Respiratory Onset ALS

[lgelb]

It is not "hard to refrain" from getting a trach in the US (or anywhere, really). Most PALS do not end up getting one. The highest published rate is 45% in Japan; the US is certainly <10%.

This article makes some good points to consider, though I think it over-dramatizes the decision to discontinue ventilation given that most P/CALS even without a trach ultimately direct the timing of death to some extent.

Typically, PALS after being trached live at most a few years, and some studies suggest that at a population level there is as yet no to little survival benefit, possibly because of changes in timing of the procedure over the years and differences among centers. Japan, unsurprisingly, reports the best survival benefit, with robust government and family support for post-trach care that is less assured elsewhere in the world.

Landis, since doing everything you can with BiPAP can slow respiratory decline, let me know if you'd like help optimizing your PALS' settings.

Best,
Laurie

 

[KimT]

I'm going to be blunt and honest. I will not get a trach for a number of reasons. First, I don't have family and don't have enough money to provide 24/7 care. Second, I'm 68, and I've lived a good life. Third, I've seen many PALS suffer so many infections, emergencies, and complications from having one. I still think everyone should have the choice and I'm in awe of people who do well on them.

Laurie makes a very important point and that is use a BiPap as much as you need it. It will no doubt give you a better quality of life. Landis, take Laurie up on her generous offer. She has tremendous expertise it BiPap settings.

 

[Landis02]

Thanks , Laurie. Chuck got his NIV last Thurs and is doing good on it. He has a follow up with the resp tech next week to review all the settings. We’ll also meet with a pulmonologist at the clinic next week. But we may take you up on your offer.

 

[Doing My Best]

Thank you all for this discussion, and Laurie, thank you for the link to the article. To me it is an excruciating topic and I worry about my own fears and motives getting in the way of my PALS' wishes. I don't think he is very close to having to make this decision but his pulmonologist told us we should be thinking about it and talking about it, so I appreciate having some kind of reference point. I dread getting closer to reaching that decision point.

 

[Nikki J]

For people who are leaning toward having a trach don’t wait until the last minute. There is always the danger of a sudden decline but also i think you need a little reserve strength to handle the immediate period after. It is a tiny procedure- that isn’t a problem but there is period after with increased secretions. I can think of at least three late members who did the trach but could have handle the immediate post op period and died or gave up within a couple of months. I am not saying rush to trach asap but be a little proactive if you want one and don’t wait until it is emergent.

 

[oldgeek]

I guess I'm the opposite of several of you. My bulbar problems came first. I was on a bipap/NIV for several months until it could no longer push enough air through my collapsing throat. I got my trach 14 months ago and it has been a tremendous help. If I had to do it again, I would do so with no hesitation. I'm on two vents (one standing and the other portable) since I can no longer breathe and the trach makes it great.

I live in a retirement home and can enjoy supper with friends as the vent helps me breathe, and I set the trach cuff so that enough air goes around so I can speak. Lately, I cannot eat and talk at the same time since the muscles give out, so I'm finally going to bite the bullet and get a PEG. Frankly, I've been more hesitant to get the PEG than the trach. It is my hope that I can still sit down for supper with friends and not try to eat with them (may have a martini or two) but will still be able to speak with the help of the vent.

My ENT surgeon was great. I have a permanent trach so no granulomas, I still have a great cough reflex so no need to suction. I changed my trach tube this morning and only needed help with the trach ties since I can't feel my finger tips. I wear a scarf over the trach and the vent sits on the back of my power chair and so folks have to look close to see either. My biggest complaint lately is that my limbs are starting to fail. Last night, I happened to wake up and the calves in both legs were having crazy fasciculations which I could feel with my hands but not in my legs. So like I say, I guess I'm the opposite with bulbar first and now limbs.

But remember each of us is different. For me, my trach has greatly improved my quality of life and frankly given me several additional months of enjoyable living. So let me tell you the downsides - which for me are not a big deal.

I was getting monthly infusions in the hospital and with the trach and vent, they said I could no longer come, I was too fragile. So, I give myself the infusions now at home.

I tried to move into an assisted living home and was told their insurance would not let me move in there with a trach and vent - I was too much of a risk. So, I have home support coming in.

After the trach, I was told I needed 30 days in a rehab facility; But in my area, none would take me with a vent and trach. So, I got a great team of home health folks - much better than a rehab facility.

And the weirdest complication: I was told by my health team I needed to go onto Hospice Care. But it's a "catch-22". Hospice is not supposed to withhold a ventilator; but, they cannot supply trach supplies or vent supplies while on hospice. In other words, stay on the vent with a trach but we just can't supply you with the things you need to support either. So I'm not on hospice and with my home health assistance, can manage everything at home. I get my supplies from my DME and again, am happy and comfortable.

I"m primary care giver for my wife who has terminal cancer which has moved into her brain causing dementia. But we manage to laugh out loud several times a day and we are both filled with joy and happiness. I'm thankful for my trach, my vents, and soon my feeding tube (if I can get my "big boy pants on" and get that done) so that I can care for my best friend and wife of 50 years.

So for us, faith and attitude sustain us and we don't let little things like a trach or terminal cancer knock us down. But again, we are all different. Blessings to each of you.