Graft v Host Disease; Day + 32

This week started off with the decision to remove Ellie’s central line due to the presumption that it was infected and the reason for several positive cultures. She had another positive culture on 29 July – another staph infection and was highly febrile for about a week. That was enough to make the call on removing the line which occurred on 2 Aug. This was done under general anaesthetic, and everything went well, though Ellie was extremely nervous about the procedure.
 
Ellie now has two ‘long cannulas’, one in each arm, for IV medications. While not ideal, its workable considering we are in the process of weaning her off several medications and transitioning most to oral in preparation for an eventual discharge. Ellie now takes most medications through her nasogastric tube which will continue when she is released home.
 
The bad news is Ellie is now suffering Graft v Host Disease – one of our major concerns with transplant. The day after removing her central line, Ellie broke out in a rash covering 25-50% of her body. Rash is one of the first indicators of GvHD and she was soon diagnosed with the disease. This is coincidental to the line removal – really bad timing, but not related. The rash coincided with a steady decline in her white cell and neutrophil count which, to this point, had been steadily improving after successful treatment for Engraftment Syndrome. GvHD is a bone marrow suppressant and therefore impacts graft growth and this accounts for the decline in her counts. The donor cells are now attacking various healthy cells in Ellie’s body which at this stage, seems to be just limited to her skin. This results in a skin rash which is extremely itchy and very distressing for Ellie.
 
Ellie is being treated with three different antihistamines, four different skin creams, and importantly, Methylprednisolone steroids to treat the GvHD and reduce inflammation. Unfortunately, treatment with Methylpred is likely to run over 8-10 weeks. While it’s not such a big deal in the short term, we know there are several long-term side effects and can cause diabetes. Ellie already has a concentration of glucose in her urine and if this continues, she will need insulin. When Ellie was being treated with Methylpred (0.5mg p/kg) for the Engraftment Syndrome, the team reassured us by saying we shouldn’t be too concerned because it’s not as if she is having GvHD-size dosage (1.5mg p/kg). This reassurance has clearly backfired as Ellie is now being treated with 2.0mg p/kg. We understand this will continue for 7 days before being reduced to 1.5 for 7 days and then reassessment.
 
Critically, we need to see Ellie’s counts recover to demonstrate the graft is recovering from the GvHD. Her symptoms of GvHD could last for weeks however, the Methylpred should allow her graft to grow and her white cell and neutrophil counts to recover.
 
Thankfully, Ellie’s GvHD is currently Grade 1 (mild): Skin rash over less than 25% of the body (though its actually 25-50% in Ellie’s case but with no other symptoms). We need to be watchful for additional symptoms which characterise the following grades:
 
Grade 2 (moderate): a skin rash over a more than 25% of the body, accompanied
by mild liver or stomach and intestinal disorders.
 
Grade 3 (severe): redness of the skin, similar to a severe sunburn,
and moderate liver, stomach and intestinal problems.
 
Grade 4 (life-threatening): blistering, peeling skin, and severe liver, stomach, and
intestinal problems
 
The silver lining in dealing with GvHD is that we don’t expect any severe GvHD because Ellie’s cord donor is perfect 8/8 HLA match. Also, a degree of GvHD indicates the possibility of a degree of Graft v Leukaemia (GvL) effect. So, the hope is that while the graft is attacking Ellie’s healthy cells, it’s also attacking any residual Leukaemia cells which would clearly reduce the chance of another relapse.
 
To us, it feels like setback after setback. Since first diagnosis, Ellie has had several unfavourable outcomes, ultimately leading to relapse and BMT. Now, during BMT she’s had six infections, central line removal, Engraftment Syndrome, and GvHD. It’s extremely fatiguing for us and for Ellie, and sometimes defeating.
 
However, in our perpetual search for silver linings, we know things could be worse. The team continue to reassure us that in the context of BMT, she’s actually doing reasonably well. When we were preparing for BMT, our head oncologist said to us “there’s a chance Ellie will go into that room and never come out again”. Those haunting words revisit us every time we have a setback. But we’re still here and Ellie is still fighting.
 
With any luck, we will be able to get on top of the GvHD soon, wean down the Methylpred, and transition all her medications to oral. So long as the infections stay away and Ellie doesn’t spike a temp, we could have her home within a week or so. We’re anxious to get her home as soon as possible, purely for her mental health and we have seen how well she bounces back when she’s home with her family and her puppy. In the last 17 months since Feb 21, Ellie has spent 10 months in hospital.
 
PC