Progressing a bit faster than hoped for

[Brad H]

Hello to all and a thank you for your support. Its been some time since I have last posted but you all have been very supportive in the advice you've given to me (and to others coming to this community for the first time. I was diagnosed in Oct 2022 and had my first clinic (also Oct 2022). This is limb onset as I had gradually lost use of my left wrist/hand as well as left foot drop during the Spring/Summer of 2022.

Among other testing at first clinic, I had a full respiratory/pulmonary workup. At that time, my FVC was 109%, my FEV1/FVC% was 70% and my MEP was 60 CMH2O. Not bad numbers at all and I didn't notice any respiratory symptoms.

Updating to 4 months later, this week was 2nd clinic. Since New Year's, I have noticed more fatigue and obvious shortness of breath. I've been trying to keep everything in our lives slow and steady- so as not to over-stress my body...avoiding stairs, sitting instead of standing. We were a bit freaked out by the fact that in just a 4 month period, the respiration had been so affected. 2nd clinic numbers in Feb 2023; FVC=35%, FEV1/FVC%=58% and MEP=10 CMH2O. Neuro also noted that I've moved from 40 to 33 on the ALSFRS-R scale in those 4 months. Hopefully, this progression will slow down.

I do have a question... They have put in the scripts for non-invasive positive pressure ventilation, cough assist and PEG. In the order, the Doctor mentioned that "Bipap was considered but ruled out due to its inability to support max/min IPAP settings >25cm H2O." I'm not sure what all that means but thought that Bipap was the standard for ALS patients. Thanks again to everyone.

 

[affected]

Strange wording, as bipap is type of positive pressure ventilation.
Bipap is actually a brand, even though it is used as a type of machine in everyday language.
So maybe they just meant they would use a different brand as there are ones considered better.

 

[Nikki J]

I am so sorry about your progression. It must have been a very difficult visit

we talk about bipap though as Tillie correctly points out it is really a brand name however it is commonly used in a generic sense and you are right we say bipap here often. PALS absolutely need bpap/ bipap vs cpap but what your doctor ordered is a third category with more capability to deal with your weakened breathing. A trilogy would be but one example of such a machine

 

[lgelb]

BiPAP is commonly meant as a type of machine, like CPAP and ASV. The brand denotation "went generic" a long time ago. However, there are multiple modes that a NIV, which is what you are getting, can run for people with more complex needs, not just a standard BiPAP mode/pressure range as would be true for the smaller, less sophisticated machines.

Most PALS do not need those other modes, but get the more powerful machines in order to have portability with the internal battery instead of relying on AC/mains power. As a byproduct, these NIVs have greater pressure ranges as your doc mentioned.

The vast majority of PALS don't need and shouldn't use those higher IPAPs, because the muscles you use to breathe can only handle so much pressure as they grow weaker. Instead, we can use more precise timing and strategic synch with your own respiratory drive to optimize the machine's effectiveness.

So the doc was just justifying the order for NIV (usually Astral or Trilogy; ask for the Astral if possible) instead of a "standard" bed table BiPAP that has lower ranges for IPAP, such as people with certain sleep apneas, COPD, and myopathies, among others, would use. S/he didn't have to -- with ALS and the numbers you've provided, they could have just written for a Trilogy or Astral. But plans/forms/habits differ.

Documentation aside, I am concerned that the doc seemingly plans to start you at what could be too high a max IPAP, if they are seriously planning on >25 as IPAP max (let alone minimum!) that could do more harm than good. Settings should not be determined by the PFTs nor by speed of progression. Check in when you get the machine, if you like, and I can help you adjust the settings to both efficacy and comfort.

Best,
Laurie

 

[MupstateNY]

I can attest to Laurie's helpfulness in this area.

Would it be helpful for you to request a telemed or ask some questions via the portal?

Do you receive physical therapy? I was given a home routine involving some gentle squats, sideways walking, and foot movements, some with resistance. I guess one can request in-home PT if one is kind of past going to appointments. (I'm about to do that transition.)

Are you taking any of these? Riluzole, Radicava, Relyvrio? I call them the 3 Rs. I guess in studies, progression was found to be somewhat slower. (With the magic of statistics.)

I think I read here that progression can come in a spurt and then kind of plateau for a while.

 

[rao]

I was dx in Nov 22 with limb onset and my progression seem fast too... changes I had to adjust to since:

usin walker for short distance
usin mwc for longer
cant turn keys
difficulty turnin in bed
getting up from a chair/bed
typing with mostly thumb/ring fingers
needin assistance for shower+clothing

I checked my alsfrs r score n in last month it fell from 40 to 38... faster than the 1/month u here in studies. ouch.

i just hope i can still do my overseas trip late apr/may. fingers crossed no more point loss until june!

 

[Tomswife]

Brad. We are here to support you. It is a challenge to understand what is happening to your body , enjoy life each day, and also try to anticipate and prepare for the future. Keep reaching out and asking questions. Peace.

 

[Brad H]

Thank you to all for your very helpful replies and support. The explanations on the ventilator are very understandable. I will ask for the Astral if its available and will definitely follow up with the Doctors about more detail concerning the settings of >25 IPAP. Once its in place, I will take you up on your gracious offer Laurie and check back in here for help on settings for efficiency and comfort. After 1st clinic, I was doing PT but this week, the clinic discontinued that. I'm still doing ROM at home but no strength or balance. It was just too exhausting. I am on Riluzole-since November and this week the Doctors asked if I would consider starting Relyvrio. There is a concern that I would no longer be able to continue with my Clinical Trial (MN-166; Ibudilast) if I added the new medication at this point - so that is something I need to consider. I do feel grateful for every day. I'm still able to work (1/2 days), I can still enjoy eating and have no swallowing issues and Lydia and I are looking forward to some much needed trips with the kids in the summer. I understand that we're all progressing differently and hopeful that this spurt of progression might plateau for a while. My best to all and thank you.

 

[Mary2]

Brad..Great that you have been able to continue working part time! My PALS....husband...was also able to continue working for awhile. My Pals went through a stage where he had a scooter and we attended ice hockey games at my son's college. We also got to Hilton Head once with the scooter. Our big trip with the wheel chair was to my son's wedding last May and we are so glad we did that trip. It was only a couple of hours a way but some planning went into it. My PALS was on bi-pap at that point.