What kind of information would you like to know?

[Mary]

Hi I just joined i haven’t had a definite diagnóis yet appt with 2nd neurologist end of month hoping to no exactly what is going on I am finding it hard to cope all I read on the Internet are horror stories with this illness it scares me I am on my own with a 11 year old daughter how does everyone manage?

[Amanda]

@Mary, Hello. I was just wondering how your second appointment went. How are you feeling? Was the doctor able to provide you with any definitive information?

Amanda

[Mary]

Hi Amanda thanks for asking well clinical exam was fine I had my hand looked at the neurologist doesn’t feel it is mnd as I have had 2 emg which show abnormality in just the 1 muscle he said it would b NY now its nearly 8 months more widespread he think I damaged muscle but no answer what to do plus he saw the muscle waste I am abit in limbo do I go to a 3rd neurologist or just leave and get on with life mayb muscle will sort out itself surely 2 neurologist wd pick up by now

[Jean-Pierre Le Rouzic]

Hi Mary,

The term ALS may imply very different life stories, some are horrible but it seems many people are able to cope with their condition. Basically it depends if one has a fast or a slow progression. If it is slow one can cope with it by adapting their environment. If it is a fast progression, then one has only months to make the transition easy for oneself and for the family. Nobody can predict in advance how ALS will evolve, so you can hope for the best outcome.

Dagmar has some really good advices!

[Mary]

Thanks Jean Pierre for your input hopefully if I am diagnosed it is v slow?

[Susan Pueschel]

I’m the care coordinator for my best friend, age 65, diagnosed 5 years ago.  Hers was bulbar onset and very, very slow progression.  She is now mostly in bed, unable to bear weight, trached and on a vent 22-24 hrs/day, feeding tube.  She struggles with anxiety and occasional depression and is taking meds for that which are occasionally adjusted depending on her condition.  I’ve seen some discussion about anxiety/depression and meds, but I’m more interested in what I perceive to be some degree of FTD.  Her lack of big picture thinking and insight occasionally leads to problems within our caregiving circle.  I’ve tried to educate all the caregivers on the signs and symptoms of FTD, but she can appear to be quite normal in her mind function, especially to those who didn’t know her before this condition.  She has an irrational fear, but can’t communicate what/why.  She’s non verbal and can barely write on a white board.  My intuition tells me it’s the fear of end of life and she hasn’t seemed comfortable having those discussions yet.  Looking for any advice, others’ experience with a similar situation.

[Dagmar]

Hello Susan,

Let me offer a response to your question – – from the perspective of a fellow ALS patient (and only 2 years older than your friend).

You are to be commended for taking on the responsibility to be care coordinator for your friend. She is lucky to have you doing this and I am sure appreciates you immensely.

Anxiety? Depression? Remember, that she was a fully functioning, independent person with hopes and dreams who, in the span of 5 short years, quickly progressed to losing the ability to move or control her arms and legs. She lost the ability to enjoy eating food and the social rituals associated with meals. She now depends on a machine to assist with breathing, and, can no longer communicate effectively. For the majority of ALS patients, throughout the progression of our symptoms, we retain the ability to think clearly, observe the world around us, have opinions, and – feel the emotions of anger, fear and frustration. Having an “irrational fear” is really in my opinion, a rational response to the situation of living with ALS.

Question: Have you explored alternative communication devices other than a white board? Surely, the staff at your ALS Clinic have discussed an “eye gaze” device? https://www.youtube.com/watch?v=Y7_f-pR8SBY These are routinely paid for by Medicare. (Two of the ALS News Today columnists write their articles using an eye gaze device)

I sense in the description of your situation some impatience on your part. That your friend is not, maybe, going along with the plan or, passively cooperating with the care team. Rather than self-diagnosing her reactions as FTD and instructing the care team to act accordingly – – with ensuing medication. You should have your friend evaluated by her neurologist and you should have this discussion with the ALS Clinic team.

