long intro, with questions

[timrous beastie]

When memory lapses and word-finding started slipping sideways more often last spring, we thought it was his family history ramping up-- Alzheimer's, vascular dementia, FTD PPA, etc. A long slow cognitive decline, in other words, that we'd openly discussed and (somewhat) dealt with in our emotional & financial planning since 2018 or thereabouts. I watched and bookmarked countless Teepa Snow videos over the years, and even attended some PAC virtual conferencing and webinars.

After taking an early retirement offer from his 20 year tech support career, my love looked for part-time work NOT sitting at a desk. He found it working as a museum gallery attendant... and much enjoyed the short walk (1/3 mile) from the train station to the art museum, where he spent the day on his feet reminding people what not to touch, etc. He planned to stay there and to defer taking his Social Security benefits until he turned 70 at the end of this year, in part to better provide for my future financial health & well-being.

But Mousie, thou art no thy-lane,
In proving foresight may be vain:
The best laid schemes o’ Mice an’ Men
Gang aft agley

Summer/fall of last year my love started having occasional coughing fits when taking his meds & vitamins. His voice grew hoarse, and he started tiring more easily at work and home. His gait slowed, and he sometimes tilted to one side. He lost his balance and tripped a few times in the winter. The idea of working until the end of 2024 was looking less and less realistic, and by February that 1/3 mile walk from the train station to the art museum became intolerably exhausting. He quit the job, and I cut back on hours at my (part-time) job to start accompanying him on doctor visits. We both hoped & thought that the physical issues were mostly rooted in his long-term COPD and the c6/c7 spinal injury he suffered in a car crash in 2019, plus generally getting older.

*sigh*

His primary care doc had initiated a "routine priority" neuro referral in January, based on the balance issues, but nothing had yet come of that when we saw his Pulmonary PA-C in late February, for a review of his meds and recent imaging. She set us up with some additional tools and techniques to get better effect from his meds, then pushed on the neuro referral for us again, with a higher priority. She said she wanted to schedule another full function work-up if Medicare would cover it, or at least an abbreviated work-up if they balked on the timing. Her speculation was our first heads-up that we might be in for the rough ride of a neuromuscular disorder.

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NEURO DATE: 03/18/2024
Interview, physical exam and reflexes, MMSE (26/30), bloodwork.
Tests ordered: EEG, MRI Brain, MRI C-Spine

DIAGNOSIS: Chronic fatigue R53.82 ; Cognitive impairment R41.89 ; Muscle weakness M62.81 ; Ataxia R27.0 ; Neck pain M54.2 ; Tremor R25.1 ; Raspy voice R49.0 ; Dysphagia, unspecified type R13.10 ; Obstructive sleep apnea G47.33 ; Fasciculations R25.3 and Dyspnea on exertion R06.09

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NEURO DATE: 03/22/2024
Electromyography and Nerve conduction study done.
Tests/info still pending: EEG, MRI Brain, MRI C-Spine, report from Pulmonary

DIAGNOSIS: Fasciculation R25.3 ; Chronic fatigue R53.82 ; Dysphagia, unspecified type R13.10 ; Ataxia R27.0 ; Orthopnea R06.01 ; Obstructive sleep apnea G47.33 ; Memory loss R41.3 ; Anterior horn cell disease G12.29 and Idiopathic peripheral neuropathy G60.9

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We saw the Pulmonary PA-C again on 03/26/2024. She highlighted a couple of numbers from the new PFT and showed us how they compared to past numbers. She put in an order for nocturnal oximetry test kit, which we have not yet received. I'm pretty sure that order was about jumping thru the Medicare hoops necessary to prescribe a BiPAP... more on that at the end, with the QUESTIONS mentioned in the thread title.

02/21/2023 * FVC %Predicted = 63 * FEV1 %Predicted = 56 * TLC %Predicted = 86
11/28/2023 * FVC %Predicted = 49 * FEV1 %Predicted = 44 * TLC %Predicted = 76
03/19/2024 * FVC %Predicted = 50 * FEV1 %Predicted = 51 * TLC %Predicted = 67

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Getting the MRIs done turned into a bit of an ordeal, and some out of pocket expense, but that's another story. Mainly, that delay is what led me to discovering this forum after I deciphered some of the neuro notes in the patient portal. It also led to the discovery that our Neuro is someone who specializes in ALS. So... however very very much we'd hoped otherwise, we were not completely unprepared for the news we got on Thursday.

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NEURO DATE: 04/04/2024
EEG was normal. MRI brain showed normal aging. MRI C-spine showed some narrowing, but nothing significant. Bloodwork ruled out autoimmune disease. 50% pulmonary indicates immediate need for (at minimum) BiPAP respiratory support, and consideration of PEG.

