Straight, No Chaser: ALS (aka Lou Gehrig's Disease) – The Reason for the Ice Bucket Challenge

Neurology, , , ,

ALS icebucketchallenge

It is a beautiful thing to see the national craze of folks bringing attention to ALS (amyotrophic lateral sclerosis, aka “Lou Gehrig’s Disease) via the ice bucket challenge. However, I have to ask… Do you have any idea what the disease is? In the overwhelming likelihood that you do not, this Straight, No Chaser is going to provide you with a few facts about ALS, so you can at least know why you’re getting all wet. Oh yeah, and thank you. The efforts and donations are meaningful. In the last month as much has been raised for combating the disease as was raised all of last year. Keep it going.
ALS is actually a very complicated disease for the layperson to understand (and it’s even worse for the 12,000 or so Americans with definitive diagnosis), so I’ll break it down in five questions.

 ALS Lou

What is ALS?
The medical description of ALS is a progressive neurodegenerative disease that affects those nerve cells in the brain and in the spinal cord that control voluntary muscle movement and power. Decomposing that medical jargon, this disease
1) creates a dysfunction of certain areas of the brain and nervous system and the associated nerves and muscles controlled by them,
2) worsens over time, and
3) it often culminates in irreversible deterioration and loss of function in the affected organs or tissues.

 als hawking

So what’s the dysfunction?
Remember that the brain controls the activities of the rest of the body. If nerve cells in the brain and spinal cord are dying, your functions associated with those cells go away. In ALS, based on the type of cells affected (motor neurons), what is affected results in a loss of the ability of the brain to initiate and control muscle movement.
Does ALS involve all muscles?
No. There are organs and tissues of the body (most notably the heart and digestive system) that aren’t under voluntary control. These areas are spared from the effects of ALS.

 ALS symptoms

What are the symptoms?
Since the brain can’t control those muscles under your voluntary control, symptoms are somewhat predictable and include the following.

  • Early symptoms often include increasing and widespread muscle weakness. This can include the arms and legs and also those muscles allowing you to speak, swallow and breath.
  • Additional symptoms include thinning of the arms and legs resulting from lack of muscle use.
  • Eventually, paralysis can occur. Paralysis of muscles involved with swallowing and breathing can lead to a rapid death.

What’s the treatment for ALS?
There is no cure for ALS. There is also no treatment that halts or reverses progression. However, there is a drug named riluzole that modestly slows the progress of ALS. The remainder of treatment efforts involves providing episodic relief of symptoms.
This is why your contributions are of value. There is ongoing research that is promising. Even if you don’t take the ice bucket challenge, you can still make a difference. Go to www.alsa.org  to learn more.
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