Duchenne muscular dystrophy (DMD) is a severe, progressive genetic disorder primarily affecting males, characterized by muscle weakness and wasting. While there is no cure for DMD, proper management, including nutritional support, can significantly improve the quality of life and longevity of affected individuals. This article provides a comprehensive overview of nutritional considerations for individuals with DMD, covering various aspects from caloric intake and macronutrient balance to managing common gastrointestinal issues.
Understanding Nutrition in Duchenne Muscular Dystrophy
Nutrition is the act or process of nourishing or being nourished. In the context of DMD, where muscle function is compromised and metabolic needs may be altered, nutrition plays a crucial role in maintaining overall health. Neuromuscular diseases (NMDs) represent a heterogeneous group of acquired or inherited conditions. Nutritional complications are frequent in NMDs, but they are sometimes underestimated. With the prolongation of survival in patients with NMDs, there are several nutritional aspects that are important to consider. Appropriate nutritional care can help to improve the quality of life of DMD patients, and a multidisciplinary team is needed to support nutrition challenges in DMD patients.
Caloric Intake and Weight Management
One of the primary considerations in DMD nutrition is managing caloric intake to prevent both overnutrition and undernutrition. Specialists suggest that daily caloric consumption should be 80% of what individuals without Duchenne muscular dystrophy consume. This recommendation stems from several factors:
- Decreased Physical Activity: As DMD progresses, individuals become less active, leading to reduced energy expenditure.
- Decreased Resting Energy Expenditure (REE): Studies have shown that individuals with DMD often have lower REE compared to their healthy counterparts due to reduced muscle mass. Skeletal muscle metabolism is a major determinant of REE, and it is altered by the severe muscle loss that characterizes NMDs.
- Steroid Use: Corticosteroids, commonly used to manage DMD, can increase appetite and promote weight gain. Chronic treatment with corticosteroids increases the risk of becoming overweight, insulin resistance, and type 2 diabetes mellitus.
Overnutrition: Decreased caloric needs, decreased resting energy expenditure, decreased physical activity, increased appetite due to medication, and lack of caloric restriction can all cause over nutrition in Duchenne. Overnutrition can lead to obesity, insulin resistance, dyslipidemia, hypertension, and obstructive sleep apnea. Complications of being overweight are the acceleration of disease progression due to the exertion of extra force on already weak muscle groups, increased respiratory involvement with worsening pulmonary and cardiac function, and deterioration of skeletal malformations with increased need for orthopaedic surgery. In addition, obesity worsens the ability of parents and caregivers to transfer and assist the patient in daily activities when patients lose their independence.
Undernutrition: Decreased muscle strength is the main cause of hypoalimentation. Dysphagia, gastrointestinal problems (i.e., constipation, delayed gastric emptying), prolonged mealtime, and dependent feeding are all consequences of muscle weakness. Furthermore, the presence of respiratory failure in the late stage of the disease can cause increased energy requirements. Moreover, a variety of swallowing difficulties are reported in patients with NMDs (i.e., facial weakness, reduced mastication, and poor tongue coordination). Undernutrition can deteriorate respiratory function and blunt immunological responses with increased risk of chest infections and a negative impact on quality of life.
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To determine individual caloric needs, tools like the MyPlate.Gov widget can be helpful. Regular monitoring of weight and body mass index (BMI) is also essential, although BMI calculations can be challenging in DMD due to the replacement of lean muscle mass with fat and scar tissue.
Macronutrient Balance
Maintaining a balanced intake of macronutrients - proteins, fats, and carbohydrates - is crucial for individuals with DMD.
