A lot has happened since our last post, but thankfully not much in the context of Ellie’s treatment and recovery. She continues to progress well, despite a few small hiccups along the way.
 
Year 6 has been an exciting start to the year and Ellie is enjoying the face-to-face academics and introduction to new activities. She participated in her first school camp recently which was an overnight adventure camp where she did rock-climbing, abseiling, zip-lining, and other fun activities. She was proud of herself for participating in every activity when some other kids didn’t because they were scared. She absolutely loved it and couldn’t stop talking about it. She was so excited leading up to camp however, the day prior she spiked a temp while at school, had dizzy spells, almost fainted, and had to come home early. We were concerned she wouldn’t be able to attend camp but she would NOT take no for an answer, so we allowed her to attend (her school was very supportive/understanding) and she had an absolute blast.
 
Her temperature and dizzy spells are an ongoing thing which we just put down to ‘unknown weirdness’ associated with bone marrow transplant and having enough chemo to fill a bathtub, but this time was likely related to the ongoing cold/flu symptoms she’s been experiencing for several months. She was confirmed to have Rhinovirus twice, and just recently, RSV. It’s been very hard for her to shake the illness as her immune system is still very immature. However, she is largely able to manage it with Panadol when needed, but she has constant mild to moderate cold/flu symptoms.
 
During a particularly bad few days of symptoms where she couldn’t recover from being highly febrile and having shortness of breath, we were advised to take her to the ER where than ran several tests and scans. It seems she regularly has some small crackling in her lungs which may be fluid, may be infection, may be something else, but not enough to do treat properly. However, this time they were concerned as her CRP (inflammation markers) were hugely elevated (>200) which is a strong indicator for sepsis. Ellie otherwise wasn’t presenting with sepsis symptoms so it was determined the combination of a viral and possibly minor bacterial infection, and her immature immune system was causing an inflammatory response. After some antibiotics, she was released with no other significant complications.
 
While her symptoms have been ongoing for several months, she doesn’t let it phase her too much and just gets on with things. She doesn’t let it slow her down much. On the other hand, I recently had man-flu and wanted someone to take me to hospital but nobody would listen to me at all!
 
Ellie was also recently confirmed to have chemotherapy induced alopecia. We had suspected this for a while as her hair doesn’t seem to be filling-in. What little hair she does have is growing slowly, but not filling-in. We’ve discussed wigs but she isn’t interested. Not sure how this will play out over the years but she is the type of person who probably won’t let it affect her too much. She is aware but not overly sensitive about it and sometime even makes jokes. We wonder how she will go during her high school year and beyond but for now, she is not too phased which is a characteristic about her we love.
 
Other exciting events for Ellie have included our American family coming to stay over Christmas, our good friends from Brisbane staying with us over the Easter long weekend, Ellie’s first sleepover birthday party at a friend’s house with lots of other kids, finishing her childhood immunisations (for the third time), and a family weekend trip down on the South Coast with Camp Quality which included her first body boarding experience at the beach.
 
Ellie continues to have great energy levels but her physical abilities are clearly limited. We are trying to get her into daily cycling on the exercise bike and weekly physiotherapy treatment due to some ongoing lower limb and back/neck issues. Despite some of these relatively minor ongoing challenges, we are so happy to have her with us, relatively healthy, and enjoying life and school. We regularly comment how incredible it is to have our family together under one roof, almost like a normal, happy, healthy family. There is so much to look forward to over the next couple of years with both girls in high school, a move to Brisbane, building our forever family home, and many more years of health ahead of us. 
 
 
PC[...]

We’re pleased to report another great couple of months in terms of Ellie’s overall progression and health. It has been largely uneventful (except for COVID, but we will get to that…) and Ellie continues to progress exceptionally well. She is fully immersed in schooling activities, including stand up paddle boarding, physical education, limited sport, and of course still loving her academics.[...]

