AA Diet Info for SMA

Based on Families Experience

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.

This website explains the accurate amino acid diet for spinal muscular atrophy patients. For over 17 years, this diet has been used successfully in many SMA patients.Those following this diet appropriately are typically healthier and stronger than others similarly affected by SMA. Because this diet hasn't been studied by medical professionals, it is often not properly prescribed. I believe this is a disservice to SMA families striving to access all available care options for their children. This website has been established with the hope that more families can understand how to safely and optimally use this diet. Any information contained here is based on the opinion and experience of many families.