questions for the community

[daddypix]

1.why do they claim at bulbar onset als patients dont usually live as long as limb onset. 2.are there any studies about the effects of tobacco on one s progression. 3.is there anyone in the forum that had multple symptoms at onset...bulbar,limb onset etc... and are still hanging n there 4.what is the avg cost for a patient to use internal ventilation..... is it the cost that has some people decline or the maintnenace......

 

[BarryG]

I can only answer #1. Because we can't swallow and often die from aspiration pneumonia. I can't even swallow my own spit and have to take drugs to dry my mouth (not excess saliva, I just can't swallow the normal amount that my mouth produces) and suction a half a dozen times a day so I don't choke.

 

[stephie]

My husband's symptoms started with slurred speech and twitching in his biceps at about the same time. We have never been sure whether it was bulbar onset or not. He lost his speech at about the same time he lost the use of his limbs. He was diagnosed four years ago and is now paralyzed. He just recently lost the ability to swallow, which is causing a lot of trouble with mucus.

We also checked into the cost and difficulty caring for someone on a vent. With our insurance, we would have paid about $500/month for the actual equipment. The big expense is in the home health care. We would have paid thousands of dollars for 24 hr. care, which you need on a vent. My husband questioned whether 24 hr. care is really necessary, but we found out it is. Someone on a vent needs suctioning anywhere from 3-20 times a day and it can not wait or the patient will choke. If family members are available to take turns and be with you, it would be much cheaper. We were told that it is a little tricky to learn how to do at first, but it gets much easier with practice.

Because my husband was not sure of where his ALS started and which abilities he would be losing first, he waited until it progressed quite a bit before he decided about a vent. He wanted to see what his quality of life would be when his breathing became a problem. Because he can not eat, speak, or move, he has chosen not to go on a vent.

 

[Katie C]

They don't "claim"..it's one of the few things about this stupid disease that is actually quantifiable. Barry is absolutely right.. aspiration pneumonia is a big reason. Also, dementia for some reason is more likely to occur in bulbar onset PALS than other types of onset. With the dementia comes a lower level of compliance that can preclude pegs and/or venting, and also again increase the likliness of aspiration pneumonia due to trying to eat things that can no longer be handled.

No idea about tobacco studies.

Glen had issues with "clearing his throat" for a long time, as well as noticeable fasiculations in his arms. Looking back now, there were cognitive signs that were misdiagnosed at least 3 times. By the time we got a diagnosis, he had limb and breathing involvement as well as typical bulbar symptoms.

We were among the lucky ones... excellent health insurance, excellent disability coverage from his job, and a nice nest egg from 30 years of careful saving and some advantageous real estate purchases. Finances had nothing to do with vent or peg decisions. Quality of life and a desire to honor his wishes did.

 

[notme]

Smoking will certainly add to respiratory problems. Studies show that there may be an increased incidence of ALS in smokers--one suggest the risk of this was higher in women. I researched this as I've been a smoker for 30+ years. For me, I decided it's one of my pleasures--right or wrong--and if I do end up with ALS--I honestly see no point in quitting--but found no studies showing that it hastened the actual ALS progression

All my research shows that distal onset (limbs) usually starts in one area, then progresses. As I don't have a confirmed diagnosed, I can't swear to that. When I would talk to my residents that could speak--they all said it started in one are--some bulbar, others in the limbs.

For many that decline vents--it's due to various things--for some, they decline when they are 'locked in' as they feel it won't improve quality of life. For others, with use of limbs AFTER breathing muscle involvement, they choose to vent to prolong their lives. Personally, I would not choose to vent if I was totally locked in. Have no clue if medicare/medicaid pays for vent (search some of Joels old posts, perhaps)

Some countries seem to discourage the use of vents (which I feel should be a personal choice) Again, Joel's website has info on vents.

I am curious--why are you asking?

 

[Alyoop]

Smoking may not hasten progression of ALS, but it will certainly hasten death from respiratory issues. As respiratory complications are ultimately what causes the death of an ALS sufferer, smoking would of course hugely increase that risk.

It's not that some countries discourage the use of ventilators in MND patients, it's that they do not have it as a choice at all. So you can't vent even if you want to.

Some forms of midiastinal tumours cause ALS symptoms, including atrophy of the hands. These types of things are of course commonly caused by smoking, as are paraneoplastic syndromes. Which as you know can cause ALS like symptoms months or years before the cancer is found. Sometimes the cancer is never found.

 

[daddypix]

hey guys as always thankyou for ur input.........this site has really given me alot of get help and information.......

 

[Phil's wife]

Some forms of midiastinal tumours cause ALS symptoms, including atrophy of the hands. These types of things are of course commonly caused by smoking, as are paraneoplastic syndromes. Which as you know can cause ALS like symptoms months or years before the cancer is found. Sometimes the cancer is never found.
Alyoop, this is true. My husband's ALS is strongly believed to be caused by Kidney cancer found 5 months after ALS diagnosis. The cancer has spread and therefore the ALS continues to progress. There are 3 documented cases that have shown ALS symptoms to stop and sometimes reverse due to removing the tumor and thus getting rid of all of the cancer.

 

[Phil's wife]

Alyoop - "Some forms of midiastinal tumours cause ALS symptoms, including atrophy of the hands. These types of things are of course commonly caused by smoking, as are paraneoplastic syndromes. Which as you know can cause ALS like symptoms months or years before the cancer is found. Sometimes the cancer is never found."



Alyoop, this is true. My husband's ALS is strongly believed to be caused by Kidney cancer found 5 months after ALS diagnosis. The cancer has spread and therefore the ALS continues to progress. There are 3 documented cases that have shown ALS symptoms to stop and sometimes reverse due to removing the tumor and thus getting rid of all of the cancer.

 

[Alyoop]

Wow, you have a lot to deal with, cancer and ALS. I have an aunty with severly atrophied hands. She is a heavy smoker ( and an alcoholic). Her EMG is normal, but ibsuspect she has a paraneoplastic syndrome. I asked her last year to go have a chest X-ray, but she never did.

It's important, that if people smoke and have ALS symptoms, they get a good chest work up as well

Best of luck Philswife