It’s been quite a while since our last update as not much is happened over the past few months. This is great news as it means Ellie is tracking steadily and continually improving. 
 
Now that Ellie doesn’t have a central line, we have reverted to monthly appointments for a clinical checkup and bloods. Prior to Ellie’s central line being removed, she was having occasional fevers and there was a thought her line may be infected. Since removing the line a few months ago, she hasn’t had any fevers and is feeling great. Of course, one of the first things we did after removing her line was go to the rock pool and beach for a swim! 
 
Ellie is doing great being back in school full time. We were quite surprised with this and thought that it may take her a few weeks to ease back into it by doing half days. However after the first few days it became obvious that she had plenty of energy left and was eager to be back in class and not miss anything. She’s loving being amongst her friends and getting involved in all schooling activities. 
 
Ellie’s physical abilities are still slowly improving. She still has obvious muscle atrophy in her legs and she tires quickly when doing anything strenuous. However she does get on her bike and rides 10 laps of the neighborhood weekly as per of her routine. She is otherwise fairly active, participating in nearly everything at school – even standup paddle boarding classes at Malabar Beach as part of school PE!
 
We’re unsure if her hair is growing back yet - possibly a little but it’s very slow. We’ve spoken with the team and dermatologist about chemotherapy induced alopecia. Not sure if this is the case as they say even after BMT it is extremely rare however, her lack of hair has us wondering.
 
Next month we will have another PET scan to confirm no presence of PTLD or other issues but clinically, Ellie is presenting very well so there isn’t any reason to suggest anything is going on. 
 
We also had a very special visitor recently – Ellie’s first (and favourite) oncologist, also named Ellie (commonly referred to as Ellie # 2). Ellie # 2 has been on exchange with the Cincinnati Children’s Hospital Cancer and Blood Disease Institute (CBDI) for the past year, and she came back to Sydney for a short visit, making the time to catch up with us. It was so great to see her and for her to see how well Ellie is progressing. Ellie #2 has been with us since day 1 and has become like an extended family member. The Sydney Kid’s Cancer Centre has a habitual relationship with CBDI with several of our oncologists having completed exchange. CBDI is where our team learned CTL treatment which was pivotal in Ellie’s PTLD treatment, Sydney KCC being the only hospital in the country that has access to this treatment.
 
We’re overall very happy with Ellie’s progress and Ellie is very happy being back to doing most normal things. She’s looking forward to more beach days, summer in Sydney, and Christmas time with lots of upcoming family visits to Sydney.
 
 
PC[...]

After a few months of slowly progressing and not much to report, we’ve had a big few weeks with the exciting news that Ellie is in metabolic remission from her Post Transplant Lymphoproliferative Disease (PTLD). Her recent PET scan showed no signs of PLTD and the team are comfortable to declare metabolic remission. The scan wasn’t entirely cold and there were some areas that required some in-depth discussion with several specialists but they eventually determined the PTLD is gone.
 
The other big news is that Ellie had her central line removed this week. This is another big step towards normalcy but one that does make us a little nervous. The logic behind removing the line was based on metabolic remission and no plan for continued treatment at this stage. However, it’s a little pre-mature as a significant influencing factor is the suspicion of an infection starting to influence the line. Over the last couple of months, Ellie’s line site and the surrounding skin has been very raw and oozy. She’s spiked a few temps and has taken a few courses of antibiotics which have helped but not stopped the symptoms around the line. The line has been in for a year now which is a long time, so the team were keen to remove it. I’m sure if the line was in better condition, we would have kept it for a little longer until we are more comfortable that we’re in the clear.
 