DIAGNOSIS: Amyotrophic lateral sclerosis G12.21 ; Muscle weakness M62.81 ; Chronic fatigue R53.82 ; Ataxia R27.0 ; Dysphagia, unspecified type R13.10 ; Dyspnea on exertion R06.09 ; Orthopnea R06.01 and Memory loss R41.3

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Long story short (too late!), here are my questions:

I plan to contact the Pulmonary PA-C on Monday, making her aware of the diagnosis and asking her to expedite an Rx for BiPAP. I guess we could still use the noc ox kit when it does arrive, for the data, but it's my (google-based) understanding that this Dx eliminates the Medicare requirement for completion of sleep study prior to device Rx. Correct?

It's also my (google-based) guess that once we have the Rx, we should further expedite things by paying for the first machine out of pocket, thru SecondWind or some such, and letting the presumably slower Medicare process provide for an additional device as back-up. Yes?

Most importantly, how can I best figure out what device to buy and where?

Also, is there anything else that I should ask the Pulmonary PA-C to do right now... in regard to the BiPAP or otherwise?

I know we will still have much to consider and research in the coming weeks/months, but getting my love some NIV respiratory support ASAP is my most immediate concern.

Thank you for any advice you can offer. This is a lousy situation to be in, but I have perused enough of these threads in recent days to know if one HAS to be in a lousy situation, it's better to do it in good company.

 

[rmt]

I'm so sorry to welcome you here.

I don't have advice for you about getting devices and care (my husband was retired military so his care was mostly at the VA). But if your PALS is having trouble with word finding and memory, I recommend talking with him in detail to find out what his wishes are for the rest of his time here. My husband lost all of his language by the end and I was glad that I felt confident that I knew what his wishes were.

This forum was incredible supportive and helpful for me. I'm so glad you found this community.

 

[lgelb]

Very sorry to hear this news, and a sad welcome to you and of course any support we can provide.

Technically, Medicare should buy two NIVs if asked properly, one as a backup, but that doesn't seem to be happening lately, probably in part because supply is tight due to Respironics' having to stop marketing the Trilogy. Most PALS work with one, unplugging it and taking it out when they leave. The batteries are portable so you can even carry extras, while cumbersome.

But yes, a machine from SecondWind or elsewhere is a definite option if your clinic/Medicare/your DME (the supplier) takes too long to get its act together and you can afford the freight. Currently, the SecondWind site has a refurb ResMed AirCurve 10 ST-A that is the best machine available in the cash market, equivalent to what Medicare will buy in every way except portability (no rechargeable battery; must be plugged in).

Any BiPAP will do in a pinch, and obviously the less feature-rich ones (volume control is the feature most applicable to PALS since it keeps the volume constant by varying the pressure) are less pricey. I would look at both their new and refurb stock. For some PALS "ST"/a backup rate becomes important, but generally that feature isn't necessary if the settings are appropriate and adjusted for progression (e.g., reduced volume/mandatory breaths per minute when needed so the airway doesn't collapse/spasm and prevent breaths from getting started, exhalation is supported as fully as possible, etc.).

You should not need overnight oximetry data. And I wouldn't do it bc the test could be kicked out as unnecessary and billed to you. It was never necessary anyway, in ALS, and that makes me ask what clinic you are going to? We can suggest other options if you'd like, or you might look at this map.

You may also want to look at the respiratory sticky if you haven't.

 

[timrous beastie]

Thank you, rmt.

... I recommend talking with him in detail to find out what his wishes are for the rest of his time here. My husband lost all of his language by the end and I was glad that I felt confident that I knew what his wishes were.

I am glad that you had the relative comfort of making difficult decisions for your loved one based on KNOWING instead of guessing his wishes. My love and I have had a number of past conversations about medical interventions and EOL issues. We've both consistently emphasized quality over quantity, but now need to revisit those topics within the specific framework of the complexities involved in this Dx.

This resource that I found at I AM ALS looks like a good place for us to start:

https://www.iamals.org/wp-content/uploads/2020/10/Identifying-Your-Values-Checklist.pdf

 

[timrous beastie]

[aargh. note to self: the EDIT window was only 5 minutes.]

Laurie -- thank you so much.

I had some inkling about prior supply issues and recalls from a few years back, when our son got a CPAP for sleep apnea, but didn't know that was still ongoing, and affected the (more versatile/portable, albeit far pricier) Trilogy.

... Currently, the SecondWind site has a refurb ResMed AirCurve 10 ST-A that is the best machine available in the cash market, equivalent to what Medicare will buy in every way except portability ...

Noted, thanks.

