Tough clinic visit today

[FamilialALS?]

Today mom had her clinic visit. Her fvc numbers were really low. She tested at 55 two months ago, and before that she had maintained a constant 60 since October of 2014. Today she tested at 17. I was unprepared to hear such a low number since she doesn't outwardly show such marked decline. When her oxygenation has been checked by the home health nurse she is always close to 100%, she does not complain or show signs of headaches. She is still mobile and hasn't shown any significant change in her stamina to walk around the house and fiddle around. Her neuro gave us the option to shift to hospice care which I agreed to. Mom was discharged from home health service last Thursday so we lost the weekly check ins from a nurse starting this week. I'm not sure what to expect with the introduction of hospice services. I'm really hoping we have nurses that understand ALS in particular so I can feel confident that they can see and understand what changes in her mean and how to help me keep her most comfortable and anxiety free.

Thankfully my sister is here from out of state and she was able to go to her appointment with us. She did pull mom's neuro aside in the hall to ask what her breathing number meant as far as how much more time mom has. The doctor explained to her that when she sees a capacity of 30 she usually anticipates six more months. My sister asked if that meant my mom may have three more months, but the doctor said she may have a month because her regression has been so steep. I'm just trying to work through the shock of hearing this timeframe.

I asked the neurologist again about genetic testing. She explained the study she had in mind for entering my mom to was not yet accepting patients. I asked her about cash paying for some of the genes to be tested. She agreed it was a good idea to be looking at that because she feels time is our biggest challenge. She said they cannot draw blood to be tested later or to be entered to a future study. So our only option to find out if my mom has a genetic link is to order the testing now. We already missed this chance with my aunt since she passed away in 2013 not knowing anyone else in her family had als. I was quoted $1500 to test 21 genes. I did ask about testing for the most suspicious genes C9 and sod1, but her neuro explained that it would take up to 3-4 months for those results to come in and if it was negative we will have probably lost the opportunity to test for any of the others.

I know I need to move forward fast with having the testing started but I want to hear from some of you about what to take care of and when before I get any official answers to the genetic testing. I have term life insurance with about ten years left on it, and have not purchased disability or long term care insurance yet.

The only thing more gut wrenching and devastating than watching my mom lose her faculties and abilities because of als is the thought that I may see this monster again in one of my siblings or myself.

 

[affected]

That is a rapid drop in breathing. One thing with bulbar PALS however is they cannot maintain a lip seal so their numbers are not always accurate in the sense of watching numbers exactly.
They are accurate enough to know her FVC is dropping now.

Remember that PALS often have more problems with expiring CO2 than getting enough oxygen so good sats are not always a complete indicator.

I would advise encouraging her to use her bipap as much as possible and the cough assist whenever it's needed.

So sorry for the shock it has been. I'm afraid a sudden rapid decline is fairly common and it's always a shock.

 

[Nuts]

Absolutely get the disablility and long term care insurance lined up. I don't think they can deny you based on your mom's history or genetic testing, but I'd get it now anyway. My husband has both, for which I am extremely grateful.

 

[Atsugi]

Be prepared. My hospice nurses knew nothing about ALS or paralysis. One told me she spent her whole career in administration. That's just one experience of course.

As it turned out, it all worked out quite well. I trained each nurses in what they needed to do doing their shift, and I was able to get some sleep.

Good luck.

 

[FamilialALS?]

Is there a measure for knowing if their CO2 is too high? The goal in switching her straight bipap machine to a trilogy two months ago was that she would tolerate it better and wear it more. She still is not wearing it more than four hours per day. I constantly prompt her and encourage her to put it on but she just shakes her head. It's tough to leave her to her own devices but she has clearly expressed her will:neutral:

 

[Atsugi]

Regarding CO2: If a doctor wanted to, she could measure the Arterial Blood Gas. But here's a question: What would you do with the information? I'm sure someone else will have better advice.

You might disagree, but I believe that the PALS gets whatever they want. It's their life--and death. They get to choose how they're gonna go. Some may disagree. --Mike

 

[lgelb]

Probably the Trilogy is set too high for her comfort. If you are willing to check the current settings, we can probably make it better. The other comfort question is the mask itself. PM me if you want.

As for the FVC, I agree it doesn't jibe with the clinical picture, air hunger/mobility/energy being much more indicative than sats, as Tillie notes, and what the neuro said in terms of survival wasn't likely accurate, either. Sounds like sloppy work, to not correlate the pt with the test result. Of course, it's good to have affairs in order, but I find both FVC=17 and a month unlikely, unless your mom is flat out tired of living.

Anyway, Larry lived for nearly 2y at FVC <30, and others here similarly, so don't spend a lot of time on that prognosis.

And don't let it scare you into hospice, either. If it's something your mom needs and wants, fine, but I'm not hearing that. Once in hospice, Medicare won't pay for DME, and if your mom is still mobile, one of these days she may reach a point of actually needing a mobility device. You can hop in and out of hospice, but it is a hassle. Again, if it's the right thing, do it, but not just because someone whose prognostications are suspect said to.

Best,
Laurie

 

[Diane H]

The FVC of 17 is without a doubt an error. No one with O2 Sats consistently above 95, not having headaches, not complaining of shortness of breath, and still up and about and puttering around can possibly be at 17. At 17 she would be in severe, obvious distress and on the verge of respiratory arrest. I can't believe the neuro believed that number! Either she didn't spend any time with your mother and observe that that number could not be her true FVC, or she is not qualified to be a clinic neurologist. The one month to live is nonsense and calling in hospice is probably premature.

Want a rough estimate of her FVC? Have her take a couple of deep breaths (while not using the Trilogy) then count as far and as fast as she can on a single breath. The number will correspond to her FVC; getting into the 40's would mean her FVC is in the 40's. Not totally accurate but obviously better than an incorrectly tested FVC!

 

[FamilialALS?]

Diane what you are describing is what I would expect too. Her levels have been tested by the same Resp therapist each visit, and she always needs to use the face mask. The hard part is my mom is bulbar with advanced ftd so it is so hard to figure out if she is having some of these symptoms or if she's trying to hide them or if she's in denial. Her Neuro is an ALS specialist operating in a major ALS clinic. Her bipap settings were turned down weeks ago to try to get her to wear it more, but that didn't help either.

It's just so frustrating to figure out from her how she really feels. I don't put a lot of stock in a time frame because I have come to accept that you just don't know with this disease. I don't have a problem with hospice being introduced because at least it means I have someone to look in on her before the two month Neuro checkup.

 

[Barbie]

don't look at the numbers too closely as you have been told by others. my husband could not seal his lips and we have never in my opinion had an accurate FVC number. once he came in at 9...and that was years ago! while his situation is different than your mom's, base your decisions on how she feels. even if she cant really tell you because of the FTD, if she feels bad you will be able to tell by how she acts.

I know how stressful it is to wonder, "how long?" we all do that. but no one can tell you, certainly not the Neuro. someone here once said they had looked on the bottom of their foot and could not find the expiration date --your mom doesn't have one either. Each PALS is different and each journey is different.

I agree if you think you may have FALS then get the disability and long term care insurance lined up now! those can really help and you can drop them if you find out that you are not a FALS later. I hope you can get the genetic testing done soon.

 

[van1]

Sorry you are going through this.

 

[FamilialALS?]

Thank you everyone for your input. The one thing this disease teaches us is you have to take each day as it comes.

 

[GilWest]

I'll give you my advice about full-time ventilation when and if the time comes....don't.