Treatments
for ALS
In December 1995 the U.S. Food and
Drug Administration approved the drug riluzole (brand name
Rilutek) for ALS. The drug seems to have a modest effect,
prolonging survival by a few months. It’s the only prescription
drug currently listed for ALS treatment.
Riluzole works by partially blocking
glutamate, a nervous system chemical that carries signals
between cells in the brain and between the brain and spinal
cord. Excess glutamate or abnormal glutamate clearance may
be involved in ALS.
Another glutamate-inhibiting drug
is topiramate (Topamax), which is on the market to treat
seizures. It’s now being tested for possible benefit in
ALS.
Neurotrophic Factors
An additional theme in ALS drug
development has been a group of substances known as neurotrophic
("nerve-nourishing") factors. In laboratory experiments,
these natural nervous system chemicals have been shown to
help nerve cells survive under adverse conditions. But results
of trials of neurotrophic factors conducted in the 1990s
in people with ALS were disappointing. As of 2003, the neurotrophic
factor known as insulin-like growth factor 1 (IGF-1) is
still under investigation.
A drug that increases the body’s
own production of neurotrophic factors, xaliproden, has
shown some positive trends with respect to survival and
disease progression in ALS after preliminary analysis of
data from a large study. Further analysis is under way.
Other Drugs
Other types of drugs being studied
in ALS include celecoxib (Celebrex), which is mainly employed
to treat arthritis. This drug may be able to interfere with
glutamate and with the production of "free radicals,"
toxic chemical by-products of various cellular activities.
It’s known as a COX-2 inhibitor and anti-inflammatory medication.
The antibiotic minocycline (Minocin,
Dynacin) is another subject of study in ALS. This drug can
block caspase enzymes, which are part of a "cell death"
pathway that could be involved in ALS.
Other researchers are studying tamoxifen
(Nolvadex), which is used in breast cancer therapy. Preliminary
evidence suggests it may be helpful in ALS through its action
against a substance called protein kinase C.
In addition to these prescription
drugs, many physicians recommend various over-the-counter
substances, including vitamin E, coenzyme Q10 and creatine.
These compounds have shown potentially modest benefits in
ALS, and studies are continuing.
Symptom Control
Although there are few drugs now
available that substantially influence the course of ALS,
doctors can prescribe several treatments to help control
some of the symptoms of the
disease.
For example, there are medications
to treat muscle cramps and spasms, help clear respiratory
secretions from the bronchial tree, dry up excess saliva
(a problem when mouth muscle weakness results in drooling),
relieve constipation (a problem when abdominal muscles weaken),
and minimize anxiety and depression.
One drug, a combination of dextromethorphan
and quinidine, is being tested for its potential to reduce
unwanted excessive crying and laughing. These reactions,
essentially muscular in nature, are thought to occur in
ALS because of some loss of neurologic control over these
muscles.
Antibiotics can be prescribed to
treat respiratory and other infections. Your doctor will
guide you about flu and pneumonia immunizations.
Therapies and Nutrition
Physical therapy to maintain full
range of motion of the joints and as much mobility as possible;
occupational therapy to help with adaptive equipment and
new methods for accomplishing daily activities; and respiratory
therapy and the prescription of ventilation devices to assist
breathing, may all be involved in the treatment of ALS at
various stages.
Speech therapy and devices that
substitute for speech can help compensate for loss of muscle
control in the mouth and throat.
Maintaining optimal nutrition is
also an important part of sustaining health for as long
as possible. The advice of a nutritionist or dietician,
as well as specialists in swallowing and sometimes a gastroenterologist,
can be helpful for many patients and their families. In
some situations, a feeding tube into the stomach is offered
as an alternative to using weak swallowing muscles, which
pose a choking hazard.
Psychotherapy or counseling to help
the patient and family adjust to a very difficult situation
may become part of the treatment program at different stages
of the illness.
Practice Guidelines
The American Academy of Neurology
has developed a "practice parameter" for physicians
treating people with ALS.
Published in 1999, this set of guidelines
lists standards, guidelines and options for a variety of
treatment issues in ALS, ranging from diagnosis to respiratory
care to pain management.
If you or your doctor would like
to see the guidelines in detail, go to www.aan.com/professionals/practice/pdfs/g10058.pdf.
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