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Lung Transplant With Esophogeal Dysmotility


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#1 delkins33

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Posted 27 January 2012 - 04:12 PM

Hi--
I'm looking for any stories about lung transplants conducted on people with "scleroderma esophogus"--limited motiliity and signficant acid reflux. After several rejections from various programs due to this complication, (Columbia, U of P, Johns Hopkins), University of Maryland has accepted me. Now I want to understand how likely they are to be able to manage this complication successfully---on top of all of the other lung transplant risk factors. Thanks in advance for your stories.
Debbie

#2 judyt

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Posted 27 January 2012 - 07:34 PM

Hi Delkins,

Sorry to read that you have such significant lung disease. Personally, I don't have lung involvement but I do have a badly affected esophagus. Hopefully somebody will chime in with some thing for you to discussl

Warm hugs and best wishes from the upside down side of the world.
JudyT

#3 Joelf

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Posted 28 January 2012 - 12:58 AM

Hi Debbie,

I am pleased to hear that you've been accepted for a lung transplant.

I have lung involvement but thankfully it was not so serious as to require a transplant. I don't have the gut involvement either. However, I have included a link for you to our page on lung transplants and also the personal story of a Scleroderma sufferer who received a transplant.

I know Shelley's husband underwent a lung transplant and I expect that she will be able to give you some advice.

Best wishes,

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#4 Shelley Ensz

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Posted 30 January 2012 - 05:58 PM

Hi Debbie,

My husband had a single lung transplant (for Alpha-1 Antitrypsin deficiency, not scleroderma) two years ago. Before he was listed, they required him to have a Nissen fundoplication surgery because there are bad outcomes in lung transplant patients who still have heartburn. Since the Nissen he has never had even a single episode of reflux. Now he is fit as a fiddle, looks decades younger than his near-70 age and lives an active life without any oxygen.

It's my understanding that the heartburn absolutely must be completely well managed prior to the transplant, whether that is by lifestyle changes, diet, surgery or meds -- somehow or other, it must be dealt with successfully or there will very likely be bad outcomes with the transplant. If you are eligible for a Nissen, you'd want to get it right away because you don't want to be having any other surgeries right after transplant.

Here are some extra warm hugs, just for you, as you go through what I know is a very challenging time. My heart goes out to you!

:emoticons-group-hug:
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#5 delkins33

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Posted 31 January 2012 - 02:45 PM

Thanks for the input everyone. Just to clarify if that influences anyones comments---my issue is not so much reflux, as it is dysmotility---eg. muscles don't push food down. It's weird because I am not symptomatic---I do eat normally and process food. So fundiplication is not indicated because they're afraid it will not only keep acid from coming up, but also from keeping food from going in.

#6 Shelley Ensz

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Posted 01 February 2012 - 10:36 AM

Hi Debbie,

Well, perhaps we're talking about a different procedure? A Nissen doesn't prevent food from going into the stomach. Sometimes they wrap it too much, which can cause difficulty swallowing, but that is correctable by another procedure, and could possibly be avoided by having a very experienced surgeon in the first place. However, I may be wrong (I often am) but my husband has never had any trouble eating (or swallowing pills) since his surgery. (It's not like gastric bypass, which makes the stomach smaller.)

My husband went through the transplant qualification process twice, since he was inactive on the list for over 5 years and then decided to reactivate, but as it had been so long, they had to make sure he was still qualified. On the second round of qualification testing, they added a gastric emptying test, which tests the amount of time that food stays in the stomach. They said that was important due to all the pills that are taken after transplant (to make sure they go through in a timely manner).

Since you've already been approved by Maryland, it would seem that they are totally satisfied with your status and test results, wouldn't it? But, I'm assuming by "accepted" you mean that you've been entirely accepted and are on the UNOS list (not just accepted to have an appointment in their transplant clinic.) Right?
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#7 delkins33

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Posted 01 February 2012 - 11:03 AM

Thanks Shelley--

Yes I've been approved and U of MD, but haven't listed on Unos yet. We're in discussion about if this is the right time or should I wait, as listing will require that I move to Maryland from NYC to be closer to the facility. Yes, they've said they'd could do it, but as 3 institutions have said no, I'm just looking for confirmation/results of lung transplants performed on people with similar issues.

