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Mihail

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Greetings to all and before I go into our situation I want to express my good fortune and gratitude for finding such a valuable resource as this online forum.

My 82 year old uncle fell ill and was hospitalized due to a hypofunctioning esophagus. 15 years ago he had ruptured his esophagus due to vomiting caused by an inflamed appendix. The ruptured esophagus was then stitched up using a Nissen fundoplasty, and has had no problems with it since.

When he fell ill recently (mid November 2015), he had been dehydrated because he never drank enough fluids and had not eaten in a few days after getting sick from a flu shot.

Long story short, he was diagnosed with ALS, then developed pneumonia and was intubated and then placed on a ventilator with a tracheotomy. . Were fortunate to have a couple of really good doctors in our family and they were surprised because he developed ALS so late in life, and it apparently struck his throat and esophagus first, instead of his extremities which seem unaffected. Were told that the doctors observed twitching in his tongue, however I care for him daily (he is in a local respiratory facility) and have never noticed any such twitching.

His weaning was going well for a while but he has been stumbling lately and is now considered unweanable and we are looking at the next.

The reason he is considered unweanable is due to the alleged underlying cause of his respiratory weakness being ALS.

However, he has other underlying problems, and were puzzled by the doctors insistence on an ALS diagnosis. After much wrangling, the doctors consented to perform an electromyography which did not confirm the diagnosis, but the doctors are going with it anyway. They told me that the electromyography was not conclusive because my uncle was not conscious enough to cooperate.

This was further confusing as other doctors have told me that an electromyography is basically an electroconductivity test that doesnt require the patient to participate.

As you can tell there are a few complex issues at play here, but my question for this forum is regarding the actual ALS diagnosis. It seems inconclusive but the doctoras are very insistent that it absolutely is ALS.

Is it possible to develop ALS this late in life and to have it not affect his extremities first and not be detectable through an electromyography? I dont want to be in denial, Im aware that he may not survive much longer regardless of ALS but I just want to know the truth of the matter.

Thank you for considering my question,

Mihailo
 

Nikki J

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I am very sorry about your uncle
To try to answer your questions
Yes people can develop ALS at 82. We have had family members of such patients as members of this board
Yes ALS can and does start in what we call the bulbar area ( speech swallow). This type of onset is more common in older PALS than younger ones. There is also a very rare form that is called respiratory onset where breathing is affected first
The EMG might not show anything if it was done on the limbs and he has only bulbar involvement.
 

Mihail

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CALS
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I am very sorry about your uncle
To try to answer your questions
Yes people can develop ALS at 82. We have had family members of such patients as members of this board
Yes ALS can and does start in what we call the bulbar area ( speech swallow). This type of onset is more common in older PALS than younger ones. There is also a very rare form that is called respiratory onset where breathing is affected first
The EMG might not show anything if it was done on the limbs and he has only bulbar involvement.

Thank you very much Nikki. You not only answered my questions but you saved me the effort of reading through the boards myself. And I will tell you that just now for the very first time I said to myself that he actually may have it. I will research these forums further, if I can find one thing that helps me understand and communicate with him better I will be eternally grateful to the good people that make this forum possible.
 

CiCi

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My husband was diagnosed with bulbar onset ALS at the age of 85 at Johns Hopkins. The first neurosurgeon that we saw said he had a patient that was 90 yrs old with ALS.

Sorry to see you here, but you will find much info and help here.

Claire
 

Mihail

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My husband was diagnosed with bulbar onset ALS at the age of 85 at Johns Hopkins. The first neurosurgeon that we saw said he had a patient that was 90 yrs old with ALS.

Sorry to see you here, but you will find much info and help here.

Claire

Thank you Claire.
 

lgelb

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Mihailo,
First, let me ask that you not quote text in your replies, since this is a threaded forum and our members face many difficulties in reading.

I'm sorry to hear about your uncle. Though it's true that very elderly onset is possible, the fact that he has been vented essentially at dx, which happens only in a small percentage of cases overall, continues to argue for careful consideration of the diagnosis, so I would seek a second opinion outside his current group of physicians, as we advise everyone to do anyway. There are viral differentials, for example, that could mimic what you have described. I'm unclear on why he was not fully alert during the EMG?

You speak of underlying health issues, but if there is any chance he could be weaned, he deserves that chance. And to that end, I would ensure that his respiratory support team is very familiar with ALS, as at most such facilities they would be more familiar with stroke, TBI and SCI, for example.

Best,
Laurie
 

Nuts

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I don't usually comment on bulbar issues, as we have not experienced that yet and there are very knowledgeable people here. I am bothered, however, that he would be considered unweanable because he has ALS. If I'm wrong I know someone will jump in, but I don't think ALS makes him automatically unweanable. Stumbling happens even without ALS. I have a friend who spend months being weaned from a vent and he had no neurological problems.