Finding a way for her to fully communicate her feelings and opinions will help. “Big picture thinking” also changes for ALS patients: we now think in terms of months, weeks and days. Our world is smaller and yes, we fear the future. Do you have a spiritual counselor who can meet with her? Someone who has Hospice training and can help you both with this issue?

Again, I applaud your care and compassion for your friend… She is a human being, feeling normal human emotions, caused by having to live in an “un-normal” body. I send hugs to you both!

[Susan Pueschel]

Aloha Dagmar, thank you for your thoughtful response.  I send positive energy and gratitude to you.

I’ve been beside Jenn from before Day 1 of her ALS diagnosis.  I also have a background in education, psychology and healthcare, so I feel I have a broad perspective and pretty good understanding of what Jenn is going through. Thank you for acknowledging my gift to my friend.  She would do the same for me.

I’m aware that the majority of ALS patients retain clear thinking, but sadly Jenn is not one of them.  I started noticing changes 3 years ago in the way she processed mentally.  Hard to explain, but I knew her well enough to know that her mind wasn’t working the same way. Yes, we cried, cursed, I researched, went to every neurologist appt with her, encouraged her to learn, advocate, ask, express.  She chose to turn her back on the term “ALS” and basically hide from friends and family.  When folks saw her and asked “what’s going on” she told them she’d had seizures (brain scans picked up some mild activity but she never displayed symptoms). It was easier for her to deal with that idea than the realization of ALS. That likely wouldn’t have been my reaction (who knows really?) but it was her path, not mine, and I’m only there to walk it beside her and do whatever I can to help her.

We live on Hawaii island with limited resources. Her initial neurologist was on Oahu – we flew to her appts.  Now he’s on Kauai, so seeing him is not possible. But we have an incredible ALS state coordinator who literally has been with every single ALS patient in our state over the years, a total of well over 100. In describing the changes in Jenn’s behavior and personality, she has no doubt that Jenn shows symptoms of FTD.  From Day 1 of Jenn’s ALS diagnosis, she looked to me to do the research – not her thing. She was fearful (lack of information) and in denial. I was the only person she’d listen to about the disease.  I took it upon myself to learn all that I could, share it with her, and I’m still learning.  Thank you for your valuable input.

As her symptoms recently got worse, I started looking for a neuropsychologist to do testing.  What I learned is that the ALS neurologists in our state don’t diagnosis FTD with ALS.  Don’t know why.  I did attend a conference recently where the neuropsychologist’s opinion was that “if you know, you know” (her long time friends know, her family knows) so there’s no value in putting someone through the battery of testing because it can be stressful – it doesn’t really change the course of care.  I’m also working hard to try to get a psychologist to come visit her for “talk therapy” which she’s agreed to, with no results so far (not Medicare participants), knowing that pay out of pocket is probably the only route.  Her husband is already paying approx $10,000/mo for caregiving, meds, supplies, etc.

I’ll just add here that to take Jenn to an appointment locally has necessitated ambulance transfer in the past. To seek specialists would require air travel – not even possible except by private air ambulance. So we try to find people who can come in. That limits the pool even further.

We work closely with our island ALS coordinator (speech pathologist) and also our Home Health agency’s speech therapist.  We’ve given her every opportunity that exists for ACDs.  She tried the Eyegaze 6 months ago, and gave up, even with everyone cheering her on. (A symptom of FTD is apathy/lack of motivation). She started with SpeakIt apps and used those to some degree until she lost right arm/hand function, but always chose to go back to the white board which she can barely use now.  She’s trying the Eyegaze again but after 2 visits from the speech therapist, she’s starting to give up. We listen to her, honor her feelings and give her choices over everything possible. She is fully aware and cooperative re: her daily routine.  Her long and sort term memory is intact and as good as mine, if not better. When she first came home from ICU I gave her a pillow that said “Girl Boss” and we honor that.  But being the boss of her healthcare decisions is not in her best interest now.  Her husband, her legal guardian, is painfully aware of this.  Her medications she takes for anxiety /depression have helped her dramatically – she’s the first one to acknowledge it. If she could, she’d take a “pink pill” (Klonopin) 10 times a day (we have it on hand for only rare use, when anxiety is not helped by other means).  She’s gotten obstinate when caregivers have had to tell her “no.”  Same with oversuctioning, Tylenol, and even stacked breathing with the ambu bag (all give temporary relief).  She’s desperately seeking something/anything to help her feel better.  I get that, but requesting overdoses or therapies with real risks if overused is not rational thinking. She was nothing like that in her past. I’ve known her for 25 years.

