Amytropic Lateral Sclerosis (ALS) is a fatal, progressive, neurodegenerative disorder that strikes one in every 100,000 Americans. ALS is also known as Lou Gehrig’s disease, in recognition of the famous baseball player who died from the disease in the 1930s.ALS causes the motor neurons in the brain and spinal cord to die. As a result, muscle use and control are lost. ALS does not affect the ability to think or reason, so the patients are aware of their suffering throughout the course of the disease. ALS usually occurs in middle age. ALS causes death within two to ten years of its onset with a usual duration of survival of three to five years.
As the nerves controlling muscles shrink and disappear, the muscle tissue deteriorates. Subsequently, muscle strength and coordination decrease. The progression of the disease begins with the voluntary muscles, and usually focuses on a muscle group, like the hips and thighs, or the shoulders and arms. The onset is gradual, and progressively worsens. As the disease progresses, more muscle groups deteriorate. Semi-voluntary muscles that are used for breathing and swallowing begin to deteriorate. As muscle use deteriorates, the patient loses the ability to care for himself, and risks death from respiratory distress, aspiration, or pneumonia.Other symptoms of ALS include paralysis, painful muscle cramps, difficulty in swallowing and breathing, hoarseness, speech impairment, urinary frequency, and muscle atrophy.
There are no definitive screening tests although certain laboratory tests, electromyography, and muscle biopsies may help in the diagnosis.
A genetic link exists for about 2 percent of ALS sufferers; however, the causes of ALS for the remaining 98 percent remain a mystery, although certain factors have been implicated in the cause of the disease, such as environmental (heavy metals), viral and possibly autoimmune causes. People with a family history of ALS can undergo genetic counseling to determine their risk.No treatments exist to reverse or halt the progression of ALS. Rilutek (Riluzole) is a drug that slows ALS progression and extends life for an average period of three months. However, it is very expensive— a 30-day supply costs six hundred dollars. Other drugs have been tried in this disease to try to decrease progression or extend survival but in controlled clinical trials, there were no benefits to these drugs. Antioxidants are now being studied in clinical trials and interestingly the use of Vitamin E, and antioxidant was proposed 60 years ago. Coenzyme Q10 is currently undergoing trials. At this time, the use of the antioxidants such as Vitamin C, Vitamin E and betacarotine are used and will probably not do harm in supervised dosage settings.
Most drug therapy aims to treat the symptoms that accompany ALS. Drug exist to ease depression, muscle cramps, stiffness, excessive salivation, and in later stages, breathing.
A variety of therapy aids everyday living with ALS. Physical therapy helps the patient’s mobility. Although it cannot restore muscle function, it can help with joint movement, and help the patient’s family learn how to move the patient in and out of bed. Occupational therapy is similarly helpful, as it helps patients and their families work together to help the patient be as independent as possible in their environment.
Speech therapy and swallowing therapy are crucial to helping patients utilize their abilities and to prevent aspiration and choking, two common dangers of ALS.
Finally, a respiratory therapist helps assess the patient’s breathing abilities and make decisions on respiratory support devices and life support.
