Questions Regarding New Diagnosis

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ALSquestions

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Hello, all,

My mom, age 71, was officially diagnosed with ALS last Thursday. It came as a shock to her (and she still doesn’t actually believe it—she doesn’t want to tell anyone until she is seen at the ALS clinic and they confirm the diagnosis), though it’s the diagnosis I was expecting.

I have a few questions about this and that. First, a little background.

My mom is very weak, fatigues very easily, and has lost 40 pounds in the last year—her BMI is just over 16. Her symptoms began two years ago in her left foot. Her hands became involved a little over a year ago, and her voice began to change more noticeably this past summer. In retrospect, her breathing was also showing changes during the summer, though it looked like it was just fatigue and just generally being weak and out of shape (though, tellingly, as she was having to do a lot of physical activity to get ready to move from her house to an independent living setting for seniors, I do remember thinking that it was odd that she didn’t seem to be getting stronger or gaining any stamina with all that exercise).

Over the last month or two, her decline has been alarming. Her voice seems to weaken almost by the day and her speech is quite slurred and slow and often difficult for others to understand. Her resting respiration is 26-28. She becomes dizzy and feels faint when she does things like trying to change her sheets. Last weekend, she woke in the night feeling odd, rolled over, and fainted in bed, and earlier this week she woke at 2am with a bad headache and couldn’t get back to sleep. I think this happens regularly, but she isn’t always forthcoming about it.

She has some cognitive impairment, which makes it difficult to tell how much she understands and even how anxious she is. I’m not sure how much to explain what is happening or what her symptoms mean. For now, I try to just make suggestions, like “Why don’t you try sleeping more upright?”, so I don’t add to her anxiety.

It’s certainly sounding to me like she needs a biPAP at night, and she might benefit from using it periodically throughout the day. We are getting the ball rolling on making the move to assisted living. She says she would like a PEG tube. Her swallow study showed only mild dysphagia, but she does choke frequently, perhaps partly due to a past history of choking, due to a medical condition unrelated to ALS, and a fear of choking again. Her cough is very weak. But she can generally handle ice cream and yogurt pretty well.

One thing that has surprised me is that she is still relatively mobile. Though I wish she’d use her rollator all the time, she can walk around her apartment without it. Her mobility has greatly decreased, but she can still stand for long enough to shower independently and she can walk short distances unassisted. She can’t really do stairs at all now, though she could with only a little help a couple months ago, so I guess that has declined pretty fast recently too.

Her first appointment at the specialty clinic hasn’t been scheduled yet, but I’ve been told it’ll likely be within a week or two, which I’m grateful for, as I’m quite worried about the breathing.

That was a lot of background. Thanks for reading this far.

My questions are as follows:
1. What should we expect at her first ALS Clinic visit? (We are in Baltimore and are going to the Johns Hopkins clinic, in case anyone has direct experience.)

2. Is there anything to be made of the fact that she’s still mobile and had limb-onset, but her breathing seems really crappy really quickly?

3. Am I naive to think her breathing is pretty bad? By which I mean, are you all thinking, “Oh, you poor, blissful idiot, you think THIS is bad?!”

4. She has heard a doctor and a nurse both mention that people can live with ALS for many years. I think that is her expectation for herself right now. I don’t wish to give her a different picture unless she wants a different picture, but I myself would like a realistic picture. What does this sound like to you in terms of where we are in this journey? No way to know? Will the clinic provide more data that could give me a clearer picture?

5. Can things get better? Like, with biPAP and a PEG and once we put some more targeted care in place in assisted living, could things stabilize, allowing her some more time in relative comfort to plan and do things she enjoys?

Thank you all for the gifts of your time and your wisdom.
 

Foodie1

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Thank you for sharing. I can relate to you because my mom passed away from Bulbar onset at 74. 2 years after her diagnosis at the Mayo Clinic.
My mom was mobile up until about 2 months before she passed. Once she was permanently in her wheelchair she really went downhill fast because she wasn’t moving her body/lungs/legs. was not able to clear out mucus, feet swelled, bed sores etc.
one of the biggest pieces of advice to you is to be pro addictive instead of reactive. Don’t wait until she can’t eat to get the feeding tube put it now. Let her get used to it while she still can eat a little bit. Or don’t wait to order a custom wheel chair that can take up to 8 weeks to be made when your mom can‘t walk anymore. Order it before. It sounds like since your mom is fainting and having headaches her blood oxygen levels are low which means she would benefit majorly with a BiPAP machine which will help put less strain on her diaphragm and possibly extend your mom’s life The clinic will be of great resource and will help you navigate with your mom. Also calling your local chapter ALS Association and getting someone assigned to your mom will be a big help and will have resources. I personally was a cheerleader to my mom always trying to give her hope and not to give up.
 

