How Diets and Diet Behave within the Therapy of Pompe Illness


Pompe disease is a rare metabolic disorder. Alpha-glucosidase is marked by lysosomal deficiency. The body needs this enzyme to break down stored glucose, which is called glycogen. When your body can’t break down, glycogen builds up in your muscles. This elevation makes it difficult for the muscles to function properly.

Symptoms of Pompe disease can appear in childhood or adulthood. Acid maltase deficiency or glycogenosis type 2 can also be called. The rate of progression varies from person to person.

The main symptom is profound muscle weakness. This vulnerability can be so severe that it impedes mobility. In some cases, people with Pompe disease may have difficulty sitting without help. The condition can also affect the respiratory system and the muscles used for chewing and swallowing.

There is no cure for Pompe disease, but there are several treatments that can help with symptoms and overall quality of life.

Treatment consists of enzyme replacement therapy (ERT) and supportive therapies. Ensuring proper nutrition is also essential, especially for the development of children.

Read some key information on Pompe disease and nutrition.

Making sure you get the right nutrition is key. But diet alone is not enough. It should be part of a comprehensive treatment plan, including ERT.

They are all different. A diet that works for one person with Pompe disease may not have helped another. Your doctor can assess your nutritional needs and deficiencies and make specific recommendations:

  • diet and dietary supplements
  • physical exercise and physical therapy
  • other supportive therapies

Consult your doctor before making any drastic changes to your diet or adding new dietary supplements.

Studies 2020 and 2021 suggests that many adults with Pompe disease may benefit from a low-protein, low-carbohydrate diet combined with aerobic exercise and ERT.

A high protein diet may also be beneficial for children, but it may not be suitable for everyone with Pompe disease.

One reason to eat more protein is that foods high in protein can provide amino acids. When proteins are broken down in digestion, the byproduct is amino acids, which can slow down muscle breakdown.

A high protein diet can help reduce the amount of carbohydrates a person consumes. Less carbohydrate means that cells have less sugar to store as glycogen.

Generally, 10 to 35 percent of calories should come from protein. Everything at the top of this range is considered a high protein diet. Of course, protein needs can vary depending on factors such as age, gender, and level of physical activity.

You can get protein from plant and animal sources, such as:

  • fish and other seafood
  • birds
  • unprocessed lean meat
  • eggs
  • seeds and nuts
  • lentils and beans
  • tofu
  • dairy products

Add protein between meals

Although you should always talk to your doctor before making any dietary changes, here are some easy ways to add protein between meals:

  • yogurt
  • milk, beaten
  • cheese
  • almonds, peanuts, pineapples, pecans
  • sunflower seeds, pumpkin seeds, flax seeds
  • hard-boiled eggs or demons
  • peanut butter
  • eating beans, hummus

You can also add some protein shakes, powders or supplements. Again, check with your doctor before trying anything new.

The role of carbohydrates

You need carbs in your diet, but not all carbs are the same. Carbohydrates are made up of sugar, starch and fiber. You can reduce carbs by avoiding those that are high in calories that are low in nutritional value. It includes foods made with highly processed white flour and added sugars, such as chips and cookies.

Instead, choose healthier carbohydrates in foods such as:

  • like whole grains, cereals, whole grain breads and pasta
  • whole fruit
  • beans

2019 research Lack of exercise and poor nutrition can accelerate the progression of the disease in people with muscle disease. And in terms of balance, protein, nutrition and exercise are independent and interactive factors.

According to A 2020 studya high-protein diet combined with moderate-intensity aerobic exercise improved quality of life in people with late-onset Pompe disease.

Proper exercise with Pompe disease can be difficult. Consider talking to your doctor about physical therapy. They may order physical therapy from the therapist with specific instructions.

Some children and adults with Pompe disease have trouble chewing and swallowing. This can increase the chances of food being inhaled into the lungs.

And low calorie intake can become a problem, especially for children and toddlers who are still developing. It can be said that there is a lack of protein and important nutrients that help maintain muscle mass.

The same 2019 research They showed that inadequate food consumption can increase physical activity and accelerate the progression of the disease. You may be more vulnerable to other metabolic stresses.

Here are some tips on how to easily chew and swallow food:

  • Cut solid foods into manageable parts.
  • Grind the food, or mix it with the sauce or gravy.
  • Take small bites, and chew slowly and thoroughly.
  • Drink plenty of fluids.
  • Replace meals with nutritional drinks that add vitamins and minerals.

In some cases, the only way to get enough food is through a feeding tube. Some feeding tubes enter through the nose, down the esophagus, and into the stomach. Some go directly into the stomach through a surgical opening in the abdomen.

Your doctor will tell you if this is necessary.

Your healthcare team may recommend working with a speech therapist who can help improve chewing and swallowing. You may also want to consider working with a dietitian who knows Pompe disease.

Pompe disease is a genetic disorder that affects the muscles.

Because protein is essential for muscle health, some people with Pompe disease may benefit from a high-protein diet. If you or your child has Pompe disease, talk to your doctor to ensure proper nutrition.

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