Feeding difficulties in children and adolescents with spinal muscular atrophy type 2

Disease course of feeding difficulties in spinal muscular atrophy type 2 is not well documented. Disease-modifying therapies rapidly change the trajectory motor function and survival atrophy, but effects on co-morbidities like bulbar are unknown. We analysed data concerning problems their standard care treatment 146 patients with 2. Data were collected from two separate cohorts: one single-centre retrospective chart review study United Kingdom (London), prospective questionnaire-based multicentre Italy. Cumulatively present 88 (60%) these cohorts. Median age at onset was 6.5years (range 0–16.5 years). Eighty-two showed periods underweight according to adjusted body mass index, thirty-six (25%) malnourishment a significant drop weight curves. Enteral indicated 23 out 72 UK cohort (32%) because loss, oropharyngeal dysphagia or aspiration. Gastrostomy its placement generally tolerated, uncomplicated 96%, never reversed performed without Nissen fundoplication 66% patients. After gastrostomy chest infections improved 80% nutritional status (e.g., Body Mass Index) 84% These results show that common problem Treatment strategies should be tailor-made symptoms needs individual patient.