Lou Gehrig’s disease, or ALS, is debilitating, degenerative, and incurable. There are several options in managing this disease’s progression.
What is Lou Gehrig’s Disease (ALS)?
Amyotrophic Lateral Sclerosis (ALS) is commonly known as Lou Gehrig’s disease. It is sometimes referred to as Motor Neurone Disease (MND). It is a disorder of the motor neurons and leads to muscle spasticity and atrophy, difficulty speaking and swallowing, trouble with breathing, and progressive weakness. All voluntary movement can cease due to the degeneration of the “upper and lower motor neurons” and muscle weakness. The parts of the body often spared in ALS are bowel, bladder, and eye control, as well as cognitive functioning. The autonomic nervous system and sensory nerves are also typically not affected. This means that ALS patients can still hear, see, smell, touch, and taste. Symptoms start with muscle atrophy and weakness as well as stiffness, cramping, twitching, and slurred speech. The limbs may show the onset first, with awkwardness, tripping, a “dropped foot”, or trouble with manual dexterity, for example. Other patients may have a “bulbar onset”, with swallowing and speech problems. Some patients may have “pseudobulbar affect”, where uncontrollable emotional outbursts occur. No matter how the disease starts or spreads, patients will generally all be unable to use their limbs, speak, swallow, and breathe properly on their own. Most ALS patients will end up dying from pneumonia or respiratory failure. Lou Gehrig’s disease, progresses, generally, within about 39 months. Rarely do patients live longer than 10 years, with Stephen Hawking, a physicist, living for over 50 years with the disease.1
Possible Causes
ALS tends to run in families. There are several genetic mutations that are thought to associate with ALS types. Mutations in a gene that produces a particular enzyme (SOD1), an antioxidant that protects from free radical damage, may be linked to the disease. 90% of the cases don’t have a familial link. There are
many suggested causes of ALS, including military service, contact sports, and head trauma. Blue-green algae contamination of food can also be a factor. Many ALS patients also have more glutamate in their spinal fluid. Glutamate is a neurotransmitter in the brain.2 While many factors are being explored in their links to ALS, there is new research that suggested that there may be a common cause to all of the variants of Lou Gehrig’s disease. Northwestern University researchers have found that patients with inherited and non-inherited ALS share an “inability of a protein system (ubiquilin2) to repair the nerve cells that tell the muscles what to do”. When ubiquilin2 doesn’t do its job, then damaged proteins build up in the spinal cord and brain. These accumulated proteins look like “twisted balls of yarn”.3
Treatment and Support
There is no cure or treatment of ALS that can reverse or stop the disease. There is only one medication, riluzole, that can slow the progression of ALS. Patients can also have support therapies to help them maintain some independence and “prolong survival”.4 Teams of health care professionals that can support ALS patients include physical, speech, and occupational therapists, nutritionists, and hospice care. Various medications can be prescribed by physicians to ease symptoms, and pharmacists can assist in monitoring drug interactions. Some exercises, such as walking, stationary cycling, and swimming can help, as can range of motion stretches and exercises. Patients may also require a BIPAP machine or other respiratory assistance, or even a tracheostomy (a breathing tube inserted into the windpipe).5
References:
1,2 http://en.wikipedia.org/wiki/Amyotrophic_lateral_sclerosis
3 http://www.webmd.com/brain/news/20110822/common-cause-of-lou-gehrigs-disease-found
4 http://www.alsa.org/about-als/what-is-als.html
5 http://www.medicinenet.com/amyotrophic_lateral_sclerosis/page5.htm#how_is_als_treated
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