Removing the line is fantastic news however, if Ellie does need more treatment such as a blood infusion, that will require a cannula which is very difficult to achieve due to Ellie’s tiny veins that collapse very easily, turning the whole process into a significant emotional event for everyone. Also, if it turns out the PTLD is not entirely gone, she will need a new central line inserted. We’ve discussed this in detail with the team and all acknowledge there is no perfect decision, and we need to closely monitor things over the next few months. Ellie will have PET scans probably every 2-3 months for a while. We are also hoping that Ellie’s immune system finally starts to kick in as this will prevent any reoccurrence of PTLD. To date, her immune system is still ‘terrible’ as our oncologist describes. We knew it would be around 12 months post-transplant for it to start to develop, but we’re beyond that now and as we know, Ellie has had several complications along the way. For now, we continue to ‘wait and see’ like always.
 
Metabolic remission and central line removal is obviously fantastic news and a big step in the right direction. We just don’t feel that sense of excitement this time around and the belief that everything is finally behind us. We’re very happy but cautiously optimistic. What REALLY makes us happy, is seeing how happy Ellie is. It’s like she has a new lease on life with her line being removed. We often talk with her about not letting cancer define her and to try and turn the negative events into positive influences in our life. She is truly responding to this advice and determined to get on with her life. It’s incredible to see. I think Haley Arceneaux had a significant influence on her, just at the right time a few months back.
 
So here’s an example of just how incredible our little Ellie is. We know that she completed NAPLAN in year three – over two years ago and in the middle of chemo – and she knocked it out of the park. Earlier this year, she did the year five NAPLAN, again from hospital, this time while in protective isolation after a bone marrow transplant. She was being treated for PTLD and in severe pain to the point where she was on Pregabalin and Fentanyl PCA. She was extremely anxious about doing NAPLAN after not having any consistent formal education for the last two years aside from about one hour per day of ad-hoc hospital schooling. Being a perfectionist, she was terrified about not doing well. The school teacher sat with her in isolation to supervise the tests, while Ellie self-administered Fentanyl boluses. Again, she knocked it out of the park, scoring right at the top of the highest banding. We couldn’t be prouder of her.
 
Ellie is super keen to get back into everything – swimming, cooking, wearing regular clothes, and getting back to school. Our oncology team are still nervous about letting her out of the house but we’ve been able to sneak a few excursions in! We even got her into school for a couple of hours for assembly (carefully and wearing masks etc) – she absolutely loved it. We just need her immune system to get moving before we unleash her on the world again!
 
 
PC[...]

It’s been a while since our last post so we wanted to check in and let everyone know that Ellie is progressing well. She’s getting stronger every week and seems to be feeling happier and more comfortable with herself.
 
We have definitely seen her mature considerably this year; she’s taking more responsibility in her schooling and learning, showing more emotional intelligence, and obvious awareness of her treatment and mortality – which she speaks about. It’s quite eye-opening to hear a 10 year old verbalise understanding of her mortality.
 
Earlier in the year, Ellie was really struggling emotionally and had verbalised that she wasn’t happy and wished she could trade places with anyone else. She certainly had some very difficult. However, over the last few months, her health has been improving weekly and her emotional state improves with it. She’s enjoying seeing her friends for weekend playdates, talking with Annabelle, and now even loves cooking with Kara. Her strength is also improving well, and she no longer needs the wheelchair to get through the hospital – she can walk the whole way which is a significant milestone.
 
In terms of her treatment, there isn’t a great deal of change; it looks like we will continue CTL immunotherapy for a while. We did have a scare a few weeks ago which thankfully turned out to be a false alarm. Ellie had a PET scan to check progress and the impact of CTLs on her PTLD which we know is still present. Interestingly, around that time we had noticed some regression in nausea and diarrhoea symptoms. The PET was conducted on Wednesday, and on Thursday they called to say we needed to come in for an urgent meeting on Friday. That is never for good news.
 
