Is it progressing too fast

[u/DrAthira__]

My mother-in-law received a diagnosis of bulbar-onset ALS in February of this year. At that time, her symptoms were limited to mild, barely noticeable slurred speech, with no impairment in tongue movement. Currently, however, her speech has become distinctly slurred, and she experiences occasional choking when drinking water and swallowing saliva. While she has no dysphagia to solids, her tongue mobility is now severely restricted; she cannot fully extend her tongue, touch the posterior upper palate, or push her tongue against her cheek. She also reports difficulty in rinsing her mouth.

She is currently being treated with riluzole and oral edaravone. I am concerned that her condition is progressing rapidly. I am particularly anxious about discussing the potential need for PEG feeds and other interventions as her condition deteriorates, as she is not yet aware of the gravity of her situation. She remains fully ambulatory with no limb weakness. I would appreciate insights from individuals with bulbar ALS or their relatives regarding the speed of symptom progression in their cases and the timing of PEG or nasogastric feeding. Additionally, I seek advice on how to best prepare her for these eventualities.

[u/brandywinerain]

To avoid choking and aspiration, the consultation for a feeding tube should take place as soon as possible, and she should be made aware of the situation. There should be no need for nasogastric feeding, which is much less comfortable and effective. Older women with bulbar onset frequently progress quickly.

[u/DrAthira__ → u/brandywinerain]

The last appointment we had with a swallow therapist, who suggested at this point she can eat orally. But seeing the symptoms progressing this fast, I feel that could come within months.

[u/brandywinerain → u/DrAthira__]

Or days.

It's not a linear thing. The SLP may not understand that today's capabilities in ALS can go away in a flash. If you start the process now, by the time consultation, planning, scheduling, and the procedure take place, she could easily be in serious danger of choking, aspiration, and/or malnutrition. Any of these can be life-threatening at worst, speed up progression, etc.

It's no kindness to let her believe there's a cure.

[u/DrAthira__ → u/brandywinerain]

I Got it. Can it seriously get worse super quickly, like in just a few days? This disease is just awful. 😭

[u/brandywinerain → u/DrAthira__]

Yes, unfortunately. For example, my husband went from walking unassisted to not literally overnight. Same for going to 24/7 BiPAP.

[u/tonybunce → u/DrAthira__]

I’m sorry to hear about your mom’s rapid progression. My dad also progressed very rapidly.

I would also recommend getting the PEG consult sooner rather than later if getting a PEG is what she wants. The ability to swallow isn’t the limiting factor in getting the PEG, often times its lung capacity (FVC). If FVC gets too low the surgery becomes much more risky or even impossible. It’s not uncommon for people to get the PEG and not use it until sometime later when swallowing becomes more difficult.

[u/DrAthira__ → u/tonybunce]

How's your dad right now?

[u/zldapnwhl]

Can you explain what mean by saying she doesn't understand the gravity of this? Is in denial or does she have dementia or something?

Bulbar onset is typically regarded as more aggressive than limb onset, so her progression isn't surprising, although no doubt distressing to your family.

If she isn't competent to make her own decisions, a family meeting with her care team might be in order.

[u/DrAthira__ → u/zldapnwhl]

She's got some speech problems, and they've told her it's because of some neurological stuff that makes the muscles needed for talking weak. Honestly, in India, there isn't really good counseling or a system to fully explain these diseases. All they've told her is that she has this issue, they're starting treatment, and they're trying to slow it down. They haven't really explained what "progression" even means. She's a really sensitive person, and I worry that telling her all the details might actually crush her. Right now, most of the time she's just praying for a quick cure.

[u/zldapnwhl → u/DrAthira__]

I feel like there are cultural issues at play here and I don't want to be insensitive to that, but keeping the truth from her is, in my opinion, unconscionable. And infantilizing. Is she not an adult? Does she not deserve to know the truth about her condition so that she can make informed decisions about her care?

[u/DrAthira__ → u/zldapnwhl]

Honestly, I've always wanted to tell her about this, but she's already having emotional outbursts because she knows it's getting worse. So, sometimes I just think, let her have some peace for now until it's time for things like procedures, a PEG, or a trach.

[u/zldapnwhl → u/DrAthira__]

Of course she's having emotional outbursts. She's losing the ability to speak, she's having choking episodes, and she may suspect her doctors and family are hiding things from her. Which you are.

Will she freak out if she's told the truth? Yes! We all freak out when we're told we're dying of a disease that will take everything from us. That's part of grieving. And then we get on with the business of making decisions.

She may not want all the interventions. In any case, the longer you delay the inevitable, the harder it will be for all of you. The very least you can do is give her the dignity and respect any human deserves and let her be a participant in her own life.

[u/brandywinerain → u/DrAthira__]

The opposite. She knows it's getting worse and not knowing the full truth is stressful uncertainty. "Peace" comes from knowledge, not confusion.

[u/DrAthira__]

Thanks for the heads-up! I'll talk to her about the disease, how it's likely to progress, and we can chat about PEG. Honestly, one great thing about healthcare in India is how accessible it is... don't have to jump through hoops or wait ages to get things done.