Engraftment Syndrome

This week has been another week of ups and downs. We were on track for early release until Ellie spiked a temp last Friday. As we know, a temp is the first sign that something is brewing and unfortunately, it never lies.
 
It is now believed Ellie does have engraftment syndrome. This is not good news and not something we were expecting. Engraftment syndrome is very difficult to define and there are some academic parameters but nothing that is black and white. This is why the team initially thought it was just some form of immunologic response.
 
As we understand, it is not GvHD (graft attacking Ellie’s body), and not graft failure (Ellie’s body rejecting the graft) as we saw the early signs of engraftment. However, since the engraftment signs of strong white cell and neutrophil recovery, we then saw a sharp decline back to pre-engraftment levels. I posed the question to the team “If it’s not GvHD, and we know engraftment commenced, to what extent can this immunologic response result in subsequent graft failure; can that happen?”. Unfortunately, the answer was yes, it can happen, and yes, it’s possible that is what we’re seeing. They then explained the concept of ‘secondary graft failure’. Again, something that was not initially explained to us.
 
What it looks like for Ellie is high temps and reduced oxygenation, requiring oxygen support. There doesn’t appear to be any fluid in her lungs and she doesn’t have pulmonary edema. The team is now in the middle of another juggling act. Steroids to calm the immunologic response, but not too much that it completely inhibits her developing immune system and damages her kidneys. The steroids have had good effect so far – her oxygenation has improved and counts have begun to recover, but her temps are inconsistent. We need to ‘wait and see’ what happens over the next few days when the steroids wear off.
 
There is also the ongoing suspicion that there may be another infection brewing or that we were never able to properly clear her previous infections. They have been doing viral tests which have all been negative and blood cultures daily. Yesterday a blood culture tested positive for micrococcus luteus again and her temps have been high but inconsistent. Interestingly, we stopped Vancamycin the day before she tested positive again. Now she’s back on the Vanc and the plan is to again ‘wait and see’ what happens in the next couple of days. The fact that she’s had irregular ‘fleeting’ positive cultures is concerning in that it potentially points to a small infection that is unable to be cleared. We know that Ellie isn’t growing the infection due to the antibiotics, but it keeps inconsistently popping up in blood cultures.
 
Ellie also recently had another echocardiogram to see if the infection was attached to one of her heart valves. The echo shows that her heart is looking ok and no trace of the infection. However, there is some fluid surrounding her heart which is consistent with inflammation and recent infection.
 
The current thought is that the micrococcus luteus possibly never went away but was just suppressed by the Vanc, hence it hasn’t showed on every culture. If we can’t get rid of it in the next 48 hours, its presumed that it’s stuck on Ellie’s central line. As we have previously written, Gram-positive bacteria stick to plastic. In some cases, they stick to the central line and are extremely difficult to remove with antibiotics. The only way to remove it, is to remove Ellie’s central line. The Infectious Disease team is already advising to remove Ellie’s central line however, our oncology team are treading cautiously and will only remove it if absolutely necessary due to the risk involved in a general anaesthetic, given Ellie’s current condition.
 
So, again, the plan is to ‘wait and see’. Wait and see what happens when the steroids suppressing the engraftment syndrome wear off and wait and see if we’re able to clear her infection.
 
It looks like we’re going to be in hospital for a little longer than expected. We really don’t know what will happen in the next few weeks and we feel like we’re back in limbo, just playing the waiting game.
 
Despite the fact that we’re all suffering ‘transplant fatigue’ Ellie is otherwise doing well. I can see that spark in her eye starting to come back, and she occasionally fires up with her cheek and sharp wit. Today she logged into her school robot and whizzed around the class while they were having a free-play hour. Seeing her friends brought a big smile to her face and reminded her about what to look forward to.  
 
PC