Our oncologist explained that the PTLD is clearly still throughout her stomach and bowel and there didn’t seem to be much change since the last PET scan. It seems as though the CTLs had good affect early, but the results had plateaued in recent treatments. More concerning is the fact the PET showed what appeared to be some new activity around the base of her skull. This had the team particularly concerned given Ellie’s previous AML chloroma in her brain. They weren’t exactly sure what it was but had concerns it could be something more sinister “eating the bone in her skull”. Our oncologist can sometimes be a little more descriptive than is needed…
 
The team ordered more CTLs at an increased dosage and an urgent MRI. Thankfully, the MRI showed no signs of PTLD at the base of Ellie’s skull, and no mysterious ‘skull-eating’ cancer. Interestingly, the increased dose of CTLs seemed to have significant and immediate effect on Ellie. Her diarrhoea and nausea symptoms subsided within 24 hours and she was feeling much better.
 
Also interesting, the MRI gave us a clear image of Ellie’s brain so we could see what is happening in the area of her previous AML chloroma. Turns out she has a big beautiful brain! In the image attached, you can see only the slightest scar tissue where the chloroma was. We can’t tell you how reassuring this is. It’s something that still haunts us – whether we made the right decision not to agree to brain radiation therapy.
 
Since the last dose of CTLs of increased strength, Ellie had been doing great. We joke that it’s the magic dose of CTLs! That said, we only have one dose left which Ellie will be administered in a few weeks. The team still say they don’t know why the PTLD is persisting, nor do they know exactly why the CTLs work the way they do. However, all agree that if its working, let’s keep doing it. I think it’s likely that unless Ellie’s next PET is completely clean, they will order more CTLs, though this time, from a different donor which comes with some risk. The only alternative is more chemotherapy which, was being discussed if the MRI results weren’t good.
 
Chemo is clearly counterintuitive right now. The problem is the PTLD is hanging around because Ellie doesn’t have an immune system. The CTLs – donor T-Cells – are effectively artificially boosting her immune system. Chemo would kill the PTLD but also destroy any recovery her immune system is trying to make, setting us back a long way.
 
It’s easy to fall into complacency – pretending everything is almost normal – until a Thursday afternoon phone call saying we need to come in for an urgent meeting, slaps you right back into reality.
 
The important thing is right now, Ellie is doing great and feeling great. She impresses us every day with her intellect, determination, and sassy wit. And she is HILAROUS. There’s not much that makes me burst out laughing these days, but Ellie comes up with the craziest funniest things sometimes – usually paired with an impromptu theatrical routine for effect.
 
We feel like we’re in a steady routine now, and Ellie’s continued progress has us feeling the most hopeful we’ve been in a long time. We’re enjoying having her home, watching her grow, and only thinking positive thoughts.
 
PC[...]

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[...]

We finally have some more good news – after another 5 weeks in hospital, Ellie has been released home and back to being an out-patient. She has seen some good improvement over the last couple of weeks, successfully weaning off the hydromorphone and several other medications.
 
We are very lucky the team has let us come home as technically, Ellie has not met the criteria for release. She is still regularly febrile and that is one of the key data points that almost always keeps an oncology patient in hospital. We have seen other kids preparing to be released from hospital, only to have their final temperate check show 38.0 and then be told ‘sorry, can’t let you go now’ and then the cycle of antibiotics, blood cultures, and tests starts all over again. We saw this happen to a boy on his birthday! It was devastating for them as the family had a big party arranged for his return.
 
We’ve been released with the caveat that we go back for review Mon/Wed/Fri before hopefully dropping back to one or two days per week. The reason for release is that aside from being regularly febrile, Ellie is otherwise clinically stable. Specifically, her pain and nausea are mostly under control. She still needs oxycodone, pregabalin, and three anti-nausea medications around the clock, but we consider that ‘under control’!
 
Ellie has been febrile since we started the CTL therapy but we have never determined the cause as nothing has grown on her blood cultures and there are no other signs of infection. It’s entirely possible that its some low-level inflammatory response to the CTLs, or just the way Ellie’s body responds to them. No-body can explain it, but also, we can’t rule-out an underlining infection or other problem. While the team have let us come home, they are clearly nervous and not entirely comfortable with it. We know the decision is largely because of the relationship we have developed with the team over the last 2 years; they know we’re responsible people; and they know we live 15 mins from the hospital. This has worked in our favour in the past.
 