Protein
Protein is an important part of any diet, especially in Duchenne, and can help you feel fuller for longer so you don’t need to supplement with unhealthy snacks. You should aim for 1.5 grams of protein per kilogram of body weight. Protein is essential for muscle maintenance and repair. A protein diet is recommended for people with Duchenne muscular dystrophy. The most common way to satisfy your protein requirements is through lean meat, like chicken, grass fed beef, and lamb, and fish. However, you can also get the proper amount through beans, yogurt, and nuts. Examples of high-quality protein sources include:
- Lean meats (chicken breast)
- Fish (tuna, baked teriyaki salmon)
- Eggs
- Dairy products (cottage cheese, milk, yogurt)
- Legumes and nuts
Fats
Healthy fats are found in foods like salmon, avocado, olive oil, and meat. Fats are necessary for energy, hormone production, and overall health. Try to include monounsaturated fats and polyunsaturated fats in your diet with Duchenne instead of saturated fats where possible. Focus on incorporating healthy fats from sources like:
- Avocado
- Olive oil
- Salmon and other fatty fish
- Nuts and seeds
Carbohydrates
Carbs, or carbohydrates, are found in much of what we eat, including starches (pasta, grain, and potatoes), dairy (yogurt, cheese, and milk), fruit (bananas, oranges, and grapes), and veggies (cabbage, carrots, and bell peppers), and processed food (cookies, candy bars, chips). It is ideal to choose foods with lower glycemic indexes so we maintain our energy over the day. So instead of having potato chips for a snack, go for an apple. Carbohydrates provide energy for the body. It is ideal to choose foods with lower glycemic indexes so we maintain our energy over the day. Focus on complex carbohydrates with lower glycemic indexes, such as:
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- Non-starchy vegetables
- Fruits
- Whole grains (oats)
- Legumes
Micronutrients: Vitamins and Minerals
Minerals such as calcium, magnesium, and zinc can activate thousands of enzymatic reactions, assist with vitamin functions, help metabolize carbs, proteins, and fats, and regulate water balance. It’s an important part of nutrition for Duchenne. More than 3,800 minerals have been identified by scientists. Vitamins and minerals are essential for various bodily functions, including bone health, immune function, and energy metabolism. It is recommended to take a daily multivitamin that includes mineral supplements.
Calcium and Vitamin D
Calcium is important for building strong bones and teeth, as well as a host of other functions. 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.). 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. 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. Given the risk of bone fragility associated with DMD and steroid use, adequate intake of calcium and vitamin D is particularly important. Daily calcium needs are based on actual intake. The first step is to collect information on what intake is obtained from food and then determine if it is sufficient. When under the ideal amount, supplements can be added. It is important to determine the actual calcium intake from food and then decide if additional calcium is needed. A body can get TOO much calcium so it is imperative to work with your health care team to determine a plan best tailored to your child’s needs.
Other Essential Minerals
Minerals such as calcium, magnesium, and zinc can activate thousands of enzymatic reactions, assist with vitamin functions, help metabolize carbs, proteins, and fats, and regulate water balance. It’s an important part of nutrition for Duchenne. A daily multivitamin can help ensure adequate intake of essential minerals like magnesium and zinc, which play roles in muscle function and overall health.
Managing Gastrointestinal Issues
Complications of the gastrointestinal (GI) tract are relatively frequent in NMDs. Several gastrointestinal issues are common in DMD, including constipation, gastroesophageal reflux (GERD), and gastroparesis (delayed gastric emptying). Constipation is an extremely common problem in Duchenne, and becomes more common with age.
Constipation
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. Pane et al. reported that 36% of patients with DMD experienced constipation, and this problem was more frequently reported after 18 years (60%…). To avoid Constipation by consuming adequate water and increasing fiber consumption. Adequate water intake helps to eliminate waste from the body, maintain a normal body temperature, lubricate joints, and maintain healthy tissue. The general guideline for the consumption of water in ounces (10 oz. = 1 glass of water) is your weight divided by two. Example: 200 lb / 2 = 100 oz of water daily.
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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). To 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.
Gastroparesis
Gastroparesis, or delayed gastric emptying, can also occur in the later stages of Duchenne. The altered function of gastric smooth muscle cells in NMD patients causes delayed gastric emptying.
Addressing Swallowing Difficulties (Dysphagia)
As DMD progresses, muscle weakness can affect the muscles involved in chewing and swallowing, leading to dysphagia. 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.
Practical Tips for Implementing a DMD Diet
- Start the day with a protein-rich breakfast.
- Increase water intake. Amount of daily water needs is equal to ½ your body weight.
- Avoid foods high in sodium. Grill, bake, & broil proteins.
- Meal planning is often an effective means with which to involve all members of the family.
- Use behavior management techniques i.e. eat slowly and recognize satiety cues
- Ensure mealtimes are relaxed and enjoyable. Mealtimes should not exceed 30 minutes. Ensure your child is comfortably seated.
- Being a positive role model. Eat together as a family as much as possible. Children learn by copying what others do, including eating what they see others eat.
- Continuing to offer new foods even if rejected. Encourage but do not pressure your child to eat certain foods.
The Role of a Multidisciplinary Team
Managing nutrition in DMD often requires a multidisciplinary approach involving:
- Registered Dietitian Nutritionist (RDN): An RDN can provide personalized dietary recommendations, monitor nutritional status, and help manage weight and gastrointestinal issues.
- Gastroenterologist: A gastroenterologist can diagnose and treat gastrointestinal complications like GERD and constipation.
- Speech-Language Pathologist: A speech-language pathologist can assess and manage swallowing difficulties.