Introduction
Living with Spinal Muscular Atrophy (SMA) presents unique challenges, and nutrition plays a vital role in maintaining overall health and well-being. This guide, developed based on a comprehensive review of medical studies and expert recommendations, aims to empower you with the knowledge to make informed decisions about your diet and manage your SMA symptoms effectively. We'll explore the connection between SMA and nutrition, discuss general dietary guidelines, delve into specific nutritional needs, and provide practical advice, meal planning tips, and recipes.
Understanding Spinal Muscular Atrophy and Its Impact on Nutrition
Spinal Muscular Atrophy (SMA) is a genetic condition that affects the motor neurons, the nerve cells responsible for transmitting signals from the brain to the muscles. This can lead to muscle weakness, atrophy (shrinkage), and difficulties with movement. SMA can affect individuals of all ages, and its severity varies. While the primary impact of SMA is on the muscles, it can also indirectly affect other bodily functions, including digestion, metabolism, and bone health [1].
SMA can affect the muscles involved in swallowing, leading to a condition called dysphagia. Dysphagia can make it difficult to consume enough calories and fluids, increasing the risk of malnutrition and dehydration [2]. In some cases, it can also lead to aspiration, where food or liquid enters the airway and lungs, potentially causing aspiration pneumonia [2]. Dysphagia can be caused by muscle weakness in the throat and mouth, as well as damage to the bulbar motor neurons that control chewing, speech, and swallowing [2].
Furthermore, SMA can affect the enteric nervous system (ENS), which is a network of nerves in the digestive tract. This can lead to various gastrointestinal symptoms, such as constipation, gastroesophageal reflux (GERD), and delayed gastric emptying [3]. These digestive issues can further complicate nutritional management.
It's important to remember that proper nutrition is not just about consuming enough calories. It's about providing your body with the essential nutrients it needs to function optimally. Good nutrition can support lung health, improve breathing, and enhance your overall quality of life [4].
Read also: Diet and Ogilvie Syndrome
SMA is a neuromuscular disorder, which means it affects both nerves and muscles. It attacks the motor neurons, the nerves that send signals for movement to the muscles, leading to muscle weakness and atrophy (shrinkage). Recent research has found that SMA also affects how the whole body breaks down or metabolizes fatty acids. Abnormal fatty acid metabolism can lead to nutritional deficiencies, which can cause many physical problems in people with SMA.
General Dietary Guidelines for People with Spinal Muscular Atrophy
While individual needs vary, some general dietary guidelines can help you make healthier choices:
- Prioritize nutrient-rich foods: Focus on a balanced diet that includes fruits, vegetables, whole grains, lean protein, and healthy fats [6]. These foods provide essential vitamins, minerals, and antioxidants to support your body's functions. Eating a variety of foods and whole grains can provide healthy sources of nutrients: fruits, vegetables, lean protein, unsaturated fats.
- Choose whole grains: Opt for whole-grain bread, cereals, and pasta over refined grains. Whole grains are higher in fiber, which can help with digestion and prevent constipation, a common issue for people with SMA [7].
- Include lean protein: Protein is essential for maintaining muscle mass and strength. Good sources include poultry, fish, beans, lentils, and low-fat dairy products [8].
- Incorporate healthy fats: Include sources of healthy fats like olive oil, avocados, nuts, and seeds in your diet. These fats are important for overall health and help your body absorb essential vitamins [1]. Fats that are consumed should consist mostly of polyunsaturated fats. Polyunsaturated fats are typically liquid at room temperature and come from plants. One example of a good source of unsaturated fat is olive oil. On the other hand, saturated and monounsaturated fats should be eaten in moderation. Saturated fats are usually solid at room temperature and are found in meat and dairy products, such as butter.
- Embrace quercetin-rich foods: Quercetin is a natural compound found in various fruits and vegetables that has antioxidant and anti-inflammatory properties. Some studies suggest that quercetin may have potential benefits for people with SMA [6]. Include foods like apples, berries, onions, and leafy greens in your diet.
- Limit added sugars: Minimize your intake of sugary drinks, candies, and desserts. These foods provide empty calories and may contribute to weight gain [9].
- Reduce sodium intake: Limit processed foods, canned soups, and salty snacks. High sodium intake can contribute to fluid retention and high blood pressure [9].
