MCADD - Treating MCADD

A comprehensive plan will be drawn up for your child by a specialist with experience in treating medium-chain acyl-CoA dehydrogenase deficiency (MCADD).

The plan will cover:

how often your child should eat
what your child should eat
what you should do if your child is not eating normally due to illness or similar – this usually involves giving your child high-glucose drinks

You will be given a number of sachets, consisting of glucose and oral rehydration solutions, which can be used in an emergency.

As part of this plan, you will regularly meet with a dietician so that your child’s progress can be tracked and you can discuss any issues. A dietician is a health professional who works with people that have special dietary needs.

Other members of your child’s care team may include:

a doctor who specialises in the treatment of children (paediatrician)
health visitor
a specialist nurse

You will also be given telephone numbers you can use in case of an emergency.

Dietary advice will change as your child gets older. Speak to your care team for more information.

The only food your child should avoid eating is coconut, as it contains large amounts of fatty acids that they will be unable to digest.

What to do if your baby is unwell

If your baby becomes unwell and starts refusing feeds, you should first contact your care team for advice.

They will usually tell you to make a drink using a sachet of glucose mixed with 200ml of cooled boiled water.

If your child is also experiencing diarrhoea or being sick, you should make a similar drink consisting of 200ml of water and a combination of glucose and oral rehydration solution.

The fluid can then be poured into a bottle or cup, depending on what your baby finds easiest.

Your dietician will provide more detailed instructions and information sheets.

You should contact your care team for further advice if your baby:

is experiencing persistent diarrhoea and/or vomiting
is unable or unwilling to take the recommended fluid intake
is breastfed and will not accept fluid from a bottle or cup
has not resumed normal feeds within 48 hours

Older children

Older children should be encouraged to eat three meals a day: breakfast, lunch and dinner.

Snacks between meals are not usually recommended unless your child has a poor appetite.

If snacks are required, they should be given at regular intervals to prevent constant snacking (grazing), which could affect appetite.

It is usually safe for your child to sleep through the night without needing to be fed, as long as they eat something small before going to bed, such as milk and cereal or toast.

Your child should eat breakfast soon after waking up. If they refuse breakfast, a sugary drink (see below) can be given as an alternative, with a mid-morning snack before lunch. However, this should not be encouraged on a regular basis.

You should never let a child over the age of 1 fast for more than 12 hours.

What to do if your child is unwell

You will need to give them an emergency drink.

Alternatively, you can give them commercially available sugary drinks that contain high amounts of glucose. These include:

Fruit Shoots
J20
a carton of Ribena (the original type, not the reduced sugar or sugar-free types)
Lucozade energy (but not the sport variety, as that does not contain enough glucose)
fizzy drinks such as cola or lemonade (again, make sure they are not sugar-free or diet versions)

(Note – the use of brand names is for information purposes only. It does not imply any endorsement for these products by NHS Choices or any related organisation).

The long-term use of these types of sugary drinks is not encouraged as they can cause health problems in later life, such as tooth decay and diabetes.

Contact your care team for advice if your child refuses the fluid or is unable to keep it down.

Advice for adults with MCADD

There are a number of issues that adults with MCADD need to be aware of. These are discussed below.

Alcohol

Heavy alcohol consumption, especially binge drinking, can be dangerous if you have MCADD because:

people often pass out after binge drinking, so the time before eating your next meal could become dangerously delayed
alcohol can cause your blood sugar levels to drop, which can trigger the symptoms of MCADD

If you do choose to drink alcohol, consume in moderation and never on an empty stomach.

Weight management

If you are trying to lose weight, you should discuss the safest way to do this with your care team.

It is especially important to avoid fad or crash diets, as a sudden change in the amount and type of food that you eat could trigger symptoms.