This sounds grim, but the reality is that 90-95% of the time she’s happy and content.  She goes through cycles and we use all kinds of modalities to help her get through them.

Yes, I was frustrated when I posted in the forum – it’s the reason I did – looking for anyone else who may have a similar experience. Those are fleeting feelings for me, usually due to discord among the team caused by Jenn’s irrational placement of the cause of her anxiety onto one specific caregiver or another.  After spending months trying to figure it out, this is what I/we believe.  Her fear is of dying. Her anxiety comes from her fear and no amount of rational conversation, prayer, comfort makes it better.  We always ask her why she’s feeling anxious, she says she doesn’t know. We acknowledge it, and the discomfort she experiences, so that she always feels heard, then we support her through it. The only RN and clinical leader of our care team is always considering the 360 view of Jenn’s life – psycho-social-spiritual – and is aware there are unresolved (not spoken of) family dynamics.   She has initiated very “light” conversations re: things like: Anyplace you’d would you like to go?  Any family members you’d like to stay in better touch with – can we help you with that?  Sort of bucket list items.  She told Jenn a story about her mom, who lived many years after being told she only had a few months to live, and shared the lessons her mom taught her and her siblings: that there is grace in dignity in the life you live, all the way to the end.  The next day Jenn told a caregiver that this nurse said she only had 3 months to live. Imagine the firestorm that started and I had to carefully, diligently put it out.

We’ve been to our local hospice. Jenn’s not there yet and they don’t offer any services to non clients, nor do they know anyone in our community to recommend.  She needs to address the end-of-life issues and her husband told me awhile ago he is not the one to do it.  A pastor she’s worked with (someone she knows and trusts) came to visit her.  She agreed to have him come back for regular visits.  After the 2nd visit, she asked to have him stop coming.  I still don’t know why but I suspect he may have approached her “forbidden zone” (end of life). It may be that I’m the one to try to have those conversations with her. I was the only one she’d talk to about ALS.  I can do it, but feel I need some preparation and a plan before I do. If I can find a licensed professional, they will be the first in line for this job.

I offer you all of this in hopes that you may learn something from her/our experience. “She is a human being, feeling normal human emotions, caused by having to live in an “un-normal” body.”  I agree 100%.  The difference in her and you, from what I can tell, is that she lacks the insight or big-picture thinking of someone whose brain is not affected by this disease.  This is not that uncommon as I’m learning.  Science used to believe ALS didn’t affect cognition.  That science is changing now.  There is definitely a link between ALS and FTD, particularly in patients with bulbar onset.  The brain is too complicated for me to understand, but I suspect she may have actually started with another more obscure disease which then led to the ALS diagnosis. For now, I will continue to do all that I can to learn, lead, support and continue to be the best friend I can to Jenn.

Thanks for taking the time to reply and your good wishes. O ka maluhia no me oe.  I wish you peace beyond all understanding.

[Dagmar]

Susan, thank you so much for filling in “the rest of the story.” We all have so much history and ins-and-outs, that it hard to tell it all in one post. So, thank you for sharing! Now, I understand much better.

You certainly are providing excellent hands-on care and coordination of care – – despite the challenges of location, limited access, and more… along with a pALS whose mental and physical health brings daily challenges as well. And you certainly are allowed to write in frustration – – this forum is for members to share, listen and support each other. 🙂

I hope one or two of our forum members can offer you insight or share their own experiences with FTD (Frontotemporal dementia)…. perhaps you could also post your question in the sub-forum “A Forum for ALS Caregivers” to elicit some helpful responses in that arena?  Thank you for the aloha! 🙂 Dagmar