- Stay hydrated: Drink plenty of fluids throughout the day. This is especially important if you have difficulty swallowing or experience constipation [10]. Aim for 1 ½ ounce of fluid per pound of body weight for adults and ½ ounce per pound for children [12].
- Eat smaller, more frequent meals: This can help with digestion, prevent reflux, and maintain stable blood sugar levels [7]. Many people find that eating small, more frequent meals instead of three larger ones helps them manage appetite, weight gain, blood sugar, and energy levels. Some medical providers suggest that children with SMA may tolerate smaller, more frequent feedings better than larger meals. This eating schedule may also help reduce acid reflux, bloating, or fatigue after meals. As long as small, frequent meals don’t lead to overeating, this may be a helpful adjustment to consider. Some children with SMA may better tolerate smaller, more frequent feedings better than fewer, larger meals. It’s also important to remember that people with SMA don’t tolerate fasting well, especially during illness. Eating regularly and staying hydrated is especially important when sick or recovering from surgery.
Specific Nutritional Needs for Spinal Muscular Atrophy Patients
SMA can affect your body's ability to metabolize nutrients, so it's essential to pay attention to specific needs:
- Vitamin D and Calcium: These are crucial for bone health, as individuals with SMA are at an increased risk of osteoporosis and fractures [1]. Ensure you get enough vitamin D through sunlight exposure, fortified foods, or supplements, and consume calcium-rich foods like dairy products, leafy greens, and fortified plant milk. Vitamin D (and calcium) are critical for bone health in people with SMA, who are often at risk for bone-related problems like osteoporosis and fractures.
- Essential Fatty Acids: These fats are vital for brain development and overall health. Include sources like fatty fish (salmon, tuna), avocados, and plant-based oils (flaxseed, canola) in your diet [10].
- Fiber: Adequate fiber intake is essential for digestive health and preventing constipation. Aim for your age plus 5 grams of fiber per day [11]. Good sources include fruits, vegetables, whole grains, and legumes.
- Hydration: Staying hydrated is crucial for overall health, especially if you have difficulty swallowing or experience constipation [10].
- Protein: Protein is essential for maintaining muscle mass and overall health, but requirements can vary widely among individuals with SMA depending on disease progression, physical activity, and any concurrent health conditions. People with SMA should keep track of the calories they eat to maintain appropriate body weight and composition (the balance of muscle and fat). Monitoring caloric intake is important, but so is making sure that you are getting calories from a balance of protein, carbohydrates, and fat.
For individuals with higher muscle activity levels or those with milder forms of SMA, protein intake may be in the range of 1.5-2g per kg of body weight, as this can support muscle strength and growth.
For individuals with lower activity levels or advanced SMA, the body's need for protein might differ. In some cases, excessive protein intake may not be beneficial and could even stress the kidneys or contribute to other metabolic concerns. Therefore, protein intake should be carefully monitored and adjusted, especially for those with kidney issues, metabolic disorders, or other complicating factors.
Read also: Understanding Cushing's and Weight
Consulting with a registered dietitian is crucial to determine the appropriate protein intake based on your specific needs, especially if you have underlying conditions like renal disease or metabolic concerns that require special dietary considerations.
Practical Advice and Meal Planning Tips for Spinal Muscular Atrophy Patients
- Modify food textures: If you have difficulty chewing or swallowing (dysphagia), consider modifying food textures [1]. Choose softer foods, puree or mash fruits and vegetables, and thicken liquids as needed. If you have difficulty chewing, avoid foods like bread, seeds, peanut butter, and meat with cartilage or tendons. Choosing softer foods can help make eating and swallowing easier. If you are having more trouble than usual, you may want to switch to softer foods while you wait to see a dietary specialist. Soft foods and thickened fluids may be recommended for people who have dysphagia (difficulty chewing and swallowing). Your SLP or OT might also suggest adjusting food textures to make swallowing safer and easier, such as: Easy-to-chew foods (not tough, chewy, stringy, fibrous, or crunchy), Soft foods (foods you can cut with the side of a fork, like fish or bananas), Minced foods (finely chopped, such as ground beef, creamed corn, or rice), Pureed foods (smooth, thick liquids like yogurt or mashed potatoes), Thickened liquids (foods with a “nectarlike” consistency, like tomato juice or smoothies). Some foods can be especially hard to chew or swallow and should be avoided if eating is difficult. These might include: Raw vegetables or fruits, Large pieces of meat with cartilage or tendons, Sticky foods like peanut butter or sticky rice
- Adaptive utensils: If you have difficulty with fine motor skills, consider using adaptive utensils to make eating easier [12].
