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NothingButLove

Distinguished member
Joined
Dec 25, 2016
Messages
106
Reason
Lost a loved one
Diagnosis
11/2016
Country
US
State
CA
City
Anaheim
My wife’s doctor doesn’t want to place a PEG tube. Today the doctor told me I might want to get hospice involved. I’m fully wrecked crying my heart out. Here’s how things happened…

My wife started showing symptoms 1/16 and was diagnosed 11/16. She lost both her legs 3/17. Things seemed to slow down but in July, things started to visibly progress. She has lost some function in her right arm and her left arm is twitching. But in general, things appeared normal. She has never complained about her breathing.

Yesterday was our “every four month” visit to the clinic. Apparently, things are not OK. My wife’s FVC is 44% and she lost six pounds. She is 5’ 2” and skinny and has never been over 115 lbs her whole life. She weighed 90 lbs at her last visit to the clinic and weighed in at 84 lbs yesterday. The doctor prescribed a Trilogy, wrote a prescription and left the room without discussing a feeding tube.

As you may know, you see a bunch of people at the clinic. When the speech therapist came in, I said, “I forgot to mention it to the doctor but I want to talk to her about a feeding tube.” She said she would let the doctor know. After we saw everyone we had already been there for 4 hours. I still wanted to talk to the doctor but my wife wanted to leave so we left.

No one ever explained FVC to me and I didn’t understand its importance. When I got home from the clinic, I Google’d it. That’s when I saw that doctors don’t like to place PEGs when FVC is below 50% and there has been weight loss. I immediately wrote the doctor an email had said, “Can my wife still get a PEG with her current weight / FVC?” Here is what she said (paraphrasing):

“I think a PEG would do more harm than good at this point. I didn’t want to bring it up during your visit because I could see you were in shock with the news of the progression. I’ve seen hereditary (C9orf72) cases like your wife’s and they can progress very quick. You might consider getting hospice involved.”

The doctor is very nice and didn’t explain it so bluntly but that’s the meat and potatoes of what she said. Of course, I had a complete mental meltdown after this conversation with the doctor. So here is what I need to know:

Should I push for a feeding tube? Should I follow what the doctor says? Do I have a choice?

It seems like we’re going from “everything is ok and we’re having fun” to “you’re going to die soon”. From the sound of it, it seems like we’ll be missing the middle part of the ALS journey. I’m super sad and super scared right now. What should I do? (besides calm down)
 
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Rob, that 50% threshold is a myth. You're reading Web sites that haven't been updated in a while or are cutting and pasting from last year's reworking of the year's before literature.

Remember, it's not your doc who would place the tube anyway. So let's not worry too much about what she thinks about tubes, because s/he may not know nearly as much about tubes as she should.

Some clinics refer only to gastroenterologists for feeding tubes, meaning an operating room, and that's where the FVC issues kick in more because of risk aversion. Here is an example of how things in interventional radiology, where the procedure is done in a radiology suite, can be different. Search on RIG and PEG and you'll see the difference in the procedure. The abstract I linked to shows success with much lower FVCs esp. staying on the Trilogy during the procedure if needed, which since she just got the rx, may not be the case. Far more important is the position of her stomach.

Lately, even the OR has been more accepting of low FVCs for PEG insertion. You can see from these abstracts 44% is hardly the end of the discussion (look at the tables showing the FVCs).

Has your wife been eating less because of choking, swallowing, chewing issues? I'm not clear on why the weight loss was unexpected. At any rate, there are 2 issues here. One is, does her FVC rule out a feeding tube? On the basis of studies like these (there are more) and many experiences you'll hear about here, I'd say no. But she needs an experienced team on this. And with rapid progression, since anatomy is important, I would get a consult soon if question 2 is a go. 84 lbs and 5'2" is pretty skinny and hypermetabolism with malnutrition is going to result in more muscle loss.

Question 2 is, does she want a feeding tube? You and she should talk about this, and we have threads about this too. If your clinic's institution doesn't place complex feeding tubes in radiology and/or under the gastro service, you may need to do some networking and explore options in your area. Are you going to Cedars? UCLA? Where? Remember, you don't have to get the tube where your clinic is.

As for the doc's experience, not all C9 ALS progresses at the same rate any more than sporadic ALS does. Don't be scared to the extent that everything stops. The tube is still most likely doable. The doc could be way off. If her breathing has accommodated to her FVC thus far, it's a good time for the Trilogy and her breathing has not decompensated as yet. Maybe things are speeding up past the middle stage, but we don't know that.

