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Embed code for: MCADD_Dietitian_letter_April_2015_v0.4 (1)
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Dietitian Letter MCADD
Re: [name of child], [address of child], [NHS number]
[Name of child] has been detected on newborn screening to have a positive (abnormal) test for medium-chain acyl-CoA dehydrogenase deficiency (MCADD). Confirmatory testing is in progress.
MCADD is a rare inherited disorder causing a block in the metabolism of fat into energy. A child with this condition is at risk from hypoglycaemia, coma and death with fasting and particularly during intercurrent illnesses when the body’s demand for energy increases and food intake is often reduced. S/he may appear drowsy or lethargic, vomit, have seizures or have a deteriorating conscious level. Hypoglycaemia is a late sign, and therefore treatment must be initiated if [name of child] is unwell even if the blood sugar is normal.
MCADD requires no special dietary treatment when the child is well apart from avoiding long periods without feeds or food. Parents/carers are instructed on maximum ‘safe fasting times’ for age (these times get longer with age). Babies can be breast or bottle fed. Specialised infant formula or enteral feeds with added medium-chain triglycerides (MCT) should be avoided. When intercurrent illnesses occur, MCADD is treated with an Emergency Regimen (ER) of high energy feeds/drinks (glucose polymer) or, if unable to tolerate these for whatever reason (such as vomiting or diarrhoea), with intravenous 10% dextrose and electrolyte additives. The aim is to supply readily available energy to avoid decompensation. If the ER is not tolerated, or the child’s condition deteriorates, then urgent admission to the local hospital should be arranged for an intravenous 10% dextrose infusion. When the child is well, they should return to their usual feeds.
The family have been instructed on maximum ‘safe fasting times’ when well and preparation and use of the Emergency Regimen (ER), details of which are enclosed. Oral Rehydration Solutions (ORS) do not contain sufficient energy to avoid decompensation, and therefore require fortifying with glucose polymer. The recipe for this is contained in the ER. The Dietary Management Guidelines for MCADD are available at www.bimdg.org.uk/mcadd.asp.
The long-term prognosis for MCADD is very good once the diagnosis is known as treatment is straightforward. The condition is inherited in an autosomal recessive fashion, with a 1 in 4 risk of recurrence in each pregnancy.
If you have any questions, please do not hesitate to contact the metabolic dietetic team on [contact number/details].
With kind regards
Newborn Blood Spot
Content last updated June 2015. Format last updated April 2015
Public Health England is responsible for the NHS Screening Programmes