Any suggestions (regardless of BiPAP model) on how to best guess at first style of mask to try?

Any BiPAP will do in a pinch, and obviously the less feature-rich ones (volume control is the feature most applicable to PALS since it keeps the volume constant by varying the pressure) are less pricey. I would look at both their new and refurb stock.

What about the ResMed S10 AirCurve VAuto?

Definitely less pricey, but also less user tweakable? I'm working my way through the respiratory sticky now, but my brain's on overload trying to grasp the significance of various tech specs & mode differences at this point.

You should not need overnight oximetry data. And I wouldn't do it bc the test could be kicked out as unnecessary and billed to you. It was never necessary anyway, in ALS, and that makes me ask what clinic you are going to? We can suggest other options if you'd like, or you might look at this map.

I will ask the Pulmonary PA-C to cancel the noc ox order. To be clear-- the order was placed about 10 days *before* we got the ALS diagnosis, by way of the pulmonary care facility that was already treating my love's COPD. We haven't yet been scheduled into the multidisciplinary ALS clinic associated with our Neuro, although I expect we will get a scheduling update early this week.

Thanks, too, for the map link. There are three (Non-VA) facilities in our general vicinity. As it happens, one of them is where we got the Dx... although we didn't originally go there with ALS on our radar. My love has expressed interest in getting a second opinion, based in part on reading an article at ALS.org... and also, I think, from an understandable element of wishcasting. Assuming we can get the Medicare clearance, we will probably pursue checking out one of the other two facilities on that map as soon as we can.

I'm more inclined to the dread certainty that the Dx is correct, based on both where we got it, and on this (newly discovered) think als tool. Either way, the ASAP pursuit of BiPAB remains my top actionable item.

*sigh*

 

[Nikki J]

Everyone should get a second opinion. Not only to confirm diagnosis but they can give another point of view about approach. And you might like clinic 2 better. If you have original medicare ( not an advantage program) just go ahead and make an appointment

 

[timrous beastie]

Thanks, Nikki. Yes, it's original Medicare, with one of the better supplement plans (Plan F).

Do you think it will make any difference on timing if we call directly versus asking Neuro #1 to provide a referral?

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hello, operator... i mean moderator: could you please point me toward info on the specifics of my apparent link faux paus in re the "think ALS tool" page? thanks.

(i tend to prefer making links more obvious, when possible, both for my own future reference and in case they help others, but i don't want to go out of bounds here.)

 

[Nikki J]

Links to sites that have a donation button are not allowed.any fundraising That was the issue here. Also links to unmoderated sites and product ( sales) listings are not allowed. Every link also puts your post in mod You are currently being modded anyway as a new member but that will end soon.

If you call and state you have an ALS diagnosis that might be enough. Mass General wanted a copy of my EMG. Call If it seems like they aren’t going to push your appointment ask if a referral will help

 

[timrous beastie]

Links to sites that have a donation button are not allowed.

Ah. Got it, thanks. (I didn't even notice the "donate" button there until you mentioned it.)

My "after moderation" edit, btw, was just to insert punction and spacing after the hyperlink-ectomy.

 

[lgelb]

The AirCurve VAuto does not have volume control or a backup rate. But as I say, it's distinctly better than no BiPAP. You would want to have one set of settings for when awake, another for at night, to get the most out of it. Just a few taps.

 

[swalker]

For bipap, I am using a Resmed Astral 150. It works extremely well for me and I highly recommend it.

I like it because it is relatively small, easy for me to configure (in coordination with my healthcare team), and has an internal battery that will last about 6 hours. I can connect external batteries (I have 2), each of which provides an additional 8 hours of power.

I have mine mounted on a stand that was provided by the DME (Durable Medical Equipment Provider) along with a Fisher and Paykel humidifier.

The combination works very well for me. I would not be doing as well as I am without it.

When the bipap was first prescribed, I was lead toward a Trillogy. I am glad I did my research and was able to get the Astra 150 instead.

I am dependent enough on the Astral 150 that my DME just provided me with a backup, so I now have two.

Medicare with a Supplement F policy paid for all of this (except the modest deductible associated with the Supplement F policy).

Steve

 

[lgelb]

Steve, I would always recommend the Astral for a portable machine, and Trilogies cannot legally be marketed right now, though DMEs are still distributing them. The OP was asking specifically about a "right now" option, however, so this was about what can be obtained in a shorter time than going through the Medicare/DME process, without co.mpletely breaking the bank.

 

[KimT]

Welcome. I was so glad to hear you had Plan F supplement. That is the very best. Medicare usually rents BiPaps. Your Part B will pay for 80% and your supplement will cover the additional 20%. Second opinions and other visits will also be fully covered.

