MCADD Dietary Guidelines - the IMD Scotland Website

Transcription

Dietary Management Guidelines for
Medium chain acyl-CoA Dehydrogenase Deficiency
(MCADD)
This folder has been put together to give you the information you need to support you
following a diagnosis of Medium chain acyl-CoA Dehydrogenase Deficiency (MCADD). It
will support other information given to you by your specialist team. It has been developed
with parents and healthcare professionals to give you a good understanding of the
condition, the dietary treatment when unwell, the monitoring required and where support
can be found.
Over the coming weeks and months your knowledge and confidence will grow. The team
are here to support your learning and to help with issues or concerns you or your child may
have. We hope you will ask questions if anything is unclear.
The pack contains information you need to start with and then add to as time goes on.
You may find as your child grows and circumstances change, such as starting school or
nursery, there may be issues you need to discuss with other families. Although each
person with MCADD is an individual, issues can arise that other families have experience
with.
The pack is useful to keep all the information you are given in one place for easy reference
and as your child grows up they will find it a useful resource too.
IMD Scotland MCADD – Dietary Management Guidelines
What is MCADD?
During long periods between eating, the body breaks down it’s own fat stores to produce
energy. People with MCADD lack one of the enzymes needed to do this. They can break
down the stored fat partly but not completely. There is a hold up at the ‘medium chain fat’
step where the enzyme needed to complete the breakdown is not working properly. This
causes a build up of medium chain fats.
Sometimes we need to break down fats quickly, for example, when we have not eaten for
some while or when we have an infection. People with MCADD can’t do this. The medium
chain fats can build up and make toxic substances that may lead to serious symptoms.
If this condition is not diagnosed early or is ignored and not treated by following simple
advice from a specialist medical team, it could lead to serious illness and possibly death.
Fortunately- once diagnosed- MCADD is usually quite straightforward to manage and
children with this condition usually lead healthy normal lives.
What treatment is available for MCADD?
MCADD is treated by diet. When the child is well, there is no specific dietary management
apart from avoiding long periods without food. The well child can be on a normal, healthy
diet.
The information in this folder will outline what you should expect your child eat and drink
when well and also the special measures you need to take when they are unwell. Your
child will have an Emergency Regimen (ER) to take when unwell, depending on their age
the recipe will change.
Medium Chain Triglyceride’s (MCT’s) –
MCT’s are a type of fat not commonly found in foods, it is found in coconut and coconut
oil. Some specialised infant formulae also contain MCT’s. A small amount of coconut as an
ingredient may be eaten but pure coconut and coconut oil should be avoided.
It is very important that you make sure that any healthcare professional looking after your
child knows that your child has MCADD, especially in the event of an accident or surgical
procedure. Fasting for a long time will result in the build up of toxic fatty acids leading to
serious complications. Your metabolic team can be contacted for advice if you are not in
your usual hospital.
You should expect to have regular contact with your specialist team. You will be in contact
with your Dietitian at least 4 times in the first year and then at least yearly after that. Of
course if you have any worries or concerns there are contact details in the folder for your
team, which you can use in between visits as necessary.
It is advisable to keep this information in a safe place, you will need to refer to it and as
your child grows, they may find it useful, as they gain greater independence.
To make the information easy to use it has been divided into sections based on the
individuals age as follows-
Contact numbers
Infants under 1yr
Children 1-2yrs
Children 2-9yrs
Children over 10yrs & adults
Your Specialist Team Contacts
Metabolic Specialist:
……………………………………………………………………….
Contact telephone number:
……………………………………………………………………….
Metabolic Dietitian:
………………………………………………………………………
………………………………………………………………………
Clinic Times:
……………………………………………………………………….
Appointments contact number: ………………………………………………………………………..
Your Local Team Contacts
Consultant:
…………………………………………………………………………
…………………………………………………………………………
Dietitian:
…………………………………………………………………………
…………………………………………………………………………
Other contact numbers
GP:
………………………………………………………………….......
…………………………………………………………………………
Health Visitor:
………………………………………………………………………..
