Kirsty here.  I decided that it was time I made an effort to update the blog as James has done the past few entries.  I guess I kind of lost the motivation to do it.  Sometimes it’s like re-living the whole nightmare and becomes too much.  Olivia’s health has completely consumed me, so when I’m not busy being mum to Oli and Sarah, I am Olivia’s carer and I tend to throw myself (somewhat obsessively) into researching, contacting doctors, parents, anyone who can give me even the   smallest bit of information that might help Olivia or lead me in the right direction in terms of finding the best possible treatment we can for our Oli.  I am a woman on a mission and find myself running on adrenalin pretty much 24/7.

So much has happened since James’ blog entry.  It’s hard to believe that anymore could actually happen to us!  We were completely devastated by Olivia’s relapse in January but found the strength to move forward and devise a treatment plan for Olivia, with the help of her doctors.  We were to start MIBG therapy with a view to commence local, external beam radiation two to three weeks post MIBG.  The biggest frustration for us was the wait.  The high dose Olivia needed for MIBG therapy was not available until March 1^st because the nuclear reactor at Lucas Heights just couldn’t provide it any sooner.  I have since found out that the reactor only operates at 30% of its capacity due to lack of government funding which is just disgusting.  In the meantime, the people who need any radionuclide therapy for medical purposes need to wait, ie. Olivia!  James and I found ourselves constantly wondering what this disease was doing to our girl while we had to wait all of those weeks before she could commence treatment.  It was an incredibly anxious and frustrating time to say the least.  However, during that time, we did manage to collect stem cells from Olivia which was a little unexpected as she is so heavily pre-treated, we thought it would be extraordinarily difficult.  We collected enough for three potential stem cell rescues which may open up other treatment options for Olivia in the future. 

James has previously mentioned much of the above so I’ll skip to the latest news.  A couple of weeks ago, Oli began to complain about a sore molar.  We just assumed that one of her adult molars was pushing out one of her baby teeth and that it would take its natural course and fall out in due course.  I should also mention that in the week or so before that Olivia was constantly waking at night or going to bed feeling scared.  She was terrified of the dark and was seeing things, thinking that someone was in her room.  Even after constant reassurances, she found it difficult to settle and usually ended up in our bed.  Her tooth then became a bigger issue and she was unable to sleep comfortably because of the pain.  We ended up having to give her round the clock Panadol.  She struggled with the pain at school and required pain relief.  I managed to get an emergency appointment at the dentist.  They checked and cleaned her teeth and said that the tooth should fall out soon on its own accord and to just keep up with the pain relief.  I left the dentist feeling somewhat concerned that this pain was going to continue or escalate.  As it turns out, we had a sleepless night and struggled to keep the pain under control.  The next day, Olivia was too unwell to go to school.  I decided to ring the dentist and ask them to extract the tooth to make her more comfortable.  We returned to the dentist immediately.  They numbed the area and extracted her tooth.  Oli was her usual brave self and I was her very proud mum. 

Unfortunately, Oli continued to complain of pain that day which seemed to stem from the extraction area.  I had also noticed that from a couple of days before, she had an itchy, watery right eye and it appeared puffy.  We put it down possible conjunctivitis.  I decided to take her to the local GP who thought that she had hayfever (affecting only one eye?!?) and she prescribed her $30 eye drops.  All along, something was worrying me.  Deep down inside I just felt there was more to it than that and wasn’t at all convinced that hayfever was the problem.  I was worried there was something more sinister going on but I didn’t want to admit it.  The next day, the problem with her eye continued, as did the pain.  However, Oli pointed out that the pain was coming from around her right cheek and orbit.  Although I felt a little concerned by this, I began to think that this was possibly a sinus infection.  Anyone who’s had one can tell you that sinus pain can be quite bad.  The only thing that made me think that it may not be was that she didn’t have the green, mucousy discharge you get with sinusitis.  I also recalled the doctor in Melbourne had pointed out that there was slight uptake in the sinus cavity in her PET scan.  He asked us if she’d had a sinus infection or a cold as sometimes there can be increased uptake in sites of infection.  She hadn’t been unwell but then she started blowing her nose so we put the increased uptake down to infection.

In the evening, I decided to call the paediatric registrar at Canberra Hospital.  She said that Olivia needed to be checked out and that we should bring her in immediately.  James took Oli to hospital that night while I stayed with Sarah so I could get her off to school the next morning.

She had to have a cannula inserted at Canberra hospital.  She hadn’t had much to drink and the doctors really struggled to find a vein.  It took about 10 goes on one arm, 10 on the other, and then about 5 on the hand before they could find the vein!  Olivia is normally fine with needles, but she was clearly very traumatised by this.  She has also recently had a few bad experiences with her Port-a-cath needle insertion, so is starting to get more anxious whenever she has to have a needle.

The next morning, the consultant at TCH felt that what Olivia had wasn’t as simple as a sinus infection so an MRI was booked for later that day.  We didn’t have to wait too long for results.  James had left the hospital to get some take-away for us all when the registrar came in to break the news to us.  You always know that you’re about to receive bad news;  Firstly, their faces say it all and then they lead you down the corridor to some isolated room.  Luckily, I had my good friend, Caroline, with me.  We were told that the MRI showed a large mass in her right maxillary (cheek) that was extending into the sinus cavity, ethmoid air cells and orbit (the bone around the eye).  Although I partly expected this result, I really was hoping for the best.  It blows me away every single time.  We know that in our situation Olivia’s disease is likely to progress but for some reason, our brain never seems to really believe it.  It is just so UNBELIEVABLE!  The most difficult thing for me is seeing Olivia finally become symptomatic of her disease.  She hasn’t been symptomatic since she was two years old.  It’s heartbreaking watching her in so much pain all because of this monster we have no control over.

I was an emotional wreck.  I decided to ring Olivia’s oncologist (so glad I had her mobile number stored away in my phone for moments like these!).  She was fantastic.  She didn’t have much to go by as she hadn’t seen Olivia, nor did she have the scan pictures and results but I felt a little better after speaking with her.  She suggested we head up to Sydney the next morning so they (she and some other doctors) could review the scans and determine a plan of action.

James and I looked at the scan pictures with one of Olivia’s other doctors and it wasn’t good.  It appears that this tumour is taking up the whole cavity behind her maxillary.  It appeared to us that it had grown very quickly in the past few weeks.  The discussion then revolved around the next step.  After the doctors had consulted with a couple of radiologists, it was decided that a biopsy should be taken.  Olivia’s doctor seemed to be certain that the mass was neuroblastoma but couldn’t rule out fungal sinusitis, which can also be life threatening!  A biopsy of the site was organised very quickly with the ENT doctors jumping on board to help make this happen very quickly.  It was organised for the very next morning which also happened to be a Saturday.  In order to take a biopsy from the site, the doctor would need to go through the right nasal cavity to access her maxillary, hence the mass.  We were told it should be fairly straight forward surgery.  Despite fungal sinusitis being a very serious infection, we were hoping that that was all we’d be dealing with:  A clean out of the fungus, followed by a course of antibiotics.

