FVC at diagnosis and progression rate

[Kellbellab]

My mom’s FVC was 50% during her initial respiratory evaluation at the ALS Clinic at Mayo. Because of this, she is not a candidate for any clinical trials. She will be receiving a cough assist and trilogy.
Does her diminished pulmonary function indicate ALS progression is aggressive? She is considered bulbar onset ALS.
If you have any thoughts, I’d appreciate.
Thank you.

 

[Nikki J]

I am sorry you got this news. Obviously 50 percent is not great. Sometimes with respiratory assist the breathing stabilizes for a while. She will certainly feel better.

If you don’t have previous numbers you don’t know how fast she has been declining. And numbers can vary a bit depending on fatigue, recent meal , quality of seal.

Did they bring up a feeding tube and what is her wish?

How bad is her bulbar disease? Does she have limb involvement? How long has she noticed symptoms? How old is she?

No one can predict how things will go but the other factors will tell you a bit.

I don’t know if this is comforting or the reverse but we have had bulbar/ respiratory onset members who were able to live independently until the end because their respiratory disease took them before they lost limb function

 

[KimT]

I'm an outlier as regards FVC. Before my diagnosis my FVC was 68. It took about 9 more months for a diagnosis but my FVC was 80 at diagnosis (Mayo.) Then it went down to the mid-70s. I've been tested at four facilities (all very highly regarded) and my numbers are all over the place. I got so frustrated I bought my own testing equipment with a grant from ALS Guardian Angels.

I can measure FVC, slow vital capacity (which many ALS doctors prefer) MIP (inspiratory strength) MEP (expiratory strength) MVV (the amount of air you can move in 12 seconds, and much more. Although my FVC is never normal, it varies by time of day. Best before food. Same lying down as sitting up. My breathing muscles are still strong (MIP way above average.)

My only point is that other things need to be considered. I've been denied clinical trials because I've been diagnosed too long, I'm over 60, and/or my FVC was under 80. I do understand why but it's still frustrating.

FVC is just one number. Getting a Trilogy and cough assist and getting used to them is important. The cough assist can be used in reverse to expand the lungs and I have had success in increasing my lung volume by doing that.

There are so many factors to measure when talking about progression. I know one PALS who has never had any breathing issues yet he is totally paralyzed in all limbs. I know another who walked and ate up until the day he died from respiratory failure.

Also, progression speeds up, slows down, and even stops for months at a time.