title: Dysphagia in Duchenne muscular dystrophy: practical recommendations to guide management.
abstract: Duchenne muscular dystrophy (DMD) is a rapidly progressive neuromuscular disorder causing weakness of the skeletal, respiratory, cardiac and oropharyngeal muscles with up to one third of young men reporting difficulty swallowing (dysphagia). Recent studies on dysphagia in DMD clarify the pathophysiology of swallowing disorders and offer new tools for its assessment but little guidance is available for its management. This paper aims to provide a step-by-step algorithm to facilitate clinical decisions regarding dysphagia management in this patient population.
  
  
  
  This algorithm is based on 30 years of clinical experience with DMD in a specialised Centre for Neuromuscular Disorders (Inkendaal Rehabilitation Hospital, Belgium) and is supported by literature where available.
  
  
  
  Dysphagia can worsen the condition of ageing patients with DMD. Apart from the difficulties of chewing and oral fragmentation of the food bolus, dysphagia is rather a consequence of an impairment in the pharyngeal phase of swallowing. By contrast with central neurologic disorders, dysphagia in DMD accompanies solid rather than liquid intake. Symptoms of dysphagia may not be clinically evident; however laryngeal food penetration, accumulation of food residue in the pharynx and/or true laryngeal food aspiration may occur. The prevalence of these issues in DMD is likely underestimated.
  
  
  
  There is little guidance available for clinicians to manage dysphagia and improve feeding for young men with DMD. This report aims to provide a clinical algorithm to facilitate the diagnosis of dysphagia, to identify the symptoms and to propose practical recommendations to treat dysphagia in the adult DMD population. Implications for Rehabilitation Little guidance is available for the management of dysphagia in Duchenne dystrophy. Food can penetrate the vestibule, accumulate as residue or cause aspiration. We propose recommendations and an algorithm to guide management of dysphagia. Penetration/residue accumulation: prohibit solid food and promote intake of fluids. Aspiration: if cough augmentation techniques are ineffective, consider tracheostomy.
authors.affiliation: a Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre and Centre for Home Mechanical Ventilation, Vrije Universiteit Brussel-Inkendaal Rehabilitation Hospital , Vlezenbeek , Brussels , Belgium ;
authors.forename: Michel
authors.initials: M
authors.lastname: Toussaint
authors.affiliation: b Department Nutrition and Dietetics , Monash University , Melbourne , Australia ;|c Murdoch Childrens Research Institute , Melbourne , Australia.
authors.forename: Zoe
authors.initials: Z
authors.lastname: Davidson
authors.affiliation: a Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre and Centre for Home Mechanical Ventilation, Vrije Universiteit Brussel-Inkendaal Rehabilitation Hospital , Vlezenbeek , Brussels , Belgium ;
authors.forename: Veronique
authors.initials: V
authors.lastname: Bouvoie
authors.affiliation: a Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre and Centre for Home Mechanical Ventilation, Vrije Universiteit Brussel-Inkendaal Rehabilitation Hospital , Vlezenbeek , Brussels , Belgium ;
authors.forename: Nathalie
authors.initials: N
authors.lastname: Evenepoel
authors.affiliation: a Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre and Centre for Home Mechanical Ventilation, Vrije Universiteit Brussel-Inkendaal Rehabilitation Hospital , Vlezenbeek , Brussels , Belgium ;
authors.forename: Jurn
authors.initials: J
authors.lastname: Haan
authors.affiliation: a Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre and Centre for Home Mechanical Ventilation, Vrije Universiteit Brussel-Inkendaal Rehabilitation Hospital , Vlezenbeek , Brussels , Belgium ;
authors.forename: Philippe
authors.initials: P
authors.lastname: Soudon
mesh_terms: D000465:Algorithms; D001530:Belgium; D001784:Blood Gas Analysis; D003679:Deglutition; D003680:Deglutition Disorders / Q000175:diagnosis* / Q000209:etiology / Q000534:rehabilitation*; D019468:Disease Management; D006801:Humans; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000503:physiopathology*; D017410:Practice Guidelines as Topic
keywords: Duchenne; dysphagia; swallowing