- Meal timing: Avoid lying down flat for at least 30 minutes after eating to reduce the risk of reflux [7]. If you have trouble swallowing, it’s also important to stay upright for 30 minutes to an hour after eating or drinking. If you need to lie down after eating or drinking, keep your head raised at least 30 to 45 degrees to help food and liquid go down the right way.
- Food safety: Be mindful of food safety, especially if you have a weakened immune system [12]. Wash your hands thoroughly before meals, and ensure food is cooked properly.
- Avoid prolonged fasting: Do not go for extended periods without eating, as this can lead to low blood sugar, muscle breakdown, and other complications [9]. Aim for no more than 6 hours between meals for SMA Type I and 8-12 hours for SMA Types II/III [10]. If you aren’t able to eat for an entire day, you may need to go to the emergency room or an urgent care center, if your doctor is not available. This is because people with SMA can’t tolerate fasting as well as those with more muscle reserves, especially if they are sick.
Sample Meal Plans
Here are some sample meal ideas that incorporate the principles discussed above:
Breakfast options
- For normal chewing/swallowing:
- Scrambled eggs with avocado and soft whole-wheat toast
- Greek yogurt with soft berries and honey
- Oatmeal with ground flaxseeds, banana, and almond butter
- Smoothie with Greek yogurt, banana, spinach, and protein powder
- For dysphagia or difficulty chewing:
- Pureed oatmeal with mashed banana and almond butter
- Greek yogurt blended with fruit puree and honey
- Smoothie with protein powder, nut butter, and soft fruits
Mid-morning snack
- For normal chewing/swallowing:
- Cottage cheese with soft peaches or mango
- Mashed avocado on soft whole-grain crackers
- Soft-boiled egg with mashed sweet potatoes
- For dysphagia:
- Pureed fruit smoothie with Greek yogurt
- Cottage cheese blended with soft fruit puree
- Soft mashed avocado mixed with mild yogurt
Lunch options
- For normal chewing/swallowing:
- Soft turkey and cheese sandwich on whole-grain bread
- Lentil soup with soft whole-wheat bread
- Quinoa salad with soft-cooked vegetables and shredded chicken
- For dysphagia:
- Pureed lentil soup with soft whole-grain bread (dipped for easier swallowing)
- Blended turkey and vegetable soup
- Mashed quinoa with soft vegetables and protein puree
Afternoon snack
- For normal chewing/swallowing:
- Hummus with soft pita bread
- Nut butter on soft whole-wheat crackers
- Mashed avocado with soft-cooked sweet potato
- For dysphagia:
- Blended hummus with mashed soft pita
- Nut butter blended into a smoothie
- Soft avocado and yogurt blend
Dinner options
- For normal chewing/swallowing:
- Grilled salmon with mashed sweet potatoes and steamed broccoli
- Chicken stir-fry with soft-cooked vegetables and brown rice
- Soft turkey meatballs with whole-wheat pasta and pureed marinara sauce
- For dysphagia:
- Blended salmon with mashed potatoes and soft vegetables
- Pureed chicken and soft vegetable mix with rice porridge
- Blended turkey and marinara over mashed pasta
Evening snack
- For normal chewing/swallowing:
- Soft cheese with whole-wheat crackers
- A soft-boiled egg with smooth guacamole
- Chia pudding with mashed berries
- For dysphagia:
- Blended chia pudding with soft banana puree
- Mashed guacamole with mild soft cheese
- Smooth yogurt mixed with soft protein sources
Recipes
Here are a few simple and nutritious recipes suitable for individuals with SMA:
Creamy Tomato Soup [16]
Mix 2 cans of cream of tomato soup, 1 can of water, 1 cup half and half, 1/2 cup milk, 1/4 cup sour cream, and 1/4 cup shredded cheese in a large saucepan.
Stir over low heat until the cheese melts.
Read also: Nutrition for Sjögren's
Reduce heat to very low and heat for 20-30 minutes to blend flavors, stirring frequently. Do not boil.
Puree if needed.
Shepherd's Pie [16]
Heat oven to 375ºF.
