Care for the GI System & Nutrition

The information on this page represents the recommended standard of care for Duchenne muscular dystrophy. Most of the care recommendations also apply to Becker muscular dystrophy, but at older ages. Most, but not all, people with Duchenne are males—but the care recommendations apply to both males and females with Duchenne.

If you don’t understand any of the medical terms and concepts, ask your healthcare providers. Take notes and ask questions during your clinical visits.

Diet, Nutrition & Swallowing Facts to Remember:

  1. Height and weight should be checked at each visit.
  2. Registered Dietitian Nutritionists (RDN) are essential members of your healthcare team and should be seen at every visit in order to help promote good nutritional status (BMI or weight for age) and prevent overweight/obesity and underweight/malnutrition.
  3. It is important to have a well-balanced diet, especially one that includes the right amount of calcium, vitamin D, and other nutrients.
  4. People with Duchenne should be evaluated if they have signs of swallowing problems (dysphagia).
  5. Insertion of a permanent feeding (gastrostomy) tube is important for maintaining weight in adolescents and childhood. It should be discussed during routine care assessments if underweight and malnutrition are suspected.

Nutritional Evaluation

It is important to have a nutritional assessment and see a Registered Dietitian Nutritionist (RDN) at every visit. People with Duchenne should eat a healthy, well-balanced diet and have regular visits with a RDN who can:

  • Check weight
  • Make sure your diet includes the right amount of calories, protein, nutrients, fiber, and fluids
  • Suggest supplement use for any deficiencies
  • Address any problems such as too much or too little weight gain, dehydration, swallowing, or digestion in general

Weight and Body Mass Index Measurements

Weight is measured in pounds/kilograms and body mass index (BMI). BMI is a calculation of fat related to height. BMI calculations are challenging in Duchenne, due to the replacement of lean muscle mass with fat and scar tissue. People with Duchenne may have a normal BMI, however, the measurements may not be  accurate in terms of body composition or of lean body mass or fat.

Diet Considerations

Maintaining a Healthy Weight

Both undernutrition and overnutrition can be issues in Duchenne. Overnutrition, and subsequent obesity, tends to be a problem earlier (late childhood through mid-teen years). Working to prevent obesity before it is a problem is much easier than restricting calories later.

Calorie requirements can be calculated using resting energy expenditure (REE). REE is the least amount of energy needed while at rest. The recommended formula in the Care Considerations is the Schofield weight equation. An online resource for this calculation can be found here.

Choose MyPlate models the amounts of each of the five food groups that should be included in a healthy diet. This website discusses recommendations for healthy eating. Safe physical activity, prescribed by a physical therapist (PT) is also important to maintain a healthy weight.

For more tips on managing and preventing weight gain, please see these resources:

Hydration (drinking fluids)

Hydration is extremely important in Duchenne. Adequate hydration will help to prevent constipation, kidney dysfunction/stones, and is generally good for overall health. Fluid requirements can be calculated two ways:

Using the Holliday-Segar method

Patient WeightDosage
patients weighting 1 – 10 kg100 mL fluid/kg
patients weighing 11-20 kg1000 mL fluid + 50 mL/kg for each kg over 10 kg
patients weighing >20 kg1500 mL fluid + 20 mL/kg for each kg over 20 kg

Based on age, fluid requirements are:

AgeMetric MeasurementEnglish Measurment
4 – 8 years1.2L5 cups/day
9 – 13 years1.8L8 cups/day
14 – 18 years2.6L11 cups/day
19+ years3.0L13 cups/day

Protein

Protein is an important energy source and component of diet. Protein recommendations are as follows:

AgeRecommendation
4 - 13 years.95 gm of protein/kg of body weight
14 - 18 years .85 gm of protein/kg of body weight
19 years +.80 gm of protein/kg of body weight

Calcium

Calcium is important for building strong bones and teeth, as well as a host of other functions.  The recommended daily doses are:

AgeRecommendation
4 - 8 years1000 mg/day
9 - 18 years1300 mg/day
19+ years1000 mg/day

Dietary sources of calcium are absorbed more effectively and efficiently than taking calcium supplements. Foods rich in calcium include: dairy products (milk, yogurt, cheese, etc.), leafy green veggies (kale, broccoli, spinach, etc.), canned fist (salmon and sardines), and calcium fortified foods (cereals, orange juice, etc.). Be sure to work with the your RDN  at your neuromuscular center to make sure that your diet includes adequate amounts of calcium. If your diet is found to be deficient in calcium, supplements may be encouraged.

Vitamin D

Vitamin D works with calcium to protect bones and teeth. The recommended dose of vitamin D is 600 IU/day for people of all ages (after infancy).  Many foods are rich in vitamin D (dairy products, fortified foods, canned fish), but the best source is the sun. Getting outside everyday (if possible) will help to improve your vitamin D levels.

A serum 25-OH vitamin D level (a blood test that shows the level of vitamin D in your blood) should be drawn each year after steroids are started to make sure that vitamin D levels are appropriate.  If levels are low, it is important to take supplements to boost your levels. Keeping your vitamin D levels within the normal range will help prevent fractures.

Common Gastrointestinal (GI) Issues

Duchenne affects your muscles — including those used to chew, swallow, and breathe. There are several gastrointestinal (GI) and nutritional issues people with Duchenne may have.

