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worrieddaughter77

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My dad has been diagnosed with "MND likely ALS " a month ago in North Western University hospital in Chicago. In 4 days we are going to his second ALS clinic visit. During our last visit, i was too overwhelmed and unprepared to ask questions, but i want to make sure i am all of the important questions this time.

I would like to run these questions by you, and see if you have any suggestions/ additions.



1. During the 9 months it took to diagnose my dad, his local doctor prescribed prednisone, thinking he has myasthenia gravis. Could prednisone have accelerated his progression?

2. 4 years ago my mom and dad moved from Ukraine to the US because of the war in Ukraine with Russia. Could stress of moving to another country have triggered ALS?

3. When do you recommend inserting PEG?

4. Is dad's progression fast,* or average?

5. How do we know if his breathing is getting worse or not.* How can we monitor it* at home?

6. When can we start on rilutek, and why haven't we started yet?

7. When can we start radicava?

8. Can we do genetic testing to see if it is genetic?

9. Do you recommend taking supplements?

10. Are there drug trials that we can join?

This is it for now. My brain is scrambled really...

Thank you all! This forum so far has been an invaluable resource. I have learned so much from it.

Anna
 

lgelb

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Anna, no sarcasm intended, but my first suggestion is to keep referring to your dad as "he," not "we." He is the PALS and these are his decisions to make, with your input, despite his lack of speech. Some thoughts about some of your questions --

1) No way to know; don't look back.
2) Ditto.
3) A PEG (RIG in Radiology is most often recommended) is recommended when eating and drinking become so difficult that the PALS is at risk for weight loss, choking and/or aspiration.
4) We talked about this before -- the answer won't tell you much, if you get one.

5) When he is on BiPAP, the machine saves data about how his breathing is, and shows it on screen if set up properly. Your dad will also be able to feel how well he is breathing. You will also be able to see when breathing is taking more energy than usual, different muscles are being used, fatigue gets worse, etc. That will help you know when his settings need adjustment, or when he needs BiPAP to begin with.

If there is no history of ALS in your family, there is no point in genetic testing.

I would add questions like,

How do we communicate with the clinic for needs between visits?

What other local resources would you suggest we contact?

What equipment do you think he will need next and what will cue us in?
 

Nikki J

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Are these your questions or your dad's? Do make sure his concerns are addressed Laurie does make some good points.

I thought we discussed that as he has not yet had had an EMG they trust to confirm their suspicions that is the likely reason no riluzole originally

You can research clinical trials in your area on clinicaltrials.gov. You can start typing amyotrophic for condition and choose your state, then seeing what may be recruiting. You can see the inclusion/ exclusion criteria too. You can still ask the question but knowing what is currently out there ahead of time would allow some advance consideration.

I am all for preparing for appointments and making lists is good. I would let the doctor say their piece first and see what remains unaddressed. I don't think the doctor will speak to the trigger questions but would likely answer we don't know what triggers ALS in susceptible individuals. If the doctor did say it might have contributed would it make anyone feel better? I doubt it.

Re supplements have you researched them? Are there ones that your dad is interested in? It is fine to ask. The answer may range from waste of money to specific recommendations but it could also be what were you thinking of trying.

Is this a clinic visit or a separate consultation? If the former and you are meeting pt, ot etc. get contact info for everyone. As Laurie says find out who the main contact is. At my clinic it is my nurse. I email her and if she does not know / can't help she passes it on. It is worth knowing how to reach each team member though. I recently specifically needed an answer from PT and I contacted her rather than go through the nurse

Your dad is lucky to have your support. There is so much to discuss and absorb at initial visit. Try to focus on the things that realky will make a difference
 
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Nikki J

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On the genetics front if your dad is interested Dr Teepu Siddique at Northwestern studies ALS genetics. He has led research identifying several of the FALS genes and has an ongoing study of PALS https://www.clinicaltrials.gov/ct2/show/NCT00821132?recrs=ab&cond=Amyotrophic+Lateral+Sclerosis&cntry1=NA:US&state1=NA:US:IL&rank=1

I believe if they find anything they tell the patient. I have found the first contact for the study Nailah Siddique very helpful and patient in answering questions on genetic issues. That said , if there is no other family history of neurogenerative disease current testing is unlikely to yield any information
 

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I have found that emailing a list of questions/concerns to the clinic coordinator before the appointment helps. Then the clinicians have time to look at your questions and do any research necessary.

