This is the question that has haunted us throughout - what will the next step be? (Is it right? What are the benefits and risks? How will Elin cope with it? What is the evidence behind it? What are our options? Do we have any options?)
This website was written at a time when Elin's situation was so desperate we were told there was nothing else that could help her. We had no options in this country after whole lung radiotherapy. I sat in a dark room through sleepless nights with the glow of the computer screen for company, searching for possible paths through research papers, studies and trials - and the practicalities of it all also led me to guides that helped the technically challenged create a website (this is it, mark II).
Those who have been on this website before will know that this is the page that has needed the most updating (aside from the astounding fundraising events page). Elin's treatment has progressed, faltered and progressed again - and we are so grateful to the teams in London and Brighton who have kept Elin so healthy despite her illness. Doctors and nurses with intuitive understanding, patience, kindness and huge expertise have helped us all when dealing with the day to day and the questions that keep us awake at night.
When the appeal began, the only option we found that was suitable for Elin was a drug therapy in Bethesda, Maryland, and we set up specific links with the team there. This was the treatment described as one on the most promising agents at a research summit in 2011, and was the only one available to Elin; she went through whole lung radiotherapy in London in May 2013 and we then braced ourselves for the scan results. Progression would mean a decision over whether to move to such a treatment, outside the UK system.
Scan results remained steady and we continued to research treatments over the spring and summer. The time that passed was crucial; a meeting with a Research Professor revealed that things were starting to open up after what he described as 'a drought'. We also started building links with a doctor at MD Anderson in Texas, through a family from Jersey with experience of Ewing's Sarcoma - we will be forever grateful they took the time to get in touch with us, as this became such an important link for us.
This brings things to the point I put the first update on here, in August 2013. Scroll down for the most recent updates - please read in the knowledge that this appeal helps us get through every day, as it means we have choice and hope. I am ever conscious that my ramblings fail to convey how significant you all are in supporting us, but please know I thank you silently every day. In awe of the strength I see daily in my daughter (and her little sis) and ever thankful to so many.
If you think you could organise a fundraiser to help our appeal, we would love to hear from you!
Thinking of doing a sporting event soon? You could get sponsored...
Work for a company that could support us with raffle prizes or a donation?
Would a 'non-uniform'/themed day be possible at your place of work?
Are you creative? Could you sell/donate a piece of your work in aid of the appeal?
We would love to hold an auction of celebrity items - can anyone help with this?
Any ideas/plans/events to publicise, please email us at email@example.com.
We met Carol and Christian Keenan in our lovely NCT group in Jersey, shortly before Elin was born. Christian is a wonderful photographer and came to stay with us in July 2013 for a few days. His pictures are so precious to us and capture that summer.
The pictures below are from our time in North Carolina for Elin's treatment at Levine with Dr Pete Anderson, August 2014. Thank you to Ronald McDonald House of Charlotte for the lovely park pictures just before we flew home.
The following pictures are from Jan 2014, when very special family friends the Zwalfs stayed with us for a weekend - when Elin was on the up. Freya is Elin's oldest friend and her mum Katie takes amazing pictures! Thank you Katie x
UPDATE: AUG 2013
Elin's first scan after radiotherapy showed an encouraging response: the scan report states that there is 'significant improvement in the size and appearance of bilateral lung metastasis'. Elin is fit and strong at the moment and the radiotherapy has allowed her to have the most normal summer she has had since she was 5. Incredibly special - and not a hospital in sight since post-radiotherapy treatment. She can also start the school term with her little sister who is now reception age - a moment the two of them have been looking forward to since they have been old enough to talk about it (a teary moment for Martin and I when we realised it could happen).
We now go scan to scan as we wait to see how long the effects of the radiotherapy will last. The next scan result is in November. Thanks to the completely incredible support of wonderful family, friends, neighbours, colleagues, companies and the unbelievable kindness of people we have yet to meet, we have now raised a sum we didn't dare dream was possible when we began the appeal in May this year. This fundraising means we are now able to choose to travel and commence treatment as soon as needed, and gives support to further work into treatments for Ewing's Sarcoma. We are completely humbled by the incredible generosity and kindness you have shown our family in support of our beautiful girl.
UPDATE: NOV 2013
We have received the devastating news that the disease in Elin's lungs has progressed. Elin was also examined - and despite the awful news, we are so relieved that she continues to be symptom-free. She is as smiley, chatty, active and busy as ever. Very hard to match up the scan result with our fit and seemingly healthy and strong daughter - and even harder to have to tell her she needs more treatment.
