FVC 27%

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kljack16

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I apologize in advance. The search engine seems to be having issues; so if there are already threads on this, I couldn’t find them.

We just went to the ALS Clinic in Houston, TX. My husband’s FVC is now at 27%. I’m trying to get an understanding of what this means in his progression.

He does not want to do the trach ..: or at least previously has said no to it. In the 20s is what they have always said was a line in the sand, so to speak. Is it?

Background - his upper body is where the disease first presented. He was officially diagnosed 8/31/2020. His FVC then was in the 90s. January 2021, it was in the 80s. In July 2021, it was right about 50%. Late March 2022, it’s 27%.

He can still stand and shuffle his feet to get from the chair to the bed or to the car.

What kind of timing are we facing? The clinic didn’t say anything other than to up his use of the Trilogy/AVAPS/BiPAP machine to all night (as we’ve been doing) and 3-4 hours each day. He’s so tired all them time. We were attributing this to the Baclofen, but now I’m wondering if it has to do with his breathing.

They put him on added O2 a month or two ago … this has upped his pulse ox to mid 90s; so all of this is pretty confusing to me.

Any experience/input would be greatly appreciated.

As always - thank you for all the support and guidance!!
 

lgelb

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May I ask which Houston clinic this is? There is no arbitrary threshold for BiPAP hours. There is no such thing as too many hours, if he's comfortable with them, but a lot of harm can come from too few, if he needs more. It's also true that the respiratory impairment and/or baclofen can lead to fatigue.

Does he have a feeding tube?

Let me know if you want help adjusting the settings to his comfort. Another common reason for fatigue is settings that are too high or low, or not synched with natural breathing. I don't know why they added oxygen before maximizing the BiPAP hours, since it can increase excess CO2 (did they discuss their reasoning?), but he should only use oxygen while he is using BiPAP, and usually, especially at low altitudes like yours, PALS do not need it. So that's another reason you might consider re-evaluating his settings.

As for the trach, the point of no return is not a given FVC (and I should note that the FVC in bulbar onset in particular is often misleading since the ability to form a seal is limited and the test requires the use of different muscles than PALS can access in a more natural setting). The key is his stability on BiPAP -- it's an individual thing. It is certainly worth staying up to date on his preferences so that when he cannot be stabilized on BiPAP you know what to do, but most PALS do not choose the trach. The right settings and the right hours maximize the time that he can stay stable on BiPAP.

Best,
Laurie
 

kljack16

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Thank you for the quick reply Laurie. I truly appreciate your input. I asked the pulmonologist about the numbers, the decline, and to your point, the settings on his AVAPS. He said the settings were good, and right now, our best option at slowing the decline is increased usage; so, that’s what we’ll do.

We are going to Houston Methodist for the ALS Clinic. No - he doesn’t have a feeding tube yet. His ability to eat is getting more precarious, but he wants to hold off as long as possible on the PEG. Methodist didn’t push for it yet, but definitely encouraged him to start giving it serious consideration since it’s better to plan it than have to do an emergency insertion.

As always, your input and caring are so welcomed and appreciated.

Sincerely,
Kelli
 

wishmobbing

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If your husband is not opposed to getting a PEG at all, get it now. The sooner the better, as in: it's there when he needs it. You don't want to get in deep water and have time where it's hard for him to swallow meds, drink enough and eat enough. Just having a minor cold might make him feel weak enough to not be up for the task.

With the PEG he can still eat and drink all he wants but he doesn't have to. If drinking enough fluids to have good digestion is getting hard, it's no problem with the PEG. It helps him preserve energy. Just as more Bipap hours will help preserve energy.

My PALS decided pretty early on to get the tube after I read some good reports on here. I was a bit worried about the extra work but it's really no big deal compared to Bipap or worries of choking fits and the risk of pneumonia they entail.
It keeps eating more fun because it's not a necessity anymore.
Sounds like good advice Houston is giving.
 

kljack16

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Thank you! We actually had schedule the PEG procedure for early February. Then, the week before we were supposed to have it done, they cancelled due to the uptick in COVID cases. Then, the nurse here in MS encouraged us to wait since it’s just another area for possible infection. Seems there are pros and cons all around, but I truly appreciate your insight.

I hadn’t thought of it in the terms you said (especially the energy conservation), and now, I’m back on the “sooner than later” plan. I just have to convince him. And I appreciate you saying you were worried about how difficult it would be but found it easy and well worth it. I will pass all of that along to him. I really like the idea of him being able to eat for enjoyment again.

Thank you!!
 

wishmobbing

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You're on it, you got this! (y)
Trying to dodge Covid is a concern I support. This pandemic messes up timing of so many things and procedures.
In the long run the tube is also helpful to avoid scary hospital stays.
My PALS only had to stay in the hospital twice. Once to get diagnosed (he hated being there, alone and with bad food) and then a planned stay in an ALS clinic to have his Bipap set up. He grabbed the opportunity to have the PEG placed at the same time so never needed to go back.
 

Samkl

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Laurie, what sort of downside is there for not being on the BIPAP as often as you might comfortably need to. Our PALS is off his quite a bit because he feels better able to “be” with people.
 

lgelb

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Just that the "battery" if you will, the muscles that help him breathe, are working harder without support, and therefore research suggests that could affect their lifespan.

Has he considered a sip 'n' puff interface for social occasions, if he has some head movement and he doesn't want to wear a mask when with people?
 

Samkl

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I’m not sure what that is, but is primary CALS may. I do know that he feels he has no sniff capability. Would that rule out the sip n’ puff ?
 

lgelb

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Not necessarily. Sip 'n' puff is drawing in air from a little tube or mouthpiece that is attached to the air hose instead of a mask, and breathing out into the open air. So it's not the nose muscles but the lips and head/neck that are more key. If they have the capacity, all you need to try it is an angled mouthpiece that will fit your hose, usually a 15mm size, and then kludge a mounted position on the wheelchair or somewhere else where he can access the mouthpiece when he wants a breath.
 

Samkl

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Thanks Laurie, I’ll follow that up!
 

Samkl

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I’ve checked the Resmed website, for sip and puff, and found just this. There doesn’t seem to be specific attachment for it, so I assume we just try to find a 15mm mouthpiece?
 

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lgelb

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You don't need an attachment, just the mouthpiece. The hose holds it in place. If he likes it, you can get a stiffer piece down the line for better positioning.

Google 15mm angled mouthpiece and you will find one that Respironics makes, and lots of others. You don't need a brand or anything -- it's a generic part.
 

Samkl

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Thanks Laurie
 

JohnHMich

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We just back from our ALS clinic visit on Wed and they reported a FVC 25% of predicted. I found it very demoralizing. My wife has a PEG and keeping her hydrated and giving meds is way easier. The wound care has been straightforward.

I find the pulmonologists are vague about the relationship between FVC, paCO2 and Sats. Her sats are in the 90s, CO2 was 37.5 and yet the sharp decline in FVC/FEV. It seems like it ought to show up on the pulseoximeter…
 
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