Constipation

Constipation is an extremely common problem in Duchenne, and becomes more common with age. Constipation is defined as having fewer than 1 bowel movement every 2 days (48 hours). Constipation can result for many reasons, including inadequate fluid intake, weak abdominal wall muscles, and dysfunction of the smooth muscles in the colon. It is important that you eat a healthy, well-balanced diet with adequate nutrients, fiber, and fluids to prevent constipation.

Constipation is a very underreported GI problem in Duchenne, but needs to be discussed. Chronic constipation is not only uncomfortable for the person experiencing it, it can also lead to other issues such as difficulty breathing. Treatment for constipation varies by the individual, and will depend on the symptoms experienced. A variety of medications, including stimulants, oral laxatives or stool softeners, may be helpful. If you are having issues with constipation, or are not having a formed stool at least once every 48 hours, speak with your primary care or neuromuscular team.

Metabolic Acidosis

A recent, life threatening, finding of patients who are treated for chronic constipation is the phenomenon of metabolic acidosis. Metabolic acidosis results from a loss of bicarbonate in the gut, which can be caused by diarrhea or laxative therapy. Dehydration and not eating enough can also contribute. The symptoms of metabolic acidosis include reduced food and fluid intake, abdominal pain, and abdominal distension. This is a preventable condition and monitoring is important.

Gastroesophageal reflux (GERD)

Gastroesophageal reflux describes the normal occurrence of stomach fluid coming back up into the esophagus, also known as “heartburn.” When reflux causes injury to the esophagus or damage to the lungs, this is termed gastroesophageal reflux disease, or GERD. GERD is a common issue in people with Duchenne who take steroids (corticosteroids). These symptoms may occur in younger patients who are taking steroids. GERD may also occur in older patients who have smooth muscle dysfunction and delayed gastric emptying (moving stomach contents into the bowel), regardless of whether they are taking steroids.

You can help control GERD by making changes to your diet and/or using medicines (usually proton pump inhibitors, or PPIs) that help reduce or block stomach acid. If you are having these symptoms, discuss management of reflux with your neuromuscular team.

Gastroparesis

Gastroparesis, or delayed gastric emptying, can also occur in the later stages of Duchenne. This can cause abdominal pain after eating, nausea, vomiting, loss of appetite, and feeling full quickly.

Problems chewing, swallowing, breathing

It is important for people with Duchenne to eat a healthy, well-balanced diet and have regular checkups. Weakness of the muscles responsible for chewing, swallowing, and breathing may occur as people with Duchenne grow older, making it more difficult for them to take in fluid and nutrition. As the jaw weakens, it becomes tiring and difficult to chew enough food to maintain the calories needed for your body to function. If you find that your mealtimes are taking much longer than they have in the past, or that you are having difficulty swallowing (dysphagia), talk to your neuromuscular team about scheduling an appointment with a speech language pathologist to evaluate your swallowing, and for tips and assistance to aid with eating.

Gaining too much weight

Weight management can be very challenging, especially in young children. It can be difficult for parents to help their child to feel included while also monitoring their diet. Fortunately, your registered dietitian (RDN) can help you.

A review of daily dietary intake and weight management guidance should be offered at or before the start of steroids. A RDN can evaluate your baseline eating habits and can help you to make healthy, sometimes alternative, choices. The US Department of Agriculture has developed a website to help families make healthy eating choices. ChooseMyPlate.gov helps to illustrate the five food groups needed in a balanced diet, as well as recommended portions of each of those food groups.

In order to lose weight, there needs to be a decrease in calories and increase in safe physical activity. Your RDN will evaluate your daily dietary needs and habits, and will guide you carefully toward nutrition/diet therapy. A physical therapist should always monitor physical activity to ensure it is safe for you.

Losing too much weight/undernutrition

As people with Duchenne grow older, many begin to lose weight. This is principally because as chewing and swallowing muscles weaken, you may be unable to take in enough calories by mouth. If you notice that you are losing weight and swallowing makes it difficult to consume enough calories, it may be time to consider using a permanent feeding tube known as a gastrostomy or g-tube. A feeding tube will allow you to still eat for pleasure, but will give you another means by which to maintain your calories and nutrition. If weight loss and maintaining your weight are becoming an issue, the RDN should evaluate and guide carefully in terms of nutrition/diet therapy.

Osteoporosis and fractures

People living with Duchenne at all ages may have weak bones, especially if they are taking daily steroids. Steroids cause bone to have a lower mineral density (osteoporosis) and are at increased risk of fractures (broken bones).

When you start steroids, it is also important to check blood for baseline vitamin D and/or calcium levels to make sure they are high enough to optimize bone health. Your serum 25-OH vitamin D level should be checked yearly. If your level is less than 30 ng/ml, your doctor may recommend starting vitamin D supplements. If calcium intake is less than the recommended daily allowance for age, calcium supplementation may be recommended as well. It is a good idea to include food with good sources of calcium and vitamin D in your diet as well.

Dental Issues

It is important that your teeth, gums, and mouth are healthy so that you can eat. Be sure to brush your teeth at least once a day, floss daily, and see your dentist at least every six months for regular check ups.