Contact telephone number
.…………………………………………………………………………
Pharmacy:
………………………………………………………………………….
Contact Number:
………………………………………………………………………..
Home Delivery Contact Number (if used) ………………………………………………………………
Parent support
These parents have given permission for their details to be printed. They are
happy for you to contact them if you would like to speak to another parent.
1. Parent’s Name:
Child’s name & year of birthContact Number:
2. Parent’s Name:
3. Parent’s Name:
4. Parent’s Name:
5. Parent’s Name:
Useful contacts
Managed Clinical Network for Inherited Metabolic Disease, Scotland www.imd.scot.nhs.uk
Climb – Children Living with Inherited Metabolic Diseases
A national organisation working on behalf of children, young people and families affected
by metabolic disease.
Freephone: 0800 652 3181’
www.climb.org.uk
Infants under 1yr
When well
Infant feeding
Your baby can be offered breast feeds or normal infant formula feed depending on your
choice, just like any other newborn. To begin with, you will be establishing a regular
feeding pattern; usually 3-4hourly feeds (6-8 feeds/day). You should not allow your baby
to sleep for more than 6 hours between feeds.
The time between feeds is sometimes called fasting and your baby can fast for up to 6
hours. You may have to continue to feed your baby during the night, even if this means
waking them for a feed. As your baby gets older they will be able to fast for longer but
may still require a feed overnight. This should be discussed with your specialist team.
If your baby will not wake or take a night feed then contact your hospital for advice.
Some specialist infant formulae contain MCT’s. If you are advised to change your baby
onto another formula, please discuss with your specialist team first to make it is suitable.
Weaning
Solid food should be introduced at the usual recommended age, ideally around 6 months or
26 weeks of age, but no earlier than 4 months (17 weeks). If your baby was premature
please discuss the best time to wean with your specialist team. If you plan to start
weaning before 6 months discuss with your Health Visitor or your specialist team. There
are some foods which should be avoided until your baby is 6 months. Your Health Visitor
will be able to provide you with advice and support about starting and progressing with
solids. You can use homemade, jars or packet foods.
Initially your child will only take small amounts of solids and will receive most of what they
need from their milk (breast or normal infant formula). You will try puree vegetable or fruit
to start with then move on to introducing starchy carbohydrate foods such as baby rice or
potato. If your baby is over 6 months, wheat and gluten containing pasta, cous cous,
cereals and breads (including chapattis) can be introduced. Remember not to add salt or
sugar to your baby’s food.
Try to encourage a wide range of tastes for your child. The only foods your child should
avoid are coconut (although as small amount as an ingredient in a food would be ok) and
coconut oil.
If you experience any difficulties with weaning your child, please discuss these with your
Health Visitor and Dietitian.
Your child can move onto full fat cows milk at 1 year of age unless you are advised
otherwise by your specialist team.
When your child is well, there is no need to encourage sugary drinks, you can use sugar
free varieties. However, you may want to give your infant a small amount of Emergency
Regimen when they are well to establish the taste, this will help them to accept it when
they are unwell. It will also help you to become familiar with the recipe so you feel
confident preparing it when your child is unwell.
Infants under 1yr
When unwell
Offer your baby their usual feed first (breast/formula) every 2-3 hours. If you are
concerned your baby is unwell and is refusing feeds, Emergency Regimen should be given.
At this age you should contact your specialist team first for advice and support.
Emergency Regimen drinks will supply electrolytes (sodium, potassium etc) from the
rehydration powder (Dioralyte / Electrolade) and sugar from the glucose polymer (super
soluble Maxijul / Polycal / S.O.S.), this will keep your baby’s blood sugar levels up and also
replace any electrolytes lost from vomiting or diarrhoea.
Oral rehydration solutions – Dioralyte / Electrolade / Rapolyte should not be given without
the addition of a glucose polymer- S.O.S. / super soluble Maxijul / Polycal powder
If you are advised to make up the Emergency Regimen at home(If you have been advised to use super soluble Maxijul or Polycal, your Dietitian will have
completed an instructions sheet, which advises on the use of scoops or scales)
Emergency Regimen (ER) 10% Carbohydrate
1. Start with a clean measuring
jug, open sachet of S.O.S 10
and empty all the powder into
the jug.