Jimi

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I'm not going to try to answer all of your questions. But I can assure you that using a bipap can make a huge improvement in the comfort, energy and overall quality of life. I was limb onset. Dx 6 years ago. Symptoms about 2 years prior. I have been dependent on niv (bipap) 24/7 for a little over 2 years now. I am paralyzed from the shoulders down. Once you get used to it, it's not bad. I had headaches in the morning and kept fighting the bipap use. Really silly of me. I felt much better once I got proper sleep/breathing. So in my experience, my progress has been very linear. But the improvement in breathing does buy more time and is so worth it. All my best, Jim
 

lgelb

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1) You may want to read this sticky about the first clinic visit.
2) No.
3) No.

4) They may prognosticate if you ask, but it's a crapshoot. There are online tools from large datasets. They're not all that, either. Nutrition, breathing and state of mind are the ultimate longevity factors. Definitely get an rx for BiPAP from what you say. It does not have to be from the clinic; any doc can write one. But you are talking about assisted living and that level of care is usually not equipped to deal with BiPAP and a feeding tube. Maybe you are thinking about another level of care? Can she have in-home caregiving -- why are you jumping to a facility? (Not saying you don't have reason, but it would help us help you to know what it is.) Are you planning for private caregiving support at the facility you're talking about?

5) Yes, she can be more comfortable with respiratory and nutrition support, but it's a progressive disease. Her progression sounds "medium" to me, but she is behind the curve for BiPAP and a feeding tube, which could turn "medium" into something else. That is a very low BMI, suggesting she is malnourished (the dizziness/faintness may be about breathing, starving or both) and there is a point past which a tube can't pull her back. If she can handle yogurt, I'd be making her high-speed blended high protein/high fat smoothies (eggs, nut butters, extra cream, etc.) by the boatload. A wide bore straw may or may not be easiest.

It sounds like she needs someone with her 24/7 at this point, her imminent move notwithstanding. Otherwise, you may not have her long at all. Sorry to be blunt.

Best,
Laurie
 

ALSquestions

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I really appreciate the straight talk and honesty. Thank you all so much.

Regarding a facility: Where she lives now is a continuum-of-care setting—they can accommodate a lot (including, specifically, biPAP and PEG—I asked) in their assisted living setting, and that’s where they are recommending she move. They said if she moves soon, she can stay there for a “long” time before having to move to the skilled nursing unit (a traditional “nursing home” setting). She’s in independent living now, and I think she would prefer having a nurse down the hall rather than trying to make the most of scheduled visits (really tough with cognitive impairment) or someone living in her apartment with her (I know she would try to “host” them and make them tea all the time! And it’s also really not set up to accommodate a nurse living with her).

I know I will have guilt for the rest of my life that I cannot take care of her in my home, but I have two young children (5 and 1), no spare bedrooms, and the kids will be home once summer starts and I will be returning to work full time in the fall. (Only reason I’m not working now is that I’m undergoing cancer treatment. I’m fine, and I’m going to be fine, but when it rains, it pours!) My sister could have her move in, but she’s an hour away, making clinic visits tougher, and temperamentally, I just am not sure that would be best for either of them. As it is, she’s just 10 min from me, which I also like.

Thank you all again for your help. Be well.
 

Nikki J

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I am sorry.

re assisted living That is great if they can really meet her needs. I would push them on exactly how much care she can get from them and how much extra they are going to charge. There is also value in not moving twice and if she is going to the skilled section getting there while she can still speak a little so they can get to know her.

the fainting in bed sounds a bit atypical. see what jhu says but I wonder if she has a second issue too and should see her internist.

my sister had her second opinion at jhu and went to their clinic for a while. She ended up at mgh but not because she hated jhu. I expect you will have a long visit both because you are new and because your mother has several pressing issues. They will test her breathing ( the diagnosing neurologist did not?) address her wishes re feeding tube and advise on swallow precautions hopefully. Pt should see her It sounds like communication is another priority made more urgent by her cognitive issues and the fact she lives in a facility. The clinic for all the communication tech is a separate appointment / referral.

I think you are planning to accompany her? It looks like you need to apply for permission as a care partner of someone with a disability which you probably know? Visitor Guidelines for Patient Care Partners During COVID-19 | Johns Hopkins Medicine
 

KimT

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I went to Johns Hopkins and they did a thorough exam. Even though I went for a second opinion, they did a Pulmonary function test, offered genetic testing, and did a very long clinical exam with Dr. Rothstein. He talked about NIV. I'm sure they can get your mom a Trilogy in short order. You might even be able to schedule the feeding tube procedure while there. Please ask.

Good nutrition and BiPap will give your Mom a much better quality of life. It's good that you're not far away. Also, ask about the Covid vaccine while you're there.
 

ALSquestions

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Thank you all so much for your help. The diagnosing neuro did not do any tests of her respiration. We weren’t able to get an appointment at Hopkins for three more weeks, which greatly worries me. I’m on the cancellation waiting list, so here’s hoping.

I’ve reached out to Hopkins to explain what I’m seeing and ask if they can recommend a respiratory therapist that we can see in the meantime but I haven’t heard back. I’m not sure that would even be helpful since that person might suggest something that Hopkins disagrees with, and that would just further waste our time.

I called U of MD to see if their clinic would be quicker to get us in, but they would require another hoop to jump through, in that they won’t accept this neuro’s diagnosis and would require that she see one of their neuros before being seen at the clinic—by the time that could happen, we certainly would’ve eaten up these three weeks.

