Bulbar ALS - what will death look like?

Bhope1006

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Good evening everyone,
Please excuse the long background info, I wanted to give a clear understanding of our circumstances. My father in law is 65 and was diagnosed with Bulbar onset ALS about 7 months ago but has had symptoms for a total of about 16 months. Due to a variety of choices and circumstances he is currently only on Rivuzole, Tudca, and a variety of other supposedly helpful supplements to slow progression. He had considered Radicava infusions but has decided not to go ahead with them.

He has stated that he does NOT want any tubes, no feeding tubes or breathing tubes, he doesn't want to prolong the inevitable. He has had significant increases in his symptoms recently and my husband and I are concerned that he is nearing the end but have no idea what it is going to look like. He is currently still able to eat and drink, he eats very slowly and still has choking episodes and is now far more limited in the foods he feels he can eat than he ever has been.

He seems to be breathing okay, though the concern is that he is having a much harder time clearing his secretions, he has a lot of phlegm and has been up at night sitting up trying to clear them from his throat. This is resulting in him napping more during the day which is highly unlike him. He has no feeling in his tongue and has lost feeling in one of his lips and moving to his throat. He drools and notices sometimes and wipes it away, he has fasciculations in various parts of his body. He is now incredibly difficult to understand when he speaks, sometimes we can understand a word here and there but it is getting significantly more difficult to communicate.

We have just printed DNR paperwork for him to sign so that if something happens suddenly, we are able to legally respect his wishes. I am an RN and he and my mother in law moved in with my husband and I to be able to help care for him as things progressed but we have no idea what to expect next. I know no one can give us a time line but can anyone shed light on what their loved ones passing looked like if they did not have any interventions such as feeding or breathing tubes? I cannot seem to find anything to help us try to prepare for this. Thank you in advance!
 
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lgelb

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Hi, B, sorry that you find yourself here.

I moved your post to "Current Caregivers" because even though your question is about death, there are still things that can happen in life to help your FIL.

If he is choking on food, for example, switching to soft/curdled food/smoothies can help. A large bore straw will help him eat at his pace if he can avoid taking in too much air.

If he continues to eat solid food and if his swallowing continues to deteriorate, whether his breathing is impaired or not, most likely he will aspirate something and die of asphyxiation or pneumonia, whether in his sleep or awake, we cannot say. I am assuming if he's declined a feeding tube and BiPAP (does he understand the difference between a vent and BiPAP? many people don't at first), that a suction machine or CoughAssist are also off the table.

If he is swallowing supplements in any form other than liquid, that could precipitate the last episode also. If he doesn't want to delay the inevitable, I would ask if he wants to reconsider the supplements, which are taking energy to swallow and metabolize, risk choking, and accelerate his nutritional deficit to the extent that there is one. Their benefit is unlikely to outweigh the lack of a feeding tube and BiPAP.

I would certainly make sure the DNR is signed and that you have a clear read of whether he would rather die at home or be transported to a hospital. Texas tends to transport and there is not always EMS support for DNRs in small communities such as yours (I grew up in Houston and know people in Magnolia), so you will not want to call EMS if transport is not desired.

If you want him to be comfortable at the end, I would have morphine at hand from here on, and let him be the judge as to when there is no more reason to measure it. He and your MIL are fortunate that you have taken them in; thank you for doing that.

Best,
Laurie
 
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Bhope1006

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Hi Laurie,
Thank you SO much for your response, I’m so grateful. They were in NY until recently and moved down here because we are the only support that can physically help right now. He’s having insurance issues now after having to go on Medicaid or Medicare (It’s whichever you have to get at 65 I always forget) and saying he needs to get co-insurance but there has been a huge time delay and they’re both not working and the deductibles are insane. So he has not been to an ALS clinic here only in NY and it doesn’t sound like he wants to go back, they are so incredibly intense and overwhelming. I don’t know if it’s possible to find a primary closer who will let us just get comfort prescriptions like the morphine to support him at home? These are all excellent points that I didnt necessarily think to talk to them about so thank you for bringing them up. He is very difficult to talk to about various interventions even the non invasive ones he just shuts down so I will try to offer the bipap and cough assist and see how he responds to that. He doesn’t want anything staying in him but maybe if he can take it off whenever it might be something he’s more comfortable with.