2. Add cooled boiled water to
200ml, make sure your jug is
on a flat surface
3. Mix well
If your child has vomiting or has diarrhoea or both vomiting and diarrhoea
add rehydration powder (dioralyte or electrolade). Instructions overleaf.
4. Add all the powder from 1
sachet of electrolade /
dioralyte to 200ml of S.O.S.
10 & water
5. Mix well
Pour into a bottle or cup, whatever you feel your child will take best.
Your infant should be offered & encouraged to take the volumes recommended in the table
below.
Age
0-3 months
4-6 months
7-9 months
10-12 months
Volume in 24hrs
45-80ml x 12 (2hourly)
85-100ml x 12 (2hourly)
90-100ml x 12 (2hourly)
100ml x 12 (2hourly)
70-120ml x 8 (3hourly)
130-150ml x 8 (3hourly)
130-150ml x 8 (3hourly)
150ml x 8 (3hourly)
As you will have contacted your specialist team when giving your infant Emergency
Regimen they will be able to advise and support further. Often your child will not manage
these volumes and may need to come to hospital. If your infant is vomiting frequently,
offer small amounts of feed/Emergency Regimen and contact your hospital.
Once your infant starts to recover they can return to their normal feeds, usually within
48hours of starting the Emergency Regimen.
When



should you contact the hospital?
Before you start using the Emergency Regimen (ER) to get advice and support
If your baby has diarrhoea and vomiting
If you are advised to use the Emergency Regimen at home and your baby does not
manage to take the volume of the Emergency Regimen it requires
 If your baby’s normal feeds have not restarted within 48hours
 If breastfed and unwilling to take the Emergency Regimen from a bottle
Supplies of the Emergency Regimen
You will receive ongoing supplies of the glucose polymer (S.O.S / super soluble Maxijul /
Polycal powder) and rehydration solution (Electrolade/Dioralyte) on prescription from you
GP. You should always have an in date supply at home in case it is required. Please check
the use by dates regularly and arrange for replacements in good time.
Holidays
It is advisable to take emergency regimen supplies (oral rehydration solution & glucose
polymer) with you when you go on holiday. If you are travelling abroad your specialist
team will be able to provide you with a letter regarding the importance of these products
for your child for customs.
Children 1-2 years
When well
From around 1 year of age, your child will be eating 3 main meals (breakfast, lunch and
evening meal) and should be given a snack before bed. Continue to include starchy
carbohydrate foods (bread, chapatti, rice, pasta or cereals) at each meal.
Suitable bedtime snacks you may want to try include- cereal & milk, sandwich, toast. You
can offer water, milk or sugar free diluting juice.
Snacks between meals are not normally required but you may want to offer snacks in
between if your child is a picky eater or has a poor appetite. It is preferable that snacks
are offered at a regular time between meals (1½ - 2 hours after a meal and before the
next) to prevent grazing (constant frequent snacking) which can affect appetite.
It is likely your child will manage to fast overnight (approximately 12 hours) but it is
important to make sure they have a snack before bed and breakfast shortly after waking.
They should not miss or have a late breakfast. They should not be allowed to fast for
longer than 12 hours.
Avoiding Medium Chain Triglyceride’s (MCT’s)
What if they will not eat breakfast?
Occasionally, your child will not take breakfast, you can offer a suitable alterative drink
(see list). You should offer a snack mid morning rather than waiting until lunchtime. If
your child appears unwell you may want to try Emergency Regimen instead.
You can offer your child a range of suitable alternative drinks
 Ribena carton (ready to drink, not reduced sugar)
 Fruit Shoot (not reduced sugar)
 Pure Apple juice (ready to drink)
Other drinks may be suitable, check the label-
Carbohydrate (CHO) should be at least 10.5g per 100ml (labels above show
examples)
It is important not to depend on sugary drinks. When well your child should get all they
need from their meals and snacks and should not require the suitable alternative drinks,
you can offer water, milk or sugar free diluting juice. If your child is refusing meals or is a
picky eater but otherwise well and you find they are drinking the alternative drinks in large
quantities, please contact your Dietitian for advice.
