PALS ROLL CALL NEW THREAD (PALS ONLY) [Wednesday or whenever]

[wmilo]

It's been a minute since I last posted here so I went back a couple months to catch up. I was happy to see that I am still about where I was in October. On BiPAP most of the time, arms and hands very weak. I found the Windows on-screen keyboard (part of Windows) which I find helpful. It is slow but with "sticky keys" on you can use keyboard combinations, and it has a look -ahead option. I had been using speech -to -text software, but it can't understand me with the BiPAP on.

Greetings to all.

 

[KevinM]

So good to hear from you, Bill, and thrilled that you haven’t progressed much at all since October. May it continue.

I continue with very slow progression but have run into other health problems that we are trying to address. My wife, daughter and a few friends continue to bring me great joy and comfort.

 

[Clearwater AL]

I need some help with this… been a while since I’ve posted here.

There seems to be mixed messages concerning Riluzole. Originally, as it
came out, below was the introduction.

Rilutek (riluzole) is approved for use in treatment in ALS and has been
shown to prolong life by three months on average. Three months?

Even neurologists have differing opinions of its value, versus cost and
effectiveness, possible side effects. Many have said it might possibly be
more help for those in the later stages of ALS. Some prescribe it when
someone is first diagnosed.

Has it been clinically proven it slows progression if it is prescribed early?
I couldn’t find anything definitive. Maybe one of our more skilled
members can.

My progression has picked up. I’m spending more time at my roll top
desk and sitting in my office chair like a potted plant. I miss working
down in my shop. But, I read some of the CALS posts… I’m ok for now.

 

[Nikki J]

Sorry for your increased progression. Here is what I have been told and or read/ heard research on riluzole. My neurologist said when it was first prescribed to me it slowed progression by 10%. The 2-3 months came from the survival times of the trial population in the 90s. So 10% means slow progressing PALS get longer benefit ultrafast PALS very little.

I was also told getting it very early increased the benefit ( this from an NIH ALS specialist). I believe there is data from an Irish study supporting that

There was also at least one paper that said it works best early and late stages and less so in the middle. Not that it does nothing midstage

finally at the big MNDA conference a few years ago (2020 I think) Professor Al Chalabi from Kings London said in a presentation riluzole slows by 30%! This was a virtual conference and the sessions were available for a short time for review. I listened twice to be sure I heard right, then asked at mgh as I was sure he misspoke. They said it was true and why they had started prescribing it for people they suspected had ALS while waiting for test results. Professor Al Chalabi is world known and respected and the MNDA is probably THE top attended ALS conference by the leaders in the field

you are right Al that prescribing patterns vary. I can’t imagine wanting to wait to prescribe until people are worse. If it is going to help at all ( even aside from the helping early evidence) why not take it when people are somewhat functional? And although i am not there yet I don’t plan on taking it to prolong the end stage

 

[Nikki J]

Laurie shared this article Riluzole: real-world evidence supports significant extension of median | DNND
they found benefits up to more than19 months though other factors may play in. It would interesting to compare survival of a clinic population with high utilization and one with low during the same time period though there would be other factors there too

thanks Laurie for finding this

 

[Smjacobsdmd]

At clinic today, Dr. O told me that of the three available meds, Riluzole is the only one that research shows actually prolongs survival. Others may but shorter research times rely on ALS-FRS which is such a flawed or at least imperfect evaluation form.

 

[wmilo]

I find this interesting. When I was diagnosed in 2017, Radicava had been approved for 5 months. I was also told that Riluzole offered 2-3 months extension, whereas Radicava showed something like a 30% decrease in decline. I think this was based on an average of 7.5% per month decline for the placebo group versus 5% decline for the drug group, based on ALS-FRS scores. (7.5-5.0 is 2.5, which is 30% of 7.5) This was based on a six-month study of 137 early-onset patients, who I believe were also taking Riluzole. Radicava-dot-com is currently claiming to "slow physical function loss by 33%". I started Radicava right away and Riluzole two years later.

