What is ALS?
Amyotrophic Lateral Sclerosis (ALS), sometimes referred to as "Lou Gehrig's Disease", or Motor Neuron Disease (MND)" is a progressive fatal neuromuscular disease that attacks nerve cells and pathways in the brain and spinal cord. Motor neurons, among the largest of all nerve cells, reach from the brain to the spinal cord and from the spinal cord to muscles throughout the body with connections to the brain. When they die, the ability of the brain to start and control muscle movement dies with them. With all voluntary muscle action affected, ALS patients in the later stages are totally paralyzed; through it all, however, their minds and intellect remain unaffected.
50% of ALS patients die within 18 months of diagnosis.
80% of ALS patients die within 5 years of diagnosis.
10% live more than 10 years.
ALS occurs in all races and all around the world.
Men get ALS more than women (1.5 to 1.0 ratio.)
After the age of 60 the ratio of men to women is nearly 1 to 1.
The incidence of ALS is 2 per 100,000.
The prevalence of ALS is 8 per 100,000.
The average age of onset is 55 years.
80% of ALS cases begin between the ages of 40 to 70.
How long do ALS patients live?
The standard answer I hear from doctors, books, and Internet sites is "2-3 years" or 2-5 years" or "3-5 years" or "2-7 years" on "average." I then wondered if these "averages" were from the time of the first patient observed symptoms, the first visit to the doctor, or from the time of "official" diagnosis of ALS. I have not seen a consensus of opinion on this matter, although most survival studies use the date of diagnosis.
What does the term "average" mean? Anyone who has studied statistics knows there are three common averages in use: Mean, median, and mode. The median is the most useful in survival statistics because it is the exact midpoint. That is, if you say the median survival of mice with ALS is 21 days, that means that half of the mice with ALS died by the 21st day. I am not sure that all of the statistics floating around are using the median as their "average."
To further complicate matters, some longevity studies define "death" from ALS as the point where the PALS requires ventilatory assistance. The reasoning for this is that the PALS would have died if they had not been placed on a ventilator, and these studies are measuring PALS "natural" life span. Such studies ignore the multitude of PALS on ventilators, many of whom live happy and productive lives.
More confusion to muddy the waters: ALS is difficult to diagnose and some individuals my be incorrectly diagnosed. That is, they may have another neuromuscular disease but be diagnosed with ALS, or they may have ALS but be diagnosed with something else. So there is often pressure from the medical community in cases where PALS have had remission or reversal of symptoms, to state that the PALS must have been misdiagnosed and did not really have ALS, because "everyone knows ALS is invariably fatal usually in 2-5 years."
How old are the statistics being reported about ALS survival? Everywhere you look you see the same expectancy, "2-5 years." The same figures were reported 10 or 20 years ago. Medical procedures, medical thinking about ALS treatment, and assistive technology have changed dramatically over the past ten years, all to the PALS benefit. I would love to see some new studies on longevity.
Treatments for ALS
Treatment of ALS is primarily a process of managing symptoms. As PALS get weaker, their symptoms change, their needs change, and consequently their treatments are always being modified. This material is presented for informational purposes only. Please see your health care professional for further information.
FDA approved drugs to slow ALS progression:
Rilutek is the only drug approved by the US FDA to slow the progression of ALS. Rilutek has been shown to extend life for 3 months in 18 month trials. While the effect of Rilutek is modest, it is a significant development in that is the first ALS drug proven to be effective in over 130 years of research. Rilutek is expensive, over $600 for a 30 day supply, but it is covered under most health insurance policies. NORD (National Organization for Rare Disorders) may be able to assist you with purchasing Rilutek if you do not have insurance.
Experimental drugs to slow ALS progression
The good news about ALS research is that there are a number of drugs currently being studied. I recommend every PALS consider participating in clinical research trials. All clinical trials have an experimental group and a control group. The experimental group receives the active medication and the control group receives the placebo, a pill with no active medication. Neither the doctor nor the patient knows whether or not they are receiving the active medication or the placebo. This is called a "double blind study."
