High-protein Diet for Pompe Disease

Emily Malcolm, PhD avatar

by Emily Malcolm, PhD |

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high-protein diet for Pompe disease

Pompe disease is a rare disease in which glycogen, a complex sugar molecule, builds up in cells and tissues. This buildup interferes with function, especially in muscles.

Changes to diet and feeding methods may help alleviate or slow the progression of some disease symptoms. One dietary change that may help some patients is switching to a high-fat, high-protein, low-carbohydrate diet (often called a high-protein diet). However, before starting this diet or making any dietary changes, always consult your doctor and a registered dietitian.

What is a high-protein diet?

A high-protein diet for Pompe disease consists of 25%–30% protein, 30%–35% carbohydrates, and 40%–45% fats. Protein from meat, fish, and eggs is better, in part because these foods are rich in alanine, an amino acid that plays a role in carbohydrate metabolism. Fats should include both omega-3 fatty acids (found in fish and supplements), and omega-6 fatty acids (found in olive oil, dried fruit, and cereals).

Carbohydrate consumption should be spread throughout the day and be in the form of whole grains, wholemeal pasta, and legumes, and not come from processed sugar found in soda, candy, and sweets. Fruits are a good option because they also contain fiber, which can help with constipation, a common symptom in Pompe disease.

How might the diet help patients?

The aim of a high-protein diet is to reduce the amount of glycogen made by cells. By reducing the amount of carbohydrate intake, cells have less sugar available to store in the form of glycogen.

Is there evidence that the diet works?

There is some evidence that a high-protein diet, in combination with exercise, can reduce the symptoms of Pompe disease. In a small study, 13 patients with Pompe disease were divided into three groups: a control group, a group that had a structured exercise program, and a group that received a high-protein diet in addition to the exercise program. At the end of the study, which lasted 26 weeks, there was no change in the control or exercise group. However, the exercise-plus-diet group saw improvement in their quality of life and lung function.

Other studies have shown that not everyone responds to a high-protein diet. More studies are necessary to draw a conclusion.

A small clinical trial (NCT02363153) at the University of Florida is recruiting 26 participants, 15 to 55 years of age, with Pompe disease to test the effects of diet and exercise on disease progression. Patients will receive an individualized diet provided by a dietitian and an exercise plan created by a physical therapist. Researchers will evaluate the quality of life and lung function of patients over 16 weeks. Each participant is expected to wear an activity tracker at all times during this 16-week period, and will be asked to enter data manually, such as daily food intake and weight, into a phone app.


Last updated: July 28, 2020


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