I have the same impression as Al on mixed-messages on Riluzole. Maybe as more clinical data is accumulated the results are looking better for Riluzole.

 

[KevinM]

Always good to hear from you, Bill (and Al and Steve). As Nikki said most of the riluzole studies that only indicated a late stage 2-3 month extension were done as part of its 1995 approval. Since then newer studies, including the link that Laurie provided from 2017, have shown it to be significantly more effective than first thought.

My neurologist is the one that cited the more recent studies and is definitely in the riluzole camp as opposed to Radicava and Relyvrio. Many neurologists, even MND specialists, still cite the original data. Of course the variability of each pALS and the flawed use of the FRS-R scale raises questions about every ALS therapy trial.

 

[wmilo]

Hi Kevin. good to hear from you too.

On the roll call thread:
My CALS is scheduled for a cardiac stress test this week, so we have been doing a lot of planning to look after me depending on the results.

We had some unseasonably warm weather with highs in the upper 60s, but are now below freezing at night and 40s during the day. Hyacinths are starting to push up and trees getting pink - too early, I'm afraid.

Best to all.

 

[KevinM]

Bill, my thoughts will be with you, your wife and family on the results of the stress test.

We have had incredibly up and down weather here in N. FL as well. Last night got down to 35 degrees, next week we will be in the 80’s. The flowering plants are very confused.

 

[wmilo]

Thanks Kevin.

My CALS met the pulse rate and time objectives without serious consequences, and her doctor has not contacted her. so we are taking that to mean he doesn't feel it is urgent.

Saw some redwing blackbirds yesterday, which we usually take to mean that Spring is here.

 

[KimT]

Bill, that's great news.

As Kevin said, we're having strange weather in Florida and, I guess, everywhere.

Today I was in the pool. The air temperature was 80 and the water temperature 83. Breeze was coming from the South and it felt great....until I got out and showered. The pain leaves when I'm in the water but I do exercise there and my muscles let me know about it once I'm back inside and try to do anything. I thought about getting a rubber mat so I can stand over the stove. I have all tile and it's so hard on my feet. I've been unable to wear anything other than high-end running shoes for several years now. My legs are getting weaker but the pain is in my knees, feet and back. I've been afraid to order one because 1. I'll probably trip over it and 2. Ace might think it's a pee pad.

I hope everyone finds something that gives them peace, a good laugh, or pure joy this week.

 

[Statius@]

I've been AWOL for a couple of months--got out of the long term habit of reading the forum at least several times a week, if not daily. Re: Riluzole, I've been taking it almost since the beginning and it may account in part for my fairly slow progression. We'll be leaving Tucson for the last time in late April. My progression is making the trip from Iowa increasingly difficult but more to the point my daughter who lives here is moving to SLC to be Dean of Libaries at Univ. of Utah. My wife qualified for a VA program that provides a stipend and other perks to caregivers. The neurologist at the Tucson VA put me on oral Radicava (I used the infused version for a couple of years but stopped mainly because of the hassle and uncertain benefits). I can still speak intelligibly and eat (slowly and carefully--take lunch through PEG. Use Trilogy at night and for naps. Generally weaker but stilll managing transfers OK.

Best wishes to all who make this site so helpful and special.

Ed

 

[bhg]

Good to hear from you Ed, I don't post often, but follow your migrations south to Tucson each year. I'm a history buff. Just south of Tucson is where the first civilian settlers started their trek to Spanish California and founded San Jose, the first pueblo in 1776. In my younger days enjoyed camping in the Nat'l park just west of Tucson - hearing the coyotes howling.

My progression is speeding up, getting more complicated. Leg and arms were easy to understand and compensate for. The bulbar area is more difficult, especially now that I've lost my voice. Getting harder and harder on my husband.

 

[wmilo]

Had visits from my sister and my son and his family (including two young grandchildren) over the past three weeks. We're catching up on sleep.

I'm sorry, Betty, about the progression. It does take a toll on our caregivers too. It is a tough situation to be in. and I think that the mental challenge is hardest.