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What is PTLD?
Post-Transplant Lymphoproliferative Disorder (PTLD)
PTLD is when lymphocyte growth occurs rapidly in the body. Lymphocytes are white blood cells that are the body's main type of immune cell and play a very important part in the body's immune system.
Lymphocytes are made up of B-Cells and T-Cells and they behave like the body's army - sending the troops in to attack the enemy (a virus).
PTLD, or the rapid growth of lymphocytes, is when lymphomas develop after transplant. PTLD can be non-cancerous (early PTLD) or cancerous. George's diagnosis was High Grade T-cell Monomorphic PTLD - this means that his form of PTLD was fast-growing, aggressive and meant his T-Cells were cancerous. T-Cell PTLD is much less common than B-Cell PTLD.
Most cases of PTLD are caused by the Epstein-Barr Virus (EBV). EBV is a very common virus and around 9 out of 10 adults would have been infected with it in their lifetime. Once exposed EBV stays in your body, and, in normal healthy people will be kept under control by your immune system. However, in post-transplant patients (whether they are exposed to EBV via their transplanted organ or simply exposed to it for the first time naturally) the suppression of their immune system means that they can't always keep the virus under control and so the immune cells start to transform.
Most people who have transplants will not develop PTLD - it occurs in a low percentage of transplant patients as it is very rare (affecting between 1%-10% of those who have had a transplant and developing in between 1%-6% of post-liver transplants).
The most common symptom of PTLD is when a lump develops in the groin, armpit or neck area. Fatigue is also a common symptom. George's symptoms included sudden tiredness and a lump forming behind his ear, but as he was suffering from an ear infection at the time (its relation to the PTLD unconfirmed) our concerns weren't taken as seriously as perhaps they should have been.
Once diagnosed, George's treatment involved him being taken completely off his immuno-suppressant medication (a huge risk as it meant his transplanted liver could go into rejection, but it was the only way to allow his immune system to build itself up and have the strength to fight). He was also treated with a form of antibody therapy known as Rituximab. Unfortunately, he had an allergic reaction to this medication but once the infusion was slowed down his body was able to cope with it. However, due to the aggressive type of cancer he had developed he was transferred to Great Ormond Street Hospital where he received Chemotherapy treatment.
Haemophagocytic Lymphohistiocytosis (HLH)
Devastatingly, all these interventions were too little and too late and George was unable to recover after developing HLH (Haemophagocytic Lymphohistiocytosis). HLH is also rare and occurs when the body's immune system reacts badly to a type of 'trigger', such as cancer, and so the T-Cells go into a sort of panic. This causes severe damage and inflammation within the body. HLH is very hard to diagnose due to its similarity to other infections.
Our beautiful boy fought as hard as he could but just 2 weeks after his PTLD diagnosis and just 1 day after being diagnosed with HLH we were told that there was no more hope. His oxygen levels had started to deteriorate rapidly, meaning that the lack of oxygen would have started to affect his brain. And so Ant and I made the heart-breaking decision to remove George from life-support. We decided that having control over his passing was better than watching him suffer from a cardiac arrest and so we snuggled him into our arms, felt his heart-beat fade, told him how much we loved him and held him as tight as we could whilst he peacefully left this world.
