients
CMHI January 2010 – May 2014
Materials and results: Period: January 2010 - May 2014:
- 245 sputum samples - 63 (26%) positive fungal
cultures
- 265 throat swabs – 31 (12%) positive fungal
cultures)
-  107 nasal swabs – 15 (14%) positive fungal
cultures)
-  10 gastrostomy swabs – 10 (100%) positive
fungal cultures)
Isolated fungal species
•119 posi$ve cultures (19%), 508 nega$ve cultures (81%) • Candida albicans 75 (63%) • Aspergillus fumigatus 17 (14%) • Trichophyton schoenleinii 6 (5%) • Aspergillus candidus 5 (4%),Candida glabrata 5 (4%), Candida dubliniensis 5(4%) • Other isolates 6 (5%): Candida krusei, Candida inconspicua, Candida tropicalis, Candida famata Conclusion • Cystic fibrosis in Poland is no more only a childhood
disease.
• Survival period is still unsatisfactory
• Registry is a valuable tool of the epidemiological and
clinical analysis, however its completedness is still
inadequate in Poland.
• On the basis of fungal isolations from sputum it is hard to
differentiate between fungal colonisation and infection
• There are difficulties in designing proper diagnostic
procedures and pathogen isolation from bacterial
contaminated samples and problems with establishing
proper clinical diagnosis
Acknowledegments
• Patients with Cystic fibrosis and their families
• Polish Society Against Mucoviscidosis
• Matio Foundation
• Cystic Fibrosis Europe
• MD, PhD Sylwia Stężowska – Kubiak (Department of
Gastroenterology and Metabolic Diseases, Medical Univeristy,
Poznań, Poland)
• MD Andrzej Pogorzelski (Institute of Tuberculosis and Lung Diseases,
Rabka-Zdrój, Poland)
• MD, PhD Agnieszka Sobczyńska-Tomaszewska (Institute of Mother
and Child, Warsaw, Poland)
THANK YOU