MIP and MEP

[ECpara]

So Tom made it to another ALS clinic visit. I can't help but wonder if it will be his last, his next one is scheduled for October. His decline seems to be gaining momentum, but who knows with this disease. Anyway, I'm wondering if any of you get MIP and MEP tests done along with FVC. Tom's were so low, and I thought last time was bad! MIP (Maximum Inspiration Pressure which is the diaphragm and inspiratory muscles), a mere 6%. MEP (Maximum Expiration Pressure which is the abdomen and expiratory muscles) 20%. FVC 18%. He has to sit up to sleep now. At least he wears the Trilogy at night now, but less during the day, go figure! Signed up for Gentiva Home Health Care (he refuses Hospice), getting measured for a power wheelchair, too. He can still shuffle around the house on his feet, but basically he sits all day watching TV, legs elevated because edema is so bad in his legs. Seems pretty despondent, arms and hands are going, but not completely yet. Getting his salivary glands radiated Thursday......let's just kill those suckers dead so they don't make any more saliva! Just venting, this is a dreadful disease.

 

[affected]

thanks for updating on where Tom is at, I often wonder how things are going for you.

We had a mips and meps done, only once as Chris was such rapid progression. Three months after diagnosis his result was 30%

Sure is a dreadful disease, I hope the radiation does what he wants and doesn't cause too much dry mouth as that is another ball game!

 

[Barbie]

A dreadful disease...well said.

Sorry about the progression--it is upsetting.

 

[starente15]

I'm so sorry to hear this Nancy. As I as I was reading, it was almost like my father wrote the post. I just came from a visit and he seems to be in the same situation as Tom. I'm not sure what his actual numbers are but breathing has gotten significantly worse. He's now sleeping upright and needs to use the trilogy more and more. I've been a basket case since returning. This disease is horrendous for all involved. To top it off we had several dysfunctional family moments which adds to the stress of it.

 

[lgelb]

I'm not sure what you guys mean about sleeping upright -- hopefully you mean head at a positive angle in a hospital bed. No one should be sleeping regularly sitting up or in a wheelchair. If they are, something is missing in the bed setup.

 

[affected]

I don't know about what others meant, but for Chris, for a long time (he refused bipap) he had to sleep as upright as possible either in his hospital bed or recliner chair.

Even his hospital bed didn't make him quite upright enough in the last couple of months and I had to use lots of pillows to keep him able to breath.

I know he would have been able to recline further with a bipap at least for some time.

 

[tripete]

Seems pretty despondent, .

It is hard to be anything but as you see your life and your abilities fade away. I am sorry but I can understand as he probably just wants it over as the journey just gets to hard and for what purpose.

They do the MIP on me at every clinic with the FVC. They are both of course dropping rapidly. The numbers are just a baseline guide, as we all know, it is how we are feeling that matters. Mental distractions are my best ally.

As silly as it sounds I play football on my xbox during the day as it takes my mind off of the pitiful condition I am in. As long as my hands hold out I hope to keep having the distraction.

 

[ECpara]

Lgelb, like Tillie mentions, Tom has to be sitting totally erect.....I use 6 pillows in our bed to get him propped up to the point he's comfortable with his breathing. He is thankfully starting out on the Trilogy at night, but at some point it comes off. I really wonder of he's choosing not to wear it to hasten the end. He used to wear it more during the day but lately hasn't been wearing it at all. Tripete, Tom still has his hands, too, it is the only thing keeping him going. The remote to the TV keeps his sanity. And he types to friends on Facebook, LinkedIn, and texting, although I'm noticing not as much because he becomes so fatigued. Even as I ask him questions, he doesn't even have the energy to answer. Yes, I can tell he wants this OVER!

 

[tripete]

I am sorry. I know I am not him, but I can tell you sometimes that I feel it would just be better for my wife if she did not have to put up with this disease and what it is doing. On top of that I feel useless. I know you CALS will object to this, but none the less if you look at it objectively it is quite true. I cannot do the things I use to do, I can not support my family or even "do" my own hobbies. We are a burden we require so much help and attention.

This is not a "death wish" it is just what it feel like and in most ways is if you look at it. Why would we want to prolong this thing? This is the question I am asking while waiting for my appointment with the surgeon on Friday regarding weather or not to get a PEG and DPS. It sounds like Tom has made his choice, it is not a bad one and it is not because he does not love his family and friends, it is just what he probably feels is best. I hope for myself that when I make the same choice others will understand and just try and make me as comfortable as possible.