Our minds were spinning so much in the clinic appointment it took a moment for everything to sink in - the drug trials in the UK that we had discussed in a recent meeting have not yet started, so thoughts immediately went to booking flights. However, the consultant surprised us by introducing the names of two chemotherapy drugs that have recently been approved for UK use in this situation - and the recommendation from the sarcoma team in London is that they are the best option for Elin at this point.
We didn't make a decision immediately, but took away the information detailing the effects of Gemcitabine and Docetaxel and the trial data that orginated from Spain. The rarity of being at this treatment stage for Ewing's is reflected in the fact the trial data was only based on 10 patients. Even though the sample size is tiny, the results for a sizeable proportion were encouraging and the side effects similar to those Elin had faced before. Second opinions from the US were positive and supported this line of treatment, which also helped firm up our decision to start 2x three week cycles.
We also needed to make a decision about how the infusion would be given - having another picc line put in her arm would mean Elin would no longer be able to swim, which she loves. We looked into a portacath as an alternative - an implant in the chest which needs a little butterfly needle to access it, but swimming is possible. We decided to let Elin decide, and a child-friendly book suggested by Brighton Hospital really helped her (and us). Elin amazed us yet again with the speed with which she accepted things after initial tears and anger - her strength puts us to shame as it's hard to adjust to all this again. Elin saw the benefits of the portacath pretty quickly so a surgery date was set up a few days later, on November the 11th.
This surgery in London a week ago seemed to go well, though it was all quite sore and bulky as the port 'access' (needle and line) was left in, ready for the first chemo on Thursday the 14th. It is given over a couple of hours so
Elin was just a day patient for the chemo - and whilst she was there, managed to go to hospital school and also make a papier mache toadstool for her new toy fairy (we had taken a little distraction detour to Hamleys on the way to the hospital). No doubting Elin's order of preference though, despite distraction buying: Reddy (beloved ted) is still very much number one.
Elin and Reddy here, at hospital school:
The next London trip is this Thursday for a longer chemotherapy infusion. Desperately sad Elin will lose her hair again. More tears (from both of us) over this - however, she has already picked out hats and headscarves in a rainbow of colours. And is busy at school today. So proud of her.
UPDATE: JUNE/JULY 2014
Elin's response to Gemcitabine and Docetaxel has been nothing short of incredible. She has tolerated it extremely well, managing to get to school everyday with just a handful of exceptions, and going to morning lessons on the days that she has chemo in the afternoons. Treatment has been in Brighton - the intitial cycles in London went well, so treatment was moved closer to home - and immune system support through injections of GCSF avoided extra trips to the hospital, as we were lucky not to have to tackle any infections.
That just gives half the picture though - the consultant's use of the word 'incredible' relates to the scans we started to see as the cycles went on. The lung lesions started shrinking, and the response continued and continued. The planned cycles were completed, but a response still showed, which resulted in the decision to extend this treatment for as long as possible. As a result of Elin's response, new options started opening up; after several months, we were stunned to receive the news that surgery and high dose chemo with stem cell rescue were now being considered for Elin.
Subsequent multi-disciplinary team meetings removed the option of surgery, as it was felt that the position of the areas to be removed deemed them unsuitable for surgery and the related risks were stacked against the benefits. However, the high dose chemotherapy route (with stem cell rescue) remained, as a paper had recently been published that supported the use of high dose therapy for patients who had a complete OR partial response to prior treatment. Before this paper, research findings had suggested that high dose chemo was only a viable option for those with a complete response, with no measurable disease remaining. Elin's response had been partial.
Our difficulty with this lay in the ambiguity of the paper's findings, as there was no differentiation between the partial/complete response patients in the discussion and assessment of the outcomes. High dose treatment carries significant risks, and the weight of past research (added to the fact this recent paper did not allow a clear view of outcomes for those with Elin's profile) increased our concern. In meetings in London, this treatment was starting to be discussed as a possible cure; this was beyond wonderful but our concern was heightened by other medical opinions stating high dose would not be considered for those with measurable disease remaining.
In the midst of this, Elin's blood results started to come back with worryingly low platelet levels - a sign that Elin's bone marrow was getting tired after 12 cycles of Gemcitabine and Docetaxel. The beginning of the summer holidays brought a flurry of meetings and several crucial decisions.
