NUTRITIONAL MANAGEMENT

Nutrition may be a significant problem in severe NMDs, in which obesity tends to follow shortly after the loss of functional ambulation. Obesity is common in patients with NMDs, particularly DMD, in which a prevalence of 54% has been reported. Weight control has its primary rationale in ease of care, particularly ease of transfers and skin care.

Conversely, malnutrition may mark the advanced stages of DMD, ALS, and SMA. As previously noted, if a severe respiratory compromise is present, the increased work of breathing may drastically increase caloric needs. The situation is complicated by the fact that this is often a time when the patient loses the ability to self-feed.

A nutritionist should assess caloric requirements and construct proper dietary requirements for the patient. This should be routinely done for all patients with NMDs with a forced vital capacity of less than 50% predicted on pulmonary function testing. PEG tube placement may facilitate nutrition because it eases intake of large amounts of calories and fluids.

Patients should be reassured that they may still eat food orally for enjoyment, provided they have intact swallowing function. Another complicating factor in patients with DMD is gastroparesis, which may make feeding more difficult.