#8 Shelley Ensz

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Posted 01 February 2012 - 02:51 PM

Hi Debbie,

Oh my goodness, what a major thing with the moving!

We were extremely lucky that Gene was approved by a hometown center, and that we didn't have all that discombobulation on top of it all. You do need to be entirely ready to be listed nowdays, as some people get a match immediately (like, even the next day in some extreme cases.) When Gene reactivated, he was called 3 weeks later but at the very last second his surgeon refused the lung for him. He was called again about 5 months later. Everything went smooth as can be expected, and he was home from the hospital in 8 days. But, my goodness, it took an awful lot of home caregiving and almost daily trips back to the transplant center at first.

So, when you are looking at relocating, try to get as absolutely close to the transplant center as possible. Both you and your caregiver will be very glad you did.

I'd say, if the center accepts you for listing, just plain go for it. You really can't compare yourself to others in the same condition because everyone with scleroderma has a different set of symptoms or complications. The important thing to consider will be your own health, and quality of life, before and after transplant.

One huge consideration with scleroderma is that the longer you wait, the more likelihood something might develop that would entirely prohibit you from qualifying for a transplant. We took a huge risk, delaying my husband's transplant so long. However, when he decided to be reactivated, it was an absolute, unwavering decision -- he was 100% dedicated to having it, even being very well aware that not everyone even lives through the operation. We did wonder over the years when he/we would "know" it was time.

Of special concern to us was that when I was pregnant, I was concerned about how I would "know" that delivery was imminent. Everyone responded by saying, oh, don't worry, you'll KNOW. A lot of good that did! When I was in labor, my mother told me I was in labor, and my doctor told my mother that I was in labor, and I still didn't believe it. So I had a nice dinner and we went to the hospital only because the doctor insisted, and I was already a few weeks overdue.

I swear, until my baby was born, I was still sure it was just false labor and they would send me home any minute!

So I don't put much stock in the whole knowing thing. But I do put a tremendous amount of stock in being ready for opportunity when it presents itself.

I hope that we can all help you be ready, and ready to do anything it takes to have a successfull transplant, when your opportunity knocks!
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#9 Sweet

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Posted 02 February 2012 - 11:54 AM

No further input, but I want to wish you the best!
Warm and gentle hugs,

Pamela
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#10 Shelley Ensz

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Posted 10 February 2012 - 02:43 PM

Hi Deb,

Have you heard anything more, yet?
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#11 delkins33

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Posted 15 February 2012 - 10:55 AM

Shelley--

Thanks for asking. I just took lung function and 6 minute walk test and my results are back to where they were before my recent flare-up---and pretty consistent with last 2 years. U of Md is going to re-input into their model and then make a recommendation about when to list based on that. So we'll see. My goal is longevity, and not so much quality of life. My quality of life right now is good, all things considered, and I wouldn't risk a lung transplant if I could stay like this. However, the statics, as limited as relevant data may be, seem to indicate that based on my ILD diagnosis, I could have 3-5 more years. With a lung transplant the risk of death in the first year is about 20%, and in the first 5-6 years about 50%. I don't know how the esophagelal issues impact those statistics. And you're right about getting worse in terms of esophagus, PAH and flare ups making it either harder for a successful lung transplant or dis-qualfying me. Thwarted if you do, and thwarted if you dont. So that's the decision I'm trying to make---it'll take a few months I think to sort this out. In the end, my son is now 8 and I really, really, really want to see him at mininum graduate from high school.

Debbie

#12 Joelf

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Posted 15 February 2012 - 12:47 PM

Hi Debbie,

I'm sending you my very best wishes for whichever decision you choose to make. :emoticons-i-care:

:emoticons-group-hug:

Jo Frowde
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#13 Shelley Ensz

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Posted 16 February 2012 - 01:30 PM

Hi Debbie,

I'll be eager to hear what they recommend now that you are in a stable condition again. Also consider the survival statistics for the center where you are going to have your lung transplant, as they can vary a lot by institution. I'm sure part of that has to do with the type of patients they select, and another part in how they manage the outcomes and how diligent the patients are to their own health and recovery.

