Where are we?

[Woof]

Hi, this is my first post to the forum. My mother has ALS First symptoms presented sep 2010, as slurred speech. After several tests, she was diagnosed with ALS in January 2011. Since then her speech has gone, her respitory capacity is down, she had difficulty swallowing and has weakness in limbs. She cannot walk much and must lately be assisted with pretty much all day-to-day activities (showering, putting on clothes). She recently had a RIG (like a PEG) put in and receives 95% of her calories through that. Lately, in the last few weeks, she seems much more tired, and seems to need to spend almost every second day in bed to recover from any exertions the previous day. She has also started to use IBAP for a few hours at a time (cannot seem to get into the habit of using it at night).

Our neuro, doctors, nurses etc dont seem to be able to (or want to) tell us how far along her final journey she is, and I cant blame them for that! But it is a question that plagues me. Does anyone have any thoughts/opinions on the rate of progression? From my own research it seems like a relatively rapid progression, and we are putting that down to the bulbar nature of the initial onset.

Finally, she is absolutely emotionally tormented by this. It is not unusual for her to be upset/teary several times each day. There is not really a sense that she has accepted her fate any more now than when she was diagnosed 8 months ago. Of course, I dont blame her for this, I dont think I would accept it if it was me either. But it breaks my heart that she is so tormented emotionally. But any advice/guidance on how to help her come to terms with her diagnosis?

 

[Ms. Pie]

Everyone is different and no one can determine any time line with regard to progression. It's not a steady progression. You lose and then stay steady for awhile and then lose more and so on. I wish I could help. It's a tough row to hoe. God's blessings on you and God's comfort to you and her.

 

[Katie C]

The doctors are being honest... they really can't tell you because each journey is so different. It does sound like she might be having some CO2 buildup so the more she can use her BiPap the better she's going to feel. Also, you don't say if your mom is on any anti-depressive medication. If not, it sounds like it might help her... talk to her neuro.

 

[notme]

One other thing that might help is if she has some way to communicate. Does she have a computer system of some sort so she can let her needs/wants be known?

For me, it would be very depressing to not be able to speak and let my thoughts be known. THere is some great free software out there for windows based systems.

ALSA loaner closets might even have some of the fancier machines and gadgets.

Does she have a mask or pillows? Some do better with the pillows for the bipap--and when the pressure goes up gradually, verses full strength.

 

[trfogey]

Our neuro, doctors, nurses etc dont seem to be able to (or want to) tell us how far along her final journey she is, and I cant blame them for that! But it is a question that plagues me. Does anyone have any thoughts/opinions on the rate of progression? From my own research it seems like a relatively rapid progression, and we are putting that down to the bulbar nature of the initial onset.

Why does it "plague" you? If you've really done the research that you claim to have, you have some general ideas about the answers to your questions, but you'll also have learned about things like plateaus. You'll have learned that some people -- Stephen Hawking, for example -- live for a decade or more in a profoundly disabled condition, with little more medical intervention than what's already been done for your mother.

The medical people know this and accept this ambiguity because they have a longer view over a larger population of patients. Those of us who've had the disease for a while accept it because we've met long-lived PALS and because we've outlived people who were diagnosed at the same time or even after we were diagnosed.

Demanding certainty in a situation that has no certainty is the act of someone seeking to reconstruct reality, not someone who has accepted it.

Finally, she is absolutely emotionally tormented by this. It is not unusual for her to be upset/teary several times each day. There is not really a sense that she has accepted her fate any more now than when she was diagnosed 8 months ago. Of course, I dont blame her for this, I dont think I would accept it if it was me either. But it breaks my heart that she is so tormented emotionally. But any advice/guidance on how to help her come to terms with her diagnosis?

What does she have to do to show you that she has "accepted her fate?" And what fate would that be with what implications for what she "should" be doing now? If you would do the same if you were in her position, what is she doing wrong? How do you think she ought to be acting?

 

[Atsugi]

Welcome Woof, to the club no one wants to join.

Your PALS and mine have come to nearly the same spot in nearly the same amount of time, but from opposite directions.

My PALS tripped inexplicably in September, saw a neurologist for testing in November and had no symptoms in December when she was diagnosed by EMG. She used a cane in January, a walker in Feb, a wheelchair in March, lost use of her arms in April, could not lift any fingers in May, started having swallowing problems in June, needed to be fed during July, and has been to the ER twice in August for shortness of breath. She can't speak hardly at all.

But my PALS is, believe it or not, a medical doctor. She knew how to read the tests herself, and knew exactly what they meant. That helped her "accept" things instantly.

