Amyotrophic Lateral Sclerosis: Treatment

There is no cure for ALS, and no treatment that can significantly alter its course. There are many things which can be done, however, to help maintain quality of life and to retain functional ability even in the face of progressive weakness.

As of early 1998, only one drug had been approved for treatment of ALS. Riluzole (Rilutek) appears to provide on average a three-month increase in life expectancy when taken regularly early in the disease, and shows a significant slowing of the loss of muscle strength. Riluzole acts by decreasing glutamate release from nerve terminals. Experimental trials of nerve growth factor have not demonstrated any benefit. No other drug or vitamin currently available has been shown to have any effect on the course of ALS.

A physical therapist works with an affected person and family to implement exercise and stretching programs to maintain strength and range of motion, and to promote general health. Swimming may be a good choice for people with ALS, as it provides a low-impact workout to most muscle groups. One result of chronic inactivity is contracture, or muscle shortening. Contractures limit a person's range of motion, and are often painful. Regular stretching can prevent contracture. Several drugs are available to reduce cramping, a common complaint in ALS.

An occupational therapist can help design solutions to movement and coordination problems, and provide advice on adaptive devices and home modifications.

Speech and swallowing difficulties can be minimized or delayed through training provided by a speech-language pathologist. This specialist can also provide advice on communication aids, including computerassisted devices and simpler word boards.

Nutritional advice can be provided by a nutritionist. A person with ALS often needs softer foods to prevent jaw exhaustion or choking. Later in the disease, nutrition may be provided by a gastrostomy tube inserted into the stomach.

Mechanical ventilation may be used when breathing becomes too difficult. Modern mechanical ventilators are small and portable, allowing a person with ALS to maintain the maximum level of function and mobility. Ventilation may be administered through a mouth or nose piece, or through a tracheostomy tube. This tube is inserted through a small hole made in the windpipe. In addition to providing direct access to the airway, the tube also decreases the risk aspiration. While many people with rapidly progressing ALS choose not to use ventilators for lengthy periods, they are increasingly being used to prolong life for a short time.

The progressive nature of ALS means that most persons will eventually require full-time nursing care. This care is often provided by a spouse or other family member. While the skills involved are not difficult to learn, the physical and emotional burden of care can be over-whelming. Caregivers need to recognize and provide for their own needs as well as those of people with ALS, to prevent depression, burnout, and bitterness.

Throughout the disease, a support group can provide important psychological aid to affected persons and their caregivers as they come to terms with the losses ALS inflicts. Support groups are sponsored by both the ALS Society and the Muscular Dystrophy Association.

— L. Fleming Fallon, Jr., MD, DrPH