I would also ask what equipment they suggest to help care for your father. It is easier to have things available to you before you need them then to have to scramble to try to find things when they are necessary. See if the ALS Association or MDA is associated with the clinic. They sometimes have items available to loan rather than having to purchase.
 

worrieddaughter77

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Thank you all so much for the feedback. I will update the questions in a bit.

Definitely a great suggestion to have just one main contact person. If i had that, i could have already asked some of the more pressing questions directly. Thank you, Laurie and Nikki.

Thank you, Nikki, for the genetics study reference! I don't think my dad has anyone from his parents with ALS, however, his grandfather could have had it, he died very young and nobody knows from what for sure. But I can see how there is probably no point in generic testing.

I agree with all of your concerns about these being my questions and not my dad's. I think my dad is in a different emotional state. He almost completely lost his speech, but after his diagnosis he stopped even trying to communicate with us. I got him a new Bluetooth keyboard for his phone so that he could type faster, but he stopped using any text to speech altogether, although his left hand is still not affected.

Similarly with the supplements. I have shown my dad all of the links from here and wanted to start a discussion about which one he should start with, but he doesn't seem interested at all. That is why i left it as a question to doctors, maybe they'll instead give a recommendation from their side.

Sigh. I'm so glad my brother is coming tomorrow to take my dad to Chicago. Hopefully, my dad will be more open to talk about all of these serious problems when he is around. Otherwise, it is just me doing all of the reading, research, and anticipation.

So, my dad will go to NWU hospital on Nov 8 for ENG as well as the second clinic visit. I don't think it will be just a consultation. I remember them telling us that we will see all of the therapists again: OT, ST, and PT.

After that we will be returning back every three months. We got appointments scheduled until next June already.
 

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I empathize with where you are right now - walking that fine line between a parent sort of assigning you to get information while they cope with all that is going on, without stepping over into seeking out the information you need to cope with all that’s going on right now for you . These forums are a great resource for you . If that’s what you need , read up and set the stuff you learn on a shelf for later . It does help. Do try (voice of experience here) to set it aside and take deep breaths. That helps too.

The first six months of appointments and contacts were, for me, as frustrating as they were helpful. I started saying “snowflake” as a nasty aside. Hopefully silently, but mom and I definitely has a speaking look when we got those answers. So much was changing so rapidly for her and no one would give her or me as her assigned proxy for asking, any straight answers... and trust me I asked the wiliest cross examinations I could trying to get any information we could grab and hold on to.
I think a lot of it isn’t that the med professionals are doing cya on a moving target, individualistic disease, but more that the snowflakity of als means that they just need to get to know the PALS before they can provide the “usually” answers. Things got a lot clearer to and with mom once she got a relationship going with in home ot and pt. And at her six month clinic visit last month (ymmv, of course) we had some very straight talk with clinic team members.

Hope that’s a little helpful.

Cee
 

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So well said Ceelea!

While there are some patterns, they are obvious in hindsight, rather than able to be predicted, especially when someone is first diagnosed.

It feels like you are being fobbed off, or like they don't want to answer or some other strange thing, but in truth we simply can't predict so much.
 

lgelb

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Anna,

Your dad was only diagnosed in October, right? Likely he is still processing, esp. since there were diagnostic missteps. So I wouldn't take his lack of input as the way it will be. If you and your brother both interact with him as you always have, and remember to use "I" rather than "you" statements, most likely you will see more interaction in the months to come.
 

worrieddaughter77

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Hello everyone,


Thank you so much for your answers. It is amazing how well prepared I was for the outcomes of the second visit, because of all of the information I got from here. At the same time I wasnt prepared to deal with the emotions at all...


Here are all of the answers we got. I will go down the questions one by one:



1.
2. We decided not to spend the valuable time with the dr on questions 1 and 2

3. When do you recommend inserting PEG?

Asap. But they will start the process in January, since they need to normalize my dad's breathing first. His breathing went down since October, and is now at 61% standing and 56% laying down. Sigh


4. Is dad's progression fast, or average?

Didn't see the point of asking this question either...


