Fast progression?

[Iscah1]

My PALS feels his progression is fast. In November 22 when he was dx, he had trouble walking and his weakness in his arms, muscle twitches and tremors. Today he can still walk, but it’s gotten slow and he had a fall recently so he uses a walker. His neck is starting to droop and his posture is really bad. He has trouble breathing and constantly feels like he is struggling for air. He has lots of trouble standing up from a sitting position and getting out of bed. He can barely dress himself, but he can do it if he has to. He cannot wash his hair and can barely shower. On the upside, he can still swallow and talk. His writing is terrible but he can type and use his smart phone so he is still able to work. Today we were ordering leg braces and he said it’s a waste bc he feels he will lose his ability to walk in a few months. The thing is, I can see progression but I don’t really know what is fast and slow. My PALS knows his body and he frustrated bc he feels he’s going downhill with no plateau in sight. Plus we don’t go back to ALS clinic until June. His score was 33 in February so I’m afraid what it’ll be in June.

My question is what determines fast or slow progression? Also, do we just sit at home between clinic visits and deteriorate? I was so happy to go to clinic but I feel like we were sent home to with more questions than before. The drs at the clinic said positive thinking was very important, but insurance denied the bipap and he’s getting worse. My husband is having trouble thinking positively and so am I.

 

[Nikki J]

I am really sorry. Fast and slow is somewhat subjective and anyway progression is not linear.

i think you need to contact the clinic. Mine will see us within a couple of weeks at most for urgent needs or significant change. That will usually be with the np for us

if he is going to need a wheelchair soon the process takes awhile so you should discuss that and get the ball rolling. Why did the insurance refuse bipap? Was his svc too high?they can check it again. In my sister’s case her svc dropped suddenly but not enough for bipap- until they tested it lying down. Don’t be afraid to ask for that.

 

[poet1973]

I understand completely what he's going through. I feel mine has started progressing faster than I would like. Last week I was at home alone because my wife was still working and then she got up and ready to leave for work and I fell!!! So she quit her job that day!! I'm not able to get out of bed, dress, or shower without assistance. Howeer I can walk some and feed myself it's a struggle. Therapist at clinic told me to use my braces because it would help me walk better and could help keep what strength I have in my legs!! IIt's been a year since my dx. It is depressing when you feel your body is betraying you. I feel I don't want to be a burden to anyone because my wife didn't sign up for this 30 years ago!!! All types of things run through your mind but it does help to talk to people who are going through the same thing!!! Trying to think positively is hard because you never know when the other shoe will drop!! My family has been wonderful trying to keep me upbeat!! But this disease affects everyone differently!! Hope everything works out for you and he!! Just try to keep your head up!!🙏🙏

 

[lgelb]

Just a note that for Medicare and most commercial plans, the BiPAP reimbursement threshold is FVC<50% and/or MIP< 60 cm/H20.

The US thresholds are more restrictive than in many other countries, so if he is having trouble breathing and does not meet these thresholds, you can get any doc to prescribe a home BiPAP and pay cash while you pursue a letter of medical necessity for ultimate reimbursement (which are still likely to be rejected, so never spend more than you can afford).

SecondWind CPAP has refurbs that make that more affordable (they have a refurb VPAP ST-A with iVAPS, my recommended home model, available right now, for example). If money is tight, any model of ResMed BiPAP is better than nothing.

 

[Tomswife]

Hi. PALS Tom is fast progressing. Dx august 2022. I am sorry you are both on this journey. The infrequent visits to the ALS clinic did not meet our needs. We formed a team of local physicians and we are getting very good care. Our team is - internist, pulmonologist, neurologist. Gastrointerologist. As Tom progressed, we needed the neurologist less and the other doctors have moved to the forefront. But our team offers telehealth. After we left the clinic, we had 3 meetings with neurologist in one month rather than waiting for clinic.
I wish you the best in health care on this journey.

 

[KimT]

I also got a local team that includes a pulmonologist, pain management neurologist, PCP, and gastro. They are all affiliated with Advent Health Hospital which, if I ever need hospitalization, that's where I'd go.

Regarding the breathing. If your PALS neck is starting to droop, that could affect his breathing but, if it were me, I'd get a complete pulmonary function test to find out where his actual breathing stands. I would also insist that SVC (slow vital capacity) is done both sitting and on PALS back. Mayo also did mine standing.

It's hard not to worry about progression or focus on it, but staying ahead of your PALS needs helps lessen worrying about the rate of progression.

 

[Buglaw]

Dumb question, but if you get a bipap early, would insurance not reimburse you once it becomes medically necessarry? I know a lot of places reccomend bipap at 75% now. So if you buy one at 75%, can you get reimbursed when you drop to 50%

 

[KimT]

If your BiPap is medically necessary and you have an Rx from your pulmonologist, insurance should cover it. Whether they cover high end Paps is another question. Medicare always covered my Trilogy and my FVC was never below 68%. My MIV and MEP were normal. Medicare does change rules so it's best to go through your clinic and get pre-approval.

 

[lgelb]

No, Buglaw, that's not how it works. They go by the actual dates of purchase (the reasoning being they don't want to pay for any use before when they think you need it). Of course, you can always try -- just not the way to bet.

Kim, even commercial plans use the Medicare criteria for a RAD in ALS: FVC<50% and/or MIP<60 cm/Hg. What was your MIP (is that what you meant when you typed MIV?) when you first got the machine? "Medically necessary" is policy-defined as those numbers and only those numbers, in neuromuscular disease. I don't know what your plan is doing, but I would not advise anyone to count on the same forbearance.

Now, it's true that if you have another condition/numbers that qualify, you could possibly get a BiPAP covered under some other criteria. But the plan is entitled to apply the more stringent criteria if you have a clinical dx like ALS. They don't want to pay twice, like with the wheelchair, for a low-end BiPAP and then the Trilogy or Astral.

 

[Iscah1]

Apparently the pulmonologist asked for a higher cost machine called an AVAP or something like that. This is according to my PALS. I really didn’t understand because the denial letter said if the doctor ordered a bipap, which is what I thought he was ordering, it would be approved. The cut off was 50% (of what, I’m not sure) but Larry test was 43% so he was eligible for insurance coverage. After he was diagnosed we looked into buying a bipap ourselves, but 1) you have to have a prescription and 2) I’m sure there are more than just the cost of the machine, like the RT and getting the settings correct, etc. The pulmonologist was out for spring break (I’m assuming), so I will call again tomorrow to understand what is going on. The insurance is driving me insane. They also denied RADACAVA, which we expected, but do they approve anything without a fight? 😕

 

[lgelb]

50% is the FVC cutoff. Is Larry on Medicare? With an FVC of 43%, Medicare and most other plans will cover the kind of machine he needs, which includes either AVAPS or iVAPS functionality.

Any doc can write a BiPAP, just so you know. And you do not need an RT to use it.

But from what you've said, you should get an Astral or Trilogy paid for. So that is what I would focus on. Hopefully you can find someone in the office who understands how to submit the justification. Often, there is just some information missing and insurance kicks it back.