Diagnosed with respiratory onset ALS

[Aaronmandevill]

Hi everyone, I (36m) previously posted in DIHALS thread: Looking for advice

Today I saw Dr. Wong who did extensive testing. We spent a very long time together, part of which he did some of his own research on respiratory onset since he wasn’t as familiar with its presentation.

He agreed that the documented changes to my swallow over 30 days, my sleep study overnight oximetry being 88%, my body wide fasciculations, and weight loss was enough to start me on radicava and schedule a feeding tube.

He also wrote me a prescription for a bipap, but he said everyone is on back order. Does anyone here know about how to get one the fastest? Also he mentioned needing to go on auto-bipap for 60 days so the machine could learn my breathing patterns, should I do that or reach out to you (Laurie) for changes?

He scheduled me for a SNIFF test and said Dr. Appel’s team could do a diaphragmatic EMG but he is not skilled to do that. I have a referral still in the works to be seen at Houston Methodist.

He also said he could put in for AMX0035 but I would need to find a pharmacy that has it if anyone knows. The nurse mentioned possibly getting it from Canada as well if anyone has info on that.

Part of me wants to keep it from my family for awhile, because they have already been so stressed out, but I will probably call them tonight.

Thanks for all your support and being an amazing community.

 

[Nikki J]

Sorry to hear this.
re amx right now you can’t get it from Canada it is technically approved but not available yet. People in the US get it from a compounding pharmacy but there has been a shortage of components and last I heard even existing customers have not been able to get it. Maybe that has improved. You might google compounding pharmacies and call around. The ones I know are Johnson in MA and Hopewell in NJ. Both ship to other states but not to every state and at least for Johnson it is not solely a distance issue

 

[KevinM]

I am so sorry to hear this Aaron. Please keep us apprised, and feel free to ask anything.

 

[KimT]

Aaron, I'm so sorry. I imagine the SNIF test will be done with a complete pulmonary function test. The SNIF will show your inspiratory ability through your nose but the whole test will give everyone a better guide on BiPap settings.

This diagnosis takes awhile to process. Don't think you have to do everything or know everything at once.

Being in the military will give you many benefits. When you're ready, just ask questions.

 

[swalker]

Aaron, I have followed your story in the other sub forum. I am very sorry to see you get the diagnosis.

I use a Resmed Astral 150 bipap. We set the various parameters manually and adjusted them as needed. It took a long time to get the bipap adjusted for me, but most of that was due to COVID and me not wanting to have folks visit the house compounded by the medical facility not wanting me to make changes myself (even under phone supervision).

Once we broke that logjam, we were able to get the bipap adjusted for my needs in short order.

I really like the Astral 150. It is more compact than competing brands. The user interface is easy for me to use. It has a 6 hour battery built in and I have two additional 8 hour batteries, so I have power for 22 hours. I have tested this and I really can go over 18 hours on batteries alone (with hours of reserve).

I also have a small gas generator that can power the bipap if power is out for longer.

I was hesitant to share with anyone. Now, we joke that it is as if my wife took out an ad in the newspaper. She communicates much more freely that I do. Either way, everyone will know sooner or later. Do it at whatever pace you feel comfortable.

I know you have been going through diagnostics for awhile, so you have already had some time to process the news. But, be sure to give yourself all the time you need to do so. It may take longer than you anticipate to really wrap your head around it.

Being military, you will have many benefits available to you with ALS that are not available to others. There is a subforum here devoted to military folks, so be sure to check in there.

My sister lives in San Antonio. While visiting her, my wheelchair van and wheelchair were stolen. I can't say enough good things about the ALS Association in San Antonio. They really helped me out during that time, providing a loaner wheelchair. Don't hesitate to call them if you need anything.

Steve

 

[Aaronmandevill]

Thanks for the reply Steve, I didn’t think about reaching out to the ALS association here in town. I am trying to get the diagnosis notes from Dr. Wong to the Air Force so I can get benefits like home health care and medical equipment.

I have seen lots of people using resmed and I think I saw it in stock. I already bought a couple masks to try. Is there a specific hose I should get or any connectors in case I miss ordering something?

Thank you @Nikki J I will begin calling around tomorrow morning, lots of compounding pharmacies out there it seems

@KimT I will ask my PCM in the morning because she would potentially put me in for SNIF test so I will ask for a redo of pulmonary function as well. She ordered the last ones so she might do it again.

