It’s great having Ellie home for Easter – it’s the first Easter she’s had at home for a few years, having been in hospital the last two Easters. All things considered, she’s doing pretty well too. I would say she’s feeling the best she’s felt since before her relapse and we’ve seen significant improvement over the last few weeks since being home.
 
Ellie is now off most meds, with just pain meds, antinausea, and antibiotics remaining. It’s the least amount of meds she has needed since this all began and we’ve started talking about removing her NG tube (though Ellie is strongly opposed to this!).
 
We had the follow-up PET scan on Monday to understand what change has occurred in the PTLD. Given her pain and nausea has reduced, the assumption was the PLTD should have reduced, though not entirely cleared. The PET scan (photo attached) shows a significant reduction in PTLD with only a couple of smaller areas of likely disease remaining. This is a really good outcome and demonstrates that something is working. For us, it’s pretty clear that the CTLs are having a good affect.
 
PET Scan photo:
·      RH image dated 6 Feb shows dozens of black PTLD lymph nodes in the bowel, stomach, and throat.
·      LH image shows significant reduction in black areas. The 3 large black areas in the torso are bladder and kidneys.
 
From the initial PET scan, you can see why she was in so much pain, with dozens of large cancerous masses spread through her body. Based on the recent PET scan results, the team decided to continue with CTL treatment and Ellie had another infusion on Wednesday. The plan is for one more in 3-4 weeks followed by another PET to check progress. Additionally, Ellie’s EBV is <500 copies which is the lowest since the PTLD diagnosis and demonstrable of the CTLs having an impact.
 
Ellie has started having skin issues again in the last week with a rash slowly spreading over her face and whole body. However, it largely responds well to steroid creams so we’re not overly concerned. That said, we don’t know the cause, but the obvious concerning possibility is GvHD. We’ll just have to wait and see how it progresses.
 
What is a little concerning is that Ellie has been experiencing vision problems. She noticed quick onset of blurred vision over just a few days. We got into the ophthalmologist quickly who diagnosed Accommodation Insufficiency which is a neurological / ciliary nerve impairment issue. Essentially, Ellie’s ciliary nerve isn’t working correctly so her pupils are fully dilated and can’t constrict, resulting in poor near sight and light sensitivity. We were told the causes could be disease related (ie AML relapse in the brain again); certain treatments such as Rituximab and CTL therapy; certain medications; EBV. The oncology team suggest there is no reason to believe there is AML activity in her brain at this stage and its more likely to be one of the other three causes. Clearly, we’re hoping it’s just related to EBV or CTL therapy and there are no other signs of impaired neurological activity so we’re fairly confident this is the case.
 
Some quick Dr Google research shows Accommodation Insufficiency is common, occurring in up to 17% of children aged 8-16 years. I guess this makes us feel a little more at ease however, it’s obvious to us that it’s related to all the treatment she’s had over the past 2 years. We know of the likelihood of organ problems following transplant and it’s why they conducted several baselining tests, including vision.
 
Aside from some skin and vision issues, and residual pain and nausea, Ellie is tracking very well and we’re very happy to have her home over Easter.
 
She’s currently obsessed with science and all things space so she’s making us watch lots of space movies with her (she wants to watch Prometheus and Aliens next but I’m not so sure…). She’s currently reading a book called Wild Ride by Hayley Arceneaux, a childhood cancer survivor who, after joining the Inspiration4civilian orbital space mission in 2021, became the youngest person to orbit Earth and the first paediatric cancer survivor in space. Our friends in America who know Hayley, sent us the book and subsequently a video message from Hayley with some wonderfully inspirational words for Ellie. It was a lovely gift and very special message – something I’m sure Ellie will never forget.
 
We hope everyone is having a relaxing Easter with family – we’re very fortunate to be doing the same!
 
PC[...]