Progression

doglady125

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May 25, 2019
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CALS
My Mom was diagnosed with ALS last December. By the time she was diagnosed, my Dad had already had to purchase a used power chair to use for walking any distances.

She had initially been diagnosed with Hereditary Spastic Paraplegia a couple of years prior to the ALS diagnosis, but had problems for a few years preceding either diagnosis. She kept complaining of weakness in her legs. Then she started having frequent falls. She then got to the point that she needed a walker for distances, then power chair for any distance and walker for very short distances.

Currently, she can walk very slowly with the walker, but only from her lift chair to the bathroom. She requires assistance getting in and out of bed, getting up from anything but the lift chair/recliner, cutting food, dressing, bathing, etc. My Dad is her full-time caregiver, but I fear that it's taking a heavy toll on him. I assist when I'm not at work.

Now, her speech is affected (she can talk, but we have to ask her to repeat at times) and she chokes easily. Dad and I are both wondering what the future holds and what the typical progression is. We do not want to ask the doctor in front of her. We do not want to put her in a nursing home and are doing our best to manage at home. He has stated that she will never be put in a nursing home as long as he's alive.

He's in need of a knee replacement, but refuses to have any surgery, as he will not put her in a rehab facility even temporarily, and we are not in a financial position to be able to hire someone while I'm at work. I work full-time, and unfortunately, cannot take any extended leaves of absence and keep my job, and my income is required to keep us afloat. Some questions that we have are as follows:

1. Do people with ALS typically become totally paralyzed before needing to be put on a ventilator?
2. Do they typically lose the ability to speak or swallow first?
3. What is a common time-frame from diagnosis to becoming ventilator dependent?
4. How is the disease managed if a person refuses the ventilator? At the beginning, she told us that she never wants one. But we cannot stand the thought of her just laying there, unable to breathe, and suffering. That sounds absolutely horrible. In a way, knowing her wishes, we hope that she passes peacefully in her sleep before reaching that point.

Thank you for any advice or guidance. Our family is totally devastated. It's only been the 3 of us for all these years, as my parents were never close to the rest of the family and I'm an only child. So we are pretty much in this battle alone.
 
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KarenNWendyn

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I’m so sorry you and your family find yourselves in this horrible situation. It is devastating.

The course of ALS varies from person to person. The only “rule” is that it progressively gets worse, though the rate of progression varies and there may be an occasional plateau along the way.

The order of how things progress will also vary between people. Some people will become totally paralyzed before their respiratory muscles fail to the point of needing ventilator assistance. Others can develop respiratory failure before the limbs and bulbar muscles fail. And every combination in between is also possible.

A person can refuse a ventilator. Other options include using noninvasive ventilation through a BiPAP machine. One can refuse that too, but using a BiPAP can help conserve energy and slow (but not stop) ALS progression. ALS will still progress even with invasive ventilation. Other alternatives include going on morphine and other sedatives when air hunger develops. This should be spelled out ahead of time in an advanced directive, if possible.
 

lgelb

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Very sorry about your mom.

To clarify the terms we're throwing around, a BiPAP, which is a form of "non-invasive ventilation," is a machine that sends air into a mask or mouthpiece, to help someone get enough air when the muscles that control breathing are weak.

Most PALS but certainly not all, choose to use BiPAP as their muscles weaken. Generally, though, eventually the muscles are so weak that even BiPAP does not help enough. Most often, it is around that time that death occurs.

That is because most PALS do not choose "invasive ventilation," where a permanent opening is made in the throat (tracheostomy) to provide air when noninvasive ventilation is no longer working.

But the important point is that when someone imagines "a vent," most often they are thinking about invasive ventilation as shown in many tear-jerker movies, seldom uplifting. They may not realize that noninvasive ventilation can most often give them a year or possibly several. So it's good to double-check that everyone is talking about the same things.

The answers to the questions above answered as regards BiPAP are:
(1) No.
(2) No.
(3) Usually around halfway through the disease or earlier, except in respiratory or bulbar onset where it could be near the beginning. But not all docs and clinics are equally aggressive about testing/asking about symptoms/getting it ordered, so there's a lot of variability on that score alone.
(4) If someone decides not to use BiPAP, life is likely to be appreciably reduced but the transition can still be softened by titrating morphine and other drugs to comfort.

Best,
Laurie
 

lgelb

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Do your parents have a spare room, that they could trade for live-in help?

I take it your job isn't eligible for FMLA?

Have you checked your local/state senior services and disability offices for respite programs and other support?

Not knowing what state your parents are in, are they eligible for Medicaid or "extra help" with Medicare? You can check benefits.gov to see what they might be eligible for.
 

doglady125

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Thanks everyone for the replies. My job does have FMLA, but the reality is, when I’m not there, cases go unmanaged and out of compliance, which would ultimately result in my firing. We also want to make sure I can be there with her and Dad at the worst, but since we don’t know what to expect, it’s tough to calculate. Right now, I use intermittent FMLA.
I’ve tried to convince Dad to look into help, but Mom is very reclusive and he doesn’t want her stressed with “strangers” caring for her. It’s definitely a difficult situation that I would not ever wish on anyone. I used to be so scared of her getting cancer, but at this point, I do not think there’s anything worse than this. Right now, she chokes a lot and sleeps a lot. Short trips out of the house absolutely exhaust her. I feel pretty much helpless and so does Dad.
 

Nikki J

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I am sorry. Does she go to a clinic?

It sounds as if she is at a point where a feeding tube should be considered. A feeding tube and bipap / non invasive ventilation might make her and you feel better though the choice is hers. It sounds too as if she should be using her power chair more to conserve strength. This will help her and your dad and you.

There are many speech options depending on her abilities

You sound understandably overwhelmed. It is a very tough situation. Again I am sorry
 

nona

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It sounds like an emotional struggle. Your employer cannot legally fire you even if work goes unfinished while you are out. That's the whole point of the law.

I wholeheartedly agree with recommendations for bipap and feeding tube as soon as you can. Both should give both parents some ease of mind and even more energy. Better sleep and better nutrition (SO much easier to eat when you don't have to worry about choking or coughing) should help with overall coping.

Are you connected to an ALS clinic? Are you urban or rural? I understand your father's concerns about strangers, but even my two parents and two stepparents couldn't do it all. I reached out to a nearby nursing school and now have two wonderful young women who pitch in. It didn't take long for them to not feel like strangers. There are angels everywhere.

You and your parents are in my thoughts.
 

lgelb

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Nona, while it's technically true that FMLA, used intermittently or all at once, protects the person's job, it doesn't prevent an employer from finding a pretext for termination. In an "at will" relationship, simply citing budgets or strategy or really anything, will do.

I agree that students can be a great blessing, and like anything else, it's best to act as if and start with a small dose. Or if there is someone your parents know and like, Doglady, perhaps that would not be a "stranger" to start with? Even a high school student could help out.

And, Doglady, while everyone is right that a feeding tube and BiPAP could prolong quality and quantity of life, I am not sure where your parents stand on all this. If the ALS clinic has a social worker or there is someone in the community they can talk to about their wishes, that might help them. You might be too close to be the only one. You will also want to encourage them to put those wishes in writing.
 
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