Please find a medical team that specializes in ALS, both to be sure the diagnosis is correct and to be sure that if he has ALS he gets the proper treatment.
 

Mihail

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CALS
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Thank you Igelb and Nuts, I appreciate your insight and the information you are providing, it is the most useful of any Ive received so far, including from the doctors themselves. They seem more concerned with presenting themselves as experts and they talk about their vast experience working with ALS more than they do explaining the basic specifics of the case at hand.

My uncle was temporarily transferred to another hospital and there we asked for a second opinion and the second neurologist agreed with the first and confirmed the ALS diagnosis. The second doctor is the one that agreed to do the electromyography, the first one insisted it was not necessary. The second doctor said that the electromyography results were inconclusive because my uncle couldnt cooperate and follow commands.

Why wasnt he able to follow commands? He may have been confused or sedated, I can communicate with him fine, it just takes effort and desire. He needs to be informed of his whereabouts and have the situation explained to him as he has been bedridden and medicated for 2 months now, and apparently is starting to suffer from dementia, although its not apparent to me from dealing with him. He understands and answers questions fine, he is "all there", I honestly think they dont try very hard but thats my subjective judgement without knowing all the facts.

My general impression is that theyre treating him by the book and by the numbers, and their book and numbers arent the best around. They wont even allow me to give him tiny sips of water or tea which he asks for because of aspiration risk. The speech therapist told me he can handle ice chips but theyre so rigid about everything. In the meantime I have had doctors tell me that its ok to give him light fluids by mouth as long as its a small amount and slow and careful.

I'd really like to be able to give him sips of water, tea and juice, he has so little to look forward to and enjoy, and the respiratory clinic is so rigid and auotomated, the patients get their rounds but little else in terms of comfort or care. I dont mind doing anything at all, but the fact is if I want things done for him I have to come in and do them. Additional hygiene, especially oral and simple things like another blanket. Most times I come to see him he is waving his hand to get attention from anyone walking by in the hallway. And I see him as frequently as possible, before and after work most days and longer on weekends, and Im on the phone with his staff all the time but its impossible to rely on them to properly take care of him, its just the way the facility is staffed and run, and its the only game in town.

Thank you all again for helping, listening and allowing me to vent.
 

Mihail

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EDIT: the doctors that tell me its ok to give him light fluids by mouth are doctors I know privately. The staff at the clinic are the ones saying he cannot receive them.
 

pittsburghgal

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Mihail,

My husband was hospitalized last June for subdural hematomas from falls. He developed aspiration pneumonia which caused respiratory failure. He ended up with a trach and was on a ventilator. He was able to be weaned off the ventilator in 2 weeks. Having the diagnosis of ALS does not make you unweanable.

I would not advise giving your uncle sips of liquids at this point. Liquids are the hardest thing to swallow without aspiration if he has any swallowing problems. Usually a modified barium swallow is done to see if the patient can tolerate food or liquids without aspiration. I know this is very difficult. My husband is not supposed to take anything by mouth and this has been the most difficult aspect of his disease for him. We do break the rules very occasionally with a spoonful of pudding or thick yogurt. He swallows with his chin down and seems to do ok. This moistens his mouth and he says yummmmmm.

Sharon
 

affected

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So sorry for all that is happening.

My husband was bulbar onset. Thin liquids are the most dangerous.

My husband spend 1.5 hours coughing/gagging/trying to clear his throat the last time he took anything by mouth and ended with aspiration pneumonia. It is horrible for them to go through this and to watch helplessly.
 

Mihail

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fla
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st petersburg
Thank you all for the wonderful advice and suggestions.

After all the heartache and suffering, he just put together two strong days of weaning, 9 and 10 hours.

Thank you for not only opening my eyes regarding his diagnosis, but for teaching me that ALS is not a guarantee of unweanability as the doctors claim.

Rest assured that you are doing good work here, your kindness has been a blessing to our family.
 

helkat22

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I assume Guillian Barre was ruled out? I'm sure it was, but I just had to ask.
 

Mihail

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fla
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You know it hasnt even been mentioned but I will ask tomorrow. Im not sure how the weaning is going anymore, he's had 9, 12, 13 hour days this past week, but couldnt do 2 today. Anxiosly awaiting to see how he does tomorrow.
 

Nuts

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I'm so sorry about the setback.

We have a friend who wound up in a coma on life support for months following surgery for lung cancer (he had a small tumor removed and wound up with a bleeder). He wound up bouncing between rehab and the hospital for some time before finally getting weened. The process took many months and his recovery at home almost a year. Granted, he didn't have ALS, but even without the disease it can take several tries to get someone weaned off the ventilator.

I hope for the best for your uncle.
Becky
 
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