UPDATE: JULY/AUG 2014
We sought different opinions about the next step, and had been reading research papers about an approach called stereotactic radiotherapy. The targeted radiotherapy focuses on the areas of measurable disease, using fractionated beams to reduce the effect to the surrounding areas in the lung. Each beam is a fraction of the total dose in the centre, and the treatment usually takes place daily, over approximately one week. Our reading of the papers was very encouraging as this was a treatment that would remove measurable disease with minimal discomfort for Elin, improving her situation and allowing further consideration to be given to future high dose chemo and stem cell rescue.
Treatment of all of the remaining lesions in this way was not available to us in the UK, so we took the decision to travel to Charlotte, North Carolina on 29th July, for a consultation with Dr Pete Anderson, who had now moved from MD Anderson, and Dr Crimaldi, Radiation Oncologist. We had booked return flights for the 3rd August; however, after many meetings, calls and discussions, we decided to alter our flights to allow for an immediate start to stereotactic radiotherapy at Levine Children's Hospital and Cancer Institute.
Planning started on the 1st August, and treatment started on the 7th August, hourly for 6 days, with a weekend break in the middle. The kindness, care and compassion shown towards us by everyone involved in the planning and treatment was just overwhelming, and they could not have done more to make Elin feel comfortable and at ease throughout. Elin was so taken with Dr Anderson that we now even have a doll that looks uncannily like him, spotted in a shop by Elin and purchased at her request!
Our extended time in Charlotte was made all the more homely by the kindness of my uncle (who lives a couple of hours away, opened up his house to us and also generously lent us his car) and the Ronald McDonald House of Charlotte - two minutes from the hospital and where we were so lucky to be able to stay during Elin's treatment. Elin and Nia loved being able to see family in the midst of all the changes, and we were all quite overwhelmed by the extent of the support and kindness we were all shown, everyday. If anyone we met during our time in Charlotte is reading this, please know you made the world of difference!
We flew home the day after treatment finished, arriving back on August 16th. We are now looking at the question of how to deal with the disease that you can't see - such is the nature of Ewing's. But, just now, Elin is busy catching up with friends, planning sleepovers, going swimming and gearing up for school (she is inordinately proud of her shiny new school shoes, purchased yesterday!).
UPDATE: OCTOBER 2014
Having returned from Charlotte, planning and scan information from the US went immediately to London, to move forward with Elin's treatment. Consultations at Great Ormond Street followed and the beginning of September was set for the next stage of treatment: high dose chemotherapy with stem cell rescue. Elin had her stem cells harvested during the first stage of her treatment, when she was five; high dose treatment involves giving a huge chemotherapy dose over several days, then returning Elin's stem cells to her to allow her bone marrow to reconstruct itself over the following month to six weeks. It would mean an inpatient stay throughout this time.
Through the end of August and beginning of September, we prepared ourselves for this aggressive course of chemotherapy. Elin went through an intensive series of tests and scans, and had her port (definitely our line of choice) changed for a hickman line - as this was necessary for this type of treatment. As parents we had sleepless nights over the severity of the planned treatment and the side effects, but this was a possible way out of the nightmare of Ewing's - and Elin made it clear to us that she wanted to go through this if it could put a stop to all the years of hospitals.
We busied ourselves with finding ways to reduce the anticipated side effects and sourcing things to allow her to continue eating through the six weeks (Elin's perennial refusal of hospital food and the severe limitations of the parents' kitchen - one microwave we were not allowed to use for Elin - meant that we were looking at not being able to feed her hot food for the duration of the stay). After looking at what we could bring in ourselves, I was all set with a little crockpot rice cooker that, as well as the obvious, could steam fish and veg, and warm soup... Paediatric oncology care is so thoughtful and child-centred in so many ways but the food aspect has always been tricky (and Elin is not a picky eater).
Elin and I also sat down at the computer and went through the wonderful Djeco arts and crafts packs and ordered a batch to keep hospital boredom at bay... friends recommended apps and I refreshed our iPad and we packed ready for our stay. The final afternoon before admission came round, and I phoned the ward to check the time of our admission. Quickly, our world unravelled. Something wasn't right - the ward could not confirm anything and we immediately contacted the consultant's secretary which lead to painful evening of waiting to speak to the consultant. The conversation later confirmed our developing fears: the scan of Elin's lungs showed two new lesions, and as a result high dose treatment could not commence as the risks were too high.
