Nutrition Suggestions for Spinal Muscular Atrophy

Valerie Lawson - Exercise Physiologist

Spinal muscular atrophy (SMA) is a genetic disorder affecting the part of the nervous system that controls our voluntary muscle movement. SMA also involves the loss of nerve cells (motor neurons) in the spinal cord and is considered a motor neuron disease. There are several varieties of SMA, and the severity can affect people differently. SMA is usually divided into three categories: Type 1 is the most severe with the earliest age of onset, Type 3 is the least severe with the latest age of onset, and some doctors have added Type 4 for moderate or mild SMA that begins in adulthood.

With SMA, the muscular system is the primary organ system that is affected. Muscle atrophy can be a nutritional strain due to muscle function being an essential nutritional reserve for our body. For longer periods of fasting, our muscles become the primary source of glucose by degrading its own protein into amino acids and then releasing them into the blood stream.

Additional potential problems with SMA include respiratory muscle weakness, swallowing muscle weakness (leading to nutritional intake concerns), back muscle weakness with progressive spinal curvature, and abnormal reaction to muscle-relaxing medications. With this muscle reduction or loss, the body is limited in its ability to adjust to nutritional changes such as normal overnight fasting, or more serious conditions such as diarrhea, vomiting, and fevers.

Therefore, some nutritional therapy may be appropriate in many situations where a person or a care taker is planning meals or food intake for a person with SMA. A few simple nutritional goals are suggested in the research:

• Increased amounts of complex carbohydrates (bran, whole wheat cereals, root vegetables, peas, legumes, brown rice and pasta, lentils).
  
  
• At least 2 grams of protein per 2 kg of body weight should be consumed per day. For example, for someone who weighs 85 pounds, divide 85 by 2.2, which equals 38.64 kg, and multiply this by 2 to arrive at 77 grams of protein per day. (See the high protein recipe below.)
  
  
• Some children may do better with smaller, frequent feedings or meals compared to three larger meals per day.

Another suggestion for children includes a feeding schedule that limits overnight fasting to 6 hours for the young infant, and 10-12 hours for an older child. Children with type I and some with type II SMA may have special nutritional needs due to chewing and weakness of swallowing muscles. There might be a hazard of choking and foods might need to be altered in consistency. (For more information, read the NCPAD Factsheet, "Tasty Foods for Persons who have Problems Swallowing and Chewing" at http://www.ncpad.org/nutrition/fact_sheet.php?sheet=330)

Please keep in mind that careful attention to nutritional intake needs to occur during illness, especially when vomiting or diarrhea is involved.

Tuna and Bean Salad

3 ounces white tuna, drained

1 pound canned garbanzo beans or canned black beans

1 cup mushrooms, sliced

1 cup tomatoes (cherry), halved

½ cup yellow bell pepper, chopped

1/3 cup orange juice

3 Tbs. lime juice

2 Tbs. olive oil

1 Tbs. whole grain mustard (or Dijon)

41/4 cup mixed greens (Romaine, Spinach, Arugula)

In a salad bowl, combine beans (garbanzo or black), mushrooms, tomato, and yellow pepper. In a separate container, mix orange juice, lime juice, olive oil, and mustard. Drizzle over the ingredients in the salad bowl. Season to taste with pepper. Toss tuna with the salad mixture and serve.

Please send your comments and feedback to Valerie Lawson at vlawson@uic.edu.

This fact sheet was last updated on 08-08-2006.

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