Maybe that's a difference. When the average person gets the diagnosis, I suppose they might think "well, doctors are sometimes wrong" or "science is inexact." Some people don't get properly diagnosed for months or even years. But once you've had an EMG/NCV study showing denervation of the muscles, that's definite.

For the question, "how long do I have," you can take a clue from respiratory function. Get a Pulmonary Function Test every couple of months and you'll see the decline in lung efficiency very well.

My PALS refuses tubes and signed a DNR. We've hired hospice in-home, which, so far, appears to be a nurse on call and a box of morphine in the fridge.

Definitely get the anti-depressants or anti-anxiety meds. For both of you.

Ireland, to my knowledge, has a great high-tech industry, so you guys probably have the best in eye-gaze-controlled computers. Our Tobii eye-following device lets my PALS control the house, door, lights, TV, and radio. Soon we'll have the email, book reader, internet browser and and voice speaker ready for her.

Good luck. God bless.

 

[brooksea]

Man, what happened to my post? I posted right after Katie (as far as I know, since someone else's post could have disappeared)!

 

[Glorious]

I guess you and I are both new to this. Your first post, my first response. My Mom was diagnosed October 2010. Her speech has been affected the most and she is losing weight, the past 2 months especially. I can understand why you say the question "plagues you". I worry everyday and I want answers! I want to know exactly how this disease is going to go and in how much time! I then remember that I am not that powerful and no-one can answer that for me. I feel like if I know the answers then maybe I can control the situation or gain control over my own emotions. My suggestion to you is the Serenity prayer...God grant me the Serenity to accept the things I cannot change, the Courage to change the things I can and the Wisdom to know the difference.

My mom is also taking an antidepressant and it helps a lot. Before she was diagnosed she went on it because she was having crying jags several times a day over nothing. Apparently that's one of the symptoms.

 

[Woof]

Thanks a lot for your responses...

in answer to some of the questions posed...

you guys probably have the best in eye-gaze-controlled computers. Our Tobii eye-following device lets my PALS control the house, door, lights, TV, and radio. Soon we'll have the email, book reader, internet browser and and voice speaker ready for her.

Thanks for the tips on the eye gaze, I think that is the next step in terms of communication, and unfortunately we need to look at that sooner than planned. She currently uses an iPad with "proloquo2go" software, but weakness in arms and lack of dexterity makes that difficult to use.

Why does it "plague" you? If you've really done the research that you claim to have, you have some general ideas about the answers to your questions, but you'll also have learned about things like plateaus. You'll have learned that some people -- Stephen Hawking, for example -- live for a decade or more in a profoundly disabled condition, with little more medical intervention than what's already been done for your mother.

The medical people know this and accept this ambiguity because they have a longer view over a larger population of patients. Those of us who've had the disease for a while accept it because we've met long-lived PALS and because we've outlived people who were diagnosed at the same time or even after we were diagnosed.

Demanding certainty in a situation that has no certainty is the act of someone seeking to reconstruct reality, not someone who has accepted it.

It plagues me because I want to do anything I possibly can to help. That includes planning which is difficult to do without knowing timelines. I do understand and accept that no-one can give me that information. I guess, I was hoping someone would jump in here and tell me that their PALS had a similar rate of progression through similar symtpoms and went on to plateau. If there are cases like that, I want to learn about them, because right now it seems the progression is faster than typical (by "typical" I mean from researching on the internet, these forums, info from neuro/docs/nurses etc).

What does she have to do to show you that she has "accepted her fate?" And what fate would that be with what implications for what she "should" be doing now? If you would do the same if you were in her position, what is she doing wrong? How do you think she ought to be acting?

Of course I dont think she is doing anything wrong and I'm certainly not demanding (or even expecting) that she "accept her fate". I was simply asking for any advice/guidance on how to help her deal with such complex/difficult emotions.

Does she have a mask or pillows? Some do better with the pillows for the bipap--and when the pressure goes up gradually, verses full strength.

notme> she currently uses a mask. What do you mean by pillows?

 

[BarryG]

While it is not always the case, often the older the PALS the faster the progression.

 

[trfogey]

Thanks for the tips on the eye gaze, I think that is the next step in terms of communication, and unfortunately we need to look at that sooner than planned. She currently uses an iPad with "proloquo2go" software, but weakness in arms and lack of dexterity makes that difficult to use.

You may want to look into a low-tech communication option as well, if for nothing more than a backup for the higher tech methods.

