SMA Parent Mary Bodzo Advocates for Amino Acid Diet to Keep Disease Stable

SMA Parent Mary Bodzo Advocates for Amino Acid Diet to Keep Disease Stable

Mary Bodzo’s daughter Krista is now 27. Born with spinal muscular atrophy (SMA), her condition is stable, and Bodzo attributes that to the amino acid diet that Krista began at age 5.

“We completely believe that changing her diet was what kept her stable,” Bodzo said in an interview with SMA News Today.

Mary and Krista Bodzo
Mary Bodzo, left, and her daughter Krista. Bodzo invented the diet when Krista at age 5 began to have trouble swallowing. (Photo by Jaycie Bodzo)

At the age of 8 months, Krista was diagnosed with Type 2 SMA. As she neared her fifth birthday, she was losing her ability to swallow. That’s when Bodzo, a mother of five who lives in Ocala, Florida, decided to try a nutritional approach now known as the amino acid diet.

This diet substitutes milk and soy formulas with a low-fat, elemental, free-form amino acid formula, made from nutrients in their most broken-down form that requires very little digestion.

The diet is not an exact science. Children with SMA seem to react badly to the protein and fat in dairy and soy formulas. But when the protein and fat are replaced with free-form amino acids and reduced fat, they can breathe more easily and have fewer metabolic complications, Bodzo wrote in a description of the diet posted in 2011 on the website Sophia’s Cure.

Children with Type 1, the most severe form of SMA, who begin the diet often see an immediate reduction in airway secretions, she wrote. They are less likely to be constipated, and some actually improve their strength and regain some function.

SMA is caused by a deficiency of the survival motor neuron (SMN) protein, according to the Muscular Dystrophy Association (MDA). In many cases, although not all, it results from mutations of chromosome 5 in  the SMN1 gene. The primary symptoms of SMA are weakness of the muscles closest to the body’s core — the shoulders, back, hips and thighs. Muscles controlling breathing and swallowing can also be affected.

Bodzo described SMA as an inherited disease that reduces muscle mass, which prevents proper absorption and storage of amino acids and fat.

The MDA is less certain of dietary benefits, as it states on its website, Medical Management. “Many people wonder if a special diet will affect the course of SMA. … So far, there’s little evidence to suggest that any particular type of diet is useful against SMA — and in fact, some diets may be harmful.”

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“For example,” the page continues, “special formulas made up of broken-down protein components called amino acids — so-called ‘elemental diets’ — actually may cause problems for children with SMA who may have little muscle tissue. Some experts say blood levels of these amino acids can become too high if there isn’t enough muscle tissue to properly use them.”

Cure SMA, which posts a document describing the diet and offered by four medical professionals, is neutral toward it. The four end their description with a “strong recommendation” that any dietary plan for a child with SMA be done under the guidance of a registered dietitian or nutritionist.

According to Bodzo, the AA diet, as it’s called, adjusts for this lack of skeletal muscle. “You don’t metabolize nutrients the same,” Bodzo said. “That’s just a basic fact of biochemistry. Overfeeding and overloading is one of the biggest issues.” Intact proteins, for reasons unknown, seem to cause problems, she said. “The more affected a child is with SMA, the more reactive they’re going to be to dairy and animal-based proteins.”

Tolerex and Pediatric Vivonex, both made by Nestle Health Science, are two common used products that form the basis for the diet. Additional ingredients might include water, almond milk, white grape juice and Pedialyte mixes with ingredients that may range from safflower oil and MiraLAX, to cornstarch and levocarnitine. Bodzo says that supplements should be determined based on “personal tolerance and nutritional lab result.”

Bodzo lists some of the common supplements used to help parents on the Sophia’s Cure site. She also provides a sense of direction on the AA Diet Info for SMA website she set up.

Some children take the diet orally, others use a gastrostomy tube or G-tube. Vitamins and supplements should also be given, but fats like safflower oil, evening primrose oil or an omega-3 supplement should be added based on the individual as determined by tolerance and lab tests, she says. Some SMA patients can eat solid foods along with the amino acid diet; some parents mix breast milk into the liquid.

Bodzo said she noticed Krista reacted badly when she took her daughter off breast milk at six months of age and began feeding her dairy. “That’s when she went downhill,” she told us.

After the SMA diagnosis, she tried feeding Krista formulas based on soy, goat’s milk, and breast milk. Finally, she met another mom of an SMA patient who put her in touch with a gastrointestinal doctor who introduced Bodzo to elemental formulas, which means there’s no food source for the protein.

“I educated myself as much as a lay person can,” she said. “It’s more of a vegan-based diet.” From then on, it was trial and error to see what worked best for Krista.

All SMA patients need an individualized plan when trying the AA diet, Bodzo said. She did receive guidance from professionals while educating herself about Krista’s dietary needs, she said. The AA diet doesn’t supply complete nutrition — and that’s where nutritional guidance is needed, she said.

Parents who want to try the AA diet may have to do their own digging for information, Bodzo said. Beside the  Cure SMA site, it’s also listed by Support SMA. The University of Maryland Medical Center discusses facets of the amino acid diet at its Muscular Dystrophy website, under the heading “Complementary and Alternative Therapies.”   

No published studies have investigated the diet, according to Bodzo. The National Institutes of Health (NIH) reviewed the practice in a nutritional survey of 44 caregivers of SMA Type 1 children whose average age was 5, The 2014 survey, published in the Journal of Child Neurology, did find that most families were feeding their children, largely via a gastrostomy tube, an “elemental” — or amino acid-free — diet.

“Although formal studies are not available … to assess the potential benefits and risks of using such formulas, caregivers … list a number of potential benefits and observed improvements in their child with spinal muscular atrophy after switching to an elemental or semi-elemental formula,” the researchers wrote, recommending that research begin into such diets to support “better management” of SMA children.

