Amyotrophic lateral sclerosis

Riluzole and endaravone are the two therapies approved by the United States Food and Drug Administration (FDA) specifically for the treatment of amyotrophic lateral scelerosis (ALS). Riluzole has been available in tablet form for over twenty years. It is believed to reduce damage to motor neurons. Riluzole may increase survival by several months and also extend the time before mechanical breathing support (ventilation) is needed. Riluzole does not reverse damage already done to motor neurons. The tablet form is available in generic versions and has been approved by the drug regulating agencies of most countries.^[1][2] As of 2018, a liquid form, Tiglutik, has been approved by the FDA, which allows those who have a hard time swallowing a tablet to continue treatment.^[3] 

Edaravone (Radicava) has been available in the United States since 2017. It is an antioxidant and may slow the decline of physical function in some people with ALS. Physical function is measured by ALS Functional Rating Scale-Revised (ALSDRS-R). This scale measures problems with speech, swallowing, and breathing, as well as daily functioning such as walking, holding items like a pen or fork, dressing, and general care of oneself, like bathing. Edaravone does not improve function that has already been lost. The clinical trials took place in Japan and were six months long. As of 2018, long term effects of treatment with edaravone on physical function, survival, and quality of life are not known. It can be used along with riluzole. Edaravone is delivered by IV infusion on usually a two week on, two week off cycle. The IV infusion takes about one hour and is initially given in a hospital or clinic setting, but many have been able to transition to having the IV infusions at home.^[1][2] Edaravone is also approved by regulating agencies in Japan and South Korea, and is waiting for approval in Canada, Switzerland, and the European Union as of 2018.

Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients. This type of care is known as palliative care, supportive care that is typically provided by multidisciplinary teams of health care professionals such as physicians, pharmacists, physical therapists, occupational therapists, speech therapists, nutritionists, social workers, and home care and hospice nurses. These teams along with the patients and caregivers can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible.^[4]

• Physicians: Can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm. Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation.
• Pharmacists: Can give advice on the proper use of medications and monitor a patient's prescriptions to avoid risks of drug interactions.
• Physical therapist: Can provide physical therapy and recommend special equipment to help the patient be independent and safe during the course of their ALS.
• Occupational therapists: Can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile.
• Speech therapists: Can provide speech therapy to those people with ALS who have difficulty speaking.
• Nutritionists: Can help teach people with ALS and their caregivers how to plan and prepare numerous small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow. 
• Social workers and home care and hospice nurses: Help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease.
• Respiratory therapists: Can help caregivers with tasks such as operating and maintaining respirators.
• Home care nurses: Are available not only to provide medical care but also to teach caregivers about giving tube feedings and moving patients to avoid painful skin problems and contractures.
• Home hospice nurses: Work in consultation with physicians to ensure proper medication, pain control, and other care affecting the quality of life of patients who wish to remain at home.

Some research has indicated that use of riluzole has prolonged survival amongst individuals with bulbar-onset ALS, but not in subjects with limb-onset ALS.  It has also been demonstrated that in patients over age 70, riluzole treatment is associated with a longer median survival time and a reduction in mortality rate regardless of the site of the onset of symptoms.^[5] Bulbar-onset patients appear to particularly benefit from riluzole for unclear reasons.^[6]

Proper nutrition and a balanced diet are essential for individuals with ALS to maintain their weight and strength.^[7]  Nutritional management, which has been shown to improve prognosis, has become a focus in managing the disease.^[8]

There is currently limited information on whether specific diets may affect the progression of ALS.  The U.S. National Institutes of Health, through the National Library of Medicine, developed ClinicalTrials.gov to provide patients, family members, and members of the public with current information on clinical research studies.  Currently, 4 clinical trials are identified as enrolling individuals with ALS to gain more information on the use of dietary supplements.  To find these trials, click on the link above.