Some other key updates are that Ellie’s EBV viral copies have significantly reduced (from around 15,000 down to 1,000); her congestion has reduced; her hearing seems to be getting better; she no longer needs oxygen support; and she’s starting to eat and drink again. In fact, Ellie has practically not drunk any water since her transplant in July 2022, until a couple of weeks ago when she suddenly decided she wanted to. Now she’s drinking around 900ml per day. While seemingly insignificant, it’s a big win as she no longer needs NG fluids over-night. It’s very common for kids to not drink water for many months after transplant.
 
Also of significance is the fact that there doesn’t appear to be any sign of GvHD. This is huge. She has been on steroids for so long to suppress her immune system and keep GvHD at bay. When her PTLD kicked-off, the thinking changed to it being the cause of her symptoms, not GvHD. So effectively, the treatment for GvHD created an environment for EBV to thrive and cause PTLD. Hindsight is wonderful but we understand the team had to treat the probable cause, clearly not the improbable cause. However, it was also possible that both GvHD and PTLD were occurring concurrently. The problem with this is you can’t treat both concurrently. We had to quickly try and reverse the effects of the GvHD treatment to commence PTLD treatment. We were very concerned that GvHD may kick off again during PTLD treatment which would have been catastrophic.
 
While our team can’t actually explain why Ellie is feeling better, clearly something is working. Our thought (hope) is that the CTLs are clearing the PTLD and negate any need for more chemotherapy. Ellie’s had two CTL infusions so far and the current plan is to do another PET scan in about a week and make a decision about further treatment at that time.
 
As Kara has said several times, we’re just thankful for every day we have Ellie home with us. So for now, we’ll just continue to enjoy having the whole family under the same roof!
 
 
PC[...]

Shortly after our last update, Ellie had surgery to remove the large Post-Transplant Lymphoma Disease (PTLD) lymph node in her neck for testing. Unfortunately, by that stage her condition had deteriorated so she was re-admitted. We’ve been back in hospital for over a week now and I think we’ll be here for quite a while as there are several issues at play and we’ve had a few scares over the last week.
 
The removal of her lymph node went reasonably well, though there was some question about whether she was stable enough to have the surgery. The surgery went ahead and they were able to remove the majority of the lymph node. However, for the next 48 hours Ellie was in an extreme level of abdominal pain despite being heavily medicated, and extremely nauseated, vomiting considerable volumes of bright green, and then bloody vomits.
 
For several days her pain and nausea were not under control and we had no real explanation for what was happening. The current thinking is that it’s all related to the PTLD in her bowel and throat. After a few days of changing medications to try and find something that worked, we eventually got her pain under control by switching from Fentanyl to Hydromorphone PCA, and a few other pain medications. She is now basically back to having about a dozen medications running through her central line around the clock, plus TPN and nasogastric feeds. It very much feels like being back to square one. Other symptomology includes a head cold which she’s had for months. No immune system means she can’t get rid of it. She is terribly congested, can’t breathe through her nose, and she has fluid in her ears. Her hearing is so poor we have to speak very loudly and so she can lip read. She also can’t hear herself speak, so when she talks she sounds like she’s deaf.
 
It's also now evident that the first plan for treating her EBV and PTLD has failed. She has shown no clinical response since the Rituximab infusions commenced and her EBV count has continued to increase considerably. There were a few days where we thought we had some success as her bloods showed no signs of EBV. However, it turned out they were actually not HER bloods, but a different Ellie Carter, incorrectly entered into her profile. But we won’t talk too much about that. They were very quick to lodge an incident report, and we’re just thankful that no decisions were made to alter her treatment based on the incorrect information.
 