I can't really put into words what this news has done to us, but we are still trying to pick ourselves back up. Elin is devastated that there is now no end in sight, but as parents we feel the gut-wrenching panic that there is no obvious way in sight. Elin needs more treatment but the consultants have no further approved treatments here to offer her.
We are now looking at trials at the Royal Marsden. We have had meetings with the wonderful team up there, and a clearer path is forming as our hope grows for the two drug options that could help Elin. The places on these trials are not currently available, so Elin has just finished a course of 'holding' chemo at Brighton - the same combination that she was on earlier this year.
To look at Elin, you would not believe all this was happening. Aside from the early September trips to GOSH and now one afternoon a week for chemo, she has not missed a second of school (and adores her lovely new teacher). We are waiting on news from the Marsden, and are continuing to explore any other options here and abroad. I hope that we will be in a clearer position by the end of half-term - but in the meantime I have also been trying to make sure that we have lots of fun things going on for the holidays (the highlight of which is a last minute booking to 'Wicked'... looking forward to her reaction...!)
Thanks as ever for all your lovely messages through the Facebook page - actually we have two (I made a community page long ago, but I'm not convinced we need it) so find the page with the picture of Elin with the butterfly facepaint, if you would like to link in with us there. Thanks again x
UPDATE: DECEMBER 2014
During October and November we researched the trial options - and the Marsden research team started speaking about the possibility of a new cohort opening for a drug called Regorafenib. This was the first paediatric solid tumours trial for this drug but it had already been approved for certain adult cancer treatments; international opinions were very positive and its method of action through targeting cells and inhibiting cell growth in multiple ways gave us huge hope. Elin was at the top of the waiting list in the UK for a trial place, but we went through a very difficult period of waiting as nothing was currently open. After two cycles of holding chemo, we received the news mid-November that a new cohort of places had been approved for the Regorafenib trial we had been waiting for.
I am sketching out the events in a way that glances over the heart of the decision Martin and I faced, as I'm afraid I cannot begin to convey the soul-searching, research and worry here. Please forgive my listing of events but I hope you can understand the reason.
We signed consent forms on November 24th, and Elin went through screening tests and scans on the same day at the Marsden. Displaying no symptoms of any chest difficulties aside from a slight soreness in her side and with a performance score reflecting her ability to do normal daily activities of 100%, she looked to be in a good position for starting on Wednesday 26th. Screening tests all went well, aside from a slight concern over her temperature which was 37.6. For someone in Elin's position, hospitalisation is needed for a possible infection when the temperature reaches 38, so this temperature reading started some alarm bells.
Unfortunately, on the way home from the Marsden Elin started feeling unwell, and her temperature rose to 38 later that evening so we were home for just a few hours before going to Brighton hospital. The temperature stayed high for days and it was Friday before Elin could return home, with the infection strain identified as parainfluenza. Chest symptoms had become apparent and the coughing very tiring. Our return to the Marsden was rescheduled and screening redone on Monday December 1st - and it was confirmed we could begin on the 2nd.
Regorafenib is a tablet dose (once a day for 21 days out of 28) which has huge benefits in that it can be administered at home - but we needed to overcome Elin's huge difficulty with tablets. Despite all the years of treatment, she had never been able to overcome this... so November was all about preparing for this. We found out about different approaches and friends sent some really helpful suggestions - our winning combination involved working through different sized cake decorations and sweets (up to tic-tacs) using a lidded cup with a long spout filled with apple juice! But the main thing was Elin's incredible positivity and bravery in overcoming yet another thing she feared.
So the day came when Elin had to take the first tablets - and as soon as she was without an audience, she took them within minutes (and now you would never know she ever had any difficulty as she is so speedy).The first two doses had to be taken at the Marsden with a night of observation in-between; we returned home on Wednesday night, only for Elin's temperature to suddenly rise again on Thursday morning. A swift return to Brighton hospital followed, where Elin then stayed for another week.
Elin is now home - and was so keen to return to school that she went in for an hour shortly after being discharged on Wednesday (with the doctor's go-ahead). She had been so down about being away from her friends and normality that time in school has become the most important thing for Elin - and her teachers and classmates have been simply amazing with the practical aspects in helping to make this happen. So happy we are all home as a four again, and hoping things can be a little gentler in the run up to Christmas.
