PALS Having a Harder Time Breathing - Would Love Your Thoughts

[Allie1987]

Hello Everyone - I posted a few times in the winter soon after my best friend was diagnosed. I’ve been back here to find info many times - which was very helpful! - but haven’t posted in a while.

The quick update: P. has lost the use of one arm completely; can still walk, but barely (wheelchair and scooter are next step); and has a lot of weakness in his neck muscles so his head droops. By April his FVC had dropped to about 50% and he got a bipap, which he uses only for sleep. Up until a few days ago, that seemed to take care of his breathing problems.

But, he told me yesterday that his breathing has worsened, and he finds it is getting difficult during the day too, which causes him a lot of fatigue by the end of the day. The other related thing is that between his head droop and the breathing challenges, he finds it hard to find a comfortable position either sitting or lying down.

He is currently in the process of moving to an accessible apartment (currently on the third floor with no elevator - yikes!) and will be getting a scooter soon. So he had been feeling hopeful that once these things happened, he would be able to get out of the house more and participate in life.

But now he is worried that between the physical/postural discomfort and breathing problems, he just won’t feel well enough to be able to get out and enjoy life.

He will see his neurologist on Friday for another FVC test and ask her advice. (Up until recently he was at the UVA clinic, which was almost useless - they seem to be having a lot of problems there. Now he is at GW.)

A few things I thought might help him were: 1) find a pulmonologist; 2) maybe some sort of neck brace, or a recliner, for the head droop; and 3) maybe try using the bipap intermittently during the day to give his breathing muscles a rest. I’ve also read that there are specialized breathing techniques out there like breath stacking, but have not delved into the details.

Frankly, his progression has been so rapid in the past 9 months that it is hard to keep up - it seems as though every time we solve a problem there is a new one!

But - I think the people on this forum are some of the best experts around on living with this thing, so I’d love to hear any ideas you have on the above. Especially on his fear that he will no longer be able to go out and enjoy life - how accurate is this? I had thought we would have a lot more time to go out and have fun, and was really looking forward to having the scooter help with this.

Also, I was surprised that he is having so much trouble with breathing this early (relatively) in the process. He is still fine on speech and eating and drinking, and while walking is hard, can still get around his apartment with a cane. I guess I had expected that breathing problems like this would come much later in the disease process. So now I’m wondering whether he is closer to the end than I had thought.

Ps. I did do searches to get info on breathing and neck weakness, but have these additional questions.

 

[lgelb]

Hi Allie, needing the BiPAP during the day does not mean that P is near the end. Keep in mind another FVC isn't going to be a guide to settings or when to use the BiPAP. That guide is him, by how he feels and breathes. I'm happy to help with adjusting the settings to those needs, something I frequently do here, so will PM you on that.

Even if the FVC remains stable, it is not the best breathing test (it just happens to be one of two used in the US to justify BiPAP) and even when triangulating several tests, they are sort of like driving using the speedometer. The pulmonary function tests are not the visual field in front of you, showing you where and how fast to drive.

There is no reason he cannot go out on BiPAP, and he will be more comfortable on it, so able to stay out longer. You can turn it off if/when he doesn't need it. We went to movies, concerts, festivals, etc. even once Larry was on 24/7 BiPAP.

If walking is difficult, and given the head drop, excursions with a walker instead of a cane, and a wheelchair at some point (that can tilt/recline to support his neck), will also help conserve his energy. It sounds like it may not be too soon to make these points.

A medium firm cervical collar, which is a few bucks on Amazon, sized to his neck height, can help you see if he needs something softer, shorter, more structured, etc. without a major investment up front for something that may not work. Collars are a very individual thing. Does he use a nasal mask? That will work best with a collar.

I'm glad to hear that he is getting a more accessible home. That will certainly help.

Best,
Laurie

 

[Mary2]

The insurance companies and Medicare have restrictions on how many mobility devices they will pay for how often. We paid for a scooter out of pocket. Between your friend requiring increased time with the bi-pap and requiring neck support, he may want to skip the scooter and get into a wheel chair with tilt. The scooter can be rather a bother taking it apart and putting it back together again. I found the pieces to be rather heavy.

 

[Nikki J]

Agree with above. If he is reluctant to use bipap during the day in part because of the mask he has options for something different during the day.

Using his mobility benefit for a power wheelchair that is it to him specifically is important too. The clinic can order if they have not already. Ask tomorrow because the process takes time. Recline or tilt position will help with comfort However do ask for recommendations about a neck brace too. When the muscles are weak they can’t protect the cervical spine.

