Figure. PHOTO COURTESY CATHY WOLF; PHOTO ILLUSTRATION: ANTHONY WING KOSNER/WING AND KO.
The American Academy of Neurology (AAN) has just issued guidelines for the care of people with amyotrophic lateral sclerosis (ALS). According to lead author Robert G. Miller, M.D., of California Pacific Medical Center, “While we are waiting for a cure, people need to know that a lot can be done to make life easier and longer for people with ALS.”
Also known as Lou Gehrig's disease, ALS is a degenerative neurological disease in which motor neurons that control voluntary movement mysteriously die. Death often comes by respiratory failure. Although most people die within two to five years from the onset of symptoms, 10 percent survive for 10 years or more. I am one of them.
Dr. Miller and his team scoured medical journals for information on the treatment of ALS and then graded the studies for the quality of evidence they produced. Here are the recommendations:
You should be offered riluzole. Riluzole is the only drug approved by the Food and Drug Adminstration (FDA) for ALS. It extends life by two or three months. The guidelines recommend riluzole regardless of disease stage because, as Dr Miller explains, there is insufficient evidence to say who would benefit from it. Clinical trials typically exclude anyone with symptoms longer than five years duration, and participants must have a forced vital capacity (FVC) of 50- to 60-percent of normal. As a result, there is little data about how riluzole affects people in the later disease stages. (An FVC of 50 percent means your breathing capacity is 50 percent of the capacity of a healthy person of your age, sex, and height.)
If you have trouble swallowing and chewing, you should be offered a feeding tube. Because the insertion of the tube is done under sedation, people should have the procedure done when their forced vital capacity is above 50 percent of normal. Although the tube has been shown to stabilize weight or cause a small gain, there is no information on how much it prolongs life.
You should be offered noninvasive ventilation at the earliest signs of respiratory insufficiency. Noninvasive ventilation consists of an assisted breathing device, often a BiPaP, that stays outside your body. Invasive ventilation, on the other hand, requires a surgical procedure known as tracheostomy. Noninvasive ventilation slows the rate at which FVC drops and prolongs life. One study found quality-of-life benefits such as decreased fatigue, clearer thinking, and less daytime sleepiness.
If you have a weak cough, you should be offered an assisted cough device. As the respiratory muscles weaken, it becomes harder to clear secretions from the upper airways and lungs. If these secretions linger, they may become a source of infection. A device such as the Cough Assist™ may be helpful in clearing secretions.
You should attend a specialized multidisciplinary ALS clinic. A multidisciplinary ALS clinic includes specialists in neurology, pulmonology, speech and swallowing, nutrition, and social services. These specialists work together to provide coordinated care for the patient. Studies show that compared with a nonspecialized neurology clinic, patients attending an ALS multidisciplinary clinic were more likely to use riluzole, have a feeding tube, and employ noninvasive ventilation. As a consequence, they survived longer and had a better quality of life.
If you have excess saliva that doesn't respond to oral medications, you should be offered botulinum toxin. Excess saliva in combination with facial muscle weakness can cause drooling. If oral medications don't work, the same botulinum toxin that is used for cosmetic effect can be used on the salivary glands to reduce excess saliva. The treatment is expensive, and some insurance companies may not cover it.
You should be screened for cognitive and behavioral impairments. Estimates of the percentage of patients with cognitive or behavioral impairments range widely. A small number of people with ALS develop frontal temporal lobe dementia. Such patients often refuse life-prolonging interventions. The guidelines say nothing about the consequences of milder cognitive or behavioral impairments.
The authors found no or insufficient information about many important subjects, such as how to break the news of a diagnosis of ALS. Many of my ALS friends saythey were given no hope. While the doctor must convey the seriousness of the diagnosis, in my opinion, she should also state that there are ways of prolonging and improving the quality of life. There are also no guidelines on relieving constipation, spasticity, cramps, anxiety, insomnia, depression, or on the assessment of communication strategies.