On the 2nd June 2018, in what seems like a different life my wife Rebecca, Esme my 22 month old daughter and I flew out to a resort in Thessaloniki, Greece for a much-needed, long-awaited weeks holiday. The day before we flew Esme had taken a tumble and we were becoming alarmed at the disproportionate size of the bruising that followed. In hindsight this followed a pattern of ‘easy bruising’ and on our first night, instead of relaxing over a bottle of wine, we began a Google search that ended in a cold sweat for the both of us...
On our second day in Greece we booked a doctor-on-demand Skype call and found ourselves falling through a series of trap doors from the resort doctor’s to blood tests at a regional hospital and the irreversible moment in time we were taken into the office of a Paediatric Consultant and heard the word ‘leukaemia’.
We spent our second night in tears and in a Greek hospital with Esme on a drip next to us. On what should have been our third day on holiday we took emergency flights back to London. In my hand luggage I carried a hypodermic needle, an adrenaline shot and an oxygen mask kit given to me in case Esme showed any further reaction to the platelets transfusion she’d been given that morning to be ‘fit to fly’.
We had no idea whether we needed to be ‘blue-lighted’ from the tarmac but in the end Esme’s auntie Emily had organised a referral direct to King’s kids’ A&E and we drove with Esme’s auntie and uncle to Denmark Hill. Initially King’s seemed to agree with the Greek diagnosis; that Esme had Acute Lymphoblastic Leukaemia which has an 80% cure rate, and an even higher one for little girls. However, on our second day at King’s we were told that the Royal Marsden (our onward destination) thought Esme had something called Acute Myeloid Leukaemia. None of this made any sense to Rebecca or I (thinking back I misheard and thought the hematologist said ‘mild leukaemia’) but we listened with fresh fear to the fact that AML is more difficult to treat and has a 60% cure rate, subject to your exact type.
By the time we arrived at the Royal Marsden we knew that AML necessitates an intense six month in-patient treatment plan to induce and consolidate remission. Given the horrible anxiety and trepidation that every procedure evoked, it seems trite to recall things so briefly now but within three days Esme had had a central venous line fitted beneath her torso, enabling drugs and bloods to be administered without needles, a lumbar puncture to inject a precautionary shot of chemo into her central nervous system and a bone marrow biopsy to understand the exact nature of the leukaemia in her blood factory. Esme also had the first of twenty five nasal-gastric feeding tubes run down her nostril into her stomach.
By the Saturday of the first week Esme had started the first block of three drugs that comprised her first round of induction chemo. Writing this now some nine months later, it all feels like a blur but I remember the vomiting that accompanied the chemo and the naivety of our question to the consultants as to whether Esme had maybe escaped any major side-effects as none had presented during treatment. We didn’t appreciate then as we do now how much chemotherapy wipes out all fast growing cells in the body (hair, mucosa) as well as the cancerous cells leading to a state of “neutropenia” in which the body’s white blood cell army is suppressed and the body exposed to increased risk of infection from all quarters.
By and large, Esme bulled through her treatment with the courage of an SAS trooper. Before diagnosis she’d chosen a hand puppet lion by the name of RaRa as her cuddly and she would take delight in roaring at the nurses and doctors with her faithful sidekick, as well as learning to take her own bloods and change her own dressing to the amazement of seasoned staff.
After the first round of chemo we had two pieces of widely contrasting news; we learnt first that Esme’s leukaemia had responded to the chemo and our little warrior was in technical remission; however, at the same time we learnt that the lead test laboratory had identified a signature genetic marker in Esme’s cancer (NUP98-KDM5A) that meant it had an especially high risk of both non-response to treatment and relapse. It was around this point, after a meeting in the adults’ quiet room where our lead consultant advised Esme’s prognosis was something like 30%, that I gave up on the idea of juggling care for Esme with my job (which had been running an online start-up and had been left open for me to return to by the founders).
Rebecca and I had somehow always imagined that supporting a sick child in hospital would be a relatively calm, sedentary experience at the bedside, filled with passing the time activities. Having spent 5 months on the McElwain ward we now know the 24/7 raging stresses associated with trying to wheel a toddler around a playroom with a drip stand, encourage a child to take on nutrients when their weight is plummeting and they have no appetite, or trying to sleep when your baby vomits repeatedly through the night or the stranger’s child in next bed screams out in their sleep from pain.
By the second round of chemotherapy we knew Esme was tracking towards a stem cell transplant in first remission and had become relatively seasoned in dealing with the side effects from the drugs mainly thanks to Rebecca’s endless quest to mitigate Esme’s pain, rashes and other discomfort. Different patients respond differently to chemo but Esme’s biggest cross to bear was the load it placed on her lower digestive tract. Every poo became an agony and we rotated through a bewildering range of antispasmodics, laxatives and painkillers. After Esme’s second round of chemo, we were advised that, blood counts permitting, we could expect overnight home leave (something we failed to achieve in round one) but this time round Esme picked up bug after bug including an incidence of acute sepsis on my birthday that nearly required removal to the intensive care unit at St George’s.
