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MP's CALS

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Mar 22, 2012
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CALS
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02/2012
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Hello all,

I am new to this forum. My mother was diagnosed with ALS in February 2012 (but has had symptoms since July 2010). She is 72. She cannot breathe on her back at all, uses BiPAP at night, walks with a walker, uses a chairlift for the stairs (PWC on order), and her speech is becoming more and more slurred. We are all still reeling from the diagnosis and she is getting her second opinion at the Mayo Clinic next week. I live 850 miles away, but by June, I plan to be moved into her home to become her primary caregiver, since my sister has taken over the family business and cannot do both. I have secured a caretaker for my house; still working on convincing my job to allow me an unpaid leave of absence (beyond Family Medical Leave), so that someday I may return to my home and have a job.

Mom is wanting to use a diaphragm pacing system. I did a search on this forum and could not find any threads about this device. She has passed the first two tests which will allow her to have a consultation in a town about 4 or 5 hours from her home. The consulting physicians may still yet say she is not a good candidate. My questions are: Does anyone have this system and what are the risks/benefits of it? Do you have any negative side effects (like pain)? Is it helpful to your daily life? I can read all about it on websites, but I would like to hear from the source--people who actually know its risks and benefits from personal experience. If you chose not to have one, let me know that, too.

Sorry for the millions of questions. After I got over the initial shock and terror, I went into Find Out Everything I Can About This Horrible Disease Mode. Almost all my questions have already been answered by lurking here for 6 weeks--many thanks to you!

Thanks in advance for helping me. Compared to other forums I've seen and participated in, this one seems especially thoughtful, kind, and not-so-narcissistic as the rest of the internet!

M
 
I am interested in this also. I asked my ALS clinic about it and they were not encouraging but primarily for non technical aspects. They did not care for the developer. They did say that the concept of sending an impulse to muscle that was basically gone made no sense. Like beating a dead horse.

I too would like to know more about this from others.
 
Hi MP,

There actually have been several threads about the DPS here on the forum. You can find the links to them if you scroll all the way down to the bottom of this page. Hope you'll find them helpful!

~ Sarah
 
My Dad's initial neurologist seemed to think it would be something the ALS clinic neurologist would recommend, but that was not the case. Based solely on what I've read (not anything he said), I suspect it's because his FVC is already too low. Dad was also diagnosed just this year - a VERY late diagnosis. Sorry you have to be here. Your Mom is lucky to have you!
 
Thanks to all for the links. I don't know why a search didn't bring them up; I tried several search parameters. I didn't find the links until after I posted.

Thanks especially to trfogey who helped with finding someone who's had one implanted. There was also a couple of other patients within some of the other links, which I bookmarked for Mom to read.

Mom's right on the borderline of being an acceptable candidate. By the time the appointments are made and the consultations are finished, she might have already lost her window of opportunity. But it does sound like a viable option for those who are not as far along in the disease. We will just have to wait and see what the doctors in Nebraska say. Since Medicare has covered everything else, including the over $1K/month Rilutek script and the PWC, I am hoping that they will pay for this procedure, as well.

Thanks again for helping me.
 
Good luck, M. Wishing you and your Mom the best!
 
...the concept of sending an impulse to muscle that was basically gone made no sense. Like beating a dead horse.

I'm being considered for this procedure right now. My FVC is 49% which is close to the lower limit of 45% so it's now or never.

Anyway, I've done some research and had several screening tests done, so I thought I'd respond to the one issue raised by jb63 above.

During the candidate screening process, they look not only for an FVC range but they also do testing (floroscopy and phrenic nerve EMG) to evaluate whether the diaphragm muscle is still strong or "gone" as you say. If the muscle has already atrophied considerably they will not recommend a person for the procedure.

The idea is that the electrical impulses from the pacer will keep the diaphragm muscle strong, in the same way that a healthy phrenic nerve would. BIPAP allows the diaphragm to rest, but this does not help keep it strong. The DPS has not yet been proven to be effective in clinical trials, but this is the concept anyway.

-Tom
 
Thank you Tom for the information. It was what I was looking for and explained perfectly. I am fearful of my diaphragm is not strong. I have a weak cough, but can talk, sing. I understand the workup now. I think I will push for it.
 
Just a follow up note.

We took Mom to the Mayo Clinic in Rochester, MN last week for a second opinion. Her diagnosis was confirmed after three days of non-stop testing and consultations with four doctors. We also found out at this visit that she has no function in the phrenic nerve on either side of her neck. Therefore, she is not eligible for the diaphragm pacing system, since it needs a functional phrenic nerve to work. She was very disappointed. She is still breathing on her own during the day, even without a functional diaphragm. This baffles me, unless she is compensating with other muscles in her ribcage?

I will say this: The Mayo Clinic was absolutely fantastic and was well worth the trip, if anyone out there has been considering whether to go there. We were extremely impressed with the level of care, knowledge, thoroughness, and the compassion of the providers. Now she knows that she has "left no stone unturned" and can go forward in her journey of trying to stay a step or two ahead of this awful disease.
 
Just as a caution for those of you considering the DP: having one may make you ineligible for other clinical trials. The DP is approved by the FDA for general use in people with spinal cord injuries, but only on a "compassionate use" basis. From what I've been able to gather, some folks who were in the Dex trial had the DP implanted. When they went to their next Dex clinic or called their trial coordinator to inform the TC about the DP, they were not allowed to continue in the trial (receiving the medication/placebo), but they will be followed up for any long-term effects from the trial. At least one person was told that he would not be eligible for the open-label portion of the trial.

At this point in time, the DP is still an experimental device in the ALS community. If you decide to go through this procedure, you may be closing doors that you might have wanted to open.

My two cents.
 
AND do not feel too disappointed if you do not qualify for the DPS. Only about 10% of PALS qualify.
 
The DP is approved by the FDA for general use in people with spinal cord injuries, but only on a "compassionate use" basis.

The above should say "The DP is approved by the FDA for general use in people with spinal cord injuries, but only on a "compassionate use" basis for ALS patients." Should have proofread my text more carefully. Sorry for the mistake.
 
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