Mom finally diagnosed

[tifferbee]

Dear PLS community,

After 7 years of misdiagnosed diseases, surgeries, tests, and confusion, my mother has finally been diagnosed with PLS. Although it is wildly satisfying to know that her issue has a name, the lack of useful information is extremely frustrating! From what I've read, there are something like 500 confirmed cases of PLS in America, and I am unsure the statistics for Canada. Has anyone managed to find useful information regarding how to proceed? My Mom returned from the doctor with a prescription for an assisted living facility, and the understanding that the disease is degenerative, but not fatal. We have no further information, so anything you are able to contribute would be a great blessing to us.

 

[notme]

PLS is a form of MND that affects the Upper Motor Neurons. While it is degenerative, it's not considered fatal, as you've apparently been told.

It's more a matter of treating the symptoms. Medications such as baclofen, valium and zanaflex are used to help with spasticity, for example. (Muscle relaxers and anti-spasticity meds) It's a matter of keeping the patient as pain-free as possible. Some with PLS have severe pain from the spasticity--and therapy can help avoid damage to joints and such.

Falls need to be prevented. Most have some level of damage in their legs that tends to worsen. The time-frame seems to be different for everyone.

It can affect the face -- thus speech--but many never move beyond issues with the limbs, I don't think.

There don't appear to be any current clinical trials for the disease--at least none that are dealing with treatments. There is one trial that is interested in info-gathering in PLS patients.

The main worry for those with PLS is that they will also develop LMN signs (atrophy, lessened reflexes, EMG changes) which changes the diagnosis to ALS.

Usually, 5 years is the cut-off, and it appears your mother has gone 7 years with no LMN issues, I take it.

The other thing with PLS is that it does progress to weakness--and often will lead to the need for a wheelchair. There just isn't a lot of information on it anywhere. It's rare enough that there aren't really any clinical trials or anything planned that I could locate.

http://sp-foundation.org/understanding-hsp-pls/pls/ this site has the best information, I think.

All in all--it's less evil than ALS. That's the only good thing I can say about it, though.

Edit to add : If your mothers issues are confined to the legs only--it's possible HSP is the culprit, rather than PLS. HSP only affects the legs normally, while PLS eventually moves everywhere--including bulbar region.

Wish there was more info I could give. I'm not sure if the ALS association or MDA help with PLS or not.

 

[old dog]

Unfortunately, as notme says, there is very little information available regarding PLS. Based on what I've recently seen on the internet, researchers in Italy are doing the most work on it. On the bright side, PLS is not considered life threatening, mainly because the muscles that control breathing are not affected, I think.

The person who diagnosed me told me (in a nutshell) I needed to go home, get in a wheelchair, and wait for the end. Life isn't great, but I'm still able to take care of my personal needs--dressing, using the bathroom, showering, etc. I'm very lucky to have found someone who lives in to do cooking, cleaning, yard work, and errands. I still work part time (only 10-12 hrs./mo.--bookeeping) and was recently offered another bookeeping job which I had to decline because I'm not sure how much longer I will be able to do this work because my keyboarding speed has slowed to a snail's pace.

I'm so sorry your mom has to go through this. Tell her one can adjust, and life goes on. (I'm 73.)

 

[billbell52]

It is rare. Incidence rate is 1-2% of ALS incidence rate. About 35-70 people get it in the US/year. There are roughly 1500 people in the US with it. I agree with notme the best website for a description is

http://sp-foundation.org/understanding-hsp-pls/pls/

I will attest progression varies a lot. Some people can use a walker after 15 years and some people use a power wheelchair after 4 years. I have Bulbar PLS. It started with my Speech. It is a rare form of PLS. I was using a cane at year 5, walker at year 6 and scooter/PWC at year 7.

There is little research in PLS. It is not fatal and few people have it. ALS gets the $s. I am in a study that is collecting data on PLS. The MDA/ALS Clinic in Dallas covers PLS. They are doing the PLS study.

 

[notme]

That was the only study I found as well billbell. Just one collecting info. I'd hope that is so they can perhaps start new trials to 'treat' it? Why else would they do the info trials (I hope)

 

[notme]

Off to mod-land. I musta said a bad word

 

[tifferbee]

Frankly, it's nice just to hear other people's stories! My Mom is already using a walker/ scooter, and she is no longer able to walk more than about 10 yards without sitting down. She has pretty much all of the symptoms listed, and it seems like she just keeps losing the ability to do more and more things.

For those of you who have been diagnosed for a while, how many people live in an assisted living situation, or with a live-in caregiver? I'm 26 and I work full time, so it's hard to manage her increasing list of needs... I read her all of your comments by the way (she's in her 60s, and not very internet savvy.) She's really glad to know that there are others out there who can relate. ;-)

 

[notme]

It's never to late to learn to use a puter--and one day, it may well be her lifeline to the outside world! teach her! 60 isn't too old to learn puters. We have pals here older

And I'd suggest live-in caregiver before assisted-living. I was in assisted-living and it was a nightmare. But that could just have been my experience. There must be good ones.

With ALS--assisted living is probably not the best choice--but one of last resort in my opinion.

 

[AKmom]

Since pls is not necessarily a death sentence, but rather an obsticle that presents in our lives with new challenges to over come. We constantly have to adapt to new ways of doing things or loosing the ability of doing something. Depending on your mother's Outlook on life itself, she has loads of adventure ahead of her. So rather than putting her into a place to keep her out of harms way, help her with the opportunity to learn and adjust to ways she can still do for herself (and others) by getting the proper equipment she needs and maybe someone to come in during the days you work, to help her with the things she cannot so.

I have had pls for about 20 years and just now starting to use the power wheel chair 98% of my up time. To put this in perspective to what I am trying to say....I still do for myself and my family the best I can. Even tonight I went and did a photo shoot for a local theater, in my pwc! Yes, my hubby now has to go with me and be my "equipment" guy, but I still work through to overcome the circumstance of pls and be useful. I even started my own home based busi ness that requires me to travel sometimes. Going into a nursing home or other such arrangement would limit your mom more.

This is my opinion. Ask your mom what she would like to do with the rest of her life. Then provide the encouragement and anything else you can to support her in accomplishing those goals. It will be good for the both of you!

 

[John1]

Hi Tifferbee,

Sorry about your mom. Although I know little about your mother's situation, I am very surprised to hear that her diagnostician prescribed an assisted living facility. I wonder how much s/he really knows about living with PLS. Joyce's post eloquently points out how she likely has years ahead of her of relatively normal life since she is only in her sixties. There are all kinds of assistive devices available now. She will likely need help in her house from time to time with cleaning and washing at some point. I am in my mid sixties and have had symptoms for 19 years. Have used a scooter for the past 8; can't walk at all but otherwise get along with minimal assistance. I still drive and work.

As Patty notes, for all kinds of reasons she should certainly become computer literate. I'm sure Vancouver has classes for seniors (if your mother even is a senior).