Saturday, 21 November 2015

Q&A: Everything I know about PTLD in one post.

Since all of this has been happening relativity fast and I've been thrown a lot of information, I thought it would be helpful for me (and you) to put everything in one post to try and sort out everything.

Let's go through the conversations I've been having with people: 

You have what? What is post-transplant lymphoproliferative disorder (PTLD)? 

Post-transplant lymphoproliferative disorder is a type of non-hodgkin lymphoma most often developed through the Epstein-Barr virus (EBV). The definition on Wikipedia says it's 'a b-cell proliferation due to therapeutic immunosuppression after organ transplantation.' Basically, it behaves like an aggressive lymphoma (blood cell tumors that develop from lymphatic cells) and is one of the most serious complications - the other being rejection - for people with organ transplants. With lymphoproliferative disorders, the lymphocytes (a type of white blood cell) are over-produced or act abnormally.

But what is EBV and how did you get it?

The Epstein-Barr virus (EBV) is one of the most common viruses in the world. Most people get it as children and it transmits as a mild, brief childhood illness with fatigue and fever. In teenagers and adults, it often leads to mono. After contact with the virus, it becomes inactive in the body and for people with normal immune symptoms, that is the end of the story.

I most likely got EBV through my lungs. Pre-transplant, I was EBV- and they warned me that if I was given lungs that were EBV+, there was a 5-10% chance that I could get PTLD post-transplant as I wouldn't have any antibodies in my system to fight off the virus. I didn't care about the information at the time as 1) I was not even listed for a transplant at the time and 2) 5-10% chance seemed fairly small versus 100% dying without a transplant.

It's possible that I picked it up somewhere since the transplant but seeing as I haven't been making out with strangers and have been fairly germaphobic, I think the most likely answer is that it was already in my lungs. It wouldn't have been the doctor's top priority at the time for me to get EBV- lungs. I was dying and I was getting the lungs whether they had EBV in them or not.

Ok, so you have EBV but how come it turned into a cancer?

Without getting too technical into types of B and T cells, the EBV infects B-cell lymphocytes which then proliferate. Normally T-cells would shut that down but as my immunosuppressants decrease my T-cells to prevent them from rejecting my lungs, it also means that the infected B-cells can proliferate happily. For me, this happened in the liver, spleen, and possibly the lungs.  

Is this common? 

No, not really. 1-2% of people who get organ transplants develop PTLD. I was given a slightly higher percentage rate as I was EBV- before the transplant, therefore had no antibodies to fight off the infection if/once I got EBV+ lungs.

The Halifax CF team hasn't had anyone with PTLD before but the hematology team has had some patients with it who have been post-liver and bone marrow transplant. The treatment is basically the same but slightly different as lung transplant patients tend to be more complicated.

What's the treatment plan? 

The first step of the treatment was to cut back my immunosuppressants to see if that would increase my T-cells enough to fight off the cancerous B-cells. They could only do this up to a certain point as cutting back all of the medication would put me at a huge risk for rejection and once you go to rejection, you don't go un-rejected. I was told there was a 50-50 chance that that would be successful and, unfortunately, it was not.

The second step is immunotherapy which is an infusion of Rituximab (if you look up this medication, it was originally developed for rheumatoid arthritis treatment but is now being used to treat some lymphomas among other things). I'll get the treatment as an outpatient at the VG in Halifax once a week for four weeks. It could also be done in Moncton or Truro but all the teams seem to what me as close as possible to them so I'll be doing it in Halifax. It has way fewer side effects than chemotherapy and has been shown to work fairly well. They'll be taking a positron emission tomography (PET) scan before and after treatment so they can locate all of the cancerous cells and compare to see how it behaves under the treatment.

The third step would be chemotherapy which I don't know a lot about as they keep saying stuff like 'if the Rituximab doesn't work, we'll go for the stronger stuff.' I'm reading that a doxorubicin-based combination of chemotherapy is often used. I don't know what that means other than a cocktail of medication. There is a lot to learn about this cancer thing.

When does the immunotherapy start?

Probably in two weeks. Next week will be my PET scan and consults so I don't forsee it happening before that.

Ok great, what's the long term prognosis rate? 

Well, that's a tough question to answer. The hematologist told me that there is a 35% five year survival rate but to take that number with a grain of salt as that is the survival rate for everyone with PTLD. Most organ transplants are of the kidney and the recipients tend to be older so when they develop PTLD, they have a much worse prognosis than someone much younger. There simply aren't enough cases on record for them to have an accurate stats of people post-lung transplant with PTLD. Or people under 30 with PTLD. Or people who are post-lung transplant, under 30, and awesome.

Wow that's a lot of information. 

Yup. But now it's all in one place.

Freaking out? 


Want to play a board game to forget about it? 

Yes, always (our conversations usually tend to move on to other things after this, I have the best friends).

*Most of my information came from the following links as well as the doctors*

If you want to read more about PTLD 
Or PTLD on Wiki
If you want to read more about EBV.
What's a PET scan??


Sue said...

yea Board games!! wish I was there to come play some with you. Thanks for the narrative of information as well - answered most of my questions without my having to ask you directly. You are so right! You ARE Awesome! and definitely have that going for you. hugs and love

christina mccully said...

What alot of information to be given to you.Thanks for explaining it to us and it must be hard to answer all the questions. i was going to come see you on Friday but woke up with a cold and knew you did not need it. Take Care and lots of hugs