Dangers of Fasting

From Consensus Statement for Standard of Care in Spinal Muscular Atrophy
Spinal muscular atrophy patients are particularly vulnerable to
catabolic and fasting states. Patients with severe muscle wasting from
any disorder, including spinal muscular atrophy, are more likely to
develop hypoglycemia in the setting of fasting. A number of case
series and individual case reports have documented secondary
mitochondrial dysfunction and abnormalities of mitochondrial fatty
acid oxidation in spinal muscular atrophy patients. Significant
abnormalities are most likely in nonsitters and sitters, increasing
their vulnerability for metabolic decompensation in the setting of a
catabolic state. Thus, it is necessary to avoid prolonged fasting,
particularly in the setting of acute illness, in all spinal muscular
atrophy patients. Nutritional intake should be optimized to meet full
caloric needs within 4 to 6 hours after an admission for acute
illness, via enteral feeding, parenteral feeding, or a combined
approach as necessary. Prompt postoperative caloric supplementation is
recommended to avoid muscle catabolism, particularly in a child with
reduced fat store. If enteral intake is not imminent, then intravenous
caloric feeding should be considered.