Prevalence and Risk Factors for Feeding and Swallowing Difficulties in Spinal Muscular Atrophy Types II and III

• Published in: The Journal of pediatrics Vol. 160; no. 3; pp. 447 - 451.e1
• Main Authors: Chen, Yen-Shan, MD, Shih, Hsiang-Hung, MD, Chen, Tai-Heng, MD, Kuo, Chang-Hung, MD, Jong, Yuh-Jyh, MD, DMSci
• Format: Journal Article
• Language: English
• Published: Maryland Heights, MO Elsevier Inc 01.03.2012; Mosby, Inc; Elsevier
• Subjects: Adolescent; Adult; Biological and medical sciences; Child; Child, Preschool; deglutition; Deglutition Disorders - etiology; Eating; Epidemiology; Female; General aspects; Humans; lung function; Male; mastication; Medical sciences; Middle Aged; Miscellaneous; mouth; muscular atrophy; patients; Pediatrics; pneumonia; Pneumonia, Aspiration - etiology; Public health. Hygiene; Public health. Hygiene-occupational medicine; questionnaires; regression analysis; Risk Factors; Spinal Muscular Atrophies of Childhood - complications; Spinal Muscular Atrophies of Childhood - physiopathology; surveys; underweight; Young Adult; SMA; Weight-for-age; SMN2; W/A; SMN1; Survival motor neuron 2; Survival motor neuron 1; Spinal muscular atrophy; Pediatrics; Nervous system diseases; Neuromuscular diseases; Prevalence; Spinal amyotrophy; Swallowing; Epidemiology; Feeding; Type; Central nervous system disease; Risk factor; Degenerative disease; Spinal cord disease; Public health
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2011.08.016

Objective To identify the prevalence and risk factors of feeding and swallowing problems in patients with type II and type III spinal muscular atrophy (SMA). Study design Cross-sectional data from 108 genetically confirmed patients with SMA (age range, 3-45 years; 60 with type II and 48 with type III) were analyzed. The questionnaire survey included demographic data, current motor function and respiratory status, feeding and swallowing difficulties, and consequences. The risk factors were analyzed via logistic regression. Results The 3 most common feeding and swallowing difficulties in patients with type II and III SMA were choking (30.6%), difficulty conveying food to the mouth (20.4%), and difficulty chewing (20.4%). Current motor function status was an independent risk factor for feeding and swallowing difficulties (sitters vs walkers: OR, 7.59; 95% CI, 1.22-47.46). All 4 nonsitters (ie, patients with type II SMA who had lost their sitting ability) had feeding and swallowing difficulties. Patients with feeding and swallowing difficulties had significantly higher rates of underweight and aspiration pneumonia than those without these problems. Conclusion Patients with type II and III SMA have a high prevalence of risk factors for feeding and swallowing difficulties, suggesting that an individualized treatment plan should depend on current motor function status.