I wouldn't bring in hospice until the tube is resolved, as they won't pay for it and the reimbursement issues will become a time sink. And again, hospice is your choice, not the clinic's.

So the answers to your questions are, if your wife wants it, do it; you never have to follow a doc onto a path you don't want, and you always have choices, Rob, down to and including the end of the road. Talk with your wife. Make a plan.

Best,
Laurie
 
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Laurie,

I think a couple things contributed to my wife's weight loss. First, she got sick about three weeks ago. It was a cough with lots of phlegm. It lasted a few days and during that time she didn't eat much. The second thing is choking. Recently, it seems like if she chokes on the first bite, she doesn't feel like eating the rest of the meal. I realize that future meals need to be more ground up an harder to choke on.

My wife is going to UCI ALS and NMD clinic.

Do we want a feeding tube? Well, we don't want to starve to death. I guess it depends on what it gets us. If everything is successful, would it help my wife live another year? Then again, what would that year be like? I've heard the forum user "Atsugi" use the term "lock out". I believe his wife had C9 also. I don't think either of us want to go there.

Thank you so much for all the information. Much to think about... For now, I need some sleep.

Rob
 
Hi. I am so sorry. My family is c9 as you know and much of what you say reminds me of my sister She was the same size as your wife and, in her case, was advised the feeding tube long before she consented. By the time she had it she was about the same weight as your wife is now and I am pretty sure her breathing was worse. She had a RIG and it was very difficult to recover and to tolerate food again but she got through it and lived 2 more years- hard years yes but never locked in ( used eye gaze) and still had quality of life ( died too soon from a cold)

My sister was more advanced than your wife sounds when she had the tube. She was glad she had it.

As Laurie says c9 progression varies widely and I actually think Mike's experience was somewhat unusual. They used to think c9 was faster than average ALS but there is research that only males with limb onset c9 are faster and they were skewing statistics. I can point to exceptions in all directions of course
 
Rob, my PALS had a feeding tube put in last September. His FVC was lower than your wifes. The choice is yours and your wifes, not your doctors. Other families have had doctors that didn't recommend the feeding tube but have had it done anyways.

I would say though that I wouldn't wait any longer to make those arrangements because of you wife losing weight and how thin she is. Some definitely have a hard time tolerating nutrition especially if they have lost alot of weigh already.
 
Rob,
My husband had a feeding tube placed long after recommended and his FVC was 28%. We are glad he got it and it made mess and water so much easier and less stressful. For a few months after, his breathing improved. We bought about 10 months with the PEG, and they were difficult in many ways but he had a good quality of life right up until the end and many good memories were created during that time. It was worth it.
Tracy
 
Rob,

My wife (Darcey) had a peg put in when she was at about 40% FVC. She only allowed it to be put in so that I could give her necessary meds should she no longer be able to swallow. She stated, then, that she would not use it for nourishment or liquids… other than for meds. To this day, she continues to support that early decision. Based upon her decline in function… and particularly FVC which had dropped to 15% on her last clinic visit… her doctor suggested that he’d approve hospice if/when Darcey felt it was time. It was March of 2015 when the doc stated that he believed Darcey to be within her last 6 months of life. We’ve seen doctors from the Mayo Clinic, Johns Hopkins and several local doctors. This last doctor was as knowledgeable and compassionate as any of them. Yet even as he gave that 6-month notice, he also disclaimed it as not being an absolute… and that Darcey could certainly prove the indications as being incorrect. A couple of days ago, now 2 years and 5 months since that “6-month pronouncement”, I suggested to Darcey that she might want to begin thinking about her Christmas list. It would seem that the only absolute to this disease is that life will at some point end. But, for that matter, so will mine…


My best to you and your wife, good sir!


Jim
 
Rob,

I can't add much to all the posts from experienced PALS and CALS but I would suggest, in the meantime, that you try some very high calorie Vitamix shakes. You can make them the consistency that your wife can manage and make them in the flavors she loves. Nut butters, coconut oil, avocados, bananas, and ice cream can be mixed to equal 1,500 calories with no problem.

You're both in my thoughts.
 