UPDATE: JANUARY 2015
After a very difficult first cycle (mainly spent in Brighton hospital), Elin bounced back in her week off treatment - which, happily, was also Christmas week. We had a wonderful cosy Christmas at home as a family (relieved beyond words Elin's temperature stayed down) and treatment resumed on December 30th. This second cycle commenced at a reduced dose, in a bid to avoid the side effects of the high initial dose.
In December we feared the disease was galloping away from us before the new drug had a chance to act; added to this, Elin's breathing had become so difficult, she was struggling to eat and life had become confined to hospitals. Therefore, to be together with Elin back at home over Christmas and the new year - and then to see Elin start the new dose and see none of the negative effects... we hardly dared to believe things were starting to right themselves and life was returning to an even keel.
And term restarted - and Elin returned to school! Her breathing and appetite have both steadily improved, and she has now even returned to her skating club on Monday evenings! She finished cycle 2 on Monday 19th Jan, and the CT scan results show that things are now stable as a result of the new treatment, and the prior infection has cleared. And her blood results are fantastic (at the moment! touch wood!). So we hardly dare breathe with happiness at the semi-normality of life at the moment, and the fact that this now very manageable treatment (under the amazing team at the Marsden) can continue until our next scan in March.
Thank you for your amazing support - and all the incredible event-planning, training and collecting going on for the appeal and ESRT. The last few months have shown us the vital importance of research into new treatments that go beyond the current protocols for Ewing's. New treatments that mean children and young people like Elin can go out there and do things kids do!
UPDATE: APRIL 2015
After 4 cycles of Regorafenib, Elin had a CT scan in March. We went into the clinic day steeling ourselves for the scan report meeting, but with cautious hope that we were continuing to make headway on this treatment. We read positives into the build-up to the scan as Elin had been so incredibly well, lively and pain-free in the preceding week - added to the fact that the scan was set for the day we were due to start the next cycle (which we hoped was a note of optimism from the hospital). However, the hours that passed in the play area waiting room gave rise to alarm bells and when we were called we knew immediately.
The CT scan showed things were no longer stable and Regorafenib needed to stop. We struggled to adjust to this, but talked though the status of a trial treatment running in parallel - a drug we started reading about almost two years ago. After a washout period of 4 weeks, Elin could be screened to check her eligibility for Abraxane - a newer version in the same family as a chemotherapy drug that worked so well for Elin in 2013/14.
And so the waiting 'washout' period began, and we filled the Easter holidays with catching up with friends and grandparents, and doing some lovely things that the girls had been hinting about for a little while (like a return trip to 'Wicked' in London and a quick trip to Norfolk to see William the horse and for a run around 'Bewilderwood'). I worried that I had overdone things for Elin with these trips - so difficult to get the balance right - but a proper break from hospital trips and treatment for a few weeks did us all a bit of good.
Scans and screening then started on April 9th and eligibility was confirmed on Monday 13th. Treatment started at the Marsden the next day, four years to the day since Elin's initial diagnosis. This treatment is an infusion, given through her central line over half an hour; we stayed overnight for a series of blood tests and for observation and returned yesterday afternoon (home to a beautifully sunny garden and ice lollies on the grass).
Elin will need to go to the Marsden every Tuesday for three weeks out of every four to receive this treatment; early days but it has been a relatively gentle start with no obvious side effects (and Elin has already squeezed in a bit of school this morning). Elin asked me yesterday how many weeks are left until the summer holidays; my answer was very disappointing for the opposite reason to the one you might expect(!)... most are counting down the weeks. We are hoping her counts remain strong enough for her amazing attendance to continue - hospitals can be forgotten when she is there.
Next scan will be early summer so hopefully we can all enjoy a gentle spring. Enjoy the sunshine days wherever you are! Thanks all, as ever x
It is just a few short weeks since I wrote the last piece but things have changed so quickly. Elin finished the first course of Abraxane at the end of April but things suddenly started to change towards the end of this first cycle. Elin is having to cope with so much. Her fiercely independent spirit makes us so so proud and her inner strength is utterly inspiring. We are heartbroken to be here, and are trying to find some of that strength as we face these impossible days.
If you would like to support our appeal, you can donate through our Virgin Money Giving page - and please choose gift aid if it applies.
If you have organised an event and would like to transfer what you have raised, you can find the details on our 'How to Help' page. You can also contact us directly via email at firstname.lastname@example.org.
We are so grateful for any support you are able to give. Thank you.