Nutrition & Weight

Pompe disease can weaken muscles used for biting, chewing, sucking, and swallowing. This can make it hard for patients of all ages to eat comfortably and get the nutrition their bodies require. Breathing problems may also make people too tired to eat. As a result, many people with Pompe disease have trouble gaining weight, keeping it on, and getting proper nutrition. Weak swallowing muscles also pose a risk of accidentally inhaling food or liquid into the lungs.

Dietary therapy provides several strategies to address these problems:

  • A registered dietician can plan well-balanced meals to provide enough calories and nutrients
  • Changing the size, texture, or thickness of foods can make them easier to eat, and minimize the risk of inhaling them
  • Special exercises may help strengthen eating-related muscles

In more serious cases, such as infants who are too weak to eat at all on their own, older patients who are severely underweight, or those whose breathing problems interfere with eating, tube feeding may be necessary. A tube is inserted—either through the nose and down the throat, or surgically into the stomach (called a gastrostomy, or g-tube)—in order to provide liquid food directly to the stomach or intestines.

The Benefits of Exercise

Many patients with Pompe disease may benefit from exercise. Benefits range from stronger muscles and less stiffness to increased energy and improved mental health. Before beginning any new exercise program, it is important that it first be discussed with the patient's doctor and other care providers. Each patient's plan must be carefully designed to account for specific needs, physical abilities, and limitations (such as any breathing difficulties).

References

  1. Kishnani PS, Steiner RD, Bali D et al. Pompe disease diagnosis and management guideline. Genet Med 2006 8:267-88.

Genzyme Support Services

Learn how Genzyme can help people affected by Pompe disease with medical information, advocacy, treatment support, and more.

Learn More

Organizations & Websites

Get lists of online resources and worldwide organizations and associations that offer information, support, and more for people living with Pompe disease.

Learn More