Frequent feeding difficulties in type II SMA and effective gastrostomy

SMA is characterized primarily by loss of muscle strength. Bulbar muscles and muscles involved in gastrointestinal transit are also affected, but they have not been as studied as skeletal muscles. Difficulties in eating (swallowing, chewing, weight gain or loss, etc.) have frequently been reported in type I SMA and sporadically in type III SMA. In Type II SMA, these problems have not been studied much. 

The results of a study of feeding difficulties of 146 adolescents and adults with type II SMA, retrospectively in a British center and prospectively (by questionnaire) in several Italian centers, showed that: 

  • 60% have difficulties: swallowing disorders, aspiration, chewing difficulties, inhalation pneumonia, weight loss, malnutrition and prolonged meals, 
  • the median age of onset of these difficulties was 6.5 years,
  • 60% experienced periods of underweight,
  • 25% experienced severe weight loss,
  • 32% are under gastrostomy, well tolerated by 96% of patients, and led to a decrease in the frequency of respiratory infections in 80% of cases and an improvement in nutritional status in 84% of cases.

 

Feeding difficulties in children and adolescents with spinal muscular atrophy type 2. R I Wadman, R De Amicis, C Brusa et al. Neuromuscul Disord. 2020 (Dec).