Your odds of survival will be much better if you don't leave anything to chance. For example, if you have a great source for medications or that expense totally covered and if you always take your meds on time, with no excuses at all.

We got Gene a pill alarm with 7 sections for pills and I sewed a leather carry case to wear on his belt. This way his meds are with him all day long, and he has never missed a single dose of his anti-rejection drugs. I also made a checklist for all the things he has to do each day (even down to number of glasses of water, and weighing, temp, blood pressure, yogurt, etc.) which he faithfully fills out.

If you are disorganized, often miss pills, don't follow doctor's instructions or miss doctor's appointments on a whim, etc. then you would want to avoid transplant and take your chances as is. It takes a lifetime commitment, that you will do whatever it takes to stay as healthy as possible, post-transplant, and it is by far more doctoring post-transplant than you have ever encountered with anything else.

My general impression is that people who are careless or disorganized, or who have a negative attitude, will have huge challenges staying alive post-transplant. But since you have a goal to live, you'd have the odds in your favor should you happen to also be very organized, good with details, and dedicated.

It also helps to be a generally lucky person in general because some of the complicatons are just plain out of everyone's control. It takes a will of steel, as it is really a face-off between life and death. Once the decision is made, you just have to be tough as steel yet still roll with all the punches gracefully, never looking back and never second-guessing.

When you look at the transplant statistics, it is important to compare them to the statistics involved in not having a transplant, too. We have no doubt whatsoever that Gene would have been gone within months or a year at the outside, the rate he was going downhill and getting pneumonia after pneumonia near the end. So we count every day as a bonus day, regardless of all the complications he initially suffered post-transplant. He became insulin-dependent diabetic overnight from the massive dose of prednisone at transplant, and 4 months later (after outstanding care on our part) he was entirely off insulin. He had to take heparin shots for awhile. He had many infections, back to back, just one of which required over $2,000/month to treat. Fortunately for us, he has a great medication resources.

Anyway, he overcame all of the complications and now is past 2 years without any infection at all for months, and no sign of rejection at all, so far. He has been off oxygen since transplant. He can even run a little bit and not be breathless -- and he is almost 70 years old, but looks and acts 20 years younger since transplant recovery. Of course, he's not also dealing with scleroderma, but he does have partial Alpha-1Antitrypsin Deficiency (A1A).

My point is, you will hear plenty of stories about people who did not survive transplant, or the first year, or the first five years. But if you go for it anyway, you need to keep your eye on the prize. There are people who make it a lot longer than that, and I suspect partially it is because they put the odds in their favor by being totally faithful to the recovery program -- as though their very life depended on it, which it, indeed, does.

Only you can tell if you have what it takes. And we will all be behind you 100% no matter what decision you make.

:emoticons-group-hug:
Warm Hugs,

Shelley Ensz
Founder and President
International Scleroderma Network (ISN)
Hotline and Donations: 1-800-564-7099

The most important thing in the world to know about scleroderma is sclero.org.

#14 Amanda Thorpe

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Posted 16 February 2012 - 02:20 PM

Hello Debbie

What a decision you have to make! How are you getting on with that?

Hello Shelley

You and Gene make an amazing combo for sure!

Take care.
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#15 delkins33

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Posted 27 February 2012 - 09:31 AM

Hi all--

Thanks for your continued input. Based on my current lung function test which brings me back to or slightly better than I was before my New Year' flare which landed me in the hospital for 2 weeks, U of MD is not advising/comfortable with waiting to be on the active transplant list. So don't need to move to MD now, but am still considering it. I'll have to go there eventually, so better to go when I'm feeling better and give my son more time to adjust at his pace. BTW--to combat flare up I've gone on prednisone for the first time (now 10mg) and have had treatments with Rituximab.

Debbie

#16 Amanda Thorpe

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Posted 27 February 2012 - 01:25 PM

Hello Debbie

That's great news! As you say you have to move anyway but at least you can do it whilst you feel better!

Take care.
Amanda Thorpe
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