Please find below a link to a summary of treatment provided by Sheffield Children's Hospital up to mid September 2012.
Lex Treatment Summary.docx
Since this letter Alexander's consultant further advised the following at the end of Oct. 2012:
1) Alexander has become symptomatic of bony disease in right pelvis/femur, requiring opiate analgesia and bony swelling on forehead
2) Started oral etoposide, with response in terms of decreased forehead swelling and less pain.
3) Reviewed by Dr Gaze re: radioisotopes. MIBG not indicated due to disparity between MIBG (low level uptake) and FDG-PET (widespread uptake) Awaiting Gallium scan to decide whether potentially eligible for Lu-Dototate therapy, although this is shortly to be subject of Phase I trial and Alex will not currently meet entry criteria due to thrombocytopenia post BuMel.
4) Alexander had a place on our infusional anti-GD2 Ab trial, which has been lost due to disease progression, but which may be an option again if he enters a further remission.
5) We do have stem cells in storage, sufficient for one myeloablative procedure, or as marrow support for other treatment options.
The above was followed by LuDo treatment in December 2012 after uptake was found to be acceptable/good. Unfortunately Alexanders disease progressed through LuDo resulting in the second course planned for end of Jan. 2013 being cancelled.
The Start- December 2009
I (Mark) arrived home from working overseas on the 13th December to take the family away to Lapland for a pre-Christmas treat. On arrival at Humberside airport I was meet by the whole family, as I always was, but this time there was a difference. Max had noticed that Alexander hadn't been himself during the previous week or so, and had spent considerable time in pain, even to the point he couldn't walk. Max had therefore booked a doctors appointment for that afternoon, which I didn't understand as Alexander looked well, was happy, and seemed to be his normal smiley self......
We all went to see the local doctor as arranged and after a short consultation Alex was referred to Doncaster Royal Infirmary. After waiting for what seemed like forever Alexander was finally seen by a doctor who advised that she could feel a lump in his belly, but as the registrar was not available we should go home and go back to the local doctors in the morning.
The next morning Max took Olivia to school and then took Alex back to the Doctors. After a further examination the Doctor advised that she would speak to Sheffield Children's Hospital (SCH) regarding a referral. At that point Alex seemed his normal self so Max took him to school and Max & I sat waiting to hear back regarding SCH. An hour later we received a call from the doctors asking us to pick-up a letter from the surgery and take Alexander immediately to Sheffield. At that point the whole thing seemed surreal and a confused mess, but we did as asked, not really knowing what to think or do. On the way to Sheffield Max read the accompanying letter from their local doctor to SCH and no words can describe what it contained- 'Alexander has a possible malignant tumor'!
We arrived at SCH around 1230hrs that afternoon and Alexander immediately started tests, which were completed around 1700hrs. The results were a parents worse nightmare, but not unexpected. The Consultant confirmed that Alexander had a possible malignant tumour, and advised us to go home and wait for news after the staff had had time to digest the results and agree the way forward. The hospital provided an update the next day and the following Thursday, the 15th December, Alexander undertook an exploratory operation during which biopsies were taken for further analysis. The surgeon again confirmed that the operation had shown Alex had a tumor, but was unable to tell us more at that point until the results of the biopsy were known. As little else could be done at that stage, and as we were in a state of total shock, we were advised by the hospital to still travel away on holiday, and make the best of the situation. With a lot of persuasion and support from SCH, including obtaining specialist cancer insurance only hours before we traveled, we were finally persuaded to take the children away, which we did. As can be imagined, the time away was a blur and Alexander needed constant pain relief. but he appeared to enjoy himself, as best he could. Olivia kept him perky and she seemed to enjoy her time away.
We arrived back home from Lapland on the 23rd December and went for the test results on the 24th. The results were BAD, 'Alexander has a children's cancer called Neuroblastoma'!