Melissa Bowerman, a lecturer at Keele University in Staffordshire, England, is studying the use of amino acid supplements in SMA patients. Bowerman said in an email that she is investigating how nutritional support, such as the AA diet, may help these patients. Preliminary work, she added, suggests that AA supplementation may counteract components of AA processing that are hyperactivated in SMA muscles and other tissues. Bowerman’s work is being funded, in part, by SMA Angels.

Many families, however, work without the guidance of a registered dietitian, Bodzo said. Creating evidence-based nutritional guidelines would encourage more families to work with professionals to create a diet for their child, she added.

Bodzo began making the amino acid diet public because of the results she saw in Krista, who uses a wheelchair to get around. Krista’s muscle deterioration slowed after starting the AA diet, her mother says. When Bodzo began feeding her daughter the diet, Krista was having trouble swallowing. Today, she can eat by mouth.

“I’m just paying it forward,” Bodzo said. “I’m not getting paid. If it didn’t work, there are plenty of other things I could do with my time.”

She hopes the diet will be researched more fully, especially as advances in SMA are made. She even hopes that, one day, such attention to diet may no longer be necessary. Krista, she noted, begin receiving Spinraza in May, the only U.S. Food and Drug Administration-approved therapy for the disease.

“Things are changing with SMA because we have this treatment and more treatments are in the pipeline,” Bodzo said. “As treatments are given at birth, a lot of these metabolic issues may not be as critical.”

The NIH clinical trials website lists more than two dozen studies now underway into potential treatments or a better understanding of SMA.

Discuss this topic and much more in our forums.

 

7 comments

  1. Mary Bodzo says:

    I would like to thank SMA News Today for reporting on the AA diet for SMA. There are some inaccuracies in this article, however. I would like to correct and clarify several points.
    1) Krista was diagnosed at 8 months, not 3 yrs.
    2) I did not say that a traditional diet causes toxins to accumulate in the blood or that dairy and other animal based proteins cause overload. Those on the AA diet avoid animal protein, but it is not known why so many seem reactive to them.
    3) Most children receive the AA formula through a gtube,not intravensously.
    4)I do not believe that children should start the AA diet at birth.

  2. Monique Ryan says:

    It is irresponsible and unhelpful for this forum to be promoting unproven interventions which potentially carry a significant risk to children and adults affected by SMA.

    • michelle says:

      Monique Ryan – you are wrong – if it were not for this aa diet my niece would not be alive today. Unproven by what – medical science? not really – those formulas the base diets Tolerex and Vivonex are proven for those with restricted GI issues. SMA has restricted GI issues. There are over 100s of 1000 of people kids with SMA on it – all are thriving. I do not know who you are but proven odes not mean it needs a paper to back it. One last thing – normal people eating fast food and processed food from the middle of the grocery store are more unhealthy then those with SMA on the AA diet. Again – my niece is alive due to this diet.

    • Mary Bodzo says:

      I think if you knew, over the last 24yrs, how many SMA patients have benefited from the true AA diet, you would realize that the irresponsibility lies with those in the medical community that have ignored this important area of research.

  3. Hades Gonzalez says:

    SMA should take this subject really serious. Doctors don’t know much or nothing how to help an individual who is affected. Nutrition is very important in our quality of life specially in chronic diseases. Parents have been struggled with nutrition. Mary Bodzo has been very supportive sharing her story and her knowledge as a Nutritionist.

  4. Annette Mabe Cordes says:

    I am so glad that the AA DIET is finally getting some positive public attention. I actually believe that it is those people such as Monique Ryan and “book knowledge” medical professionals who have for 14 years continued to vilify the wonderful ladies(SMA moms) that have given freely the knowledge of their own personal observations. These ladies have NOT represented what they feed their own children as anything more than a diet that has helped their child fight this disease we know as SMA. While it may not have scientific (double blind testing) proof of efficacy it has 1,000s of SMA individuals that are not just surviving but are thriving on this diet. The lack of scientific proof falls on those medical professionals (doctors, nutritionists, dietecians, and researchers) that find it easier to poo-poo that which is unfamiliar than to conduct the research necessary to prove it works. The problems occur when a patient that is doing great diet wise comes into a hospital for surgery or illness and those medical professionals refuse to accept the diet as the parent has been mixing it and feeding it at home. This is where the old adage, if it’s not broke don’t fix it, comes into play. Unfortunately because it is unfamilar, “not proven” and wasn’t in their textbook or on their cheat sheet they throw the patient under the bus and do things their way. This wrecklessness has caused negative side effects and the death of their patients. When used correctly this diet works. My DGD who was diagnosed type 1 in 2003 has been on this diet ever since and is doing great at the age of 15, which is well beyond the doomsday forecast given on diagnoses day. Some things that cause problems is when these “medical professionals” agree to use one of the Amino Acid (Elemental) formulas but use it incorrectly for SMA. There are a few truths about its successful use
    1. The formula (tolerex/vivonex/
    neocate or others) is not the complete diet and cannot be used that way you must add juices, purees, vitamins and supplements
    2. The formula must be double diluted(no ifs, and, or buts)
    3. When using tolerex or vivonex for SMA no matter what the age or weight the maximum is 2 packets total (rather it’s a mixture or all one kind)
    4. You must obtain nutritional labs prior to starting and at regular intervals to insure that all nutritional needs are being met.
    I suggest anyone interested in the diet as a whole contact Mary Bodzo as to learn more about it while we wait on the testing/research this valuable tool deserves. It carries no more “significant risk to children and adults affected by SMA” than eating at McDonald’s daily and should not require anyone’s approval to feed it to yourself or your child. No one has to approve McDonald’s in advance.

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