We were told that it would take around an hour.  We waited and waited.  It was almost two hours when James and I looked at each other and started to think that maybe that was a good sign.  After all, it can take a while to clean the fungus out.  Unfortunately, this was not the case.  The doctor came into see us and after apologising for taking so long, he said “the conditions in there were horrific!” and that he “would’ve pulled out much earlier if it wasn’t for her doctor wanting the biopsy so badly.”  This had us worried.  He said that “it was full of blood”.  He said that that area is a vascular anyway but he was surprised at how much blood there was.  He said that reaching the site was a challenge and he finally had to drill a hole through the bone to access the mass.  Once he got in there, he said there was no fungus.  He said it looked very much like a tumour.  We were shattered once again.  We now had to wait for the official pathology results.  The doctors chose not to take any further action until the results came through.  Their immediate concern was continuing to manage Olivia’s pain so we now also had the Palliative and Pain team on board.  There was also talk of putting Olivia on steroids to help with any inflammation of the tumour site, thus reducing the pain.

We received the results on Monday.  Olivia’s oncologist confirmed that it was neuroblastoma.  However, she didn’t want to put her on steroids as she was worried that it might interfere with her planned MIBG therapy which was starting on Thursday.  The plan was to continue with MIBG therapy, followed by local, external beam radiation 2 to 3 weeks later.  Given that MIBG is not effective for approximately 8 weeks, we had initially considered using local radiation on her cheek to attempt to stop it from growing and causing her more pain, however, that would likely have delayed the MIBG therapy.

The recovery from the biopsy was not too much fun for Oli.  She had to have a pack in her nose for a few days.  It was incredibly uncomfortable for her and she experienced a lot of pain, not to mention the pain from her cheek.  She was on high doses of pain medication.  Despite the pain and discomfort and although she was unable to smile, she told me that she was smiling on the inside.  She’s so beautiful.

Until anyone has lived through what we have with Olivia, it would be difficult to fathom what she has been through.  I feel like most days I am just running on adrenalin and going through the motions to get Olivia through this ordeal.  It’s not until I have a quieter moment to sit back and reflect on the pain and suffering she has experienced in her short life that the tears really start to flow and my heart just breaks into pieces.  It’s so incredibly painful to watch her go through this journey, I sometimes find it difficult to fathom myself.  But I can’t continue on like that, so I have to smile through the tears for Olivia’s sake and pull myself together and get back to auto pilot.  I just wish I had an ounce of Olivia’s strength.  She amazes me each and every day.  She embraces all the joys in life and doesn’t let go.   She always manages to see the beauty in everything.  To her, cancer treatment is a nothing more than a big hiccup in her life’s journey that’s not going to stop her from living.  She looks to her future with such excitement and enthusiasm.  She has dreams and goals just like any other 8 year old girl.  James and I have spent the past few years searching for even the smallest glimmer of hope that Olivia might be okay but, at the same time, we’ve had to live with the all consuming fear that we will most likely lose her to this monster of a disease one day.  With every relapse we wonder, is this the one she won’t recover from.

When it came time for Olivia to have the pack removed, the ENT doctors came into her room to remove it.  In what was meant to be a relatively simple procedure turned out to be a bit of a nightmare.  Olivia panicked and became incredibly anxious that it would be very painful.  After a while, the doc managed to give it a very gentle tug but Olivia was in excruciating pain.  It was then suggested that they take it out the next day under general anaesthetic.  We thought that would be the much kinder option and Olivia was happy with that.  That afternoon, all of the kids on the ward were treated to a visit by Hi-5.  They came into Olivia’s room and although she’s not much of a Hi-5 fan anymore, they made her smile.  They played 5 second Pictionary on the white board which was a lot of fun.  I have to say Hi-5 has changed quite a lot from when my girls used to watch them – there was not one original member in the group, though four of them said they’ve been with the group for three years.

Olivia went into theatre first thing the following morning.  Apparently, it was all very straight forward with the pack coming out easily and no bleeding.  However, Olivia was a little worse for wear when she awoke from the GA.  She was in some pain with her nose (and the tumour was continuing to cause pain to the right side of her face) so she was on round the clock pain relief for the next few days, not to mention the nasal spray and ointment she has to use for the next 4 to 6 weeks!

Just when we thought things couldn’t get any worse, Olivia’s doctor came to give us more news.  Apparently Olivia’s tumour sample tested positive for ALK staining.  Whilst the tumour sample that was tested for this in Germany was negative, apparently, it is quite possible for NB to change and evolve into a more aggressive form.  It is also possible that the test used in Germany only covered the two most common mutations that make up approximately 85% of ALK mutations.   ALK is a gene mutation which stands for Anaplastic Lymphoma Kinase.  Less than 10% of all neuroblastoma patients have it and it means that those patients who do have it, have the most aggressive form of the disease!  We were very surprised that result came back as quickly as it did – we thought it would take a couple of weeks.  On the one hand, we were devastated but on the other hand, we realised that this possibly opened up another option for Olivia.  An inhibiting drug called Crizotinib has been developed to inhibit or slow the growth of ALK positive NB tumours.  We are in the process of learning more about it but we do know that a lot of work is being done at CHOP (Children’s Hospital of Philadelphia).  The drug is currently being trialled so there isn’t much data available regarding its effectiveness.  However, I have heard that, unfortunately, some forms of neuroblastoma can be resistant to this treatment.   I believe that they are also working on an ALK antibody but this is still in its preclinical stage.

Sarah came up to Sydney with us for the first half of our stay.  Although she really wanted to be with us, it really wasn’t much fun for her.  As much as we hate being without her, we know that it is best for her to stay in Canberra and go to school.  She is always spoilt for attention by my parents and I think that it helps a lot that she has Ellie to play with and cuddle. 

James here.

We travelled over to Westmead on Wednesday afternoon as Olivia was booked in to commence MIBG therapy on Thursday.

MIBG therapy uses a radio labelled isotope known as I-131 which is infused over a period of about 90 minutes.  The isotope travels to the sites of disease and attaches itself to neuroblastoma cells where it kills the cells with its radioactivity.  This is a slow process and can take up to 8 weeks or more before we can see its maximum effectiveness.  It is about 30% effective in relapsed neuroblastoma patients, which isn’t a great result but it’s about all we have right now.  We will never lose hope!   It can also assist in reducing bone pain associated with neuroblastoma, so is usually used in a palliative setting.  However, Olivia has previously shown good MIBG uptake, so in this case we used a higher dose in order to try to achieve tumour reduction.

