High-protein Diet for Pompe Disease

High-protein Diet for Pompe Disease
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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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Pompe Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

Emily holds a Ph.D. in Biochemistry from the University of Iowa and is currently a postdoctoral scholar at the University of Wisconsin-Madison. She graduated with a Masters in Chemistry from the Georgia Institute of Technology and holds a Bachelors in Biology and Chemistry from the University of Central Arkansas. Emily is passionate about science communication, and, in her free time, writes and illustrates children’s stories.
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Özge has a MSc. in Molecular Genetics from the University of Leicester and a PhD in Developmental Biology from Queen Mary University of London. She worked as a Post-doctoral Research Associate at the University of Leicester for six years in the field of Behavioural Neurology before moving into science communication. She worked as the Research Communication Officer at a London based charity for almost two years.
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Emily holds a Ph.D. in Biochemistry from the University of Iowa and is currently a postdoctoral scholar at the University of Wisconsin-Madison. She graduated with a Masters in Chemistry from the Georgia Institute of Technology and holds a Bachelors in Biology and Chemistry from the University of Central Arkansas. Emily is passionate about science communication, and, in her free time, writes and illustrates children’s stories.
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