In the USA it is known as as Amyotrophic
Lateral Sclerosis (ALS) or Lou Gehrig's Disease - named for a famous American baseball player whose progression through its debilitating symptoms proved heart-wrenching for an adoring public.
ALS or motor neuron disease is a neuromuscular
disease resulting in the body gradually losing control of the muscles.
Nerve cells in parts of the brain and spinal cord degenerate, causing
voluntary muscles to cease responding.
For this reason, ALS is also known as "the cruelest disease" since it lays waste to the body while the brain remains alert as ever to what is happening.
As the disease progresses
people, with ALS lose the ability to move their arms and legs.
Physical therapy and assistive devices allow patients to remain
mobile even after the disease begins to affect their limbs.
Eventually, speech muscles
are also affected, and speech therapy or speech synthesizers and
computers are helpful in keeping communication channels open. In
the final stages of this disease muscles needed to breathe stop
working, and respirators may be used to maintain lung function for
as long as possible.
There are two
forms of ALS. Familial
ALS is passed genetically. This type effects less than ten
of every one hundred people who develop Lou Gehrig's disease. If
you have two or more relatives with ALS, you may have an increased
chance of getting Amyotrophic Lateral Sclerosis. The more common
sporadic ALS is not a genetically inheritable disease, but there
is still very little known about what causes people to develop this degenerative
ALS occurs 20 percent more frequently in men than in women.
Stephen Hawking, diagnosed
ALS in 1963.
ALS first gained
widespread public attention in the United States in 1939, when Hall
of Fame baseball player Lou
Gehrig of the New York Yankees was diagnosed -- which is why
many people know it today by the name of Lou Gehrig's disease. Yankee pitcher "Catfish" Hunter also later died of the same disease in 1999.
Other famous people with an ALS diagnose and later succumbed to the disease include Irish-American singer Dennis Day, British actor David Niven, and Chinese leader Mao Zedong.
Hawking has been living
with ALS for many decades while producing some of the most brilliant
theories in his field. He was first diagnosed in 1963.
is unusual for a person diagnosed with ALS to survive for
many years after the diagnosis, it is possible. About 10 percent
of those with motor
neuron disease survive for 10 or more years. Early diagnosis
is difficult, but the earlier treatments can begin, the better.
While no cure
for ALS is on the horizon, there have been advances in the treatments
and medications used to slow the progress of the disease. Riluzole is the primary drug used to slow the progress of ALS while many
other drugs, such as Myotrophin (somatomedin C), are currently being
used or tested in clinical trials.
genetic testing for ALS has been initiated among patients with the familial
type of ALS and related ALS stem cell research also looks promising, but definitive
studies will take years.
People who are diagnosed with Lou Gehrig's disease can look forward to slowing down the disabling effects of Amyotrophic Lateral Sclerosis and living much longer than was the case even ten years ago. Support groups for patients and caregivers can also provide ideas for successfully coping with the changes in lifestyle and the emotional stress of living with increasingly reduced mobility and a terminal illness...
More about ALS around the Web:
The ALS Association - Information on advocacy for ALS, clinical trials, stories of personal courage in dealing with this disease, the latest research plus facts for the patient and professionals are just the beginning of the resources you'll find on this site.
International Alliance of ALS/MND Associations
- This is the home page of an international group dedicated to funding research to find better ways to treat this disease and raising public and governmental awareness of the need for research. The links and informational resources are up to date, extensive and cover many countries in several languages.
ALS Society of Canada - Lou Gehrig's Disease - The focus is Canada but the resources are international and geared towards all ALS patients and their families. Latest news and research plus advocacy and support are all covered. A Guide to ALS Patient Care For Primary Care Physicians is geared to professionals to help familiarize them with symptoms and bring about an earlier diagnosis and better treatment.
ALS Fact Sheet - Complete FAQ including information on causes, symptoms, diagnosis, treatment options and ongoing research, with related resources.
Clinical Trials. gov Amyotrophic Lateral Sclerosis
- US government site listing clinical trials. Some are currently recruiting and others have not started yet. This is a good place to find out what the current research is and to obtain access to experimental treatments.
is intended as reference and not as medical advice.
All treatment decisions should be made by medical professionals.