For About and By Caregivers
The Nature of a Beast: Understanding ALS

By  Arleen M. Kaptur


There are so many different situations which can make someone a caregiver, like a loved one getting into a horrible accident and being rendered immobilized. The years can begin to take a toll on the mind and body, and help is needed with the simplest of things. Then there are the ravages of a horrific disease, ALS (Amyotrophic Lateral Sclerosis), also known as “Lou Gehrig’s Disease” in the United States (after the baseball great), MND (Motor Neurone Disease) in England, and Maladie de Charcot in France, which robs mobility and is eventually fatal for those who are in the prime of their lives. ALS is a neuromuscular disease which begins as muscle weakness, and then slowly progresses into total and complete paralysis throughout the entire body. ALS becomes a bit easier to understand when it is broken down by its Greek meaning:                               

  • A—without
  • Myo—muscle
  • Trophic—nourishment
  • Lateral—side (of the spinal cord)
  • Sclerosis—hardening or scarring

 Although ALS was first described by French neurologist Jean-Martin Charcot as long ago as 1869, there is still no cure for this devastating disease. About 50 percent of ALS patients die within the first 18 months of diagnosis, with 20 percent of ALS patients surviving five years; however, people who go on a ventilator to help them breathe may be able to live for many years. The average age for onset seems to be around 55, but people as young as 12 and as old as 98 have been diagnosed. Around 80 percent of all ALS cases usually begin between the ages of 40 to 70. In recent years, more and more people in their 20s and 30s are being diagnosed with ALS. This terrible disease does not usually discriminate between sex or race; however, men are more likely to be diagnosed than women. The cause of ALS is still unknown, but environmental factors are a strong, suspicious factor as a possible cause, with a higher incidence among people who have been exposed to agricultural chemicals and solvents. Whatever the cause, medical researchers have discovered that an excess amount of a neurotransmitter called glutamate is produced, clogging the synapse nerve cells, preventing transmission of neural impulses. This clogging of the synapses causes eventual death of these nerve cells, resulting in motor neuron damage and muscle atrophy (shrinking), specifically to nerve cells in the brain and spinal cord that control voluntary movement. The motor nerves that are attached to muscles gradually degenerate and die from lack of receiving nerve impulses, and as a result of this nerve death, the muscles atrophy and waste away. By the time someone with ALS first notices neurological symptoms, more than half of the motor neurons may already be dead. Muscle paralysis is the end result of this type of nerve death.

 Symptoms of ALS may include tripping, stumbling and falling, loss of muscle control
and strength in hands and arms, difficulty speaking, swallowing and/or breathing, chronic fatigue, and muscle twitching and/or cramping, and drooling. Both upper and lower motor neuron damage is experienced, with the symptoms of upper motor neuron damage resulting in stiffness, muscle twitching, and muscle shaking, and the symptoms of lower motor neuron damage including muscle weakness and muscle shrinking (atrophy). The most common path for ALS to take first is to weaken either the arms or legs, making this known as “limb-onset” ALS. While the symptoms of ALS are not generally painful, some of  the effects may be, such as pressure sores, muscle cramps, joint contractions, constipation, burning eyes, swelling feet, and muscle aches. Surprisingly, there are a few muscles that remain untouched by ALS, enabling people to retain their bowel, bladder, and sexual functions. In a cruel twist of fate, the mind also remains unaffected and remains quite sharp, even when there is total paralysis. Eating can also become more difficult as muscles begin to weaken, with a loved one’s diet including softer foods and the eventuality for a feeding tube, but this can greatly extend the quality and quantity of life.

 Three types of ALS exist, with the most common type called sporadic ALS. This type
is not hereditary, but does account for about 90 percent of all ALS cases. Only about 10 percent of ALS is recognized as hereditary (familial ALS), which is passed on by a dominant gene. The third type of ALS is called “Guamian,” named for the unusually high incidence of ALS on the island nation of Guam. Diagnosis is made usually through a process of elimination and can take several weeks or even months to detect and confirm existence, since early symptoms can mimic many other neuromuscular diseases. The usual diagnostic tests include MRI (magnetic resonance imaging), EMG (electromyogram), muscle biopsy, and blood tests. A definitive diagnosis of ALS can only be made when the existence of damage is evident in both upper and lower motor neurons, and at least three limbs are
sufficiently affected.

 When it comes to treatment, the FDA approved a drug called Rilutek, which appears to slow the progression of the disease. There are also several other medications that can help with the relief of symptoms, along with different therapies, vitamins, antioxidants, and proper nutrition, which are included as part of a treatment plan. In recent years, the over-the-counter supplement creatine seems to be effective in preventing ALS in studies using mice.

 The unfortunate truth at this point and time regarding ALS is that it is almost always fatal, with the majority of patients dying painlessly and peacefully in their sleep, because of the build-up of carbon dioxide in the blood, caused by insufficient amounts of oxygen due to the shrinking of muscles which help with breathing. But there is also some good news, with the past five years seeing more research conducted than in the last 150 years since its discovery. Studies and research for other neurological diseases such as Parkinson’s are shedding new light on how to possibly treat ALS. Also, many loved ones do not have to remain in bed even though they may be totally paralyzed. Thanks to the improvements of modern technology, specially designed wheelchairs are lightweight can accommodate portable ventilators, allowing for loved ones to enjoy a lot more freedom and movement than in prior years. The most important thing that loved ones and their caregivers must remember is that they need to surround themselves by a strong network of support through family, friends, and the medical team. Look to the future, because there are hundreds of strides being made every day in research for finding better treatment, as well as to attaining the ultimate goal ... a cure.

The ALS Association

Les Turner Amyotrophic Lateral Sclerosis Foundation

Muscular Dystrophy Association (MDA)
ALS Division

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