5. How do we know if his breathing is getting worse or not. How can we monitor it at home?


Because of my dad's worse breathing, a pulmonologist saw him this time as well. He said we needed to get the bi pap machine right away. Hopefully, it will arrive soon. On Dec 20, we will go back to the clinic just to see the pulmonologist again. That's when they will schedule the peg insertion procedure. He also said the machine will come preset with the settings and that the dr will be receiving all of the data from the machine electronically to daily monitor my dad's breathing. I hope, though, that we will able to see the numbers, too, as Laurie said.

6. When can we start on rilutek, and why haven't we started yet?


Right away.

7. When can we start radicava?


More bad news. The dr said my dad is outside of the window to be recommended for radicava, and although they wrote the prescription, the insurance is most likely not going to cover it. Also, they said they only have a few patients out of all of their als clinic currently approved for radicava. So it sounded like we shouldn't really hope to be approved!!! What should we do then?

8. Can we do genetic testing to see if it is genetic?


No point.

9. Do you recommend taking supplements?

No, his dr doesn't see a point in supplements. Sigh...


10. Are there drug trials that we can join?


Not at Northwestern currently. But they recommended the same website as Nikki.


Other bad news, is that my dad got so tired by the clinic visit, that he got drastically worse in the last couple of days. He can't eat without choking and coughing anymore, although he was doing so much better with all of the suggestions on how to eat. I am so worried about aspiration.


I am very concerned about the future clinic visits, too. It is a 3 hour drive from where we live, and doing it all in one days seems too much for my dad now. Maybe we should do it in several days, with an overnight hotel stay, so that my dad gets some sleep in between the driving and the many hours it takes at the clinic. Ughh i really don't like this problem, given that we have to go back there at least twice in the next month or so. My dad really hates hotels, so he is guaranteed not feel comfortable either way.


Emotionally, it was tough as well. Thank goodness my brother did all of the driving, but I still wasn't prepared to hear the news about my dad's progression. I thought he plateaued.

Anna
 

Nikki J

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Hi Anna

Thank you for the thorough update. I am sorry the news was bad and the trip difficult.

I hope your dad feels a lot better on bipap. He is using a lot of energy to breath so helping that may make a significant difference. The feeding tube also should help him conserve energy and maintain weight. Please ask here if he struggles with the bipap. Laurie and others can help problem solve.

You were given accurate information on radicava. There were 2 trials. The first showed no benefit to the group but seemed like a small subgroup might have benefited. The second trial contained only that subgroup. The criteria for entry into the second trial are the ones many insurances are using and they include normal breathing values.

Clinic days are hard and often long. It sounds like you should be making the next several visits anyway as they are going to get something your dad needs and wants. After that you can reassess. You might ask if they do televisits. You might be able to work with his pcp to meetsone/ most of his needs. It sounds like the local neuro might not be a good choice though?

Staying over before and or after might help too if you can/ if he will. Likely the hospital has arrangements for medical discounts at one or two nearby hotels. Mine does and I found a listing on the Northwestern site https://www.nm.org/locations/northwestern-memorial-hospital#local-services. I note a couple of the hotels are suite hotels. Perhaps your dad would prefer them to a regular room?
 

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Anna,
I'm not sure of the value of the next visit -- i.e. what the pulmonologist would do/see a month later. So I'd ask about that. He doesn't need to see him that soon to schedule the PEG (RIG, I trust). For you and others where the PALS is a long drive from the clinic, it is a burden that many clinicians can't fully appreciate, but you have the right to ask "is a live visit necessary? why?" and you can assert yourself in terms of video calls (paid or unpaid, but telemedicine is increasingly reimbursable), your recording his breathing while asleep on your phone, etc. And as you say, when he gets the BiPAP, the pulmo will have that data.

What you see on the screen of the Trilogy datawise is up to you. When you get it, let us know and I can walk you through setting it up so you can see what's happening, and tweaking the settings accordingly and based on how he perceives his breathing as well.
 

worrieddaughter77

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Hi Laurie and Nikki. Thank you so much for your comments! i will definitely ask on advice on how to set up the bipap once it arrives. i hope it arrives sooner rather than later!

i will fight with the dr to see if we can do the next visit remotely. that is an excellent suggestion, Laurie. I'll update you on that.
 
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