How long can a PALS survive with extremely low FVC?

[wembleton]

When they're on a machine like the trilogy 24/7 with no desire for a trach, I mean. Below 20%, is it a constant question of when they will aspirate or suffocate? Can a PALS technically be at 0% and still survive on the trilogy?

I am friendly but not close with someone with ALS and as they have publicly mentioned their FVC was 16% last month this is something I have thought about

 

[lgelb]

There is no cut point for at what FVC someone feels air hunger or is not going to survive, as there are many muscles involved and the FVC is not a reflection of how people breathe apart from this single test, which is not a real world setting. SNIP, followed by MIP/MEP, is probably a better gauge of real world function but still flawed and not strictly predictive.

If you read here, you'll see that people start PAP 24 hours at various levels of FVC, but a lot don't know them. It doesn't matter; it relates to discomfort, not a number. Likewise, there is no set number at which people aspirate or die. At 16%, your friend could have days or years, and it also depends on what kind of life s/he wants.

I see no point in monitoring FVC beyond getting a machine script in the first place, or to be eligible for a clinical trial.

 

[Atsugi]

Laurie is our expert on air. I totally support that post.

I've seen people survive long after they "should have" been dead. The human body is amazing.
Also, some PALS die a lot sooner than I would have thought.
I've never found a way to predict a PALS life expectancy.

Toward the end, we don't measure stuff, we just keep the patient comfortable. hour-by-hour, week-by-week.

 

[wembleton]

There is no cut point for at what FVC someone feels air hunger or is not going to survive, as there are many muscles involved and the FVC is not a reflection of how people breathe apart from this single test, which is not a real world setting. SNIP, followed by MIP/MEP, is probably a better gauge of real world function but still flawed and not strictly predictive.

If you read here, you'll see that people start PAP 24 hours at various levels of FVC, but a lot don't know them. It doesn't matter; it relates to discomfort, not a number. Likewise, there is no set number at which people aspirate or die. At 16%, your friend could have days or years, and it also depends on what kind of life s/he wants.

I see no point in monitoring FVC beyond getting a machine script in the first place, or to be eligible for a clinical trial.

Thank you. From her posts it seems pretty clear that she's preparing to die & planning things with her husband; things to give to her children, etc. So is that a precaution or do PALS generally have a sense of when it's coming? I know that she doesn't want a trach

 

[starente15]

It's not really a precaution because ALS is terminal. From my own experience, once my father could comprehend what his fate was, he began doing the same. He lived at least another year but he wanted to make his wishes known to certain people. From the minute of diagnosis, everyone knows what's coming, just not when it will be.

I think people handle it in different ways. My dad lost interest in everything that meant something and for others it creates a desire to check everything off their bucket list.

I'm sorry your friend is faced with this. It's a terrible situation for the PALS and their family.

 

[swalker]

Who knows?

I don't place much importance on my FVC scores. They are low (below 30%). I was at 31% 2 years ago.

Yet, I breathe fine. I am occasionally short of breath, and just can't take a deep breath no matter how hard I try. But, I don't sense any kind of air hunger unless I exert myself.

I think FVC scores have some value in tracking progression in that they can provide a quantitative (though imperfect) measure of how my breathing muscles are deteriorating. But, at least in my case, they don't provide any meaningful insight into how long I may or may not survive.

Steve

 

[gooseberry]

When my husband was getting tested regularly, the last number he posted was 18 to 20%. That was 5 months before he passed away due to a fall. His pulmo said he had seen people use the trilogy at these numbers for years. Like everything else with ALS, each person has theiir own course.

 

[Dave K]

The measure of FVC does not itself predict life expectancy in a PALS. Case in point: my wife survived for two years after she reached zero FVC and still breathed all day without a ventilator. Her neuro called it "reflexive breathing." There's no telling which nerves will give up next, and in her case the diaphragm just never completely stopped working while she was alive, even after all the other muscles stopped.

 

[wembleton]

The measure of FVC does not itself predict life expectancy in a PALS. Case in point: my wife survived for two years after she reached zero FVC and still breathed all day without a ventilator. Her neuro called it "reflexive breathing." There's no telling which nerves will give up next, and in her case the diaphragm just never completely stopped working while she was alive, even after all the other muscles stopped.

Wow, that's amazing. Was her situation quite rare?

 

[affected]

FVC is just a bunch of numbers and many things affect how reliable the numbers are (lip seal for one thing), and are only an indication of some things. Just as has been said. I think it is too easy to get caught up in looking at numbers and similar things, rather than just focusing on the person.

Please focus more on finding ways to become close enough to offer help to this person you know. There will always be some way you can help out, no matter how small it may seem to you, it could be huge to this PALS. This is so much more important than worrying about FVC.

You will never regret making their and their CALS life a little better

 

[wembleton]

FVC is just a bunch of numbers and many things affect how reliable the numbers are (lip seal for one thing), and are only an indication of some things. Just as has been said. I think it is too easy to get caught up in looking at numbers and similar things, rather than just focusing on the person.

Please focus more on finding ways to become close enough to offer help to this person you know. There will always be some way you can help out, no matter how small it may seem to you, it could be huge to this PALS. This is so much more important than worrying about FVC.

You will never regret making their and their CALS life a little better

I barely knew this person even before ALS which is why I said I was friendly with her but not close at all. I don't even live near her anymore but my mom does things for her through her church.

I don't think it's necessary to make assumptions or to passively aggressively chastise someone for a question they ask on a forum (which I've noticed happens not infrequently here)... I think we're all aware that if you do have a relationship with someone you should help them out; it's pretty self-evident. But really has nothing to do with the question I asked.

 

[sweetmozart]

I just want to chime in because I agree with what Laurie said about no need to monitor certain things after a point.

The last time my husband had his FVC measured was to 'qualify' to have the surgery for his stomach tube placement. At that time, probably four or five years ago, he blew right around 30%. I think knowing that effected him, because it was the first time he had such a low result. He has refused getting his FVC ever since.

I've witnessed the power of the mind in anything medical, and this is a perfect example.

I read the answers here with relief because I worry about his breathing. Thanks for the helpful responses.

 

[JimInVA]

In March of 2015, Darcey's FVC had dropped to 15%. Her neurologist suggested that she was likely within her last 6 months of life and might want to consider hospice. We did not engage with hospice. And though she's continued to decline in function, we're [now] almost at 2 years since that day in early 2015. 2017 may be her last year... but I'm sure not going to count her out until she says "enough". As many have said, FVC is just a number and one of many indicators. But a low FVC is certainly no promise of an immediate passing.

My best!

Jim

 

[wembleton]

In March of 2015, Darcey's FVC had dropped to 15%. Her neurologist suggested that she was likely within her last 6 months of life and might want to consider hospice. We did not engage with hospice. And though she's continued to decline in function, we're [now] almost at 2 years since that day in early 2015. 2017 may be her last year... but I'm sure not going to count her out until she says "enough". As many have said, FVC is just a number and one of many indicators. But a low FVC is certainly no promise of an immediate passing.

My best!

Jim

Wow, that's amazing! Is she able to move any parts of her body at all?

 

[lgelb]

It's actually not "amazing," as this entire thread explains, though we are all happy for the joys that Darcey and Jim still share. Please stop gawking like a tourist, Wembleton; you have your answer. I am sure the PALS of whom you speak appreciates your mom's help.