Brown 1 pound ground meat in a large skillet and drain.
Mix 2 cups mashed potatoes, 4 ounces cream cheese, ½ cup shredded cheddar cheese, and 1 clove garlic until well blended.
Combine meat, 1 cup mixed vegetables, and 1 can of gravy; spoon into a 9-inch square baking dish.
Cover with potato mixture and remaining cheddar cheese.
Bake for 20 minutes or until heated through.
Maple Oatmeal [16]
Pour 1 cup oatmeal into a microwaveable bowl.
Stir in 1 cup milk. Mix well.
Microwave uncovered for 1-1/2 to 2 minutes until thickened.
Let stand until desired consistency.
Add 1 tablespoon butter and 2 tablespoons maple syrup.
The Importance of Monitoring Caloric Intake, Fatty Acids and Glucose Metabolism
People with SMA may require dietary modifications to provide proper nutrition and avoid eating-related problems such as acid reflux and aspiration (inhaling food or fluid into the lungs). Due to low muscle mass and limited mobility, people with SMA usually require fewer calories in their diet. Reducing caloric intake can help lower body fat and excess weight. However, reducing the number of calories can also reduce the intake of other nutrients. Vitamin deficiency is common in SMA, especially with the vitamins A, D, E, and K because they require fat to be absorbed. Ask your health care provider about checking blood levels of key vitamins and minerals and how to supplement any deficiencies you may have. Treats are fine too - just remember to enjoy in moderation!
SMA is associated with abnormalities of lipid metabolism. Compared with healthy controls and non-SMA motor neuron diseases with equally debilitating statuses, the abnormal lipid metabolism found in patients and animal models appears unique to SMA. Moreover, increased fat mass, even though relatively low caloric consumption has been repeatedly reported in patients with SMA. Several serum fatty acids and lipids have been found correlated to the motor function of patients with SMA, suggesting potential biomarker candidates for SMA. It has recently been implicated that defects in fatty acid transport and mitochondrial β-oxidation may also contribute to muscle wasting in patients with a severe SMA phenotype.
Two studies of type 2 SMA patients suggested they might be more likely to experience hypoglycemia following fasting. A recent study in type 1 SMA patients also showed a similar finding of hypoglycemia even after a short-term fasting (>4 h but <6 h). In contrast, other studies have reported hyperglycemia during fasting in patients with type 2 and type 3 SMA, some of whom were diagnosed with diabetes and ketoacidosis. The metabolic syndrome features of increased fat mass and decreased lean mass have been reported in patients with type 2 and type 3 SMA. A recent study also indicated that, in a good state, obese children with SMA type 2 were at increased risk of insulin resistance and impaired glucose tolerance, with 50% of participants showing urinary ketones.
Importance of Working with a Healthcare Professional
It's crucial to work with a healthcare professional, such as a registered dietitian or nutritionist, to develop a personalized nutrition plan tailored to your specific needs [15]. They can assess your nutritional status, identify any deficiencies, and provide guidance on appropriate calorie intake, food choices, and meal planning [17]. Early assessment and intervention by a registered dietitian can help optimize your nutritional status and prevent complications associated with malnutrition or other dietary issues [5]. Before making significant changes in your diet, always consult with a dietitian or qualified health care provider who has experience with SMA. They will make sure that a new diet will meet your nutritional needs. What is healthy for other people might not be healthy for you. Check with a dietitian or another provider who has experience in nutrition for people with SMA to find what dietary changes are best for you. Talking with a dietary specialist about nutrition and health can be an important step in helping you thrive while living with SMA. If you haven’t seen a dietitian lately, ask your doctor if you should be referred for a check-up.
Conclusion
Nutrition is fundamental to managing your health and well-being with SMA. This guide provides a foundation for understanding your nutritional needs. By following the guidelines and practical advice presented here, you can make informed choices that support your overall health and help you live your best life. Remember that individual needs vary, and personalized guidance from a registered dietitian or nutritionist is essential to create a nutrition plan that addresses your specific requirements and helps you manage SMA symptoms effectively. With the right approach to nutrition, you can improve your overall health, enhance your quality of life, and navigate the challenges of SMA with greater confidence. Eating well and staying as physically active as possible can improve the quality of life for people living with SMA. Work with your healthcare team to create a nutrition plan that supports your overall health.