Olivia had to have a foley catheter inserted prior to the infusion as half of the radioactivity is excreted in the urine in the first 24 hours.  When it came time to insert the catheter, Olivia fought it big time.  One of her favourite phrases in hospital is, “Hang on…I’m not ready” which is repeated like a broken record.  This time she worked herself up into such a state that she was very distressed and tense…she wasn’t able to relax enough for the nurses to insert the tube.  I really felt for Oli, knowing that it wouldn’t be a particularly comfortable procedure.  James and I tried very hard to keep her calm and it just doesn’t seem right or fair to her that we should be telling her everything will be okay if she just lets the nurses get on with it, when we all know that it will most likely hurt, but what else can we do…that’s our role.

In the end, the nurses suggested giving her midazolam which would relax her and make the procedure much easier.  The nurses said that she would go out like a light.  They gave Olivia one dose and we all waited for her to quickly fall asleep which never happened.  All it did was slur her speech and she continued to tell the nurses to hang on and that she’s not ready, only much more slowly.  They decided to give Oli another dose and, again, it only slowed down her speech and she continued to be aware of what was going on, though somewhat dazed and confused.

She knew it was going to hurt and fought it.  They eventually had to use gas, which still didn’t really stop her fighting – it just slurred her speech even more. 

Olivia was infused with 12 mCi/kg which is the whole amount assigned nationally for the month.  As previously mentioned, we had to wait for a month to get it and, in the meantime, Olivia’s disease was gaining momentum and spreading!

The MIBG therapy required Olivia to spend 5 days in an isolated lead-lined room because of the high level of radioactivity.  Unfortunately, we were required to very much minimise our time spent in the room with her. Olivia had to stay in a lead lined room and we were in an adjacent room.  There was a TV and camera in both of the rooms, though that meant that if we watched each other, we couldn’t watch TV.  It wasn’t until about the 3^rd day that Oli felt confident enough to actually watch the TV.  When on video, there was no sound, so if Oli wanted to talk she had to phone us.  We soon made up a series of hand signals to communicate.

As Oli was radioactive, we had to record our radiation exposure with portable radiation dose meters.  It was hard to be away from Olivia when she was all alone and not feeling 100%, so we tried to alternate going in to see her as much as we could.  We brought in a portable DVD player and iPod to keep her from going insane.  Thankfully, she was quite happy to watch the entire Harry Potter series and the first season of Dance Academy.

Overall, Olivia handled the MIBG therapy very well.  She never really complains, though we suspect that she was feeling quite nauseous for the first few days.  However, we knew she was feeling a lot better on the last day, when she was doing ballet in the mirror.

After the MIBG therapy, they conducted an MIBG scan to look for MIBG uptake by the tumours.  Whilst we were shocked to see that she now had new uptake (and hence new cancer) in her spine and leg, we were pleased to see that she appeared to have good MIBG uptake at her tumour sites, which hopefully will translate to treatment effectiveness.

Four weeks after her MIBG therapy, Olivia had local radiation on her cheek, head and hip.  This has left her with a bald spot at the front of her hair.  However, she just takes that in her stride and just wears thick headbands.   It’s strange, but for some reason, Olivia has always liked radiation treatment.  I guess it’s because the staff are always so friendly and the treatment doesn’t hurt her or make her feel sick.

Shortly after radiation, we went through a huge scare with Olivia experiencing constant back pain for over a week.  We were so worried that the pain was being caused by new tumours and decided that she needed to have a scan of her abdomen. 

We had planned to take a holiday to Brisbane and the Gold Coast in late April. As the MIBG scan in Sydney is only conducted on Wednesdays we were faced with having to cancel our holiday to have the scan or wait 3 weeks (because of Anzac day).   Thankfully, we were able to organise an MRI scan in Canberra at short notice.  The MRI didn’t show anything, which was reassuring, though an MRi is not definitive for detecting neuroblastoma so we had to wait for the MIBG scan some weeks later to be certain.   Any disease progression at this stage would be disastrous, as it would most likely result in the cessation of her treatment.   We decided to go ahead with our holiday and have the MIBG scan when we got back.

We were glad that we did go on holidays, as we had a fantastic time in QLD doing all the theme parks and swimming in the pool every day.  Thanks to Seaworld, we even got to go in the water and feed and play with a dolphin called Scooter.  Fittingly, Scooter was the mother of Nila, the dolphin that Olivia met on her Make-A –Wish trip several years ago.

Whilst we were away, we noticed that the tumour on Olivia’s cheek appeared to have reduced in size considerably and that she was looking really well.  We were hopeful that this was a sign that the MIBG treatment was working.

Leading up to her MIBG scans, we had been thinking about what our treatment options would be once the bulk of her disease has been reduced.

A sample of her tumour had been sent to the US a few weeks earlier for testing.  After many weeks, it was confirmed that she has an ALK mutation.  Whilst this does open up the likelihood of giving her the ALK inhibitors that are currently being trialled in the US, it also means that she has the most aggressive form of neuroblastoma.   We were curious as to why the ALK test in Germany came back negative.  It is possible that the Germans only test for the main 3 mutations that make up 85% of ALK mutations.  As it turns out, Olivia has one of the very rare types that falls into the remaining 15%.  So, just to put this into perspective, she had approximately a 1 in 100,000 chance of having neuroblastoma, of those kids, approximately 7% have the ALK mutation and then probably about around 1% of those have this particular mutation: that equates to approximately 1 in 140,000,000 kids in the world having her exact type of disease!  Needless to say, there isn’t a lot of data available on successfully treating this type of neuroblastoma.

We had our consultation with our Oncologist after Olivia’s scans.  At the start of our discussion the scan results weren’t available, so we discussed our treatment plan, which included an additional round of MIBG treatment, Indium and Lutician treatment in Melbourne, possibly followed later in the year by ALK therapy and a Vaccine or personalised medicine (chemo) in the US.  Unfortunately, our treatment plan was torn apart when our oncologist received a phone call with Olivia’s scan results.  Other than a reduction in the areas subjected to local radiation, the disease in her bone marrow has spread considerably.  It is now in her arms, legs, spine, shoulders and skull!   Clearly the MIBG therapy hasn’t worked.  We were just totally shattered by this news, as all our treatment options were based on the MIBG controlling her disease.  Our oncologist suddenly changed her tune and indicated that there is now no treatment that is likely to be effective and suggested that maybe now was the right time to take her off treatment.  Even though we knew that this day would most likely come it still tore our hearts out to come to the realisation that after almost 6 years of fighting this horrid disease, she is now likely to succumb to it within a matter of weeks or months.  This was without a doubt the worst day of our lives.  We tried to explain why we were so sad to Oli, without telling her about the gravity of the situation.  After hearing this, she just remarked that she’s fine, as she is still able to dance!

