what is theleading cause of death in pals

[smwelder]

i know each pals path is different. I'm a bit lost in why we die. I know our bodies wither do to mussel loss and breathing gets to become a problem, but with the use of cpap and bipap or vent problem solved right? what else goes wrong to cause death?

 

[cervus]

After a while the BiPAP becomes ineffective and then you make the decision of whether or not to have a tracheostomy. With just the BiPAP, after a while (everybody is different) ones starts storing C02 which is what usually ends a PALS life. It poisons your body but in some ways (so I've heard) can be a blessing in that the PALS just gets more and more tired and usually 95% pass away in their sleep. That's what our ALS team at the U of Alberta hospital have told us. My husband is starting to store C02 (he uses the BiPAP at night and for an afternoon nap). We had the pressures on the BiPAP upped a bit and that helped with headaches but it's just a comfort measure really. He will not trach and has decided not to become 100% dependent on the BiPAP. He does get more tired now and a couple of days ago said his breathing feels different. Yesterday he felt better but today he's tired again. Hope this helps?

 

[notme]

The other thing that can happen, unfortunately, is infections. Even those on vents can get respiratory infections/pneumonia. For those that choose to vent, it's normally recommended to do the surgery before function becomes so low that it's dangerous.

It seems doctors don't always explain things to their patients as well as they should. At least here in the US, though, vents are a real possibility, unlike some countries that won't supply them for ALS.

 

[kmendsley]

I believe also to jump off of notme's post that pneumonia can result simply from the fact that the PALS are no longer able to cough or sneeze because those muscles are so atrophied they no longer work and everything...infections, viruses, simple colds...'settle' in the PALS chest and it becomes bad enough to develop into pneumonia that becomes too advanced before it is noticed or not able to be fixed with antibiotics as well. Essentially, the lungs fill up with fluid and the person drowns in their own muscus per say by O2 levels dropping drastically.

Very sad.

 

[Ms. Pie]

Ah, so much fun to look forward too...

 

[valg]

How do you know when you are storing CO2?

 

[asantiago]

I didn't know about storing of CO2. I guess this is what happened to my mom. We talked some after her death wondering what the actual cause was for her. As described above, getting sleepier describes how she went. She hated the bipap and just wouldn't wear it. We were so uninformed about EVERYTHING!

 

[cervus]

Valg,

Symptoms of C02 retention are restless nights, morning headaches, the mind gets a bit cloudy, general fatigue (more than usual) and then there are others. My husband experienced morning headaches although they didn't follow his immediate wake up. He got them a bit later in the morning. He always took a nap but didn't feel the extreme tiredness that he does now. It's almost like he says he needs a nap and he starts closing his eyes. A few things happened before that to show he was having respiratory problems - passing out, fully body spasms, and once, we had to perform the Heimlich but it was unusual in the fact that the minute he started choking, he turned blue. For most people that would not happen till a little bit later. He hasn't been struggling with breath hunger or anything like that. I was told that doesn't need to happen. Breathing can become more and more shallow and then a little more rapid. Yesterday morning Bob slept the whole morning but then was awake the rest of the day. We'll see. We've been given a timeline of a few weeks to a couple of months at the most. BUT nobody knows.

The symptoms I've listed are just some.....

 

[notme]

The only blessing with ALS is that normally the CO2 retention does cause the PAL to be sleepy and the meds they use help with any air hunger feelings make their passing much more comfortable on them. As horrid a thought that is--most do pass away in their sleep.

The others have answered the feelings of too much CO2. Headaches and very sleepy. Those that choose to vent and use a Bi-pap should get them done sooner rather than later---before the surgery for the vent, for instance, becomes too dangerous for the PAL.

But, I'd also suggest an ALS literate doctor/pulmonologist--as I've recently found the treatment my private pulmonologist prescribed for me might not have been the best choice with my condition.

 

[valg]

Living in the Caribbean there is no ALS specialist of any sort. I have had to Google and then make suggestions to the Drs.

If you start to store CO2, then your O2 levels decrease? His are still in the mid 90's.

Also what meds are used to help with air hunger? He has had a few episodes of gasping/short of breath (usually after laughing or if someone has on perfume - he is very sensitive to any sweetish smells), but usually his breathing is just very shallow. The physio says he is only using the very top of his lungs.

 

[notme]

HI

How are his pulmonary function tests? It's very possible that he needs a bi-pap. Only blood gasses (drawn from an artery) can detect too much CO2.

Some PALS need bi-paps just at night or when they nap. O2 sats aren't really a good indicator of Co2 retention--at least not on their own.

Generally speaking, O2 sats in the mid-90's are considered okay. The problem with some PALS is they drop more at night and when they are sleeping.

While I'm not diagnosed--my O2 sats definitely drop at night and with exertion. I've been told by PALS here and by my ALS neuro that O2 isn't a good idea for PALS as it doesn't let the Co2 exchange work properly.

Look at my post on the "DO I have" section. trfogey posted some good links with explanations. It's difficult when you have doctors that aren't well versed in ALS--as my pulmonary doc was treating my O2 levels--but apparently didn't take into account the possibility that my breathing issues are muscle related verses lung related.

 

[catcaniac]

What everyone is saying about the ventilator is very true. Eric has been ventilated since March. Since that time he has been in the hospital twice and is now on his third round of strong IV antibiotics for trach/respiratory infection leading to pnuemonia. The second time he got very sick. He was unable to control his bowels for about 3 days and was very out of it. His oxygen concentration plummeted to 13 % and his blood pressure went down to 70/40 within a 30 minute period. He would have died at that point if we hadn't called 911 because we really didn't realize he was septic with infection. Now we know the signs and are trying to ward things off ahead of time. So far the antiobiotics are maintaining and improving his function but eventually, his body will develop a tolerance for the antiobiotics. He has already been through 5 different kinds. He still seems to be responding to Gentamicin and Cefepime. But it was only less than 3 weeks since he was on them and he started developing a low grade fever within 7 days of stopping them the last time. I do think the condition you are in when you decide to vent makes a huge difference. Unfortunately, Eric's illness progressed so rapidly that all we had was about 2 weeks from when he started going down to making the trach/PEG decision to save his life for the time being. At that point, his body had already deteriorated to the point that his body has no ability to fight things and they think that he has been fighting the same infection ever since the trace was put in. If the PEG and trach surgeries are done when the body still has some strength then I feel that it could be very successful like Joel's was. Even though things have been tough, Eric would still say that he is glad he did the trach. It has allowed him to have more time to process the disease and spend with us. If he weren't so sick, I would be comfortable taking him anywhere with the power chair and vent. He just got the new one and it is amazing. You just slide everything on the chair and you are ready to go. He can't move anything but the companion switch allows me to drive the chair easily.