Thank you again for being so helpful,
Brittany
 

lgelb

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However we can help, Brittany. I am sure you will hear from other CALS whose PALS didn't have both a BiPAP and a tube. (My husband had the first, not the second.)

There is a six-month time clock on buying a supplement, past his first getting Medicare, so I would look into that. After that, medical underwriting that he would not pass comes into play. You can find plans at medicare.gov. Since he moved, he may also be eligible for a Medicare Advantage plan even if it has been more than six months. He will also need a Part D plan for drugs if he does not get Medicare Advantage.

It should be possible to find a primary like that, yes. Maybe even a simpatico hospice to help out. It may take some networking -- you might tap your medical contacts. I will PM you if I have thoughts, as, I'm sure, will current area residents.
 

affected

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We were in the opposite situation, my husband had feeding tube but no bipap.
We achieved a beautifully peaceful end of life, here at home.
However I will say this is because early in the disease he was clear on his wishes. Even though he experienced quite rapidly moving dementia during his ALS, I had clear direction through his advanced health directive to make sure he could have what he wanted.

I didn't agree with all his choices, but advocated for him to make his own choices.

Let me know if I can help at all, but I feel that getting everyone on board with making his choices happen will give everyone the most peace along the way and after.
 

GreenCat

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I said I'd rather starve than get a feeding tube - even the words are horrible, but now we call it a Nutrition Transport Line, which is a little better. Before it, I had been choking to weeping exhaustion and blue lips staggering to the Cough Assist every dinner.
 

sweetmozart

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Hi Brittany!

I just want to chime in about the clinic visits. My husband went in to the clinic until it got to be a bit of an ordeal. Our clinic here in Massachusetts has a home visit program where the nurse practitioner comes to our home for his regular appointments.

So, maybe you could call your clinic and find out if home visits can be arranged.

Good luck!
 

ARCG

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In our case, we had hospice palliative care when my husband decided the clinic visits were too exhausting. They really helped make him as comfortable as possible. My husband had the trilogy but didn’t want the feeding tube, as it turned out, he was eating okay unril a few days before he passed. It was his breathing that became the Issue and he did not want invasive measures. Hospice was able to prescribe his meds, riluzole, nuedexta, etc.
 

Believer

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Hi B
I'm so glad you asked the question. I wonder the same thing. My PALS has the PEG and Bipap. We also live in Texas with a DNR. I didn't know that I might have problems with it. We also deal with a lot of phlegm. He is afraid he will die choking on the phlegm.
 

wndshr

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My wife (54) has bulbar and does not want any of the interventions either, for now. We have negotiated a FT when she loses about 10% of her dx weight or her breathing function degrades to a level they will not do the FT procedure. She has filled out a POLST that says once she is no longer able to perform daily hygiene functions, she wants off the FT and into hospice. Im scared to death what that process will look like. Will she suffer terribly? I see people talk about morphine, is that what hospice uses to mitigate the suffering?

Thank you all for your feedback
 

lgelb

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There is no reason for anyone to suffer at the end. When air hunger begins, that is a legitimate indication for a doctor that the PALS has seen in the last year (can be your PCP, does not have to involve either an ALS clinic or hospice) to write a prescription for morphine. Morphine is available in enough forms that whether someone has a feeding tube or not, can swallow well or not, dosing is not an issue. When a PALS is no longer comfortable, there is no need to measure it. The CALS' last and best gift is a peaceful passage.

Best,
Laurie
 
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affected

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If you work with your wife wndshr, you don't have to be scared of her suffering.
We had such a peaceful passing here and having the feeding tube made it easy for me to administer morphine meds, but I stopped giving any fluids or nutrition several days before he passed.

We used a combination of morphine and clonazepam really effectively.
The clonazepam works really well to relieve panic and anxiety. We had it as drops that I could just pop under his tongue. It is absorbed directly through the gums, so even though he could not swallow I could use this. Being absorbed through the gums means it does directly into the blood stream so it works really fast.

He slept a lot of the time obviously, but he was also aware of his surroundings and lucid, but calm.

On his last night we watched an episode of Breaking Bad together (this was back in 2014), then he passed very peacefully in his sleep some hours later.
 
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