As we were in no state to drive back to Canberra that night, we stayed in Sydney until the next day.  We had to constantly hold back tears watching her happily play and dance around. 

After a couple days of thinking about the worst, then seeing her do three hours straight of dance classes, we decided that Olivia still has too much strength to give up on.  It is simply dumbfounding that she can look so healthy and yet her body is so riddled with cancer.

We sat down and considered our options: standard chemotherapy is not our first choice as it has not previously proven to be very effective and it causes too many side effects.  The Indium and Luticium treatments in Melbourne rely somewhat on the MIBG therapy working and require her tumours to express Somatostatin receptors.  We thought of having her retested for suitability for this treatment (which was previously low), however, didn’t end up having the time to do so.  

As part of our original treatment plan, we were already investigating the option of moving to the U.S. for treatment later in the year.  They have many different types of treatments being trialled, most notably immunotherapy, ALK inhibitors, neuroblastoma vaccine, oncolitic viruses and customised chemotherapy. 

Just prior to Olivia’s latest scans, our oncologist had started looking into whether it would be possible to obtain the ALK inhibitor (Cryzotinib) in Australia on compassionate grounds, outside of the U.S. trial.  The drug is already available in Australia for lung cancer patients and was previously supplied by Pfizer for another patient at Sydney Children’s Hospital with a different type of cancer.  After a lot more researching the internet and making several phone calls to US specialist, we decided that the ALK inhibitor was the best initial treatment option for Olivia.   We were hoping that the request would be successful and that we would have her on the drug within two weeks.  Every day that passed, we wondered what this cancer was doing to her body.  We were constantly asking her oncologist if she had heard anything.  She told us that Pfizer had requested some more of Olivia’s scan results and background information.  We saw this as a positive sign.  We waited everyday for the approval, only to be devastated by the news that company policy was they wouldn’t supply it to Neuroblastoma patients outside of the US trial.  We couldn’t believe how it took them over 2 weeks for them to “consider “ our application only to advise that it is against company policy.  Time is something that is not on our side!

We were absolutely gutted by this latest news and were starting to feel that our attempts to save her were going to be in vein.  Fortunately, we had previously organised a phone call to the States to discuss treatment options over there.  We had always tried to keep abreast of US treatments, though had previously avoided going there due to the incredibly high cost of treatment combined with the possibility of Olivia deteriorating and being stuck in intensive care over the other side of the world.  However, we simply can’t give up on her and watch her deteriorate, and for ever wonder “what if we had taken her to the US”? 

The decision to go was sealed when we informed that if we entered into the ALK inhibitor trial in Bethesda, Maryland (instead of the one in Philidelphia), the treatment would be free.   The other good news was that we could probably go over there initially for 6 weeks for scans, the first cycle of treatment and then re-scanning, before taking the next 2 month’s supply of tablets back home with us.   We would then only have to return to the US for 1 week scans and evaluation every 2 to 3 months.

Once we made the decision to go, our lives once again became focussed on getting us overseas ASAP.  In order to fit it with the earliest opportunity to start treatment, we had 10 days to organise everything and get on the plane.  

As Olivia’s disease spreads it adversely impacts on her blood counts, requiring more frequent platelet and red blood cell transfusions.   We were deeply concerned about Olivia’s eligibility for the ALK treatment trial when we received an email from the US Dr indicating that Olivia’s neutrophils have been hovering around the 700 mark for the last 2 months, when the cut-off for the trial is a minimum of 750.  It was made very clear that this was a firm entry criteria and that the method used in the US typically produced lower results.  The only suggestion we were give was to try taking her bloods in the afternoon instead of the morning, as some patient’s neutrophil count increase with activity.  After a very stressful 24 hours thinking that Olivia wouldn’t be eligible for this last treatment option, we couldn’t believe that her count had increased to 1100!  USA here we come – if we can get our medical visas in time.

The visa application process through US consulate turned out to be quite involved and very frustrating.  Whilst they theoretically have the processes in place to issue emergency medical Visa, we had 8 days to get our visas and actually only obtained them with 2 hours to spare!  This was not helped by the fact that the interview and processing is conducted in Sydney, and the information line connects to a call centre in India, who have minimal information available to them and almost no interest in chasing anything up.  In fact, were it not for a very helpful lady at Australia Post, we would have not received our Visas in time to make our flights.  The Visa information line seemed to think that the visas had been sent, but couldn’t provide tracking numbers.  Australia Post couldn’t check to see if they had arrived without a tracking number.  Am I the only one who sees a problem here?  We were told to wait another day for the tracking number.  We waited but still no tracking number available with less than 24 hours till our flight.  After pointing out this problem to the call centre, they indicated that they would call with the tracking numbers within 24 to 48 hours!  Aaaaaarh! 

Thankfully we stumbled across the direct phone number to the sorting room at the GPO and spoke to someone who was willing to go look everywhere to find them without a tracking number.  She indicated that they had just arrived.  However, when I went into the post office a few hours later, they initially couldn’t find them and suggested they weren’t there.  They looked in another location, and still no luck.  It was only after I insisted they were there, that the helpful lady we had originally spoken to noticed the other staff looking for something and showed them where they were.

This was one of many major obstacles in the way of us getting to the US for the trial.  The last hurdle came late the night before we were due to depart, when we received an email from the US doctor that seemed to be questioning whether Olivia would be well enough to travel and would meet the criteria for being accepted onto the trial.  Olivia’s blood test results showed that her tumour marker was rapidly increasing, indicating that her cancer was getting much worse – yet she still appeared to be well within herself.  We made an urgent call to the States to discuss these issues and convinced them that Olivia was very well.

It had been an extremely stressful built up to our departure, but it was almost a relief to finally hop on the plane.  It was sad to say goodbye to our families and Olivia’s best friend Ella but it was also comforting to know that we were finally on our way to getting Olivia treatment that she so desperately needs.

 We will deeply miss our family and friends and also our loveable Ellie.  As we are only hoping to be in the US for 6 weeks initially, we didn’t have to pack our house and put our belongings into storage like we did for Germany.  Luckily Kirsty’s sister and her fiancée have offered to move into our place and look after the house and Ellie while we are away.

We have to thank Olivia’s oncologist for being so supportive and helping facilitate getting us on the trial in the US.  Considering that she was the person who originally suggested that now would probably be a good time to stop treatment, and had previously advised us against going overseas, she did everything she could to help up get on the trial.  She is probably now enjoying not having us call her every day and filling her inbox with emails!