It can be helpful to offer your child some Emergency Regimen or alternative drinks from
time to time when they are well. This way they will become used to the taste and be more
accepting when they become unwell.
Children 1-2years
When unwell
soluble Maxijul / Polycal / S.O.S.), this will keep your infants blood sugar levels up and also
Instructions to make up Emergency Regimen overleaf(If you have been advised to use super soluble Maxijul or Polycal, your Dietitian will have
1. Start with a clean measuring
jug, open sachet of S.O.S 15
and empty all the powder into
the jug.
. Add cooled boiled water to
200ml, make sure your jug is
on a flat surface
3. Mix well
add rehydration powder (dioralyte or electrolade)
15 & water
5. Mix well
Pour into a cup.
If preferred, you could offer suitable alternative drinks previously mentioned,
including:
 Ribena carton (ready to drink, not reduced sugar)
 Fruit Shoot (not reduced sugar)
 Pure Apple juice (ready to drink)
Other drinks may be suitable, check the label. Carbohydrate (CHO) should be
at least 10.5g per 100ml refer to the 1-2year ‘when well’ section for examples
of suitable product labels.
Emergency Regimen should be offered 2-3 hourly day and night. If your child is unable to
take large quantities of Emergency Regimen or is vomiting you should try offering small
amounts more often.
AIM FOR APPROXIMATELY 1200ml in 24 hours, offer 100ml every 2 hours.
If they refuse or are unable to keep down their Emergency Regimen contact the hospital.
How long should I continue with the Emergency Regimen?
Once your child has started eating again continue to offer some Emergency Regimen or
alternative drinks until their appetite has returned to normal (usually with 48hours of
starting the Emergency Regimen). Once appetite and intake has fully returned Emergency
Regimen/alternative drinks can stop.
Oral rehydration solutions – Dioralyte / Electrolade should not be given without the
addition of glucose polymer S.O.S. / super soluble Maxijul / Polycal powder.
When




should you contact the hospital:
When you start to use the Emergency Regimen to get advice and support
If your child has diarrhoea and vomiting
If your child does not manage to take the Emergency Regimen prescribed above
If your child’s normal diet has not restarted within 48 hours
Holidays
It is advisable to take emergency regimen supplies (oral rehydration solution & glucose
polymer) with you when you go on holiday. If you are travelling abroad your Specialist
Team will be able to provide you with a letter regarding the importance of these products
Children 2-9 years
When well
From the age of around 2 years, your child will be eating 3 main meals (breakfast, lunch
and evening meal) and should be given a snack before bed. Continue to include starchy
carbohydrate foods such as bread, chapatti, rice, pasta or cereals at each meal.
Suitable bedtime snacks you may want to try include- cereal & milk, toast or sandwich.
You can offer water, milk or sugar free diluting juice to drink. You may want to begin
gradually explaining why eating regularly is important for them and the need for
Emergency Regimen/alternative drinks when unwell or not eating.
Snacks between meals are not normally required but you may want to offer snacks in
between if your child is a picky eater or has a poor appetite.
It is likely your child will manage to fast for up to 12hours overnight but it is important to
make sure they have a snack before bed and breakfast shortly after waking. They should
not miss or have a late breakfast. They should not fast for longer than 12hours.
If your child refuses breakfast you can offer some suitable alterative drinks (see list). You
should offer a snack mid morning rather than waiting until lunchtime. If your child appears
unwell you may want to try Emergency Regimen instead.
You can offer your child a suitable alternative drink
 Lucozade Energy (original)
 Marks & Spencers Energy drink
 Sainsbury’s Energy drink
Many sports drinks have a low carbohydrate content 6g carbohydrate/100ml (6%) or less
and are therefore unsuitable.