We did lots of research into Alexander's condition over the Christmas and New Year period but nothing could prepare us for what we had learnt or would be told:
Cancer in Children is rare, and Neuroblastoma is very rare. Only children suffer with Neuroblastoma, and normally only up to the age of 5. Alexander was 5 the previous February. Neuroblastoma has the largest fatality rate of children's cancers, and as its so rare there is not as much known about the disease as some of the more common types of cancers. Survival rates for children with cancer have improved dramatically over the past 10 years, and long term survival (still alive after 5 years) is now around 70%. Neuroblastoma lags this statistic and stands at a little over 60% if you are one of the lucky ones that do not have the higher risk categories, when the chances of long term survival drop significantly.
The start of 2010 saw Alexander have approx. 6 weeks of tests, including various scans, blood and urine tests, and bone marrow extractions, etc. This was followed by Chemotherapy in February that lasted until May. Yet more scans followed in June to determine the impact of the chemotherapy, and to provide a base-line for any future treatment. Unfortunately the Chemotherapy failed to shrink the original tumor so another biopsy was organised for July as the hospital needed additional samples for testing. This was carried out in July and a routine biopsy operation took approximately 6 hours when it was originally planned for a couple. Fortunately, the surgeons found that the majority of the tumor was operable so took the opportunity to remove approx. 90% of the growth. The remaining tumor parts remained inoperable due to their locations close to or surrounding vital organs, but the removal of a 'small grapefruit' from Alexander was a welcome bonus that no one had expected.
The biopsy results followed, and it was found that the tumor was active and still growing, but very slowly. The Doctors could not determine just how long Alexander had had the growth but they did advise that it was possible it had been with him since birth. Not the news we wanted, but at least there was no immediate danger!
Next followed 3 weeks of Radiotherapy in August, with repeat scans in November. Unfortunately we were told early December that the cancer was still growing.
Alexander started 2011 with 4 months of Chemotherapy followed by more scans in June. The scans showed that the cancer was still active but that there was no noticeable increase in size. Alexander's Consultant therefore decided to delay further treatment and wait for a period before having more scans to establish if there had been any growth. Scans were again undertaken in December and we received the news we wanted....'There is no noticeable increase in size'. For the first time since 2009 the Strong family had little to worry about and had the 'best Christmas ever'.
On the 18th Jan. Alexander started a mild form of out-patient Chemotherapy as a precautionary measure to help inhibit any new growth. Compared to previous treatment this was a 'doddle', and apart from a few trips to Sheffield the Strong household was returning to some sort of normality. That was until a lump appeared under Alexander's left armpit at the end of January but at that time the hospital didn't seem too concerned and put it down to an infection and administered antibiotics. The lump continued to grow and became 'substantial' mid February so Alexander was examined by the Consultant and his surgical staff, and a biopsy was taken mid Feb. The biopsy found that the lump was Neuroblastoma. They also advised that the Neuroblastoma had spread to Alexander's Lymph nodes and other parts of the upper body via the Lymphatic system, including his bone marrow.
For those of you that don't know, the lymphatic system is part of the immune system and is distributed throughout the body. It acts as filters or traps for foreign particles all over the body and is in effect the bodies 'hoover' as it cleans up 'bad bits'. A lot of cancers, including Neuroblastoma, use the lymphatic system as a way of moving around the body (spreading), and this was exactly what was happening to Alexander.
This was bad news, not only had the cancer spread, and spread rapidly, but the biopsy had determined that it was very aggressive unlike the original tumor in Alexander's belly that still appeared to be static. We were told that unless the disease could be brought under control it was doubtful that further treatment would be successful, but a course of radiotherapy was decided upon even though it had not worked originally. The logic was that the new cancer was much more aggressive than the original and therefore more likely to react to 'conventional' treatments.
Radiotherapy started early May for a period of 4 weeks, and luckily it worked in that most of the new cancer visibly disappeared. Some did unfortunately remain, so the Consultants decided to give Alexander high-dose Chemotherapy, which was the last of the 'conventional' treatments available in the UK. High-dose chemo started mid June for approx. 5 weeks, during which time Alex was placed in isolation and needed round the clock care. Alexander completed the treatment in July and we received the results on the 29th August. More news that we didn't want to hear. Why can't they just tell us for once that Alexander is clear? Instead we were told that the treatment had worked to an extent but there were still areas of active disease. This bad news was cushioned by some good news in that Alexander had been accepted on a UK trial for Antibody Therapy, which is good as this is the treatment we were considering going to Germany for.