 

[lgelb]

Sorry to hear this news, Aaron.

The tests don't have anything to do with BiPAP settings (they are basically done to justify the order to the VA or Medicare), nor is any "auto" period needed. When you get your Astral (agree with Steve, it's the best portable solution) or Trilogy, let me know and I can help you get the settings where they need to be.

We will support you however we can.

Best,
Laurie

 

[Aaronmandevill]

@lgelb Thanks so much for your support on here with all the bipap settings and information. The astral looks a bit pricey so I’m going to ask my pcm tomorrow if that’s something they could cover. If not I have a very supportive aunt who has been providing financial assistance thus far which is why I was able to see Dr Wong so quickly since I bypassed insurance and referrals. Lowest cost for astral seems to be $4500-$5500

 

[lisa g]

Aaron, I'm sorry to hear about your diagnoisis. After reading the above posts your getting good advice. Take time to process everything. You'll have plenty of questions and we're here to help guide you and answer them.
As far as obtaining AMX0035 at the present time is an issue. I have been purchasing it from a compounding pharmacy for the last 6 months. They were unable to fill it this month due to a nationwide shortage of one of the components.
Just know your not alone in this journey.

 

[Aaronmandevill]

@lisa g Thank you for the reply. I wonder if it gets approved in the US if they will be able to amp up production. I’ll just keep calling around to see if I get lucky. At my progression rate it seems it won’t matter too much but I’m in the mindset I want to try and survive as long as I’m able. That may change in the future but that’s how I feel today.

Oh quick question does anyone know how I actually acquire the radicava ORS? My doctor faxed over the benefit form today.

 

[lisa g]

Aaron,
The doctor faxing over the benefit form is the first step. I've been on radicava infusions for over 2 years and am in the process of transitioning to the oral form.
Once the form is faxed you'll be given an advocate from Journey Mate. From there they will run it through insurance to see if you'll have a co-pay. They have programs that will help with or pick up your out of pocket cost. It shouldn't be a lenghty process. You can always contact Journey Mate to see where you are in the process.

I hope this helped.

 

[swalker]

Aaron,

A refurbished Astral 150 could cost less than $6,000. I believe a new one goes for around $20,000, but I have not checked prices recently so those numbers could be out of date.

There are different types of circuits that can be configured for the bipap. The arrangement of hoses you need will depend on the circuit you use.

My Astral 150, hoses, maks, humidifier, batteries, etc. etc. were all provided for me via Medicare. A respiratory therapist came out and set everything up. I receive new masks and other bits and pieces as necessary from them. So far, Medicare has covered everything. I have Medicare parts A and B along with a Supplemental Medicare policy through USAA.

You will be under Military medical care, presumably transitioning to VA. Things will be different there, but you should have excellent coverage.

Steve

 

[Aaronmandevill]

Steve, Thanks for the reassurance that once the military activates my care hopefully the support will increase. At this point my boss is saying I have to take paid time off since my records are not transferred to the military clinic yet. I told him to just have the police come pick me up at home since I can’t lift my head, figured that could get some good news coverage.

Tomorrow hopefully I can get everything in motion. Air Force won’t schedule a feeding tube until I’m aspirating/choking on liquids. So I may have to pay out of pocket to get a feeding tube sooner as well. Will ask tomorrow how much that might cost with GI.

I did see a refurbished one for $5300 so might go with that. Need to make sure my aunt is onboard with me going that route first. Us military guys don’t make too much $$$.

@lisa g Thanks for that about the process, I will need to make sure they actually faxed over the request and then follow up. The only negative about Dr Wong is his office staff is very bad about doing things in a timely manner. But I found if you sit in the waiting room all day they find time to get things done quicker.

 

[Aaronmandevill]

Ok I’m looking to purchase a refurbished Astral 150. At the bottom both websites say Can not be linked to My Air App. Does that matter at all?

 

[lgelb]

No, that's a remote configuration app. You can do all that on the machine itself. But not all these web sites are reputable. A reputable source will want a prescription, anyway (though any doc can write it). The issue is whether to wait for a script that your payer will reimburse you for.

A second option: SecondWind is selling the Aircurve ST-A for <3k, which you could use at home (the difference with the Astral is the battery for portability) while you get in with the VA or even your active duty plan, and you could use it later as a backup machine. If you buy anything for cash, make sure you get a matching humidifier, too.