However, by the end of August after living day by day some plans were falling into place; Esme was in full remission after her second round; we had a date and (an umbilical cord) donor set for transplant; and, wonder of wonders, Esme’s eating and fluid intake via the feeding tube had picked up to the extent that we were able to go home for a handful of days pre transplant.
This was also a sad time for us as we’d come to love and trust the amazing team at the Marsden but more so Rebecca and I had operated in the tightest of bubbles with Emily, Rebecca’s sister, and work-permitting her partner Mikey, but Emily now had to go back into her role as an early years teacher from September and so we knew from neutropenia perspective we’d see a lot less of her.
After a third round of intense “conditioning” chemotherapy, Esme received her new donor cells on 27th September. These were a ‘seven out of eight’ match and belonged to the umbilical cord of a baby boy born fourteen years earlier and cryo-preserved by the amazing Anthony Nolan charity.
Esme handled her transplant with all the fortitude and insane bravery she’d shown previously and we all enjoyed the fact that we had the (relative) privacy of our own room for the first time in four months. The flip side now was the challenge in keeping a curious two year old occupied across a three week period of enforced isolation. Aside from the momentous day in which the actual cell donation took place, we now mainly remember the week we were allowed to first escape from confinement and resume our relentless adventuring around the Marsden’s various car parks, lawns and walkways.
By the start of November, despite a horrific case of anaphylactic shock to a new medicine which brought a crash team of 30 staff into our room on the eve of departure, we were cleared for a permanent discharge and moved out of our second home armed with a black bin liner of drugs and syringes to face a new challenge…
Initially looking after a child at home coming out of a bone marrow transplant is terrifying not least because you don’t have a bell within reach to call a nurse. Steadily, however, Rebecca and I became proficient in administering the dozen or so meds and gradually the days rolled over and returning to outpatients at the Marsden felt less like going ‘home’. Within two weeks, despite all the trauma she’d endured, Esme announced she wanted to sleep in her own room - much to our surprise but testament to her unending courage.
With transplants come a bunch of important milestones as for every day that passes the risk of transplant failure or disease relapse diminishes. After a memorable Christmas at home (which tested our new regime of alcohol, gluten, sugar and dairy-free living!) we reached Day 100 on 5th January. Celebrating with Esme’s favourite auntie Emily we took relief in the fact that that statistically this now meant Esme was less likely to suffer from acute graft versus host disease, a complication of transplant where the donor immune cells attack their new host organs.
Due to the prolonged risk of re-admission from exposure of a weakened immune system to everyday colds and coughs, as a family we followed a groundhog day routine of days out across South London parks interspersed with community nurse visits and outpatient trips. The consultants were delighted with Esme’s progress throughout and were particularly impressed that the “chimerism” score (which tracked the percentage of original recipient cells versus new donor cells) was 100% in the bone marrow.
Two days after Esme’s six month, 180 day milestone post transplant we put Essie through a planned bone marrow under general. If the results from this were as expected, we’d be talking next about removing her Hickman line and feeding tube in the Spring to get even more back to ‘normal’.
As any oncology parent will know, the “scanxiety” from waiting on results is one of the worst emotional tests that comes with childhood cancer, however, as the first three tests came back clear we began to believe the fourth but most definitive test would be the same.
Instead in early April we were invited to the Marsden to be told that the signature NUP98-KDM5A marker is back and that a relapse will inevitably follow. Looking at our daughter at her fittest, bubbliest and happiest in months, none of this makes any sense but equally for me it’s the demon that’s come to me in sleepless nights since diagnosis and is now a reality..
Having been told all along that a second transplant would be the fallback option for a failed first cord transplant, we have now learnt that the Marsden, to whom we have inexpressible gratitude for their expertise and care, are sadly more than likely blocked from supporting a second transplant until 12 months from the first due to NHS England policy (https://www.anthonynolan.org/8-ways-you-could-save-life/campaign-us/defend-second-transplants).
Whilst we understand this policy from our extensive conversations with AML experts in the US, we know that for Esme the best course of action is try to get to a second transplant as soon as possible, rather than bide our time for 7 months with maintenance chemotherapy to try and keep the disease at bay.
This is why we have taken the decision now to fund raise to give our little lion cub the best shot at a lasting cure. Esme has inspired us every day of her life with her lion-like bravery and her zest for life. We invite you to join Esme’s pride of supporters and move mountains to give her a second chance.