So I feel stuck and helpless and frustrated. It’s helpful to hear that anyone can prescribe a biPAP. I will follow up with her primary care doctor this week to see if she can get moving on that front. I feel like I know more about ALS that she does though...

Thank you again for your time. Your patience and wisdom are invaluable
 

KimT

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My local neurologist prescribed a Trilogy but she really needs a pulmonary function test. Do you have access to a local pulmonologist?
 

lgelb

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To clarify, she needs pulmonary function tests if you want insurance reimbursement for the device. She doesn't, if you're willing to pay cash. Either way, with cognitive impairment, I would make sure she has seen video of what the mask and machine look like before she encounters the real thing.

No one can do PFTs without an order from a doc. Nor can a respiratory therapist prescribe BiPAP. But her primary care doc or the neuro that diagnosed her can do both (order testing that can be done at a local hospital or clinic/write for BiPAP). It does not require a pulmonologist. Since she still ambulates independently, a Trilogy would be cumbersome at this point; a smaller BiPAP that plugs into the wall would be fine.

With the respiratory rate, which is likely not sustainable, and is probably affecting both swallowing and speech (and possibly cognition), and issues you report, I would at worst make sure she can get PFTs at Hopkins (that isn't always part of an evaluation) when you go.
 

ALSquestions

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I was able to get my mom in for PFTs yesterday. Our clinic appt (which, it turns out, it just a diagnosis confirmation) is still a week away. However, the tech who did the PFTs wasn’t able to complete the test because my mom didn’t have the strength or ability to do the tests. She couldn’t get any results. I talked to the tech and she said she got “maybe a liter” on an FVC test, which would put her FVC at <30%.

She has continued to lose weight rapidly, at a rate of 2.5lbs per week (now 85.5lbs). Her cognitive status is also deteriorating by the day. But in many ways she is herself: I brought my daughter over for lunch and my mom had set up a cute little picnic spot for her on the floor before we arrived.

My sister and I need to make some rapid decisions about whether to go through the process of moving her to assisted living (where she’d have more supervision and help but where, because of covid restrictions, she can have only two visits per week, plus moving itself is stressful) or to think more along the lines of enlisting hospice to come where she is (and where she could become an inpatient, if needed) and plan for me to come over as much as possible.

I hate having to think so logistically, but I’ve also got to take into account that my ability to help will become really complicated and difficult when (if) she makes it to June and my kids are home again.

Does anyone have any thoughts about what I should be expecting at this point? I really appreciate your support.
 

Nikki J

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I am sorry. Where you should be depends on what she wants her life to look like. Is she going to want bipap? That might buy her time and possibly improve her cognition.
I still wonder how much support assisted living will give. and I would worry about only being allowed in twice a week.
a feeding tube may be tricky with that breathing if the numbers are accurate and not a reflection of cognitive dysfunction in cooperating. My sister was close to that number and emaciated when she had hers but she was also much younger and in excellent health aside from ALS. She had a very difficult recovery and we almost lost her.

None of us can predict the future unfortunately. I wouldn’t make any decisions before talking to Hopkins but you should talk about how she wants things
 

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I'm really sorry about the whole situation.
I truly agree, find out now what she wants with every possible thing, and talk to Hopkins about this with her to help make the best decisions. I would be concerned about not being able to see her often if she is deteriorating this fast.
 

lgelb

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If she does not have her own caregiver in assisted living, it is hard to see with staff only how she will enough support at that level. And, like Nikki, I would worry not only about care gaps with 2x/weekly visits but her quality of life not being able to see you, your sister, and your daughter more than that. If my days were numbered, I, and I suspect most people, would value family visits above all. And I have recently seen people who are cognitively impaired progress more rapidly to death when they were deprived of that, even with video chats.

With the rapid weight loss, low BMI, and what at best is probably moderately impaired respiratory function, with or without intervention, June may well not be an issue, and so quality vs. quantity of life remains something to discuss in very basic terms -- maybe boiling down to the facility vs. home. It is still possible that the BiPAP and feeding tube could extend her life, but assisted living likely will not, if that makes sense.

With what you would pay for assisted living, I would think that she could have in-home care over that period, see you all, have help with the feeding tube/BiPAP if she gets one/both. Baltimore is a big city, so there should be access to self-pay individual caregivers to avoid agency hassles/overhead, and she would likely also be entitled to Medicare-funded hospice care through an agency if you so chose.
 

ALSquestions

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This is all so helpful. Thank you for your kind and thoughtful words. I thought I’d try my best with Atul Gawande’s five end-of-life questions when I see her tomorrow so I can try to get a better sense of her thinking. We’ve talked about things a few times, but it’s so effortful for her to talk. She is able to type though, so I may suggest that, since it may be less tiring and might also allow me to tease out the cognitive piece a little more, since it’s sometimes hard to gauge if she doesn’t know what to say or if she’s out of breath and energy. I agree with what’s been said about 2x/week visiting—that doesn’t seem wise or compassionate for anyone involved.
 
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