We had our first trip to the emergency department this week with Ellie spiking a fever on Wednesday evening. She had a clinic appointment that day, and they accessed her central line to take bloods per usual. The thought is that some bacteria was introduced into her line at that time, which caused her fever just a few hours later.
 
Ellie spiked an unusually high temp which reached 40C very quickly. The team believe it is likely a gram-negative bacterium due to the high temp. Once in emergency, Ellie was administered Cefepime and Gentamycin antibiotics immediately while blood cultures were taken. We’re lucky to live so close to the hospital as we’re required to get her to the ED within 30 mins of spiking a temp. Once at the ED, we flash the ‘gold card’ which gives the triage nurse specific instructions to immediately admit her and commence Cefepime as soon as possible. It’s a pretty efficient process so there’s no waiting around in the same room as other sick kids. We remained in an isolation room in emergency for about 24hrs until an isolation room became available on the paediatric oncology ward.
 
Since being admitted, Ellie’s blood cultures haven’t grown any bacteria so we may not know what the cause of her temp was. It’s possible to have a situational temperature which isn’t caused by anything specific however, due to the very high temp, this is unlikely. It could have been a small amount of bacteria which was quickly cleared by the antibiotics and therefore hasn’t appeared on the cultures. This would be ideal as it means Ellie should be released soon. If her cultures are positive, then she will be committed to a 7-10 day course of antibiotics, and would have to stay in hospital likely for around 2 weeks. However, if her cultures are negative, then after around 5 days she could be released providing she doesn’t have any more temperatures and is otherwise stable. We are therefore hoping she can be released as early as Monday or Tuesday.
 
Aside from this minor setback, Ellie has been progressing well. We can notice the positive effect being at home has on her emotional state and she appears to be getting some of her strength back from moving around the house and playing with Chewie.
 
Fingers crossed we’ll be home again soon.
 
PC[...]

We’re excited to say that Ellie was released home earlier this week and is enjoying being back in her own environment with her puppy and her sister. It has been great having the whole family back under one roof again and not needing to share time between the hospital and home.
 
It has been another challenging transition and we’ve had some friction along the way. There was some miscommunication about the necessity for a new central line. Initially we were told a new central line would not be required and we could make do with cannulas however, this advice has now changed. The two long cannulas that were placed last week both failed within the week. Additionally, Ellie can’t be released home with a cannula, so every time we go back in for bloods, it requires more needle sticks. The team have decided that Ellie will need a new central line placed next week. This is just another GA that we didn’t want and surgically placing another line into her heart comes with more risk as it will be the third time. While it would hopefully only be needed for around 2 months before being removed, the thought is that Ellie will still need blood product support and when she spikes a temp, they will need to commence antibiotics within 30 mins, so relying on cannulas won’t be sufficient.
 
We are also required to attend clinic appointments three times a week where bloods are drawn and blood products may be required. This is challenging without a central line as Ellie has developed what seems to almost be a phobia of needles. She’s not so bad with regular needle sticks for drawing blood however, cannulas are very challenging. Her veins are extremely difficult to access, and they almost always roll or collapse. The only way to access them is by having an anaesthetist use an ultrasound to guide the needle. This results in Ellie usually being stuck 3 or 4 times before successfully having the cannula inserted. It takes considerable effort to mentally prepare her for a regular needle and we need to physically restrain her for cannulas. Ellie now says she wants to have the central line back in to avoid more needles.
 
The good news is that Ellie’s GvHD appears to be under control. It has not progressed beyond stage 1 GvHD and her rash has responded to the Methylpred steroids. The rash is barely noticeable and she isn’t bothered by itching. The Methylpred will likely last for another 8-9 weeks.  
 
It’s a full-on job looking after Ellie at home. We’re back in the routine of administering half a dozen medications through her NG tube twice daily, giving her water boluses regularly to flush the medications from her kidneys, and NG tube feeds throughout the night. She is still very weak and needs assistance walking, eating, dressing, washing, and most other activities. We’re trying to get her back into a school routine where she can zoom-in via her ‘Missing Schools’ robot which lives in her classroom however, getting her to participate is challenging.
 
Ellie’s confidence has taken a real beating throughout treatment, which is most evident with her communication and decision making. We had noticed this after completion of treatment last year and it’s even more evident since transplant. She struggles to communicate with her teacher and friends during school sessions and her ability to make simple decisions about what to do or what to eat is almost non-existent. We know she is very intelligent however, we also know she is starting to miss key learning milestones. To compensate, we are developing a plan to have a private tutor come to the house twice weekly to get her back on track and reduce any longer-term learning issues. She is also really struggling emotionally so we are looking at options for emotional/psychological support which I can see as being an ongoing requirement as she progresses through school and needs additional support coping with all the normal stresses of childhood and teenage years. We feel it is best to get on top of some support networks sooner rather than later.
 
Silver linings: Ellie is home with her puppy and her family, and this is great for her mental health; the whole family is back under the same roof and this is great for everyone’s mental health; GvHD and engraftment syndrome are both under control and we consider that there are no ‘major problems’ right now; Ellie’s counts are recovering well and her white cells and neutrophils are at a level we haven’t seen since first diagnosis (11.7, 9.36); so all things considered, Ellie is doing reasonably well.
 
We remain optimistic that Ellie will continue to improve with every day.  
 
PC[...]

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[...]