With a clinic's order, you should be able to get the BiPap quickly. They will write up the order as crucial medical equipment, at least that's what Mayo clinic does.

As Laurie pointed out, we're entitled to two BiPaps but I've found that getting a power wheelchair triggers approval. My RT confirmed.

RTs will visit you in home, too. If the clinic you choose is close, you may want to continue seeing them but know that there is the option of putting together your own team closer to home.

I wish you the best in getting BiPap ASAP.

Take care,
Kim

 

[swalker]

Here is a correction to my previous post. I have Medicare Supplement plan G rather than F. They are very similar, but G does not cover the Part B deductible.

Steve

 

[timrous beastie]

Checking in with an update/frustration/more questions.

Monday: called pulmonary care facility (reminder: this is not part of an ALS clinic, it was pre-existing relationship) and spoke with medical assistant. She said she would get message to the PA-C indicating receipt of ALS Dx and our request to move forward on BiPAP Rx pronto. (I did not mention cancelling the noc ox order, despite possibility of getting hit with an "unnecessary test" expense, cuz I just wanted to get to the main point.) No response that day.

Tuesday: Called again, spoke with same medical assistant and was told that the PA-C wanted my love to go get arterial blood gas tested. Initially the med asst. said that we could simply drop by local 3rd party lab when we finished our errands, but she called back a few minutes later to amend that. Said would need to go to pulmonary out-patient at the affiliated hospital instead, but DEFINITELY still left us with impression (both on phone, and in the patient portal follow-up message) that it was a simple walk-in situation. Between groceries and and unusually long stop at tire repair facility, we decided we'd done enough for Tuesday.

Wednesday AM: I headed out for my own doc appointment (annual) and my love went off to the hospital out-patient clinic for his ABG. Both home again, he said "no go"-- woman there said it had to be done by appointment, and she was not the scheduler, that "they" would call him. (IF she provided any more info than that, it didn't stick in my love's short-term memory.) So-- I hit up the phone again, to have words with the medical assistant. Had to be insistent to push past the first available med asst, not wanting to restart story from scratch. Also had to rein in my frustration, and did manage to keep a civil tone while asking "What gives? THAT didn't go as expected, and how do we get this expedited!?" Med asst. E said she had clinics all day, asked that I reach out again if she didn't get back to me with answer by 3:30pm. Promised she would do whatever she could to get situation moving.

Wednesday PM: I fell asleep after a new round of errands, woke just in time to call the pulmonary care facility before their switchboard closed for the day. Med asst. now basically tells me that we need to call the scheduling number for "them" that she had (supposedly) included in the original patient portal message. Nope nopety NOPE. She had not included a phone number OR name of some specific entity we were supposed to call-- so she calls back a few minutes later and gives the number to my sweetie.

Thursday AM: I am scheduled to go to my part-time job today, will be first shift back since last week's Dx. (Dx was also on a scheduled shift day, but I called out then AND on my shift that was scheduled Monday.) My love will call the scheduling number for the ABG test, and either call me home from work or have our son take him if the test can be done today.

Meanwhile... I have wandered around the 'Net again, and stumbled upon a ResMed document called "Medicare respiratory assist device (RAD) coverage guidelines" - "For services performed on or after January 1, 2024". I think this document explains why our Pulmonary PA-C hasn't written the Rx yet, despite the Dx. I think she's trying to protect our Medicare coverage for any additional or upgraded RAD in the future.

I also think that, IF my reading of the document is correct, we might (a) have to start with the AirCurve VAuto regardless of paying out of pocket and (b) NOT be allowed to tinker with the "prescribed" initial settings (d*mm*t!) in any way until certain conditions are met. Why/not you ask? Because this coverage document seems to indicate that you can only jump straight to RAD that has a back-up rate IF "Chronic obstructive pulmonary disease (COPD) does not contribute significantly to patient’s pulmonary limitation."

Aaargh. Questions for Laurie: Do you think I am reading that right? And if so, do those guidelines (in re: using the "prescribed" settings) give us little or NO leeway for making at-home wake/sleep adjustments to help my sweetie stay comfortable and more rapidly acclimate to using BiPAP? (I'm having trouble just getting him to wear the hearing aids he got this month-- in part because he forgets about them, in part because everything sounds different, and in part because this Dx now has him thinking "THAT was a waste of money.")

Appreciate the continued input and greetings, y'all... (Hello, Steve and Kim)... and I am NOT unaware that my frustration at this delay is small potatoes compared to the numerous forms of delay many here have had to contend with. Other than doing more these days so that my love can conserve his energy, and trying to get/keep the medical records more organized, I'm feeling kind of helpless as a CALS so far.