Pulmonary function tests

Robertblanchard

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Hello. This is my first post.

My wife was diagnosed with ALS last April. Her neurologist said most patients live 3-5 years.

She had her three month pulmonary function test yesterday at Barrows in Phoenix.

Her FVC went from 100% to 81%.
Her MIP stayed-35.

The new neurologist at Barrows said these results change her prognosis to less than 3-5 years. She does not want bipap for now and she will never get a trach. She is having the signs of increased carbon dioxide in her system.

Can someone please educate me on what the forced vital capacity and maximum inspiratory pressure numbers have to get to for when my wife gets to go to heaven? She is unhappy with her quality of life and just wants to go home.
 

KarenNWendyn

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Sorry to have to welcome you here and I’m sorry your wife’s quality of life is not good.

The new neurologist at Barrows said these results change her prognosis to less than 3-5 years.
I’m surprised the neurologist said that. Those numbers are still pretty good, especially the FVC. She could hit a long plateau and do reasonably well for quite some time. I’m sure others will chime in regarding the numbers. Is she struggling with other functional losses? So many factors affect prognosis. It is her choice whether or not to use BiPAP, but know that it can actually improve the quality of her life.
 

Nikki J

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My sister had an even bigger drop in three months ( from well over 100 to 75) and then to 50 three months later. I don’t know what happened after that as she refused to tell me but she lived 2.5 years after and rarely used bipap. She did have a feeding tube though and she would have died much sooner without it. Impaired breathing will burn calories and of course swallowing generally becomes an issue.

as the saying goes none of us have expiration dates stamped on the bottom of a foot. Plateaus do happen. Of course early and swift respiratory decline is concerning but she has only had 2 tests so far.

I am sorry you have to join us
 

lgelb

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I'm sorry that you find yourself here, Robert. As Karen says, regardless of what your wife's preferences relative to her long-term survival, BiPAP once needed (and with a MIP of -35 she meets Medicare criteria for getting a machine) does improve comfort, reducing air hunger and breathlessness, as well as the symptoms that CO2 retention cause, such as dizziness, confusion and headache. In fact, because of these effects, it might improve her outlook.

She would still have the final say as to when and how much she used it. If she decided to stop it at any point, knowing that would reduce her lifespan, that would be her right as well.

If you want to share more of how/why she finds life intolerable, we might be able to help or provide other perspectives.

Best,
Laurie
 

affected

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So sorry you have joined us Robert.
We really can't predict, but what we can help you with is keeping her quality of life as high as possible for whatever time she has.
 

Kristina1

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I'm sorry that you find yourself here, but welcome. <3
81 is a great score! I'm very surprised that there is CO2 retention with such a high fvc.

I understand that your doctor is basing his comments on the fact that 20 points in 3 months is a significant drop and if her fvc continued to drop that much every 3 months your wife would decline more rapidly. But ALS is not linear. She could drop 20 points this time, but you could come back next time and learn her score didn't drop at all. Plus this is just her second clinic visit, there is barely a baseline for her so soon.

I would ignore those comments about prognosis except for taking it in a general sense. ALS is unpredictable and progression is not linear. When I was diagnosed my neurologist said "The life expectancy is about 5 years for ALS, but you have bulbar onset, and for bulbar onset the expectancy is closer to 2 years." Well I am now almost 3 years out from diagnosis, and while I have experienced plenty of loss I am definitely not near death.
 

nona

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Same here, @Kristina1 . Approaching five years with bulbar onset. Breathing is decent with the help of NIV at night. I can't get my lips to seal to take FVC measurement but I kinda like not knowing.
 

KimT

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Pulmonary function tests are odd. Before my diagnosis, my FVC was the lowest. It always showed a restrictive pattern (which pointed toward ALS) but every time I was tested, my numbers were different. The one number that was constant was that my MVV, which is a measure of the amount of air you can move in 12 seconds, was above 100%. Different doctors and different centers use different numbers. Hopkins, Mayo, and U of Florida all used different numbers as expected results.

I ended up buying my own equipment. One think I notice is that my FVC, MVV, MIP, and MEP are ALL higher during the morning before I start putting things in my stomach. I also noticed that my SVC, which might be a better indicator than FVC, is just as high and sometimes higher lying down than either standing or sitting. If I do the tests when I'm anxious, tired, or full of food, the numbers are much lower.

As others have said numbers are just numbers. People have lived on FVC near zero and still gone outside in their PWCs with Trilogy on board. I consider non-invasive ventilation (BiPap) more of a quality of life decision rather than a quantity of life decision. Same with feeding tube. Both are choices, of course.

I tend to go how I feel and not what numbers say.
 
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