What It Is.
Also called Classic ALS and Amyotrophic Lateral Sclerosis, is a progressive muscle wasting disease leading to respiratory failure and death, usually 2 – 5 years from symptom onset. Al* was diagnosed in his early forties and I became his nurse when he was 65.
Pretty quiet guy, these days. Since the day he chose to have a positive air pressure ventilator do his breathing for him, he hasn’t said a word. He’s been on the vent for 15 years now. He also has a feeding tube inserted into his stomach because … he can’t swallow either, and hasn’t had a bite to eat in 15 years. He does, once in awhile, let some flavors touch his lips and, very cautiously his tongue. He likes pickle juice and ice cream. But good mouth hygiene is important for comfort as well as health. Could you imagine what it must be like for him to communicate that he has a tooth-ache? Only his eye muscles and a vestige of smile/frown muscles still worked, and his heart. It took awhile but we got it and, actually found a dentist to come to the house to extract a tooth! Good Luck if you live in a a big city.
But He Was Lucky
Having worked for The County, Al had primo Health Insurance, plus a Pension, plus Medicare, plus SSI Disability. He and his wife lived in their own home, with beautiful gardens they had planted, and 4 cats. It sounds like a lot. In fact it was. The Insurances paid for all of the 16 – 24 hrs/day of Registered Nursing Care, and a good portion of Doctor’s bills, Ambulance transportation, hospital costs, meds and supplies. The supplies took up residence in what was once a spare room: bottles of normal saline, bottles of sterile water, ventilator tubes, trach cuffs, wound drainage sponges, packages of disposable pads, cases of cans of liquid nutrition, urinals and bedpans, just to name a few.
And He Was Frustrated, and Even Mad
I only knew Al for 5 years, and I was only 1 of 4 primary nurses caring for him. I have my voice and whole body with which to communicate. Al has close to nothing. Fortunately, skilled health care professionals have learned how to read signs of the body; changes in color, tone, excretions, secretions, etc. Added to Al’s use of his eyes and smiles & frowns, it only took me about 8 months to figure out that he definitely did not want the ventilator anymore. Oh Boy.
He did have a Do Not Resuscitate Order, and his wife was his Health Care Proxy – in case he could not speak for himself. Could he? Armed with a list of pretexts to lure his M.D. (whom he had not seen in over a year) into a home visit, Al proceeded to demonstrate to the doctor that he could speak for himself. Al’s wife almost required being bound and gagged to keep her quiet, but the doctor got the message and he said “I can’t do that for you”. It may have been more possible if his wife was supportive of his autonomously made request but she was adamantly opposed.
Al’s Story Lives On
It took another 3 years before Al’s heart finally stopped and he was free to sail through the portal of his death. His wife is also free now, to live, and I really suspect that AL is the more peaceful of the two. This experience may, very well, have been more traumatic for Mrs. Al than it was for anyone else. I’m also free, to remember, interpret, and tell my story about AL and we had a lot of fun.
More Good News
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Join Date: 2006
State: West of England
Ventilation prolongs life ,but not to a full span. Obviously the disease continues and a locked in state will occur ultimately,which some people will cope with better than others.People permanently ventilated are more susceptible to chest infections , which can be fatal and of course disconnection could occur with the same result.
Stephen Hawking , I was told a month or so ago , is not on a ventilator regularly,although he does have a trachy.It is probably one of the reasons he is such a long survivor , because he can breathe by himself for the most part. I have no idea if he is vented at night. I would tend to assume he probably is.
Above copied from the ALS.org ‘question & answer’ section. A good start for learning more.
The incidence rate for ALS is .007% and there are, roughly 5000 newly diagnosed cases per year in the U.S. Less than 5% of those with ALS choose mechanical ventilation. Bill Moyers, On Our Own Terms, profiles a gentleman with ALS choosing NOT to be ventilated. Excellent.
* ‘Al’ is not the gentleman’s real name.