 

[Allie1987]

This is all super helpful - thanks so much! And thanks very much to lgelb for the offer to help with settings.

It sounds like this is maybe not as dire as he is interpreting it (in terms of his ability to continue to get out and enjoy life) - which is very encouraging.

On the upcoming FVC test - that was actually previously scheduled (before the current breathing probs started). The neurologist wants him to get a feeding tube inserted before his breathing gets worse, so I think the FVC was to see where they are for that purpose (as well as general monitoring). Sorry I was unclear - I was trying not to go on for too long.

Re the scooter vs. wheelchair: He does not have a wheelchair van, so we were thinking he could get started on the process for ordering a pwc, and in the meantime purchase a portable scooter out of pocket that could be put in the car trunk and used for outings. Once he gets the pwc he could use that in the apartment.

At some point the scooter may no longer be sufficient for outings, and then he will need to decide if he can manage to get a wheelchair van (they don’t have a lot of money). But this was our interim plan.

One question: I do think his hesitation with bipap during the day is related to the mask. What are the alternatives to that, especially if you are using bipap during outings?

Also, thanks for recommendations about getting a neck brace. I will suggest he ask his neurologist about this. His posture looks super uncomfortable so I hope that can give him some relief.

Thanks again!

 

[Nikki J]

I was thinking if he has a full face mask at night maybe nasal pillows? Just an example

Re the wheelchair transport issue. Is there paratransit where he is going to live? It isn’t as convenient as having your own van but here if you can’t use regular transit ( and our transit is almost completely wheelchair accessible - 1 or 2 stations aren’t but all the trains and buses are) there is a service that will take you and one caregiver if needed for a fairly small fee. Also ask ALSA. The way you describe his issues a scooter isn’t going to be comfortable even now. And probably impossible soon.

 

[Doing My Best]

Hi Allie, soon after my PALS got the power wheelchair we got into the question of head support because his head drop progressed rapidly at about that time. We got a soft collar first, but it wasn't enough, and the best solution was a headrest on the wheelchair. There are various styles, and the one he uses now includes a strap that goes across his forehead to hold his head up, without which it droops almost to his chest. It also rotates to allow him to turn his head from side to side. It's called The Axion, by Symmetric Designs. You might start researching different options and see if you can get one delivered right along with the PWC. My husband's was covered by Medicare. ALSA might be able to help you with the research.

 

[MupstateNY]

an alternative to the scooter idea is an electric wheelchair such as the majestic. the newest model is very lightweight and easy to fold and load into the car. i bought mine from comfy go.

it can be used with chin support.

 

[lgelb]

Chin support will likely not be enough for neck drop and a lightweight PWC does not provide the same tilt/recline options that make a less invasive neck support (often important, esp. with bulbar issues) possible.

The cervical collar may help pre-wheelchair. Post-wheelchair, it's best to use a support that works with the chair so your neck is not pushed out, and to take advantage of what the wheelchair offers in itself.

Agree, a scooter doesn't seem like a solution at this point.

Many PALS use a mouthpiece or nasal mask during the day and a full face mask at night. But often a full face mask is not needed at all. PALS are typically written one at the start, but it's not a person-specific thing, usually, but the practice's habit.

 

[MarkD]

I have had breathing issues almost from the beginning. I manage things with three settings on my trilogy ventilator. One setting for the mask I use when sleeping, and one setting for the nose pillow I use during the day when I lie down in bed off and on. And lastly the third setting which I call my puffer setting, which i use when I am sitting at my computer, and just take puffs from the ventilator. In between all this when I'm standing up I can go quite a while if I need to without needing the ventilator to help breathe, however I can't stand up for a real long time so when I need to sit or lay down I need the ventilator.

 

[oldgeek]

I'm a PAL and can related. I could not get enough air on Non Invasive BiPap that I got a trach and put on night time ventilator. However, I too became weak and tired during the day and so they added daytime ventilation as needed. It has done a super job of eliminating my fatigue. My pulmonologist told me that people with our condition burn about 80% of our energy just trying to breathe - which is why the daytime vent is helpful. He also ordered a feeding tube which I'll get the end of the month. Finally, I too have head and neck problems. I found those inexpensive airline pillows worked great, but I also have a cervical collar I wear that allows me to feed in the vent tubing and does not press against the trach tube. Bottom line, it's all trial and effort. Good luck