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Tuesday, April 3, 2012

Neurology: Day 16

I wanted to blog a little bit of information about a disease which I find to be one of the most horrible illnesses in the world: Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig's Disease. I think we healthcare workers all have a few diseases which really depress us, ones that always get us down. For some, that might be dementia; for others, it might be aggressive cancers that strike at a young age; for me, it is ALS.

Before I get into the medical side of ALS, I want to share with you a few experiences which I have had with this disease. In the past, in what feels like a lifetime ago, I was a nurse's aide at a long-term nursing facility. I loved working with the patients and being able to spend quality time with them while improving their day-to-day life. There was a kind-hearted woman I had the great pleasure to get to know during my final summer at the nursing home. She was almost unrealistically kind, a person who goes out of her way to make others happy. She never wanted to be a bother, during her life preferring to help others and put others' needs first. She had ALS, and I saw the gradual and painfully real decline in her functioning during the months in which I was involved in her care. It was disheartening to see such a beautiful spirit slowly lose the ability to move, but she accepted her illness with great tact and with an admirable perspective - she always remained positive, despite being completely aware of her slow and inevitable decline.

While on outpatient neurology, I met an incredible, yet unfortunate, man with ALS. The first muscles which he lost were his laryngeal musculature and the ability to swallow, and he was losing his ability to walk at a startlingly quick rate. This man loves to ride his bicycle, and spent many summers travelling to different parts of the country to ride his bike in the gorgeous countryside with his wife and many friends. He's unable to do those things any more, and his ability to even walk is starting to leave him. He will eventual lose all of his ability to move, but he will never lose his mind. He will be painfully aware of his loss during the entire process, even when he is unable to communicate. This disease is just so terrifying, so horrific, being a prisoner in one's own body.

Some of you may have read the novel, "Tuesday's with Morrie" by Mitch Albom, which is about an inspirational mentor who has ALS. It's a very motivating book, and it's a quick and enjoyable read if you haven't read it yet.

After meeting this man with ALS last week, I went home and ran for miles and went to the gym and danced around, enjoying every little movement I made. I felt so motivated; I have the ability to run and dance and move about so I better use it, for there are many people all around us who would love nothing more than to just be able to run and dance again.


Interestingly, there have been studies conducted which attempt to prove a link between a kind personality and ALS. Neurologists often say that only nice people get ALS...and they might actually be on to something!




Amyotrophic Lateral Sclerosis: a disorder affecting the anterior horn cells and corticospinal tract and corticobulbar tract. The hallmark sign of ALS is the presence of both upper and lower motor neuron signs, without sensory deficits. Onset is typically 50-70 years of age. Lou Gehrig was unusually young at diagnosis (aged 35). Clinical features include progressive muscle weakness, usually first noticed in legs or arms (but can begin in virtually in any muscle); spasticity; fasciculations; dysphagia; dysarthria; dyspnea; weight loss and fatigue. Bowel and bladder control, sensation, cognition, and extraocular muscles are unaffected, even in the late stages. Diagnosis is made based upon clinical evidence and EMG/nerve conduction studies showing involvement of two (probable ALS) to 3-4 (definite ALS) regions. The treatment is highly supportive. Riluzole (glutamate-blocking agent) may be prescribed, but it has only shown to hasten death by 3-5 months.


References: http://www.ncbi.nlm.nih.gov/pubmed/16546756;  Step Up to Medicine, 2008. http://www.lesturnerals.org/events/run-4-als/&docid=OMSRl4okx3nlDM&imgurl=http://www.lesturnerals.org/files/7213/0454/1904/Run4ALS%252520with%252520tagline%252520web%252520ready.png&w=1121&h=532&ei=jzp6T9vfNMGCgAfk9_TwAg&zoom=1&iact=hc&vpx=162&vpy=182&dur=984&hovh=155&hovw=326&tx=201&ty=95&sig=109125001317639959798&page=1&tbnh=100&tbnw=210&start=0&ndsp=28&ved=1t:429,r:0,s:0

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