Dylan Hartung is another kid who has also been battling neuroblastoma for a long time.  He was featured in the same 60 Minutes story as Olivia in 2008.  His family moved to the US about six years ago for life saving surgery.  He has since been on many different trials over there and has thankfully remained stable disease.  Ironically, just as we are moving to the States, they have decided that they have run out of treatment options in the US and that it is time for them to come back home.  We wish Dylan and his family all the best.            

Hi, it’s James here.  Our lives have once again been shattered by the news that Olivia has  relapsed a fourth time!  Scans taken on 18 January have revealed 3 new tumours – two in her skull and one in her hip.  The bigger one in her skull is at the site that was previously deemed to be inconclusive after her last scans in November.  If only it had been confirmed back then, we could have been treating her 6 weeks earlier and may have even still been able to collect cancer free stem cells.   It is frustrating to know that a PET scan would most likely have confirmed the relapse, but they don’t have this facility at Sydney Children’s Hospital. 

The realisation that Olivia had again relapsed was terrifying.  Whilst we have heard the prognosis from our doctors many times before, it doesn’t get any easier.  We know that Olivia is now facing the toughest battle of her life and we need to find a treatment regime in a hurry that is likely to benefit her.  Time is not on our side and we have been pushing the doctors to get the ball rolling, as the disease could be rapidly spreading throughout her body.  However, it seems as though there are so many obstacles in our way, including needing to test her bone marrow and once the results are known, discussing treatment options with specialists that don’t return from leave until February.

Given that Olivia currently has multiple lesions in different parts of her body, it is likely that her bone marrow is now infected.  She just had a bone marrow aspirate on Tuesday and we are anxiously awaiting the results which are due late this week.  We still intend to collect stem cells next week irrespective of whether she has marrow disease, as contaminated stems cells can still be of some use in an emergency if her marrow becomes too supressed – although that will be giving her cancer cells back!  She has failed to harvest twice previously, although this time, we will use Pleraxafor, the very expensive drug that we are hoping will stimulate her stem cells for collection.

If, as we suspect, her marrow is infected then we are facing her worse relapse ever, with minimal stem cells in reserve.   This will effectively limit our already meagre treatment options to those that don’t require stem cell rescue.

Once more we have been scouring the internet for treatment options and communicating with local and overseas specialists and other parents in similar situations.  Whilst there is no stand out emerging treatment, we have discovered a few treatments that we need to carefully look into.  We have always had the option of MIBG radiation therapy, although in Australia it is offered in relatively low dose, usually for palliative care.  We are mainly limited in Australia by supply, as treating just one child with MIBG requires over half of the weekly production of the radioactive material from Lucas Heights!  MIBG is used in higher doses overseas, often in conjunction with chemo, but requires stem cell rescue, which we currently can’t do.  However, we have been told that some kids do respond well to the low dose MIBG, so this will probably be our first treatment option, commencing in a few weeks time.

We met with a doctor from the Nuclear Medicine Department at Westmead hospital last week to discuss MIBG therapy.  We have been told that during this treatment, she will be isolated for approximately 4 days, with a video link to Kirsty and I in the room next door.  Thankfully, this treatment shouldn’t make her feel too unwell, although we expect that boredom will be a big problem.  We are limited by what she can bring into the room, as everything will have to be disposed of afterwards.  That definitely rules out taking in her new iPod!

It may be some months before we know whether Olivia responds to the MIBG therapy.  This should give us more time to plan some additional follow up therapies.

We have previously consulted with 2 Doctors in Melbourne who had mentioned a few new experimental treatment options.  They are trialling an Octeotide based treatment called Indium therapy that has demonstrated some effectiveness in another relapse patient.  However, this treatment requires the presence of Somatostatin receptors, which Olivia will need to be tested for.   We are taking Olivia to the Peter MacCallum Institute in Melbourne this week to have her tested for the Somatostatin receptors through a Gallium PET scan.  This scan will also give us a more accurate indication of the current extent of her disease, which could be very confronting.

Whilst in Melbourne, we will also be discussing another new treatment called Lu177 Dotate.

We still have the option of following up Olivia’s initial treatment regime with traditional localised radiation treatment, however, that will not eliminate the source of her disease, nor any bone marrow disease.   We are trying to avoid chemotherapy, as she has had so much of it in the past and has only had a mixed response.  However, if Olivia doesn’t respond to our planned radiation based treatments, we may be forced to put her back on chemo as an interim measure to attempt to keep the disease at bay whilst we arrange other treatments.

Even if we are fortunate enough to fight off the tumours that Olivia currently has, we know that more will come.  We need to keep treating her as though she has a chronic disease.  However, it’s a catch 22 situation; if our initial planned treatment regime is effective in eliminating the tumours, then she may become ineligible for most trials as it is then not possible to determine the effectiveness of any subsequent treatment.  

We are currently looking into a new vaccine trial in the US that has shown promising results in very limited patients.  Depending on Olivia’s response to the any initial treatment regime, this may be worth considering to consolidate her treatment.  We have always been aware of treatment options in the States, though we have been reluctant to travel there unless they have a proven breakthrough treatment, due to the extremely high cost of medical treatment and having to again leave our family and friends.

Treatment decisions will not be made lightly and we will do whatever we can to try to save our beautiful girl, without overly compromising her quality of life.  We are not ready to give up on her whilst she is looking so well and she has indicated to us that she is prepared to fight.  We will know if the time comes that we will need to stop treating her, but we for now, we need to do everything that we can to help her fight this dreaded disease.

The following was written prior to Olivia’s recent relapse:

We hope that everyone had a very Merry Christmas and a Happy New Year.  We enjoyed a very pleasant holiday season, with Olivia in remission and in great health.  We were fortunate to spend a great Christmas together with most of our extended families.

As previously noted, shortly after we returned from Germany, Olivia re-fractured her knee and was forced to give up dancing, which she has always been so passionate about.  Sadly, even once her fractures finally healed, she still wasn’t able to return to dancing as she was often plagued by an intermittent mystery knee pain.   We really missed our dancing girl! 

We went through a very anxious period starting around August last year after Olivia very suddenly stopped walking due to discomfort in her left knee.  She wasn’t complaining of severe pain, but simply couldn’t walk.  We could not help but think that it could be cancer returning, though that would’ve been the first time that Olivia has felt pain as a direct result of neuroblastoma.   After an initial bone scan picked up an abnormality in both knees, we had a very anxious 2 week wait for MRI and MIBG scans.   It’s a sign of how crazy this situation is in that we were praying for either a structural problem (fracture?) or treatment related bone pain.  The MIBG (neuroblastoma specific) scan came back clear, though the MRI showed abnormalities.  Whilst not certain, the doctors indicated that they didn’t believe that it was cancer and that it was most likely a side effect of the treatment, possibly the Rapamune oral chemo tablets she takes.  It was quite bizarre, though somewhat comforting when her knee pain suddenly completely switched legs!   She ended up spending several weeks in a wheelchair and then another month or so on crutches before her knee finally settled down.

