Nutritional and Gastrointestinal Management of the Patient With Duchenne Muscular Dystrophy

Abstract

Advances in treatment and multidisciplinary management have resulted in improved survival of individuals with Duchenne muscular dystrophy (DMD). Updated DMD treatment recommendations as found in the 2018 DMD Care Considerations are aimed to assist multidisciplinary care teams in providing standardized care to their patients, including attention to nutritional and gastrointestinal health. Challenges remain for care teams in accurately estimating height and nutritional status for individuals with DMD. It can be difficult for patients to maintain a healthy weight. Risk factors for obesity include glucocorticoid therapy and loss of ambulation. In contrast, in the later stages of the disease, swallowing dysfunction can lead to poor nutrition and consideration for gastrostomy tube placement. Constipation is highly prevalent, underrecognized, and undertreated in DMD. With this article, we address the assessment and management of gastrointestinal and nutritional issues, as well as clinical controversies.

Targeting gut dysbiosis against inflammation and impaired autophagy in Duchenne muscular dystrophy

“…In this regard, it has been estimated that chronic persistent inflammation, forced sedentariness, and long‐term use of anti‐inflammatory steroid drugs cause overweight and obesity in more than half of the children with DMD. On the contrary, adolescents and adults with DMD frequently experience underweight due to swallowing dysfunction, lack of dietary fiber, and reduced intestinal motility (Kraus et al , 2016; Brumbaugh et al , 2018). Similar pathological features were found also in mdx mice, a widely used experimental model of DMD (Mulè et al , 2010; Radley‐Crabb et al , 2011).…”

The Role of Taurine in Skeletal Muscle Functioning and Its Potential as a Supportive Treatment for Duchenne Muscular Dystrophy

“…Interestingly, no effect of taurine on body weight was observed in WT animals. Obesity is commonly observed in DMD patients, especially in glucocorticoid-treated patients [7,115,116]. Therefore, it remains unclear if taurine-induced decreased body weight would be disadvantageous in these patients.…”

“…Approximately 50-65% of DMD patients are using vitamins or nutritional supplements [115,116], which indicates that the quest for supportive treatment, including but not limited to taurine, in DMD is relevant. A detailed overview of nutritional supplements that are under investigation for the treatment of DMD is provided in the review of Boccanegra et al [118].…”

Transition from Childhood to Adulthood in Patients with Duchenne Muscular Dystrophy

“…As dysphagia progresses, calorie intake decreases and unintentional, progressive weight loss is typical. Malnutrition can also occur when patients lose the ability to self-feed, for example, when progressive weakness in the upper limbs or postoperative complications after spinal fusion surgery occur [ 49 ].…”