What is the Amino Acid Diet?

By: Mary Bodzo

This information is based on opinions and experiences compiled from parents of children with SMA and should not necessarily be relied upon as an alternative to medical advice from professional health care providers.

ABOUT THE DIET

The Amino Acid Diet is a nutritional approach that many SMA families believe has had a positive impact on their child’s quality of life. Using this dietary approach, milk and soy based formulas are eliminated and substituted with a low-fat, elemental free form amino acid formula. Elemental formulas are made from nutrients in their most broken down form, which require very little digestion. Tolerex and Pediatric Vivonex, from Nestle Nutrition, are the two formulas used most often. EO28 Splash, from Nutricia North America, is a ready-made, more palatable, amino acid formula sometimes preferred by children without g-tubes. Because the EO28 Splash contains a higher fat content, it may not be an appropriate formula choice for children with Type 1 SMA. These formulas contain vitamins, minerals, carbohydrates, protein and some fat. The protein is in the form of free amino acids. These amino acids are not derived from food sources. Most parents that implement this diet believe more benefit is realized with these elemental formulas, rather than hydrolyzed amino acid formulas, which contain broken down proteins from foods, such as dairy or soy. The free form amino acids in Pediatric Vivonex, Tolerex, and EO28 Splash, are in balanced amounts, and are less likely to trigger immune responses such as inflammation. They are easily digested and quickly absorbed into the bloodstream. When given frequently, in low amounts, these amino acids provide a steady source of accessible energy and may help prevent muscle breakdown.

Many children with SMA are very reactive to the protein and fat in dairy and soy formulas. Their reactions are often attributed to their SMA progression. However, when this protein and fat are replaced with free form amino acids and appropriately reduced amounts of fat, children with  SMA  experience  improved respiratory health and fewer metabolic complications during illness. Most children with Type 1 SMA have an immediate reduction in airway secretions. Constipation, which can be a major complication, is more manageable or even eliminated. Some children have improvement in strength and regained function.

Parents using these formulas vary their child’s particular diet based on tolerance, age, weight and length, and severity of SMA, but there are some basic guidelines that most children respond to favorably.

Children with SMA do not tolerate large amounts of amino acids or fat due to their reduced muscle mass. This reduction of muscle mass prohibits the proper absorption, storage and utilization of amino acids and fat, which could result in toxic accumulation in the bloodstream.

• More than approximately 2 packets of either formula, over a 24 hour period, may cause increased heart rate, arrhythmia, and/or gastrointestinal distress. It is strongly recommended that any child receiving more than this amount of formula, be carefully monitored with appropriate labs and observation of toxicity symptoms.
• %  of fat calories should be kept at the minimum requirement necessary for adequate essential fatty acid intake
• To avoid fat, protein, and calorie overload, these formulas should never be combined with soy or milk based formulas.
• Children should be monitored with the following laboratory testing: quantitative amino acid profile, fatty acid profile, basic metabolic profile

ADDITIONAL SUPPLEMENTS

Because these formulas aren’t used to supply complete nutrition in SMA patients, additional calories, vitamins and minerals must be added to the diet to ensure that their daily requirements are met. Special attention should be given to calcium, magnesium, sodium, and potassium intake.

Commonly added supplements include:

• Multi-vitamin and mineral– NanoVM from Solacenutrition.com  (may only require half-dosage)
• Additional Vitamin D3—this level should be monitored for adequate dose
• 1/8 to1/4 teaspoon salt, for sodium, many use pink himalayan salt
• Probiotic—Florajen 3
• L-glutamine powder—(dosage is 200-300mg/kg/day, not to exceed 10gm/day, if lab result is less than 500.)
• Tolerex contains 1060mg/pack, Pediatric Vivonex contains 774mg/pack, EO28 Splash doesn’t contain L-glutamine
• Based on lab reports, the amino acids L-carnitine and L-arginine are often supplemented.
• Additional essential amino acids should be added as indicated by a quantitative amino acid profile. Nutricia Essential Amino Mix can be added in small amounts to keep amino acid levels in low to mid-range.
• CoEnzymeQ10 or Ubiquinol, the more reduced form.

