Amyotrophic lateral sclerosis (ALS) and nutrition
Fortunately, in my career I have only come across a few patients diagnosed with amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, after the famous New York Yankees baseball player who died in 1941.
However, the impact of ALS has hit closer to home for me after having an uncle diagnosed with it. Much remains medically unknown about this disease, but it is estimated that five to 10 per cent of patients get ALS due to heredity. It is a progressive nervous system disease that affects the nerve cells in the brain and spinal cord, resulting in loss of muscle control. Eventually, it affects mobility, speech, nutritional intakes and breathing. Unfortunately, it remains incurable but there are some promising treatments under study.
Symptoms of ALS include difficulty with walking and the basic activities of daily living, such as dressing brushing teeth, etc. These eventually lead to tripping and falls, muscle weakness, slurred speech, dysphagia (difficulty swallowing), muscle cramping, and cognitive and behavioral changes. ALS causes nerve cells (motor neurons) to die, and the brain and spinal cord cannot send messages to muscles. The complexity of the nervous system makes treating and managing ALS very difficult. ALS is most common in those 40 to 60 years of age and affects men more than women. A multidisciplinary, team-based approach is required to manage ALS. This includes not only MDs but also dietitians, speech language pathologists, occupational therapist, physiotherapists, and social workers.
In terms of nutrition, it is important to focus on combating malnutrition, dehydration, reducing the risks of aspiration pneumonia (food going into the lungs) and choking. Texture-modified diets are often required, along with nutritional supplementation. Many patients will go for swallowing tests such as a video fluoroscopy to see what type of diet texture is required. As the disease progresses, many will be unable to swallow, and an enteral feed may be considered. Also known as a tube feed, this is where a tube is inserted into the stomach or small intestine and a nutritional formula is delivered via a pump feeding system. Medications can also be delivered via the feeding tube. A registered dietitian will calculate the amount of formula required based on height, weight, lab data, calorie, protein, and hydration needs, etc. Enteral feeds can be flexible with both intermittent and continuous feeding times based on the individual’s preferences and routines.
New medications are in trial that can slow the progression of ALS but many of these remain experimental. It is important to focus on quality of life, as my aunt and uncle have done by being as active as possible, spending time with their adult children (and their pets), remaining positive, leaning on family members for support and utilizing the community resources that have been very helpful to them such as the ALS Society of Manitoba and the Movement Disorder Clinic at Deer Lodge Centre.
Charleswood community correspondent
Lisa Lagasse is a registered dietitian and community correspondent for Charleswood. Email her at Charleswoodres@gmail.com or find her on Twitter: @LisaRD42324393