SpeakBook | nonprofit communication tools

It plagues me because I want to do anything I possibly can to help. That includes planning which is difficult to do without knowing timelines. I do understand and accept that no-one can give me that information. I guess, I was hoping someone would jump in here and tell me that their PALS had a similar rate of progression through similar symtpoms and went on to plateau. If there are cases like that, I want to learn about them, because right now it seems the progression is faster than typical (by "typical" I mean from researching on the internet, these forums, info from neuro/docs/nurses etc).

I understand the need for some planning, but this disease is far too unpredictable to get too caught up in getting everything done at the perfect moment. No matter how well you think you've done, you're going to be early with some things and late with others. If the doctors are telling you that certain equipment needs to be obtained and that the decisions for certain interventions need to be made soon, then concentrate on getting those things done without drawing any conclusions as to whether things are going "typically". You have a one-patient universe, and things aren't typical or atypical -- they simply are what they are.

Of course I dont think she is doing anything wrong and I'm certainly not demanding (or even expecting) that she "accept her fate". I was simply asking for any advice/guidance on how to help her deal with such complex/difficult emotions.

"Accept her fate" was the term you used in your original post, not mine, and it certainly sounded as if you were suggesting that she should have made some type of "progress" in that direction, which is why that I asked the questions that I did. Is there a particular need for her to deal with her emotions in a different way than she is doing at present? And whose need is that? Has your mother expressed a need for help in dealing with her emotions?

On the odd chance that there is an ALS-related cause for some of the teary episodes your mother has, you may want to do some reading here on the site about emotional lability and pseudobulbar affect, which can be treated with medicines. There's also the possibility that your mother has developed a clinical depression that also can be treated with medicines. Consult her ALS specialist for advice on these problems -- they are very common problems that many PALS face at various times in their journey with ALS.

notme> she currently uses a mask. What do you mean by pillows?

A nasal pillows mask is one in which a pair of nozzles are inserted into the patient's nostrils. The nozzles are made of a pliable material that allows them to conform somewhat to the patient's nose and seal against pressure leakage.

 

[helpingpals]

As everyone has told you there is no way to no for certain when the end will come. How is her FVC scores? (breathing test) What her FRS (functional rating scale). While neither one of them or nor anything else will give you a definitive timeline it will give you an idea at her rate of decline. I wish you all the best.

 

[Atsugi]

I agree that the questions "plague" a person. After every doctor appointment, I took the doc aside later and asked, "how long? I need to plan." They gave their best guesses.

But the reality turned out very different. Now it's day-by-day. Although you need to plan for the end, mostly, you just want to ACT for the present. Make it the best it can be. Make smiles.

 

[dian_na]

My mom began much like you have wrote. She broke down when being told as your mom has, it was sincerely difficult to comfort her because there isn't any "right" thing to say or do at that point. You need to recognize if she had this symptom before she was diagnosed some of the crying could very well be emotional libility, meaning she has no control of it and she may began laughing too. I have been taking care of mom for a long time now and I will admit I am so guilty in that I never have become accustomed to her crying. It hurts to see her cry, I jus hate it however when the laughter comes you gotta laugh too you can't help it. Once this old man was driving a vehicle next to ours at stop light I guess mom and him looked to each other at the same time and she smiled at him, and he smiled back real big, Mom just busted out laughin non stop, she was belly laughing so hard ......... I looked over at the guy who smiled again, the poor ole man had not a tooth in his mouth he looked like one of those comical birthday cards with all the wrinkles and no teeth. He was being friendly, he couldn't help how he looked, my mom laughed in his face nonstop, I was like " mom, stop gzzzz, then I would start laughing because it did look funny, but gzz she laughed the momemnt he smiled toothless at her. It was k inda embarrassing for me but then after a moment i thought hey, she's my mom so I said, "you laughing at that man, how yo know he isn't going to be your next husband?" and she laughed so hard I thought she'd pee her pants. As for the toothless man I knew I'd never see him again.
I had thngs in the house before they were needed. The eyegaze is a good way to go, so she has time to adjust to it before having to use it, you can still put mouse for her to use until she can't.
If she's having problems walking, order the AFO braces, uf those are difficult and she is having problems get a walker and have it sitting there waiting somewhere. It will save her a lot of energy to use it. At the point of needing the walker get her perm mobile ordered, it can take months to get those.
If she is choking get began talking about the PEG tube now. The sooner she gets it the better. Theres going to be a lot go on and when you get one thing accomplished 10 other things can and will dump on your lap. Just be prepared to react when you need to.

 

[dian_na]

One more thing, in the middle of doing what you have to do ...........

Stop worrying about the potholes in the road and celebrate the journey!