Although many children seem to tolerate Tolerex best, essential fat must be added to this formula.  To accomplish this, there are several different options. It is common for parents of children with Type 1 SMA to add breast milk to their children’s Tolerex. For many, this has made a tremendous difference in their children’s growth and wellness, especially their gastrointestinal health. Based on their essential fatty acid profile lab reports, approx. 8-10 oz/day of breast milk may meet the essential fatty acid requirement for some children. If a child’s mother can’t supply breast milk, many families report a positive experience acquiring it from donors, signed off as healthy by their doctors, or donor milk banks. For more information go to milkshare.com and eats on feets on facebook. There are many SMA caregivers willing to share information on acquiring breast milk from donors. Questions can be posted on the facebook group SMA AA diet.

If breast milk is not an option, families have added nut milks (be sure that a child is not allergic to nuts),  hemp milk,  or small amounts of oils containing the essential omega 6 and 3 fatty acids to the Tolerex mixture. Fat supplements should be added in slowly to check for tolerance. Children will vary in how much they tolerate, but effort should be made to provide at least 10% of calories from essential fatty acids.  This should be monitored by an essential fatty acid profile lab test. Signs of intolerance may include,  increased oral secretions,  reflux,  slow stomach emptying,  flushing,  increased heart rate. Treatments that enable babies to avoid the devastating impact of severe muscle atrophy on metabolic processes may tolerate diets much higher in fatty acids. This is because most dietary fat is metabolized in skeletal muscle.

ADMINISTERING DIET

These formulas should be diluted as much as possible (more than directed) with water and other fluids, such as breast milk, juices, rice milk, nut milks, and fruit and vegetable purees. These will also increase calories, potassium, and other nutrients. A minimum of 16 ounces of fluid for each packet of formula is a well tolerated dilution for most g-tube fed children. Those drinking their AA formula should dilute as much as possible with water and juices. Adequate hydration is extremely important, and recommended daily intakes should be met.

Additional Food:

In addition to the elemental formula mixtures, if tolerated, fruits and vegetables should be consumed.  Less affected children usually include some grain in their diets. The formula, fluids, supplements, and fruit and vegetable purees should be mixed in a blender and refrigerated. Because children with Type 1 SMA are often extremely reactive to changes in their diet, foods and supplements should be added gradually, waiting several days before adding in anything new.

Many families report that their children receive the most benefit from this diet by greatly reducing or avoiding animal protein foods. Children following the AA diet correctly are receiving adequate protein from the amino acids in their elemental formula and amino acid supplements, and can easily be overloaded if they are also eating high-protein foods. They should never be fed high amounts of amino acids over a short period of time, and any increases should be indicated by lab results.

CLOSING

Although children are affected by their SMA differently, according to hundreds of SMA families, the recommendations stated earlier have proven, over the last 24 years, to be the safest and most effective way to implement the AA diet. Parents must take responsibility for monitoring and problem solving when placing their child on this diet. Unfortunately, due to lack of studies in SMA nutrition, many medical professionals are unfamiliar with this dietary approach and tend to overload our children with fat and/or amino acid protein.  Nutritional lab testing, networking with other SMA families that are successfully using the AA diet and following the above guidelines, can provide invaluable guidance for information on the benefits and safe administration of this diet.

Personally, I have had success with this diet for my daughter Krista, a lovely 27-year old, who was diagnosed with SMA Type 2 when she was 8 months old. We placed Krista on this diet over 24 years ago and have been very pleased with her wellness and stability. There are many other children with SMA that have also experienced more quality and length of life from following the AA diet as stated above. The feedback from their caregivers has played a significant role in our ability to adjust and improve the AA diet. I would like to thank all of you who have contributed by sharing your experiences and knowledge. It has been a joint effort!  A special thank you to Jeanna Huette, who contributed greatly to the revision of this paper.