Nutritional status, swallowing disorders, and respiratory prognosis in adult Duchenne muscular dystrophy patients

Abstract

Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with home mechanical ventilation (HMV) and cough assist device, its prevalence and the relationships with respiratory muscle strength and long‐term respiratory prognosis. We reviewed the patients (n = 117, age 18–39 years [median 24]), followed in a reference center, from 2006 to 2015, to obtain clinical baseline, nutritional status, vital capacity (VC), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP). The median body mass index (BMI) was low (15.6 kg/m²). Included patients had severe restrictive respiratory function with a median VC of 10.5% [7–17] of the predicted value. All patients were on HMV. Prevalence of malnutrition, swallowing disorders, and gastrostomy were respectively 62%, 34%, and 11%. BMI and serum albumin level were significantly associated with MIP, MEP, and VC. The 1‐year/5‐years cumulative incidences of respiratory events (pulmonary sepsis and acute respiratory distress) were, respectively, 20.7%/44.5%. Using univariate analysis, predictive factors for respiratory events were swallowing disorders (p = .001), transthyretinemia (p = .034), MIP (p = .039), and MEP (p = .03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR = 4.2, IC 95% 1.31–12.2, p = .01). In conclusion, this study highlights the interrelationships between nutritional intake, swallowing function, airway clearance, and respiratory function in adult DMD. A multidisciplinary approach focusing on these previous factors is essential to optimize DMD patient health.

Congenital or Early Developing Neuromuscular Diseases Affecting Feeding, Swallowing and Speech – A Review of the Literature from January 1998 to August 2021

“…Almost all the clinical studies were observational studies and about one fifth included healthy controls. Standards of care and comprehensive overviews that contain descriptions of feeding, swallowing, saliva control and speech in NMDs were also mentioned for NMD as a group [12][13][14], SMA [29][30][31][32], JMG [41], CM [51], CMD [54], DMD [66,67], PD [87] and DM1 [92].…”

“…The relationship between eating and digestive symptoms and respiratory function has been investigated in patients with DMD who were nonambulatory and required non-invasive, mechanical ventilatory support [65,66]. Eating and digestive symptoms were more closely correlated with respiratory function than with age in patients with advanced DMD [65].…”

Year in review 2021: Neuromuscular diseases

“…In another study, Fayssoil et al 10 examined nutritional status in adult DMD patients. They completed a retrospective chart review of all adult DMD patients followed between 2006 and 2015.…”

Non-Invasive Respiratory Assessment in Duchenne Muscular Dystrophy: From Clinical Research to Outcome Measures

“…Swallowing deficit should be also evaluated in patients with advanced disease, as it represents the principal cause for decreased oral intake and it is closely associated with bulbar muscle function. A significant interaction has been reported between respiratory muscle strength, evaluated by measuring MIP and MEP, and both nutritional parameters [128] and the presence of eating disorders [129]. In addition, malnutrition may affect diaphragm thickness and function [130].…”