Rob,

My husband Brian had his PEG placed when he was trached and vented, his breathing was that bad. He could still eat at that time, but I said let's do one and done with surgery. That was 7 years ago. The last 2 he has been completely dependent on the tube for food, water, meds. He's also been mostly bed bound those same two years. The other 5 he still went and did things. He also talked around the trach until a year and a half ago. All this after his Dr said we needed to decide on the trach or not quickly if we wanted it because he could go at any time. I did agree with that assessment and had he not chosen the vent he would not be here to worry about the PEG.

All that said, your wife needs to decide what she wants. If she wants the tube find someone to do it. Brian is 6'2 and weighs about 120-125 so yes very thin and no muscle, but the rest of his organs are seemingly very strong.

Hugs as you make your decisions

Sue
 
My wife had her feeding tube placed as she could not swallow much at all and lost 20 lbs in two months, she only weighed 125. Our ALS doc was fully supportive and made all the arrangements. The tube was placed by a Intervention radiologist so she was not totally sedated. That was done two years ago and since the tube she everything, food, vitamins, meds and water has gone through the tube. It has kept her alive. Her FVC was 40% at the time, now less than 20.

It will take a few weeks to get comfortable with the process but after that it goes pretty well. She uses isosource for food, meds are all ground up and dissolved in either water or juice.

If you are up for the process, go for it.
 
This thread and others have convinced us that a feeding tube is worth it and the right choice. I will specifically ask our doctor for a RIG. I'm kind of pissed at the doctor for not bringing up the feeding tube earlier. I feel like she was making a choice for us and not the choice we would make.

Nikki J - Thanks for sharing the story about your sister who was the same size and weight of my wife. The fact that she lived two more years really convinced us to move forward with the RIG. Was she on a vent?

lgelb, soonerwife, codyclan, JimInVA, KimT, Narrowminded and ntd19 - Thank you all for contributing to this thread. It has made me feel so much better. On Tuesday when the doctor said she advised against the feed tube and suggested I might want to get hospice involved, I completely lost it. I started crying in a way completely different: loud and uncontrolled. I've shed a ton of tears since this whole thing started but this was different. I thought it was "game over". I felt completely powerless when talking to my wife's doctor. Who am I to disagree with a doctor who is in expert in neurology/ALS? This forum has given us the information we needed to make a choice. Thank you all so much. I feel much better.

I'll let you all know how it goes...
 
No vent. Nighttime bipap. I do think she plateaued for a while but I don't know her breathing stats. Once it dropped below 50 she would not tell me and she dropped below 50 well before feeding tube

We are all so different even those of us who are c9
 
Update - A RIG has been approved by our insurance company! Now we'll have to do the consult, get the pre-ops and we'll be in business.

What a stressful 2+ weeks. I wrote my wife's doctor at the clinic and said we wanted a feeding tube placed by interventional radiology. At first she said we could do a PEG with the doctor she normally refers her patients to. Then, she changed her mind. She said she was't sure if that particular doctor took our insurance and we wanted a RIG anyway. She gave me some paperwork and said to have my wife's PCP make the request for authorization to my insurance. My wife's PCP was out on medical leave and there was a nurse practitioner filling in. I really didn't have confidence in the nurse practitioner making the request for a couple of reasons:

1. Time is of the essence. FVC will continue to drop.
2. This needed to done through interventional radiology.
3. Ideally, the doctor doing the procedure should have experience with ALS patients.

Somehow, I didn't feel the nurse practitioner could convey these important aspects to the insurance company. So, I actually drove to the insurance company in person and asked to speak to a customer representative. I explained the situation and he said to have the ALS clinic call in a "stat auth". He said they would be able to explain the urgency and the important details of the operation.

I didn't realize our clinic could call in requests. I went back to the clinc and asked the medical assistant if she could call it in for me. I guess they usually don't like dealing with all the different insurance companies because it takes up too much of their time. However, she agreed to call it in. She dragged her feet a bit but it's been approved.

I just had these visions of me begging my wife to eat and her crying and saying that she couldn't. Followed by a morphine haze and the inevitable... Not the end I'm hoping for.

Thanks again to everyone here...

Rob
 
Glad you cleared that hurdle. Best wishes for an easy procedure.
 
Well done Rob - sadly as CALS we end up having to be on top of everything and advocating constantly when we just want someone to take care of the behind the scenes stuff.

With so much out of our control, a win like this is a great thing!
 
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