The Antibody trial had already started and we awaited further details, including when Alexander could join. We did however know that the trial was a Phase 2 trial that would be administered to all participants, and that it was primarily to gauge tolerance levels of the drugs involved to help set guidelines for future use should the treatment be accepted in the UK. As the treatment was already in use in some parts of Europe and the States we expected it would be approved, but until that time we were thankful that we at least had access to the trial.
We were told that a meeting was due to be held early Sept. by the controlling bodies and sponsors to discuss results to date and decide what's next for the trial. Only then would we find out when Alex can join the trial and what exactly would be involved. We were however informed that Alex and Mum will need to spend a week in hospital followed by a yet unknown recovery period before undergoing several more courses of the same, meaning that Alex and Mum would be away from home for around 2 months starting mid/end September 2012.
The week before we expected to be called up for the Antibody trial Alexander went for his weekly check at Sheffield but it was not what we expected it to be. Further reviews of earlier scans had shown that the cancer had in fact spread, such that Lex was no longer eligible for the trial. Our Consultant told us that Alexander's cancer had spread to his bones, a thigh, bottom of spine, and his skull. He further advised that Alexander's chance of long term survival was now rather slim. He did however say that we should not give up and he would continue treatment shortly (Lex was still recovering from the high dose chemo from a few months back so immediate treatment was not possible) based on drugs that Alexander has already had before in the hope that they would somehow make a difference this time round.
We did not tell Alexander or Olivia about this as they both had enough to worry about. We really wished the news was better, but it wasn't!
Sheffield discussed out precarious position with UCHL and they agreed to see us with the intent of providing mIBG treatment in London. Lex had mIBG scans before we went to meet the team at UCHL but the results were not available at that time of the meeting. UCHL were very welcoming and showed us their facilities and talked us through the treatment protocols applicable to mIBG. We had a very open and candid discussion with the main consultant at UCH, who advised us that he didn't think he could cure Lex but he hoped that mIBG would provide additional quality time with Lex, as he would expect an improvement Lex's condition for some months after. It was still not what we wanted to hear but at least it was available and something we could put our energy into whilst we looked for alternative treatments elsewhere. We left UCHL a little happier than when we arrived. Unfortunately this was to be short lived as the following Friday the UCHL team convened to review Lex's scan results and concluded that the uptake of the drug used to show the cancer, and ultimately be used to attack it, was not good enough. We received a letter in the post at the end of Oct. advising us that mIBG was no longer an option. Lex was getting worse and our options in the UK now appeared to be gone.
Our consultant at Sheffield discussed Lex further with UCHL and they agreed that UCH would try LUDO, which is similar to mIBG but uses a different carrier/drugs. LUDO is still not fully approved in the UK, and a trial was due to start early December to progress its approval, but earlier tests had shown great potential for the treatment. Luckily, UCH can provide LUDO on compassionate grounds at their discretion, so it was still an option for us as there was nothing else available and they clearly thought that Lex deserved a chance. Lex therefore had another scan to see if the uptake from LUDO was enough to justify the treatment, with the scan taking place in the middle of November. We had a nerve wracking time waiting to see if the very last realistic option in the UK was available to us, and a week later we received the news that we wanted. Lex was eligible for the treatment and he would start on the 28th November. It was all looking a little better but literally days before Lex was due to travel to London a growth appeared on his forehead literally overnight. It became quite big rather quickly and we feared that the LUDO treatment would be in jeopardy, but Sheffield started oral chemotherapy as an outpatient and UCH agreed to continue with LUDO.
