Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS), also known as “Lou Gehrig’s Disease,” is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.

There is early evidence from a small study that people with ALS who are fed a high-kcal, high-carb diet, could potentially see the progression of their disease slowed.
Patients with ALS, who have difficulty swallowing, as did the 24 people in this study, often end up requiring a feeding tube into the stomach in order to receive the adequate amount of nutrition. These patients are usually fed a standard commercially available formula. ALS patient lose weight because they are not able to take in enough calories because of their difficulty in swallowing. As patients lose weight, they are actually burning their own muscle.

Researchers found that a high-kcal, high-carb tube-fed diet was safe and tolerated- better than one designed to main weight or a diet high in fat. Patients on the high-kcal, high-carb diet gained some of the weight they had lost. At the very least, patients should avoid losing weight.

There needs to be larger trials to see if patients should be trying to gain weight. But, until this happens, all patients with ALS should be actively avoiding losing weight.
This study was published in February 2014 and experts report that the results are far from definitive. This study is also much too small to really show a survival benefit from the diets described. A larger trial would be needed to really highlight any problems. Overall, the study, and future studies, would be beneficial if it included more patients.

als

Pro-Stat Supplement

Pro-Stat is a nutritional supplement that I recently became familiar with at my MNT rotations. This supplement is used for patients who are having difficulties gaining weight or consuming energy, in general. This supplement delivers the highest concentration of protein and calories in the smallest serving size. It is ideal for patients with an increased need for protein, have a poor appetite, suffer from pressure ulcers, or who could be experiencing protein-energy malnutrition. Pro-Stat is a complete protein and contains all the essential amino acids while being easy to administer. There is no mixing required and I witnessed the supplement being used with a patient who was on a tube feeding. The patient didn’t seem to mind the supplement, which is rare. A large number of patients who are on tube feedings seem to be very particular about what supplements or formula they are consuming. So, it was to my surprise that the patient who we introduced this supplement to, actually seemed like he enjoyed the protein supplement. Plus, I think it helped that I explained to the patient why we were giving him the supplement and how it would help his health status too.

ProStat