During the October school holidays, Olivia and Sarah went on their first Camp Quality Camp without us.  Olivia’s round of chemo scheduled for 2 weeks beforehand had to be delayed due to low blood counts.  However, rather than have her miss camp, we decided to push back her chemo 3 weeks and let her enjoy her holidays.  Thankfully her knee was OK at the time.

We were a bit apprehensive about how they would go on their own, and were half expecting the phone call begging for us to come and pick them up.  As it turns out, they were having so much fun that when we called them they either wouldn’t stop talking about what they were doing or didn’t even want to speak to us because they were so busy enjoying themselves!   They were so lucky to have some such wonderful companions as Bec and Sasha, and after they got back kept going on about how much fun they had together.

It felt quite strange being without the girls, as we had spent so much time together over the last year.   We decided to take advantage of the girls being away to do some minor renovating around the house with some help from Kirsty’s Dad.  Unfortunately, due to the amount of time spent in Sydney, we still hadn’t finished unpacking all our belongings from when we finally moved back home (after 10 months away) so the place was certainly a mess for a while.  However, the end result was well worth it, although I now have a new found respect for plasterers! 

Two  days after the girls got back from camp, we departed for Queensland to see my sister Romy’s family and visit the theme parks.  We try to have at least one good family holiday a year as we know how much the girls enjoy it and we really value our family time together.  With so much uncertainty in our lives, we don’t ever want to regret not giving Olivia the chance to live her life to the fullest.  Both girls are particularly fond of theme parks, so were very excited about going to the Gold Coast.  It was also great for them to spend some rare time together with their cousins Alex and Christian. 

We certainly made the most of our time up there, going to all 3 theme parks at least once and 2 waterparks.  Sarah has really become a lot more daring and was keen to go on a lot of the bigger rides, including the BatWing at MovieWorld.  Olivia has always been so fearless for someone of her age and is fortunate enough to meet the height restrictions for most rides, except the Superman ride – there were tears when she found out she couldn’t go on it!   It was a great holiday, but very full on.  The girls must have been exhausted when they got back, starting straight back at school.

Thankfully, with the exception of her knees, Olivia was generally very well between chemo cycles and went to school most of the time we were in Canberra.  Ever when her blood counts were low, she still generally felt well.  Although that did present some risk of her catching something, we had to let her try to be as normal as possible.  It is a credit to her determination and the assistance from her school and teacher that she managed to keep up with her classmates despite missing so much school.  We are so proud of her!

We were recently alarmed to discover that Sarah had contracted Chicken Pox that was going around their school.  As Olivia’s chemo wipes out all her previous immunisation, this can be quite dangerous to her.  Thankfully she did not catch it, though she still had to endure two very painful needles as a precaution.

Despite ongoing research, we hadn’t been able to come up with a plan for maintenance or for future treatment should Olivia’s cancer return.   The sad reality of our situation is that it is almost certain that Olivia’s cancer will return and there are currently very few options left for us, anywhere in the world.  We sought treatment opinions from a specialist in Melbourne and from several prominent experts in the US.  The Melbourne Dr was probably the most positive that we have come across in Australia, and we walked away with a possible course of action if Olivia relapses.  Sadly the American Dr’s were not so positive, indicating that she has almost no chance of long term survival. 

We know that we are really just trying to buy enough time for new, more effective treatments to come along.  In the meantime, we just need to keep Olivia as healthy and happy as we can.

We are always cognisant of trying to balance Olivia’s quality of life against the likelihood of extending life.   We had wanted to stop Olivia’s chemo for some time, but we were so scared that she would relapse shortly after we did.  We finally made the decision to stop Olivia’s chemo altogether late one night in October. Whilst finishing our packing to travel to Sydney to start chemo the next morning, Olivia woke up with excruciating pain in her knee just before midnight.  We realised that the treatment was starting to impact on her quality of life and we couldn’t bear to watch her suffer through that kind of pain.  Strangely, it seemed to be a very slight increase in her chemo dosage a few months prior that made the side effects that much worse, also making her lose the last of her hair.

Thankfully it didn’t take long after stopping treatment for her knee pain to fully subside.  We knew she was back to her normal self not long after the treatment stopped, as she just doesn’t stop talking and is now full of energy and dances around everywhere, even when brushing her teeth!

Now that we have stopped treatment, we just watch and wait.  We still live a very anxious life but are trying to give Olivia a chance to build up her strength and live a “normal” life.  She is in good spirits and we are hopeful that she will remain in remission for some time.  In the meantime, we are looking into alternative treatments to help boost her immune system. 

Olivia currently looks very well.   Her hair is slowly growing back and she has a very strong appetite and has put on a bit of weight.  She has been enjoying going on bike rides and seems to have fairly good stamina.  Both girls have been spending a lot of time on the trampoline and in our little pool. 

Our number one priority at the moment is to collect Olivia’s stem cells.  Stem cells can be used to revive her immune system if it is depleted during future treatment.  Without sufficient stores of stem cells, it would not be safe to subject her to any aggressive treatments.  For example, she currently can’t undergo high dose MIBG treatment, one of the very few potential options that we have left.  In hindsight, we wouldn’t have been able to undergo the previously planned stem cell transplant in Germany as we weren’t able to collect anywhere near sufficient quantities of stem cells during the last two attempts.  It was very disappointing to put her through countless injections to stimulate her stem cells and have two lots of surgery, all to no avail.

There is a very expensive ($5,000 per injection!) drug available that we are hoping to use in February to stimulate her stem cells before trying for a third collection.  Given that she will have been off treatment for 4 months by then, we are hoping that her immune system will have had a chance to recover somewhat and the collection will be successful.

On the home front, we have been extremely busy, trying to get on with life between frequent trips to Sydney.  There just doesn’t seem to be enough days in the week to do everything that needs to be done!  I have been back at work full time since we got back from Germany and have pretty much used up all my leave during previous chemo trips, so haven’t been able to take much time off recently.

We celebrated Kirsty’s 40^th Birthday party in December.  Being a two times cancer survivor herself, she has a lot to celebrate.  She had an 80’s themed party which produced some amazing costumes, including a Smurf and a giant Rubiks Cube!  Kirsty looked a true 80’s chick in her Madonna style costume complete with frizzy hair and lace gloves, whilst both Sarah and Olivia dressed up in pink flouro outfits.  Olivia even wore a matching pink wig. 