Carbohydrate (CHO) should be at least 17g per 100ml (labels above show
examples)
It is important not to depend too much on sugary drinks. When well your child should get
all they need from their meals and snacks and should not need the suitable alternative
drinks, you can offer water, milk or sugar free juice. If your child is refusing meals or is a
picky eater but otherwise well and you find they are drinking the alternative drinks in large
quantities, please contact your Dietitian for advice.
More people will become involved in the care of your child as they grow, nursery, school
etc and they will also need to know what to do if your child is unwell. It may be easier if
school have a supply of suitable alternative drinks for your child if they are required until
you can be contacted.
It can be helpful to offer your child some Emergency Regimen or alternative drinks from
time to time when they are well. This way they will become used to the taste and be more
accepting when they become unwell. When they are old enough, this can be a good
opportunity for them to learn how to make up their Emergency Regimen, as in time they
will be making it up for themselves. This will gradually prepare them for greater
independence.
When unwell
soluble Maxijul / Polycal / S.O.S.), this will keep your infants blood sugar levels up and also
Instructions to make up Emergency Regimen overleaf(If you have been advised to use super soluble Maxijul or Polycal, your Dietitian will have
1. Start with a clean measuring jug, open
sachet of S.O.S 20 and empty all the
powder into the jug.
2. Add cooled boiled water to 200ml,
make sure your jug is on a flat surface
3. Mix well
add rehydration powder (dioralyte or electrolade)
20 & water
5. Mix well
Pour into a cup
If preferred, you could offer suitable alternative drinks as previously
mentioned, including:
Many sports drinks have a low carbohydrate content 6g carbohydrate/100ml
(6%) or less and are therefore unsuitable.
Other drinks may be suitable, check the label Carbohydrate (CHO) should be
at least 17g per 100ml refer to the 2-9 year when well section for examples of
suitable product labels.
Aim for the following volumes (these volumes are a guide only if you have concerns
contact your hospital)
Age
2 years
3-4 years
5-6 years
7-8 years
9 years
Volume in 24hrs
1200ml/day offer 100ml x 12 (2hrly)
1300-1400ml/day offer 110-120ml x12 (2hrly)
1500-1600ml/day offer 125-135ml x 12 (2hrly)
You can offer smaller volumes more frequently to begin with moving to larger volumes
every 3 hours once tolerated.
If they refuse or are unable to keep down their emergency feed contact the hospital.
Once your child has started eating again continue to offer some Emergency Regimen or
alternative drinks until their appetite has returned to normal (usually with 48hours of
starting the Emergency Regimen). Once appetite and intake has fully returned Emergency
the addition of glucose polymer super soluble Maxijul / Polycal / S.O.S.
Holidays
It is advisable to take Emergency Regimen supplies (oral rehydration solution & glucose
Children over 10years & adults
When well
From the age of 10 years into adulthood, regular mealtimes remain important, 3 main
meals (breakfast, lunch and evening meal) plus a snack before bed. Starchy carbohydrate
foods should be eaten at each meal, examples include bread, chapatti, rice, pasta or
cereals. Suitable bedtime snacks include cereal & milk, sandwich or toast.
Children
Your child will be becoming increasing independent and over the years should have
become familiar with their treatment and understand why their Emergency Regimen is
important. They will be spending increasing time away from home, at school and with
friends and you will want to feel confident that they continue to do all they can to remain
well.
They should continue to have a starchy snack before bed and a breakfast shortly after
waking. It is important that they should not fast for long periods overnight (longer than
12hours), keeping to a routine would be helpful.
By the age of 10 years a young person should know how to make their Emergency
Regimen and if necessary be able to make it themselves. They should also know what
type of alternative drinks are suitable and where they can get them. School should still
have a supply of suitable alternative drinks for your child in case they are required or when
they are older they may want to carry these themselves.
Teenagers and Adults
Teenagers and adults should avoid prolonged fasts and take care to use the Emergency
Regimen when unwell. If away from home, they should have a supply of ingredients for
the Emergency Regimen and contact details of their specialist team.