 

[starente15]

My dad recently got an adjustable bed. It's set at slightly less than upright position but he's also using pillows. For the first time this past weekend he said, "it's really hard." I can tell he's starting to get panicky. I have a call into the palliative nurse as so far they haven't provided any service. I need to get a feel for how they think he's progressing and when he may need something to relieve anxiety/air hunger.

 

[ECpara]

Tripete, thank you so much for your honesty. I know what you feel is exactly what Tom feels. He was once such a vital part of this household. Now I have taken over all the things he enjoyed (just like the hobbies you enjoyed).....driving (his new car), mowing the lawn (2 acres on a tractor), trimming the bushes, applying the weed killer and lawn fertilizer, and cutting the vines in the woods, among other things outdoors. He sits on the porch and stares, doesn't feel much like even communicating with me. Wish I had a penny for his thoughts. On a side note, I'm glad he had a PEG put in when he did, he got over two years of quality life with it. He was an avid golfer and only gave it up in February. It has been a fast decline since. I mentioned Hospice to him, he wanted no part of it, I think he wants to go out on his own terms, whenever that may be. So sorry Tripete, what agony you, Tom and all PALS must feel.

 

[lgelb]

For sure, a BiPAP improves breathing in any position. But for those of you using a stack of pillows even in electric beds, you might try just one that has dense foam or air with firm support for the headgear/mask to settle in.

If someone is getting the BiPAP off during sleep time, I think first that s/he isn't getting into deep sleep, before believing that an exhausted PALS is coming back to consciousness just to take it off (doesn't mean s/he isn't ready to go, of course).

And, yes, tweak the BiPAP settings. Slower breaths and/or deeper breaths may be the path to try first. Also, depending on what the preferences are at this point, decreasing EPAP (pressure, duration or both) may be more comfortable but of course may accelerate CO2 buildup.

Also, you really want the legs elevated as well -- I am sure you do, maybe tweak a little -- so the middle has less to support, weak as it is.

I have posted explicitly on this topic and the procedures if you opt out of hospice (and, in our case, out of clinic for 2y), but in brief even if you/your PALS hasn't had air hunger as yet, if things are heading that way, which it sounds like is the case for some of you, I'd ask whatever doc that will be asked to sign the death cert to write morphine solution so it's on hand.

Star, if the nurses aren't proactive, call the doc directly. You don't have to work through them. If your dad says it's hard, it's too hard.

 

[affected]

I am sorry. I know I am not him, but I can tell you sometimes that I feel it would just be better for my wife if she did not have to put up with this disease and what it is doing. On top of that I feel useless. I know you CALS will object to this, but none the less if you look at it objectively it is quite true.

Pete "you" are not a burden, the disease is.
BUT I wouldn't for a moment say that you don't feel all those things and I agree they are totally valid. I wondered often whilst caring for Chris and since he passed just how would I feel if it had been me? I like to imagine all kinds of things, but I suspect I would feel all these same things. Of course, grieving after your PALS is gone is no piece of cake either!

It's a damned ugly disease, no one should have to go through it. I supported Chris in making choices I did not feel were the best ones because I strongly believe each person has their own line where things are tolerable and have the right to say where that line is.

If you get all the information you can on the PEG and DPS then you can make your own informed choice on your own terms.

I had two big foam wedges and at times would try one behind the bed mattress to sit Chris a bit higher but he did not like them, we worked out many good arrangements of pillows. It seems the only common theme with this disease - what works perfectly for one doesn't always work for the next person. It's the power of this forum - ten of us may give ten slightly different solutions or strategies to something and with luck one of those will work for the person asking

I also kept Chris's legs elevated at the knees, just a little bit and that helped with pressure and to stop his sliding down the bed.

Even though we were on palliative care (Australia is a bit different it was palliative community so they came to the home but not to do any caring tasks), it was all public holidays when Chris went into actively dying at the start of Easter, so I was doing it all alone with phone support. However, we had everything in place between the palliative team, our doctor and medications on hand ready.

ECpara I hope Tom's hands hold out for a good while yet, it really sucks when they have to ask just to have a station changed, an itch scratched etc Not for the CALS, but for the PALS it must be so incredibly frustrating xx