Diet is a concern for people with Pompe disease because muscle weakness can make it difficult for patients to chew and swallow (or nurse if they are infants). This can make getting adequate nutrition a challenge.
There is some evidence suggesting that a modified diet and exercise program may slow the progression of Pompe disease.
Ways to improve nutrition
Muscle weakness affects the ability of Pompe disease patients to eat and digest food comfortably. They may have difficulty chewing and swallowing their food. Infants with the disease may have difficulty suckling and swallowing. If the body is not getting enough food, the patient’s overall health will suffer.
In addition, weakness in the muscles that push food toward the stomach can cause food and stomach acid to move back up the esophagus, leading to heartburn and acid reflux.
Muscles in the chest and abdomen also are necessary for bowel movement. If these muscles are weakened, constipation may result.
There are a number of strategies to combat these problems. For example, eating several small meals throughout the day can be less taxing and help digestion. Chopping solid food into smaller pieces or mashing it and moistening dry foods (with sauce for instance) can make it easier to swallow. Liquids also may be difficult to swallow for Pompe disease patients. These may be thickened with cornstarch, rice cereal, or thickening powders to make them easier to swallow.
Sitting upright for an hour or two after eating or drinking can help avoid heartburn and acid reflux, as can eating many small meals. Eating plenty of fiber and staying well-hydrated can help prevent constipation.
If patients are not able to take enough food by mouth, they may require a feeding tube in order to get adequate nutrition. Feeding tubes get food directly into the stomach, without the patient having to swallow it. They may be inserted through the nose and down the throat into the stomach (nasal tube), or through a surgical opening in the stomach wall (gastronomy or “G tube”).
Working with a professional dietician can help ensure that Pompe disease patients consume well-balanced nutrition that supports their overall health.
High-protein diets and supplements
Pompe disease patients have a loss of muscle tissue and there is some evidence that a high-protein diet, in combination with appropriate exercise, can slow this loss. The recommended diet is approximately 25-30 percent proteins, 30-35 percent carbohydrates, and 35-40 percent fat. Carbohydrate consumption should be spaced out in small, frequent portions throughout the day to reduce the build-up of glycogen while avoiding low blood sugar.
Proteins from meat, fish, egg, and dairy products are rich in an important amino acid (building blocks of proteins) called alanine. Alanine is important in glucose metabolism (or the body to break down glucose to produce energy) and some researchers recommend that Pompe disease patients take an alanine supplement.
It also is recommended that patients work with a physiotherapist to perform appropriate exercises to maintain muscle and help strengthen specific muscles needed for eating. Bioimpedentiometry (BIA) may be used to monitor patients’ muscle mass.
Clinical trials on the effect of diet in Pompe disease
A small interventional clinical trial (NCT02363153) to investigate the effects of diet and exercise on the progression of Pompe disease is recruiting patients, ages 15 to 40, in Florida. Participants will be given an individualized 16-week exercise and diet plan and wear an activity tracker. They will be asked to come to the University of Florida four times during the study for exercise testing, to complete quality-of-life questionnaires, respiratory function testing, and blood tests. The trial is expected to be completed in December 2018.
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