We couldn’t believe how much fun the girls had dancing and singing Karaoke and Olivia ended up stayed up until 1:30am!

The girls were lucky enough to both receive realistic mermaid tails with a proper built in flipper for Christmas that they can wear in the pool and swim around like a mermaid.  Unfortunately, our above ground pool is tiny and they get one or two kicks in before they reach the other side.  They love swimming so much, and have been begging for a bigger pool, but that will have to wait until things settle down and Kirsty is able to start work again.

Due to the extreme likelihood of a relapse, doctors suggested bringing forward Olivia’s scan intervals from the usual 3 months to 6 weeks.   Scans in November were considered to be clear, with the exception of a slight uptake in her head.  This will be monitored but for now is being treated as inconclusive.  The next scans are scheduled for 17 Jan.

Olivia celebrated her 8^th Birthday on 15 January.  Each birthday is a huge milestone for us, as we never know how many more she will have.  We intentionally scheduled Olivia’s scans for after her Birthday, as we wanted her to enjoy her special day, unlike last year when there was a huge cloud hanging over our heads.

Olivia didn’t want a big party this year, so just had a few of her closest friends over for a sleepover. We spent most of the day at the pool (with waterslide) and then went to dinner at Wagamama’s, followed by dessert at Cold Rock.  Olivia was thrilled to finally get an iPod touch that she had been nagging us about.  Kirtsy and I were pretty tired after staying up into the early hours of the morning the previous night, after our first attempt at making her chocolate smash cake ended in failure!

On a much sadder note, we were deeply upset to hear of the recent passing of two of our close friends from Germany.  Felix was a 7 year old from the UK who relapsed the same week as Olivia and his family moved to the US to pursue different treatments.  He was an amazing kid who never complained and showed so much strength for such a little body.  Maddie was also a gorgeous 7 year old from the UK, who fought a very long and brave battle against this dreaded disease.  We pray that they are both now in a better place where they no longer have to suffer and we hope that their families can find the strength to get them through these tough times. 

 

Hi, James here.

We have had a draft update that we have been meaning to finish off, but just don’t have the time at the moment.  So just briefly, this is where we are currently at:

We rented out our house in April for another 3 months after accepting that we would most likely have to return to Germany for a stem cell transplant; only to decide against the transplant 2 weeks later!  We decided that the risks were too high given that she remains in remission and that we just need to try to give her body a chance to recover. 

 In the meantime, we are continuing with the German chemotherapy protocol, that, whilst making her sick during her week in Sydney Children’s Hospital, offers her quite good quality of life for several weeks between cycles.  Overall, Olivia has actually been very well whist back in Canberra, without any fevers or need for blood transfusions.  She has loved being back at school, swimming lessons and dancing now that her leg has finally healed.

Unfortunately, we still have found ourselves travelling to Sydney a lot for chemo, stem cell collection and radiation; however she has been able to attend hospital school for some of the time.

We have tried two different methods of collecting stem cells, but both were unsuccessful.  Though this does not impact us immediately, it may limit future options. We will likely try again later this year, after Olivia has finished her current treatment.

We had to wait for Olivia’s fractured leg to completely heal before undergoing two weeks of radiation on her left leg.  Strangely, Olivia has always somewhat enjoyed radiation.  Understandably, she is not so keen on chemo, where she barely talks and refuses to lie in the hospital bed.

Although we have spent much of the last few weeks in Sydney, we are really happy to have finally moved back home and retrieved all our belongings from storage.  We can’t thank Brandon and Craig   from Canberra Removals enough for volunteering to take care of our removal and storage.

We still have boxes everywhere, and should at this rate be unpacked by around Christmas!

Whilst in Germany, we promised Olivia that she could have her dream bunk bed, with a desk underneath.  She loved sleeping in it for the first few weeks, before hurting her “good” leg last week.  We still don’t know what happened, but she suddenly wasn’t able to walk.  Initial x-rays and examinations failed to find any explanation and we of course feared that the cancer had returned to her other leg.  We were all set to book her in for a bone scan, when a couple of days ago she started walking unassisted.  We are just praying that it stays away!  Having said that, she continues to have some slight pain.

That is pretty much everything.

 

We have to apologise for the lack of updates over the past few months.  Olivia’s care and treatment schedule has taken precedence and to be quite honest, I have been consumed by it.  James and I have both contributed to this blog, summarising the happenings of the last few months.  We will endeavour to update more regularly with brief posts.

We have finally returned back home after 6 months in Germany, although there was a huge cloud hanging over our heads – we were devastated to find out the day before Olivia’s 7^th birthday that she has developed a new tumour in her leg. We were praying that the MIBG scan would be clear as the bone marrow aspirate and the MRI of Olivia’s torso prior to Christmas had both come back clear.  It appears that whilst the immunotherapy was able to initially clear her bone marrow, it was unable to stop a new tumour developing.  It is apparent that the (undetectable) source of Olivia’s cancer is extremely resilient and has once again proved resistant to all attempts to destroy it.  It seems that her immune system has been weakened by years of treatment and was unable to work with the immunotherapy to destroy all her neuroblastoma cells (immunotherapy tags neuroblastoma cells so that the body’s immune system recognises them as foreign cells and attempts to destroy them in the same manner as the body fights off most other sicknesses).

Needless to say, in light of this latest setback, we were utterly devastated and wondered how we would make it through Olivia’s birthday weekend.  I have to say that I don’t think I handled this news as well as I have in the past.  I found it incredibly difficult to deal with.  I just felt defeated, like this monster has beaten us yet again.  Nevertheless, we tried to make Olivia’s birthday as special as possible, treating her to a birthday weekend in Berlin where my sister and her boyfriend came to surprise her.  I made Olivia a butterfly birthday cake with the limited resources available to me in the house (and the supermarket) and also went out for a lovely dinner.  On Sunday, we took the girls to a lovely open air ice-skating rink before heading back to Greifswald.  I guess you could say that the weekend was quite bittersweet.  On the one hand, we were absolutely devastated and heartbroken by the recent news but we were just so happy to see that gorgeous smile on Olivia’s face.  I can’t even describe the incredible heartache I was feeling that whole weekend.

Although any neuroblastoma relapse is life threatening, with both of Olivia’s previous relapses the disease has been localised and didn’t spread rapidly as is typically the case.  We just prayed that this would be the case again.  At that stage, the disease seemed to be limited to her leg, which reduced the immediate risk to vital organs.  However, hers being bony disease meant that it could not readily be removed with surgery.  Also, neuroblastoma is a very difficult disease to detect, so it is possible that she has disease elsewhere that is not yet showing up on scans.  The German doctors were initially concerned about another possible lesion in her head, though they now believe that it was a false positive at the site of one of her original tumours.

