Amyotrophic Lateral Sclerosis: Part 1 of 2
By Ruth Werner
Amyotrophic Lateral Sclerosis: Part 1 of 2
By Ruth Werner
"Fans, for the past two weeks you have been reading about the bad break I got. Yet today I consider myself the luckiest man on the face of the earth."
- Lou Gehrig, July 4, 1939
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"I am quite often asked: How do you feel about having ALS? The answer is, not a lot. I have had motor neurone disease for practically all my adult life. Yet it has not prevented me from having a very attractive family, and being successful in my work. This is thanks to the help I have received from Jane, my children, and a large number of other people and organizations. I have been lucky that my condition has progressed more slowly than is often the case. But it shows that one need not lose hope.
- Stephen Hawking
Physicist and author of A Brief History of Time
In my last article I put out a call for massage therapists who work with Amyotrophic Lateral Sclerosis (ALS) patients to get in touch with me. I hoped to share some of their stories with Massage Today readers. Well, the response was amazing. I have lots of information to share, both on the development and latest research into this disease (part 1), and on what therapists are doing to help improve the quality of life of their clients who have it (part 2).
Amyotrophic Lateral Sclerosis: What Is It? Also known as Lou Gehrig's Disease, ALS is a rapidly progressive, irreversible condition that destroys motor neurons in the central and peripheral nervous systems, leading to the atrophy of voluntary muscles. ALS usually affects people between 40 and 70 years old; the average age at diagnosis is 55. It can occur in higher rates within families, but the distribution and incidence of this disease is usually random. Men have it slightly more often than women. Approximately 20,000 people in the U.S. are living with ALS at this time, and life expectancy between diagnosis and death for most patients is two to 10 years.
Etiology: What Happens? The primary feature of ALS is the destruction of motor neurons. In most cases, it begins in the anterior horn cells and the descending tracts in the spinal cord. Large motor axons degenerate and are replaced with fibrous astrocytes, a type of glial cell. This is the derivation of the name of the disease: "lateral sclerosis" refers to the scarring of the motor tracts on the lateral aspects of the spinal cord. Without stimulus from the upper motor neurons, lower motor neurons (that take messages from the spinal cord to the neuromuscular junction) atrophy. Ultimately, the skeletal muscles deteriorate without sufficient motor stimulus: "amyotrophic" refers to muscle wasting.
While we have a reasonably clear idea about how this disease progresses and changes function, its causes are still unknown. At this point in time, the features of ALS that are being intensively studied include abnormal glutamate levels (this is an excitatory neurotransmitter that accumulates in the synaptic cleft, killing the affected neurons); exposure to neurotoxins (including lead, agricultural chemicals and others); free radical activity (this is an issue especially when ALS runs in families); a deficiency of neurotrophic factors (these are chemicals that allow for healing or new growth of nerve tissue); and a new avenue of inquiry: Almost half of all ALS patients show signs of retroviral exposure. Further explorations of these factors may eventually open new doors to effective treatments to stop or even reverse this mysterious disorder.
Signs and Symptoms. ALS presents different early symptoms in different people. The most common pattern is stiffness, weakness and awkwardness in one body part, which slowly spreads to other parts of the body. About two-thirds of ALS patients have their first symptoms in the arms or legs; these cases are called spinal ALS. The final third will begin with "thick speech," excessive salivation, and difficulty with swallowing. This implies damage to cranial nerves rather than spinal nerves and is called the bulbar form of ALS. Fasciculations, or visible muscle twitching, may be present, along with painful cramping. One side is typically worse than the other, and the stiffness eventually moves proximally up the limbs, eventually to affect the trunk muscles for breathing. The leading cause of death for ALS patients is respiratory failure.
The nerve damage seen with ALS is to motor neurons only; sensory neurons are not affected. This can be a painful disease, however, as the body gradually collapses and gravity puts musculoskeletal stresses on muscles that have no power to respond. ALS does not influence intellectual capacity at all. While depression and anxiety are certainly a part of the process, the disease itself does not affect cognition or awareness.
How Is It Treated? Traditionally, treatment for ALS has been strictly palliative; that is, aimed at managing the severity of the symptoms only. Recently, some medications have been developed that can slow but not stop or reverse the progress of the disease. Other interventions include drugs for muscle spasms, along with moderate exercise and speech, and physical and occupational therapy to maintain muscle strength as long as possible. (This turns out to be a delicate balance. Too little exercise allows muscles to degenerate; too much exercise puts a dangerous demand on low-functioning neurons - which can also cause muscles to degenerate.)
Assistive devices such as leg braces, arm braces or wheelchairs can improve a patient's ability to function. In advanced cases, swallowing may be so difficult that the insertion of a stomach tube (gastrostomy) may be recommended. Since this disease does not impede cognitive or emotional processes at all, psychological therapy for ALS patients and their families is an important part of the treatment plan.
Massage? We'll get into specific ideas about massage techniques for clients with ALS in part 2. In the meantime, bear in mind that this is a disease that involves the degeneration of motor neurons but not sensory ones, and the client is fully aware of the changes occurring in his or her body. This combination of factors makes massage a great choice for many ALS clients because sensation is intact and the client can communicate (until he or she is very advanced) about how the massage feels and what is needed. Massage therapists who work with a client who has ALS are probably going to be working with someone in the process of dying. What a gift and privilege to be invited into this holy process.
I'm still accepting communications from therapists about their experiences with clients who have ALS - please share your wisdom with other readers through this column.
Until then, many thanks, and many blessings,
Ruth Werner, LMP, NCTMB
Editor's note: Read part 2 of Ruth Werner's article in the Sept. issue.