In the current issue of Quest magazine, our friends at MDA discuss a recent multi-center study that suggests spinal muscular atrophy (SMA) patients who are unable to walk but are otherwise considered to have “relatively high motor function” are at a higher risk for weight gain. This is when compared to SMA patient who do walk and to SMA patients who do not walk but have a lower level of motor function. The results seem to reinforce the importance of managing nutrition in the care of SMA patients.

To read the entire article in Quest, click here. To read the abstract of the study, click here.

For more information about SMA and nutrition, check out the links below.

• “Spinal Muscular Atrophy GI and Nutrition Care” on FightSMA’s website
• “Investigating the Impact of Nutrition on SMA” from the FightSMA’s blog
• A Family Guide to the Consensus Statement for Standard of Care in Spinal Muscular Atrophy
  Published by The Patient Advisory Group of the International Coordinating Committee (ICC) for SMA Clinical Trials
• Consensus Statement for Standard of Care in Spinal Muscular Atrophy
  Prepared by the Standard of Care Committee for Spinal Muscular Atrophy, a standing committee of the International Coordinating Committee for Spinal Muscular Atrophy

Nutrition is a critically important variable in the well-being of spinal muscular atrophy (SMA) patients, and there are many complex issues to be considered in the dietary management of an individual SMA patient. While as a community, we have made much ground toward a consensus on best practices for management of respiratory care issues, nutrition is still a relatively uninvestigated, and controversial, subject. This is an extremely important issue, particularly with regard to type 1 SMA children, who are extremely fragile, and often require special attention and management of dietary issues.

With support from SMA Angels Charity, the Pediatric Motor Disorders Research Program at the University of Utah has launched a new website, www.smaandnutrition.org to help SMA families provide SMA researchers with information that will help them investigate the impact of nutrition on the disease and to help SMA families evaluate their current nutritional regimen. From now until June 14, we’d like to especially ask all families with type 1 SMA patients to complete the SMA Type 1 Nutritional Survey, whether or not your child is on any special kind of special diet. This on-line survey should take approximately 15 minutes. The survey includes questions about feeding regimens and methods; select medications, equipment, and medical interventions; specific type of formulas regularly given to the patient; and how the diet is determined and adjusted. The short-term goal is to gather data on the frequency of use of special diets for type 1 children and to better identify the similarities and differences of types of special diets currently prescribed. We hope to gather information from as many possible families before June 14, and hope to present preliminary results in June.

**Please Note: Ideally, all participating families will fill out a detailed dietary record when they submit answers to the nutritional survey. The ultimate goal of this website is to serve as a tool to help provide feedback to families about their child’s diet, resulting in a report about possible nutritional deficiencies and recommendations that you can share with your local dietician and care providers. However, depending on how many responses received to this initial request, dietary record analysis may be delayed. During this initial launch period, they hope to assess how useful a tool this may be by assessing the interest of the community in this project. Ultimately, this project will only be successful with a majority of the community participating. Your participation is greatly appreciated!

The www.smaandnutrition.org also provides a link to the nutritional guidelines for SMA patients on the University of Utah School of Medicine research website. These guidelines need to be refined by real data from SMA patients. Participation in the survey requires a login and password. In order to receive login information for the website, SMA patients must be enrolled in the IRB approved research study “Clinical and Genetic Studies in SMA” which allows University of Utah to collect data about you or your child’s experiences and medical issues in living with SMA. Participants or their parent/guardian must sign a consent form to allow University of Utah to contact you to ask additional questions about you or your child’s medical history. It is not necessary to participate in the full study; you may elect to participate just for the nutritional part. To obtain a login, email smadiet@gmail.com or contact the Pediatric Motor Disorders Program research office for more information at 801-585-9717.

The material above was prepared with the assistance of individuals at the University of Utah’s Pediatric Motor Disorders Research Program and at SMA Angels Charity, Inc..

Spinal muscular atrophy (SMA) patients are often cared for by a team of specialists - neurologists, pulmonologists, orthopedists, nutritionists, etc. The Wall Street Journal reported yesterday on a shortage of pediatricians trained in specialties such as these which is causing delays in treatment for patients across the country. According to the article, “shortages of doctors across a multitude of pediatric sub-specialties are forcing 90% of hospitals to delay appointments, lose patients or refer them elsewhere” and “for families, that often means waiting for months to see a specialist and incurring heavy travel costs, often to another state.”

To read the entire article, click here.