Weight Management
If you are trying to lose weight, discuss this with your specialist team beforehand. It is
important to establish if weight loss is necessary and if so that a sensible approach is
taken, avoiding long fasts, fad diets and rapid weight loss. Your Dietitan will be able to
provide support and advice.
Alcohol
Excess alcohol could result in vomiting or lack of food being eaten. If alcohol is taken to
excess and results in vomiting it is important to go to hospital straight away.
Alcohol, if consumed should be taken in moderation and with food. It is important that
fasting periods are not exceeded due to over sleeping or lack of food as a result of excess
alcohol.
If a meal is missed then a suitable alternative drinks would be- Lucozade Energy (original)
- Marks & Spencers Energy drink
- Sainsbury’s Energy drink
Many sports drinks have a low carbohydrate content 6g carbohydrate/100ml (6%) or less
and are therefore unsuitable.
Carbohydrate (CHO) should be at least 17g per 10ml
It is important not to skip or miss meals on a regular basis. If this is an issue please
discuss with your specialist team.
Instructions to make up Emergency Regimen overleaf-
(If you have been advised to use super soluble Maxijul or Polycal, your Dietitian will have completed an
instructions sheet, which advises on the use of scoops or scales)
Children over 10years & adults
When unwell
1. Start with a clean measuring jug, open
sachet of S.O.S 25 and empty all the
powder into the jug.
2. Add cooled boiled water to 200ml,
make sure your jug is on a flat surface
3. Mix well
If the individual has vomiting or has diarrhoea or both vomiting and diarrhoea
add rehydration powder (dioralyte or electrolade).
25 & water
5. Mix well
Pour into a cup.
If preferred, you could offer suitable alternative drinks as previously
mentioned, including:
Many sports drinks have a low carbohydrate content 6g carbohydrate/100ml
(6%) or less and are therefore unsuitable.
Other drinks may be suitable, check the label Carbohydrate (CHO) should be
at least 17g per 100ml refer to the 10yr-adult when well section for examples
of suitable product labels.
Aim for the following volumes (these volumes are a guide only if you have concerns
contact your hospital)
Age
10 years
11-13 years
14-15 years
16 years and above
Volume in 24hrs
1800ml/day offer
2000ml/day offer
2200ml/day offer
2400ml/day offer
150ml
165ml
185ml
200ml
x
x
x
x
12
12
12
12
(2hrly)
(2hrly)
(2hrly)
(2hrly)
Smaller volumes can be taken frequently to begin with moving to larger volumes every 3
hours once tolerated.
If vomiting starts or continues and it is not possible to keep down the Emergency Regimen
contact the hospital.
Once normal diet has restarted, continue to take some Emergency Regimen or alternative
drinks until appetite has returned to normal (usually with 48hours of starting the
Emergency Regimen). Once appetite and intake has fully returned Emergency
the addition of glucose polymers S.O.S. / super soluble Maxijul / Polycal
Holidays
It is advisable to take Emergency Regimen supplies (oral rehydration solution & glucose
for customs.
If alternative glucose polymer is required, your Dietitian will advise on how much super
soluble maxijul or polycal is needed. Please make up the Emergency Regimen as follows1. Weigh ___g or measure ____ levelled
scoops of super soluble maxijul or
polycal powder (glucose polymer)
2. Pour glucose polymer powder into a
measuring jug and add cooled boiled
water to 200ml
3. Add 1 sachet of electrolade or
dioralyte to 200ml of glucose polymer
and water. Mix well and pour into a cup.
Acknowledgements
Dixon. M. in conjunction with Champion. M. and British Inherited Metabolic Disease Group
(2007) M.C.A.D.D. Medium chain acyl coA Dehydrogenase Deficiency- Dietary Management
Guidelines for Dietitians
MCADD is suspected leaflet, NHS Antenatal and Newborn Screening Programmes
Members of the MCADD Implementation Group, Scotland
Scottish National Managed Clinical Network for Inherited Metabolic Disease
Photographs by Laura Agnew, Medical Illustration, Royal Hospital for Sick Children Glasgow

MCADD Dietary Guidelines - the IMD Scotland Website

Transcription

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