Medications

[EPgh]

Hello- I am newly diagnosed, and had my first appointment in the ALS clinic today (U of M medicine- New Mexico). The appointment was over 6 hours and I was exhausted, which I think contributed to me not asking the best questions.

I will admit I have not done much research on the meds yet (I was only diagnosed last week). They said they can start me on Riluzole, but after the pulmonary function test (I scored a 58% although the score was not really explained to me), I was told I am not eligible for Radicava and Relyvrio. Can someone point me to more info on this? Why is this the case? What benefits will I miss out on by not having these 2 meds? Are there specific threads on this forum about the 3 meds that I should check out?

Thank you!

 

[Nikki J]

I just posted about an upcoming webinar on the three r’s but it isn’t until March Webinar on the three R’s

Insurance companies hate paying for expensive medicines which relyvrio and radicava are. They often make their prior authorizations mirror the trial participants as a way of gatekeeping. Radicava had a cutoff svc of 80% I remember.

There was a recent trial of another oral formulation of radicava in Europe that failed to show benefit. It wasn’t exactly the same as radicava ors though. I do know some people think radicava helps them others think not.

Relyvrio is two substances combined. One is otc called Tudca and it can be bought on amazon. If you do that titrate up slowly from 250 mg aiming for 2000 eventually

What kind of insurance do you have ? If commercial ( not government) the makers of relyvrio will help you. I think radicava has a program too. Federal regulations forbid them helping medicare etc. there are some grants that medicare people can get but they wouldn’t pay for out of pocket relyvrio and radicava.

I only take riluzole and tudca. Relyvrio made me terribly ill and I didn’t want radicava when it was iv because of the treatment burden and didn’t think the oral was worth the insurance fight for me

 

[lgelb]

Since either your FVC or SVC was 58%, EP, I'd check if they tested your MIP.

If it is <60 cm, you are eligible for a portable BiPAP (aka noninvasive ventilator with a rechargeable internal battery) most likely called an Astral, Trilogy (least preferred), or Luisa and should get the ball rolling on an order with your clinic or pulmonologist ASAP.

Even if it's 60+ cm, I'd give serious thought to a retest or getting someone (can be a doc, NP, PA, etc.) to write an rx for a standard BiPAP (without a rechargeable battery), that you could plug in for nights at home and have as a future backup machine), if you can afford that. SecondWind has new and refurb models; you send them an rx, they send you a machine.

I say this because there is a growing body of evidence that starting BiPAP once FVC=75% or so can prolong quality/quantity of life in ALS. Also, if your issues started in September, or even a few months before, you are progressing quickly and so I would not want you to wait for your next clinic appointment.

You might also want to browse the respiratory support sticky. Let me know how I can help.

 

[swalker]

As I was going through the diagnostic process, I was asked to do a Pulmonary Function Test (PFT). My PFT revealed a Forced Vital Capacity (FVC) of just over 60%. After diagnosis, my FVC continued to decrease, eventually dropping below 20%. It was not until it was below 20% that I was prescribed a bipap. After using the bipap, I can heartily say that I wish it had been prescribed earlier!

Since starting to use the bipap regularly, I have had increased stamina (though still quite lacking!) and have consistently gained weight. (about 20 pounds so far, after an initial 70 pound loss while going through diagnosis). The weight gain has required a dedicated effort on my part. But, before bipap, no amount of commitment to consuming more calories resulted in appreciable weight gain.

Steve

 

[EPgh]

Oh my gosh, thank you all SO much for your replies! I will look into the Tudca for sure…no one mentioned it in my many appointments today! I currently have private insurance but I will likely be having to resign from my job soon, and will have to apply for Medicaid/medicare.

Thank you for bringing up the Bipap. It was actually ordered today by my team and they said it should only be a couple weeks before I get it. I didn’t really notice any significant breathing issues, but maybe it’s contributing to the complete, extreme exhaustion I’m experiencing. I was a previously healthy, extremely active young women, and I’ve never felt anything remotely close to this level of tiredness.


My first symptoms were noticed in August/September. Now my right hand, right leg and pulmonary function are affected. My doctor did mention today that he worries I’m progressing quickly as a result of this, but I’m not totally sure what that means in terms of time/symptom progression (like closer to the 2 years survival than the 5?!). Upsetting to hear/think about for sure.

Thanks again everyone. @nikki I’m going to check out the webinar!!

 

[Nikki J]

Did they also talk about a feeding tube? It is easier to have the procedure when your svc is over 50 so even if your swallow is good it is another thing to consider. It is certainly possible to have it done later but it will be harder.

I am so sorry you have such fast onset but no one knows how long they are given. I know a number of people whose breathing plateaued on bipap. My sister’s breathing dropped pretty fast her second year but she lived another 3 and would have lived longer but a cold killed her. Avoid germs!