YET AGAIN, LIFE CHANGES IN THE BLINK OF AN EYE...
Post Transplant Lymphoproliferative Disorder (PTLD)
3 days ago it was confirmed that I have a type of Lymphoma called PTLD. This condition is a type of lymphoma that can develop in people who have had organ transplants and occurs due the ongoing immunsuppression required to prevent rejection. Although it's one of the most common cancers to occur post transplant, it is still very rare - only affecting around 3% transplanted patients.
I am not going to use this particular blog post to explain how I feel about this new turn of events, but simply to explain what PTLD actually is - so I can make sense of it myself but also in a way that hopefully my friends and family can find useful.
What is a lymphoma?
Lymphoma is a cancer of the lymphatic system. One of the main functions of the lymphatic system is fighting infection - and therefore includes parts of the body that make immune cells (thymus and bone marrow) and places where immune cells are stored ready for action (lymph nodes, spleen, tonsils). In lymphoma it is the white bloods cells called Lymphocytes that become abnormal and they tend to collect in the lymph nodes. There are about 60 different types of lymphomas, all with their own features, treatments and prognosis. I only need to concentrate on the specifics of PTLD. There are different types and I am still waiting to hear about specifics.
Why does PTLD occur?
There is usually an association with EBV (Epstein Barr virus). 9/10 people have had EBV at some point - it's the virus that causes glandular fever, but often can produce no symptoms. It stays latent within cells and our immune system keeps it under check. If our immune system isn't working properly (e.g after transplant anti rejections to suppress the immune system) then the EBV can flair up and cause the lymphocytes to divide abnormally. Alternatively sometimes the EBV is introduced with the transplanted organ. Some people with PTLD don't have EBV and the causes here are more unknown. My blood came back as EBV +ve. This will be useful when monitoring treatment response as they can see if the EBV level in my blood goes down.
Symptoms of PTLD
Most commonly people notice painless swelling of lymph node in the neck, armpit or groin. You can also experience 'local symptoms' where enlarged nodes are pressing on nearby tissues. I had presented with severe back and abdominal pain which had come on suddenly and I couldn't manage at home. I'd actually had a hospital admission a few weeks prior with similar pain but it was thought to be a bowel obstruction at the time - I think ironically there had been two things going on at same time. However when the pain returned it was so extreme, so I was admitted again and had abdominal CT. This showed three main groups of enlarged, necrotic nodes in my abdomen, along with lots of singular swollen nodes. The lymph nodes are at the back of my abdomen - hence me feeling it as intense back pain where nodes are causing compression.
PTLD can cause other body symptoms such as fatigue, weight loss, night sweats, fever. I hadn't noticed anything in particular and actually thought I was quite well over the period of lock down. I was of course exhausted but assumed that was looking after Estelle full time.
Diagnosis
A biopsy of lymph nodes is the only way to diagnose. I had CT scans of chest and neck to see if there were any nodes easier to get to, but there didn't seem to be. It's good they hadn't spread further but it did make biopsy more tricky. They did laparoscopic (keyhole) surgery under general anaesthetic and managed to find a node close enough to surface to biopsy. I has asked about removing nodes that were causing pain, but most of the large groups were lying right next to major blood vessels, so couldn't be touched. I had a night in HDU to recover but coped fine with the anaesthetic itself and the wounds are healing well. I have been back home for nearly a week now and just got the results a few days ago - much quicker than expected.
Treatment
The initial treatment of all PTLD is to reduce anti rejection drugs, in an effort to allow my own immune system to get some control. One of my drugs (MMF) was stopped right after the scan and my other one (tacrolimus) is being reduced to the minimum level - so I had some more blood levels taken today to check this. My steroids have been increased to try and protect the lungs from rejection and trivial as it may seem, I am dreading getting the steroid moon face again. It is going to be a really difficult balance of being able to mount an immune response against the cancer, without reactivating the rejection of my lungs. The chronic rejection has been stable through lock down, and in general for about the past year. My lung function is still under 1 litre, which isn't great but I am not needing oxygen and can manage what I need to do day to day. I am absolutely terrified that my lungs start declining again, after just getting to the stage where I felt a bit more secure. Although 2nd transplants had been discussed during my previous decline, I would not be eligible for this when I have active cancer (would be possible if cured in future). So I need to get this kicked into touch without affecting my lungs.
Beyond initial treatment I am now awaiting communication from the Beatson. Treatment might involve an antibody drug that would act to "mark" the affected cells for my immune system to nuke, or it might be more formal chemotherapy, or combination of both. I will update my blog once I know myself.
The future
At the moment I am just getting my head around the diagnosis. The Beatson are aware of me and I expect to hear this week if I need any more staging scans, or whether I will be meeting oncologist first. Newcastle have been in communication and they are happy for Beatson to take the lead, and they will liaise as required. I've to monitor lung function twice weekly on my home monitor. My GP is now looking after my pain meds. I had a real struggle getting on top of the pain in hospital and barely slept - I am now on higher doses of long acting opiates, with doses for breakthrough pain and another for nerve pain. Things have been under more control the last couple of days and I have slept a bit better, so hopefully I can settle into a routine now. It's impossible to comment on prognosis - there aren't clear factors that determine if I will be a responder to treatment, how my lungs will cope and how likely we are to achieve remission. My aim of course is to be cured asap with no loss of lung function - aim high!
I will update when I have more information about the next steps!
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