How long or average length before transition to PALS?

[KevinR]

I just had a swallow study that showed atrophy on my right side of my throat. I’m now on a soft mechanical diet and either crush or mix my meds with applesauce. Plus I am to turn slightly to the right when I’m swallowing. A PEG tube was discussed as I am losing weight (finally got that six pack I had back in the day). There is obvious changes in my right of my neck, shoulder, arm and leg.

I have a BiPap order but when I showed up to my appointment they had charged it to APAP.. I have no words.

My US Government issue doctors are still holding onto PLS. What type of timeframe am I looking at? I know everyone is different and it would only be a guess.

I understand if I hold my weight and use the bipap it can help me prolong my transition but I haven’t made a decision on the feeling tube.

 

[AnxiousStatsGuy]

I believe the norm is to treat PLS as a "provisional" diagnosis for 5 years, but several studies I just double-checked seem to cite 3 years with no LMN signs as the point where the confidence in the PLS dx goes up quite a bit.

That said, you're noting atrophy and clear changes in your right-sided limbs. Have you had an EMG recently / did your neurologist(s) reaffirm the PLS diagnosis since these changes started?

If it truly is PLS, I believe it does not technically shorten life expectancy. Most end up quite disabled, but do live a long life.

If you have LMN signs and it is ALS, it is harder to say. UMN-dominant ALS is on average much slower overall, but can sometimes speed up precipitously when the LMNs become involved.

 

[KevinR]

I had an EMG a little over a year ago. He said it’s coming up again. Last physical he noted changes in my right arm reflexes as they are beginning to dull. He did say it’s coming up again soon. And yeah they have confirmed PLS but it’s VA so they seem to be slow walking everything.

 

[KimT]

Is it possible to get an opinion from outside the VA?

 

[Clearwater AL]

Kevin, yes, consider an EMG outside the VA. The VA is a good thing but...
the VA bureaucracy can be overwhelming. Many of us have found
sometimes the VA appreciates getting an EMG report outside (civilian) from
an accredited ALS facility. Kinda weird... like it done them a favor.

Been a while since I have followed your posts... have you contacted the PVA?
They recognize PLS under the ALS umbrella.

Hope you get a determination soon.

 

[KevinR]

PVA? I did file a claim of PLS being a variant but was denied. When I filed that one I filed a bunch others under the gulf war presumptive’s.. I hit 100% P&T first go around so my VSO said let it go until I transitioned into ALS.

 

[lgelb]

The Pringle criteria call for 3y, Kevin, but by your account, you may indeed be ultimately diagnosed with ALS. I agree with getting an EMG and eval outside the VA as it could facilitate getting your full VA ALS benefits while you are in the best position to leverage them.

Stats Guy, PLS is not necessarily fatal -- because it's a slower train, you may die of something first -- but it is incorrect to say that it can't be. And those who present with respiratory impairment in the early years are less likely to fit into the nonfatal category, whether the final dx is PLS or ALS.


Best,
Laurie