The good news (sort of) is that the results of the lymphoma biopsy confirmed PTLD, caused by her EBV, and not a more sinister type of cancer. Granted, the PTLD diagnosis isn’t good news but at least it isn’t a more sinister lymphoma which would be bad news. We’ll take what we can get at this point.
 
The new plan for PTLD treatment is the donor Cytotoxic T-Cell Lymphocyte (TCL) treatment. After speaking with one of the head oncologists, we’ve since learned that this is a real opportunity. I’m not aware of it being performed in Australia before and we only have access to it because our oncologist has some experience performing it in America, though as I understand, it’s only been performed in around 500 patients. Our oncologist was part of a study which used CTLs to treat EBV with good results. The theory is, the donor T-Cells will boost her immune system for a short period of time and target the EBV and PTLD, hopefully eradicating it. We have secured 6 batches of infusions which are given in 3-4 week intervals and the donor T-Cells eventually die after around 6-8 weeks. While an opportunity, it also comes with risk. Cytokine Release Syndrome (CRS) and GvHD are the main ones were concerned about. There’s also the risk that it doesn’t work which would make us almost out of options.
 
Since the first infusion of CTLs on Friday, Ellie has been highly febrile, has respiratory difficulty and significantly elevated CRP (inflammation) markers. She’s now reliant on oxygen and we had a scare yesterday morning which almost resulted in another ICU admission. She’s since stabilised but we don’t really know what the cause is. By now, we’re very used to being told ‘we don’t know why this is happening’. This particular incident could be the CTLs, PTLD, EBV, or a new underlying infection that is brewing. We’ve changed up her anti-biotics and anti-fungals again and added some broader-spectrum anti-biotics to be sure. So far, nothing has grown on her cultures, but a chest x-ray shows some minor collapsing of her lungs. This is likely the reason she is now reliant on oxygen however, we still don’t know the cause of the other reactions.
 
It’s a difficult time right now, and we can see Ellie deteriorating. We’re just hopeful that the CTLs have an effect and give Ellie a chance. If not, our last chance could be more chemo but I know there are serious questions about how much we could/should give her at this stage.
 
On a brighter note, Ed Sheeran visited the hospital yesterday. He wasn’t allowed to come up to Ellie’s isolation room and Ellie wasn’t allowed to go down to the Starlight Room to watch but the nurses arranged for him to say a few words to her. He said “a big hello to Ellie Carter, I’m sorry I couldn’t come up to see you but hope you’re feeling better soon” which was televised on the internal hospital TV network. Of course Kara took a video of it all, and then the video of Ellie and the nurses dancing to Ed Sheeran was sent back to the Starlight room for Ed to see and that was also televised on the hospital TV. Ellie was very chuffed by this and it put her in good spirits for the rest of the day. The ward was buzzing all afternoon.
 
While clinically, Ellie is not doing so well and she’s under High Acuity care, she’s surprisingly in good spirits. Since her pain and nausea are under control, she’s alert, talking, eating, and maintaining a sense of humour. Despite everything else going on around her, it makes a huge difference when she’s in good spirits. There are nurses on the ward who have literally never heard Ellie talk in the last 2 years and they’ve been coming into the room just to have a chat with her.
 
We continue to look for the silver linings, but they appear to be fewer and fewer. We’re doing our best to stay positive and are hopeful that CTL therapy will give Ellie the boost that she needs.  
 
 
PC[...]

It hasn’t been the start to 2023 that we had hoped for – with lots of ups and downs over the Christmas period and first few weeks of the new year.
 
As we know, Ellie has struggled with Graft vs Host Disease (GvHD) since around the time she was released from transplant. This has been treated with Prednisolone steroids and it has been our aim to wean her off the steroids as soon as we can due to the long-term complications associated. We have not been able to achieve this due to GvHD continually reoccurring. One of the major issues with continued steroid use is the prolonged weakened immune system. Prednisolone kills T-cells (which can cause GvHD) but it also doesn’t allow the immune system to recover. The longer Ellie doesn’t have an immune system, the more likely she will develop life-threatening infection or complications.
 