Lex had LUDO treatment at UCH in London from the 28th Nov. to the 3rd Dec., and is now home recovering. Lex has been quite poorly recently due to growth on his forehead that appeared literally over night and has not stopped growing, but luckily (if you can call it that) it is no longer growing as fast as it did to start with. Lex has, and still is, also suffering from bad pains in his leg around the area we know, but he doesn't, the cancer has spread into. It has been so bad that we have had to carry him around as he can't bear to even stand on it. This is not like Lex. Even when he has been poorly he has just got on with it with little complaining, so we therefore know that things are not good right now as he hasn't been like this before. The good news is that the growth on his head started to reduce in size as of this morning (9th Dec.) so something appears to be working. Unfortunately, there now appears to be another lump forming close to the original, and if this is the case then we have further problems as Lex can't have the next lot of LUDO until the end of January 2013.We have been that worried about his present condition that we spent the 8th Dec. in hospital (8th Dec.) at Sheffield where the duty Doctor confirmed what we thought- the new lump it is most likely related to the original growth. We expect to be summoned soon by Lex's consultant, and we hope that he then agrees to scan Lex and provide beam radiotherapy to tide him over until he can carry on with Ludo. Lex is quite poorly now, and getting worse, and we fear that if there is no further treatment between now and the end of Jan. Lex may not be in a position to carry on with LUDO!On a more positive note, we are in consultation with Doctors across the globe, including the States, Germany, and Holland. I attended an educational conference on Neuroblastoma in October, and I met some of the world leaders in Neuroblastoma. The conference was arranged by the UK based charity Neuroblastoma Alliance, who managed to pull these very important people out of a European wide conference being held at the Barbican in London to speak with parents about what ishappening in the world of Neuroblastoma. I won't bore you with too much detail (already done that) but what I will say is that advances are being made, but not quickly enough. It is these very people I am now talking with, and our hope is that after LUDO we will be able to go to Germany to undertake HAPLO followed by Antibody therapy, or to the States to obtain one of any number of options that are currently being studied there. My preference based on what I heard at the conference, and what I have learnt recently, is for Lex to have the German protocol as results, although early stages, are quite encouraging.Whilst there are no guarantees in anything we are looking at, and the consultants involved make that very clear, there are at least options 'IF' LUDO brings Lex into some sort of remission!
A full body scan on the 14th Dec. showed significant disease spread in Lex. We originally thought that the pain Lex was bravely suffering was being caused by inflammation due to the recent LUDO treatment but sadly this is not the case. Lex is now in hospital with Mum whilst they try to formulate a pain relief plan so that he can at least come home, but even then we most likely only have weeks left with our son. It's unbearable at this time and we are completely lost and helpless, but must try stay strong for both Lex and more importantly now Olivia. Olivia still doesn't know the gravity of the situation so we would ask that you please continue to respect this until we are able to bring ourselves to discuss this properly with her.
We would really like to say thank you to all that have helped, and those of you who are still helping us, and we would like you to know that we haven't completely given up hope just yet but the fact is that we are most likely going to loose Lex soon.
Lex is now taking Methadone as Morphine is no longer effective, but at least he's no longer in constant pain but he is now asleep more than he is awake. He is also home, and we hope this remains the case for Xmas, but in truth we just don't know. We are all (Mum, Dad, Olivia & Alexander) going to meet the staff at Bluebellwood Children's Hospice on Thursday, and Max and I have started to let Olivia know a little more about what is happening. She clearly doesn't understand the situation but we need to start to let her know so its not a complete surprise and shock when we do end up at Bluebell.
We are still trying to seek further advice from overseas and intend to talk with Germany soon, but whilst we hope there is an 'out' for our little star Lex, we both know that it is unlikely.
We all went to Bluebellwood Hospice on the 20th Dec. to meet all the staff and see the facilities. There is a side of us that is glad we went but another that detested what it means, but unfortunately Lex is getting worse by the day, with more bad days than good. We really hope that we would be home for Xmas, but this is looking less certain as time goes by. As you can imagine, Max and I are completely devastated and so so tired of the whole thing, but needs must. Watching your son slip away in the most unpleasant of ways is just not right or fair, especially at Christmas time knowing that it will be 3 years to the day on Xmas eve that we received the official diagnosis of Cancer. He's done absolutely nothing to deservethis, yet he is suffering so much. I know there are people who will read this and who will not like this next bit but I fail to see how there can be a god to let this happen to someone so innocent. Its breaking us all!