Physical therapy can help with range of motion for the PALS. It cannot restore muscle function but it is necessary to prevent painful contractures or locking of joints. Oftentimes, insurance companies will pay for 4-6 sessions of physical therapy. Make sure the therapist instructs family members on the exercises. It would also be helpful for the therapist to show family members the safest way to do transfers with the patient.
Speech therapy can help PALS adjust to the changes ALS makes to their speech. The goal is to maintain existing speech rather than to improve it. Exercises and helpful suggestions can enable the PALS to make the best use of their remaining voice. The speech therapist also can help with swallowing difficulties.
Swallowing therapy can assist with eating and drinking problems for PALS. It is important to recognize early swallowing difficulties in order to prevent aspiration and choking. The therapist can give specific recommendations on head posture and tongue positioning in order to reduce swallowing difficulties. Changes in the consistency of food are also required as the disease progresses.
Occupational therapy (OT) has the goal of enabling a person to function in their environment with a minimum of effort and as independently as possible. An occupational therapist can come to your home and recommend the placement of kitchen appliances and gadgets to make meal preparation easier for the PALS and the safest way to transfer when toileting and showering. They can also look at furniture placement for ease of movement around the house and devices to assist with using the telephone, computer, home electronics, etc. Often the role of the occupational therapist and the physical therapist overlap when the issue is range-of-motion exercises.
Respiratory therapists are licensed health professionals who use sophisticated diagnostic, therapeutic, and life support equipment. They provide ventilatory life support, administer medical gases and CPR, measure flow rates, pressures, electrocardiograms and arterial blood gases, and develop respiratory care plans.
Respiratory therapy is essential as a PALS' breathing ability diminishes. Breathing ability is measured in vital capacity and PALS should make it a point to have their vital capacity tested on a regular basis. Opinions vary as to when intervention is needed. Some respiratory therapists recommend beginning Bi-Pap therapy when vital capacity dips below 70%. Bi-PaP is noninvasive ventilatory assistance, which insures PALS are receiving sufficient air volume, and rests the lung muscles to provide comfort during sleeping and improve performance during the day. This can be achieved with a nasal mask or nasal pillows that are connected to a mechanical ventilator.
When vital capacity drops below 50%, serious consideration needs to be given to a ventilator.
Here are some recommendations to make breathing easier:
Do not lie down immediately after eating.
Avoid eating large meals which can increase abdominal pressure and prevent the diaphragm from fully expanding.
When sleeping, use a hospital bed or pillows to elevate your head 15 to 30 degrees. This keeps abdominal organs away from the diaphragm.
Proper nutrition is essential for all of us, especially those of us who have an illness. Most Americans are familiar with the food pyramid recommended by the USDA (United States Department of Agriculture.)
Many PALS structure their diet to eliminate as many "processed foods" as possible. This means avoiding foods with preservatives or artificial ingredients, and concentrating on eating fresh, natural foods. This makes good sense, particularly since some fresh fruits and vegetables contain significant quantities of vitamins and antioxidant substances. Protein is very important for PALS and the best sources are meat, fish, eggs and cheese.
"Nutrient dense" foods are what PALS should keep in mind when selecting their meals. Steak and beans both contain protein, but steak is nutrient dense, meaning you can eat much less of it to get adequate protein. Oranges and kiwi fruits both have vitamin C, but kiwis have more, meaning they are more nutrient dense. Another advantage of nutrient dense foods is that you don't have to expend as much energy eating, because you can eat less and obtain the same nutritional value. Plus, eating less may result in the release of fewer free radicals.
Fiber is essential in the PALS diet to prevent constipation, a common occurrence among patients. If you are eating enough fiber and still have problems with constipation, consider using a bulk form of laxative, such as those made with psyllium husk.
At least one research project is looking into the effectiveness of the ketogenic diet. This is a special high fat, low protein, low carbohydrate diet that has been used to treat some forms of childhood epilepsy. The researchers believe that the ketogenic diet produces fewer free radicals and want to see if this diet will slow the rate of ALS progression. Such a diet goes against all conventional nutritional recommendations. PALS should always seek the advice of their physician before making any dietary changes.