Dietary Therapy

A dietary regimen of food and supplementation combined with delivery techniques may be used to provide adequate calories and quality protein to support growth, maintain weight, provide energy and minimize the protein deficiency due to muscle breakdown.

Infant Onset

Tube feeding may be required for infants who are not gaining weight or for children and adults who are severely underweight or have serious swallowing or breathing problems. Liquid food is carried directly to the stomach or intestines.

This can be done through a nasal tube that goes through the nose, throat, and esophagus or by gastrostomy (gas-TROSS-toe-me) tube, or G-tube, that is surgically attached to the stomach wall. Another option is a gastrojejunostomy (GAS-tro-jee-ju-NOSS-toe-me) tube, or G-J tube, that is attached to the small intestine.

Managing Diet

Some Pompe patients may find eating problems make it hard to maintain a healthy weight. A registered dietitian may help plan a customized diet for individual patients. A dietitian will consider a patient's age, height, health status, and other factors when determining the optimal caloric intake for that patient.

To help prevent muscle wasting in patients with late-onset Pompe disease, doctors may prescribe a high-protein, low-carbohydrate diet. This type of dietary therapy has not been shown to provide consistent results. Some children and adults have had improvements in lung function and muscle strength after following a carefully supervised high-protein diet. For others, the benefits have been modest at best.

Combining a high-protein diet with daily exercise (also carefully supervised) may produce better results. Some studies have suggested that adding alanine, an amino acid (one of the building blocks of protein), to the diet may also be helpful.

For example, one 27-year-old woman diagnosed with late-onset Pompe disease always had trouble gaining weight. But after starting a special diet and exercise program supervised by a metabolic specialist, her health improved dramatically. The extra nutrition she gets from overnight tube feeding helps keep the weight on and gives her more energy to get through the day.

Always consult your health care provider before changing your diet or beginning an exercise program.

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