Amyotrophic Lateral Sclerosis

The cause of ALS is unknown. It attacks its victims at random. However, it was recently discovered that five to ten percent of those with ALS show a definite genetic pattern. In this rare form, about one-half of the offspring may develop ALS. These people show a gene defect that affects an enzyme called superoxide dismutate. This enzyme eliminates toxic substances called free radicals. Free radicals can cause nerve cells to die and are associated with a number of diseases and even implicated in aging itself. For most people with ALS, the vast majority of their children are not at any greater risk of developing this disease than the general population. This type of ALS is often called "sporadic ALS" due to its unpredictable nature.

ALS researchers have found no difference between the symptoms and disease progression in the sporadic and genetic forms of ALS. Therefore, since the genetic and acquired forms of ALS appear to be similar, an understanding of the cause of the genetic form could lead to treatment for all forms of the disease.

While there is no cure for ALS, research to solve the ALS puzzle is ongoing. Scientific advances have led to approval of the first treatment for the disease- a medication that may increase survival time. Other treatments under investigation include several nerve growth factors which may help maintain quality of life by maintaining nerve function. While each of these therapies represent a step forward for people with ALS, a cure remains to be discovered.

For the majority of people with ALS, the primary treatment remains the management of ALS symptoms. Patients need to take an active role in the design of their treatment regimen. Ideally, ALS management involves physical, occupational, speech, respiratory and nutrition therapy. For instance, certain drugs and the application of heat or whirlpool therapy may help to relieve muscle cramping. Exercise, however, is recommended in moderation. Drugs also may be used to help combat fatigue, but in some patients may worsen muscle cramps.

As the disease progresses, various assistive devises will help persons with ALS maintain their independence and endure personal safety. For example, an ankle/foot brace can improve function and conserve energy, as well as help avoid injury. When neck, trunk and shoulder weakness makes walking or sitting difficult, cervical collars, perhaps with an additional chest and head strap, provide helpful support. A reclining Chair is preferable to a headrest to relieve fatigue of neck muscles. There are also numerous devises to assist in feeding, dressing and maintaining personal hygiene. Eventually, more substantial equipment, such as wheelchairs, scooters, lifts and hospital beds may be required.

It is important to know that speech therapists can help with speech and swallowing difficulties as they develop. Also, drug treatments can help patients who develop excessive saliva and drooling. Family members of people with ALS should be instructed in the Heimlich maneuver to provide assistance in a life-threatening choking episode. Feeding tubes may be necessary to maintain nutrition, as may breathing devises when the disease affects the muscles of the chest. However, with these supportive devices, there are physical, emotional and financial implications, and their use should be discussed with a physician well in advance of when the need arises. Managing the symptoms is a process that is challenging for people with ALS, their caregivers, and their medical team.

Of all the disabilities that affect a person with ALS, one of the most devastating and most common is the progressive loss of the ability to communicate. However, advances in computer technology mean that persons with ALS today have vital new electronic communications options that can be adapted to their individual capabilities.