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Scleroderma & Lung Transplant - UK

Lung Transplant

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5 replies to this topic

#1 hw75

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Posted 18 October 2012 - 02:06 PM

Hi, I am new in this forum and I am looking for some information. My Dad has scleroderma, he is 58 years old and was diagnosed about 13 years ago.

His breathing has been getting steadily worse now for the past 2 years and he is now on oxygen full time. He has Pulmonary Fibrosis and also Pulmonary Hypertension although the PH is undercontrol with medication.

We have just been informed that he will not be assessed for a lung transplant despite his consultant asking for an assessment, we don't yet fully understand the reason for this and are waiting for more information.

What I really wanted to know is if anybody knows of scleroderma patients in the UK who have received lung transplants and how many of these have been done in the UK ?

Any information on Lung Transplant for Scleroderma patients in the UK would be great.

Thanks

#2 Joelf

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Posted 19 October 2012 - 01:36 AM

Hi Hw75,

Welcome to these forums!

I'm sorry to hear that your father has Pulmonary Fibrosis and Pulmonary Hypertension and is in need of a lung transplant. I'm in the UK and have interstitial lung disease as well, so can empathise with him, although I underwent cyclophosphamide treatment which thankfully did improve my lung function and so therefore I'm unable give you any first hand information about lung transplants.

However, I've included a link to our medical page on Lung Transplants and there is an article on the Outcomes of lung transplantation in patients with scleroderma which I hope you'll find informative, although it doesn't specifically relate to the UK.

I see that you're awaiting more information from your father's consultant about the possibility (or not) of a lung transplant for him and I would definately pursue his consultant for more information. Although I received excellent treatment, I have found that it's very important to be pro active with the doctors and consultants I deal with (certainly being informed and knowledgableable about lung disease caused by Scleroderma has helped me to understand the course of treatment.......but I am rather a control freak!! ;) :rolleyes: )

We may have other members who can give you some more advice and now that you've found our forums, do keep posting and let us know how your father is progressing.

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#3 Shelley Ensz

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Posted 22 October 2012 - 08:50 PM

Hi Hw75,

I'm sorry that your father has scleroderma and such bad lung involvement.

My husband had a lung transplant almost 3 years ago (but not due to scleroderma), so I know first hand that an awful lot of qualifications must be met in order for someone to qualify for it. I heard one person say that they evaluate 100 people for every 1 that is finally approved, and even then, many of the folks who are listed die waiting for a match. They only accept people who they can figure have good odds of living for many years after the transplant -- to make the the huge and very costly endeavor worthwhile.

Here, they evaluate total health (every test imaginable, and then some!). They refused to list my husband until he had a Nissen fundoplication surgery because there are bad outcomes in lung transplant patients who have heartburn (reflux). They did a gastric emptying study to make sure his post-transplant medications would be processed in a normal time period. Scleroderma patients typically have heartburn and g.i. motility issues, which may toss them out of the ballpark if the issues cannot be fixed or treated.

They check to see if the patient has always been compliant with doctor's orders, for what good would it do if they got a transplant and then didn't follow the post-transplant protocol, which entails many medications, many times a day, without fail. They make sure the patient can afford all the after-transplant care, including very expensive medications for the remainder of their life. And that they have a sure-fire support system able to help them and support them through the entire process. The patient and family are even evaluated to see if they have a positive attitude, as depression can worsen survival odds and cause a lack of compliance with treatment regimens.

On top of that, the person has to qualify at the top of the heap as being the absolute sickest, to get enough points to get at the top of the list. Someone can be very sick, for a very long time, yet still not be sick enough to even bother trying to qualify for listing -- if there are a great many even sicker people ahead of them in line. When my husband finally asked to put his listing active, he was on 8 liters of oxygen at rest, and up to 15 liters with activity -- and even then, they said his numbers might be "too good" for him to be called. He was called fairly soon, though, so they may tell people things like that just so they get on with their life pre-transplant and aren't sitting on pins and needles all the time, waiting for a call saying that a match has been found.

So it's never any surprise if someone is eliminated from even trying to qualify; the real surprise is when someone actually succeeds in getting screened and qualifies and then somehow manages to live until a match is found, and then survives the whole transplant process.

If you can find out why he didn't qualify to be evaluated, you may find it is something that can be remedied so that he can be reconsidered. Here in the U.S., people can apply at any transplant center and each of the centers have their own criteria for listing. Your father's age is going to be working against him so the sooner you can get to the bottom of it, the better.

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Shelley Ensz
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#4 Amanda Thorpe

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Posted 24 October 2012 - 04:36 PM

Hello Hw75

Welcome to the forums, unfortunately I don't know of anyone who's had a transplant here in the UK. I suggest that you try to get information from the Royal Free hospital as it's a scleroderma centre of excellence and they may know who to refer you to if they can't help you themselves. Their scleroderma clinic is headed up by Prof C Denton. Alternatively have a look at this list of scleroderma specialists throughout the UK and maybe contact the pulmonologists to see if they can help you.

Other than that contact the PALS (Patient Advice and Liaison Service) at the hospital in question and see what they can come up with, especially in respect of getting a detailed answer as to why you father has been turned down.

Finally I found an NHS site about transplants, NHS Blood & Transplant and, as it happens national transplant week has just ended!

I hope some of this helps and take care.
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#5 hw75

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Posted 14 May 2013 - 01:02 AM

Many thanks for the replies to this post and sorry for not replying sooner.

 

We never really did get a complete answer why he was refused for assessment although his consultant did offer to refer him to another centre. The centre he was referred to was Papworth originally and they turned him down solely on the basis he has scleroderma. He does take losec daily although his test for reflux was clear, he has always complied with the doctors recommendations and has no other secondary symptoms from the scleroderma other than his lungs.

 

After long discussions he has now decided he does not wish to pursue the lung transplant avenue as the complications and risks are severe and although he struggles daily there is no certainty that he will continue to detoriate rapidly, he may even stabalise and stay like this for years.

 

He is under the Royal Free and sees Prof Denton (Prof Black before that) at least once a year and has also had various treatment and tests at the Royal Brompton Hospital. He local lung specialist is Mr Woodhead who I believe also has worked at the Brompton prior to coming to Coventry

 

He has received fantastic care at the both the Royal Free & Royal Brompton and I believe they have done everything possible to try and help him. My earlier post said he was diagnosed 13 years ago however after checking with my Dad it was actually 17 years ago and has been under the Royal Free for the entire time.

 

He has had most of the treatments I see discussed on this site including, cyclophosphamide, mycophenolate and rituximab, none of which appear to have made any difference to his lung symptoms

 

If anybody knows of any new treatments or trials that could of interest it would great to hear from you



#6 Amanda Thorpe

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Posted 14 May 2013 - 03:27 PM

Hello HW75

 

Sorry your dad has had to cease pursuing the transplant and I hope he stablises or even improves. I must admit I would not take the transplant route, I am to lazy and disorganised! It takes a lot to have a transplant.

 

Thank you for filling us in and please keep posting.

 

Take care.


Amanda Thorpe
ISN Sclero Forums Senior Support Specialist
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(Retired) ISN Email Support Specialist
International Scleroderma Network (ISN)





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