So here we are. Ellie has now been diagnosed with Post-Transplant Lymphoproliferative Disease (PTLD). It is essentially a form of lymphoma disease. While it is cancer, it’s caused by EBV and therefore different to most forms of cancerous lymphoma. (I don’t really know what this means but I think it sounds like a softer blow). She has dozens on PTLD lumps present in her neck, tonsils, and bowel. As I understand it, this is also not a relapse in that it is not AML. It's a different type of cancer, basically caused by the treatment for her other cancer (AML).
 
We have been tracking that Ellie has tested positive to Epstein Bar Virus (glandular fever) for a few months, but her levels weren’t significant enough to treat. Then about 2 weeks ago, her EBV levels spiked significantly. This was cause for concern, and the team ordered a CT scan, followed by PET scan. The PET scan confirmed PTLD with a significant number of lumps in her bowel, tonsils, and a large lump in her neck.
 
Unfortunately, many of her symptoms are consistent with both GvHD and PTLD (diarrhea, nausea, stomach pain, sore throat, skin rash etc). Ellie’s stomach pain has been extreme for a couple of months, so PTLD in her bowel makes a lot of sense. GvHD is much more common and therefore we have been treating her with Prednisolone. Also unfortunate is that the Prednisolone has essentially been working against her, allowing EBV and therefore PTLD to grow. It is likely the Prednisolone has been masking the growth of PTLD.
 
In the past, our oncologist has referred to this phase of treatment/management as a black art, because there is no play book and not enough data/evidence to support what we’re doing. We also know Ellie has had some rare complications, and our oncologist reinforced that we are again in ‘the rare of the rare’ territory. Engraftment syndrome, GvHD, EBV, and now PTLD is incredibly rare. There simply is no evidence to clearly guide us through the next steps.
 
I asked the oncologist if it was possible that Ellie has GvHD and PTLD. His response was “Yes, and if that’s the case, we’re in real trouble.” This would be a real problem as we’re about to stop the drugs that fight GvHD so we can commence the drugs that fight PTLD.
 
Notwithstanding, our oncologist has a plan – and a backup plan – like he always does.
 
Ellie will undergo four infusions of Rituximab over four weeks. Each infusion takes six hours and will destroy her B-Cells. Note that we have already destroyed her T-Cells with Prednisolone however, EBV apparently hides in the B-Cells. We will cease Prednisolone to try and grow her T-Cells again.
 
Ellie will also undergo EBV-cytotoxic T Lymphocyte (CTL) therapy. This is ‘kind of like another transplant but not really’. It’s a type of immunotherapy which ‘transplants’ donor-matched T-Cells to fight PTLD. Again, not much data available to support but apparently, it’s the new thing to try. We’ve been told we don’t know what the optimum dose is, but we’re going to get her as much of the good stuff as we can. Apparently, there is a donor-matched quantity in a Brisbane donor bank and we’ve placed an order for as much as they’ve got.
 
The back-up plan is to have an operation to remove the large PTLD lump in Ellie’s neck for the purpose of biopsy and tissue testing. This will likely happen in the next week. It won’t necessarily change the way we immediately treat however, if the treatment plan fails, then we can confirm the exact DNA makeup of the cancer and therefore treat with the correct chemotherapy. We hope it doesn’t come to that, as it will be another very delicate dance, given the extent of toxicity already administered over the course of the last 2 years. The tissue testing will also be used in (yet another) ‘Rare Cancer Sub-study’ to better understand what causes and how to treat rare cancers.
 
Risks? Yep, lots. All the normal stuff – none of which we really want to think about. But at this stage, we just need to try to remain positive and try everything that’s available. As Kara said in her last post, we are ever grateful to kiss and hug Ellie, and have her home with us every day that we can.
 
PC[...]