FVC Standing vs Laying down

[Cherise77]

Hi everyone. I hope you're all staying safe during these uncertain times. I had a question regarding my Dad's recent visit to his respirologist and FVC test.

His numbers standing have dropped from 66% 6 months ago to 62% but his laying down numbers have gone from 38% 6 months ago to 32%. My question is the 30% spread between standing and laying down normal? What does it mean?

The respirologist is talking to him now about a feeding tube which he is very upset about. His only weakness after three years of symptoms is still his right hand and arm this is worrying us that it's jumped to his lungs before the other limbs. I guess just not what we were expecting and it's scary. Have any other pALS or cALS on here had this same experience with progression?

Thanks for any advice you can provide. We appreciate this forums support and guidance during these last couple years.

 

[Nikki J]

The normal drop from sitting to lying is about 5%. Not sure standing might be more but 30% is not normal and is usually indicative of diaphragm dysfunction. Has he not been offered bipap?

arm seems to go bulbar or respiratory sometimes as a next step though anecdotally I have seen more bulbar than respiratory. However every PALS is unique. I am sorry

 

[Cherise77]

Thank you for your reply Nikki.
He is on Bipap at night. Doesn’t need it in the day but can get out of breathe if he’s running around with the Grandkids.
He says he can’t lay down at all anymore without feeling like he can’t breathe. He went to the dentist a month ago and said that was really hard and doesn’t know how he’ll go back for another appt as he was struggling to catch his breathe the whole time.

 

[lgelb]

He could use BiPAP with a nasal mask at the dentist, if that comes up, and could also start using it before or after running around with the grandkids.

The dentist will often be flexible with chair positioning if he asks. Some chairs can do more than others -- he might need a particular treatment room.

As for the spread, he's had it for a while and the FVC is really not much of a telltale sign overall because it only measures extremes in breathing. You do not go around breathing like on that test.

I would be more concerned with real-world breathing, like running out of breath during the day, and that's why I suggest starting to use BiPAP other than at night.

Over time, some muscles tend to compensate for others that are weaker...for example, the abdominals taking over in part for chest muscles...until there are not enough "good" muscles left. Letting the BiPAP do part of the work, gradually increasing the time as needed, can extend the time before that "until" time kicks in.

 

[richdees23]

good morning cherise.
i'm not good with percentages and like nikki said, every pals is unique, so all i can offer is my experience. i was diagnosed in feb 2019 and since then my limbs have declined steadily, at a relatively slow pace (i think?). other than that, my only issue has been my breathing.
around the beginning of this year i couldn't lay flat on my back anymore and if i recline too much i lose my breath. i use my cough-assist
regularly which helps. i do experience shortness of breath (especially wearing a mask in the heat), but overall, my condition seems to be stable for now. i definitely would look into the cough-assist as a therapeutic tool, it might prove to be very helpful.

 

[KimT]

We're all so different.

Some doctors use SLV and MVV to get a better idea but it sounds like your PALS needs some BiPap support during the day. Some PALS use sip and puff while watching TV. If the machine is hooked up when playing with kids that might work for awhile.

My breathing was so unpredictable I ended up buying my own testing equipment. So, at least I can measure relative progression and muscle strength.

The BiPap is an amazing piece of equipment and PALS can live much longer when it is used as needed