We have once again spent countless hours looking into treatment options available in the US and Australia, but as we already knew, there is little documented success in treating relapsed neuroblastoma patients.  The Children’s Hospital of Philadelphia (CHOP) appears to have numerous trials on offer, though are all phase 1 (preliminary) trials that may not be of any benefit to Olivia, as they test the safest tolerable dose rather than the effectiveness of the treatment.  Germany offered us a novel type of chemo called RIST, combined with local radiation.  The German doctors believed that this chemo should be able to get Olivia back into remission.  They have suggested following up this chemo with a haplo-identical stem cell transplant.

We decided to proceed with the RIST chemotherapy and Olivia completed the first 2 out of 4 cycles of this tightly scheduled protocol in Germany.  It was decided during this time that we could continue with this treatment in Australia, though Olivia’s Australian oncologist had to seek special approval from hospital administration.  It was eventually approved for the remaining two rounds of the first phase and if she had a response, they would continue to fund the treatment into the second phase.

Before leaving Germany, Olivia had a biopsy to see if they can determine what type of neuroblastoma she actually has.  As her disease has always been in her bones, it has never been possible to get a biopsy without a degree of risk.  It is likely that there are numerous different types of neuroblastoma, however only a few have been identified to date. Identifying Olivia’s tumour may tell us whether it is a particularly aggressive type or a slower growing type.  Unfortunately, it is only in relatively few cases that they can actually use this knowledge to formulate a better treatment.  They are still trying to work out how to target specific types of neuroblastoma with specific treatments, however, this is probably still some years away.

Olivia underwent a biopsy on her lesion on January 20.  We were initially told that Olivia would be a bit sore for a few days after her biopsy.  Unfortunately, she ended up being in quite a bit of pain and was still limping quite badly when we left Greifswald two weeks later.

We left on February 8, our Sarah’s 5^th birthday.  It was an incredibly busy day but we did try to make it as special as we could for her.  She was spoiled with presents in the morning although we had to spend much of our time cleaning up our room.  We then headed off to hospital as Olivia required a blood test and James and I had to be tested to see who would be the most suitable donor for Olivia’s planned transplant.  We also had to wait for the doctors to supply us with a letter regarding Olivia’s current condition and treatment just in case she required any hospitalisation whilst in transit.  I have to say that I really felt for Sarah given all the time we had to spend in hospital on her birthday.  Our plan for the afternoon was to travel to Berlin and stay the night there and take Sarah out for her birthday.  I even had one of our German friends order a birthday cake for her.  We had to go through quite a process.  All I wanted was a simple kid’s birthday cake without cream.  It was impossible!  I don’t think there is a single cake in Germany that exists without cream.  In the end, our friend did manage to order a cake without cream…I had no idea what we were in for.  Once we arrived in Berlin, I headed off to pick up the cake which turned out to be a strawberry fruit flan…huh?  Well, I suppose I did say no cream!  I was a little disappointed as I knew it wouldn’t be Sarah’s dream cake but being the happy-go-lucky girl that she is, she didn’t complain, and actually really enjoyed it.  We stayed in a nice hotel with a pool so the girls got to have a little swim even though the water was absolutely freezing.  They still seemed to enjoy it.  Although it wasn’t much of a birthday for Sarah, we did promise her a special birthday cake once we returned to Australia (still haven’t got around to organising it yet).

Thankfully, the flight home went quite smoothly with Olivia feeling well and both girls on their best behaviour.  Due to the tight scheduling of this chemo, we had to take Olivia straight to hospital in Sydney upon arrival back in Australia to commence her third round of chemo.  The combination of jet lag, heat (coming from a German winter) and the faulty air conditioning at Ronald McDonald house resulted in a very sleepless week for us.

After one week in Sydney, we finally returned home to Canberra. It was so heartwarming to see Olivia re-united with her best friend Ella. Olivia was also able to return to school, ballet and swimming, which she loved.  Sarah started kindergarten at Burgmann and also commenced ballet lessons and swimming.  Sarah became quite interested in ballet having spent time with Olivia dancing around the hospital in Germany. Whilst we aren’t sure if it is her ’thing’, we thought that she could give it a go for a term to see how she goes.

It was strange coming back home, particularly not having access to any of our belongings other than the winter clothes we brought back from Germany!  Given that we will be spending half our time in Sydney, we decided that it was not worth getting all our belongings out of storage and moving back into our house when it become available a month after we returned. We knew that we could possibly be moving out again less than 2 months later.  Thankfully, my parents have very generously opened their home to us so we have somewhere to live in between treatment before we return to Germany.  They also continue to care for Sarah whilst we are in Sydney with Olivia and have been looking after our gorgeous dog Ellie whilst we were away.  We were so happy to see her again and she just loves all the extra attention.

Whilst we were in Canberra, my dad and I had a meeting with Senator Kate Lundy in an attempt to rally the government to provide some financial assistance to patients, such as Olivia, who are forced to travel overseas for clinical trials. Sen. Lundy seemed quite receptive and asked that my dad draft a new public policy in conjunction with Senator Lundy’s office to propose change for pediatric cancer patients who require financial support to access clinical trials overseas.  My dad is currently in the process of seeking various professional opinions to support this direction and his proposed policy change.

We became very concerned when we saw that at the end of Olivia’s first week back at school that she started limping quite badly on her left leg.  It had been some 5 weeks since her biopsy in Germany and her leg had been okay for the last two weeks.  In our minds there were really only two possibilities, either she had overdone it and injured her leg, or the tumour had grown significantly to be causing her pain.  The latter would be devastating as this would mean that the chemo was not working and we would need a plan B which we didn’t have!  Our options right now are seriously limited and we don’t know what we would do if we had to stop the chemo.

Olivia was originally due for scans two weeks later, but we pressed her oncologist to bring them forward in light of this latest concern.  We really couldn’t wait that long to find out if we were dealing with disease progression.  Thankfully, Olivia’s doctor arranged a CT scan later that week and an MIBG scan and bone marrow aspirate for the following week.

After a very anxious wait, we were incredibly relieved to find out that it was not the tumour causing her pain as the CT scan indicated that Olivia’s femur had a suspected fracture.    Unfortunately, the CT scan didn’t provide us any information about the cancer.  We then had to follow up the suspected fracture with an x-ray which confirmed that Olivia had a healing fractured femur.  It was about 7/8 of the way through the bone. It may seem strange to most normal people how happy we were to find out that she “only” had a fractured leg!

As the fracture seemed to be healing, they only put her on crutches, not in plaster.  Trouble is Olivia loves dancing around so much, that it was difficult for her to not put any weight on her fractured leg.  We were initially perplexed as to how she could have fractured her leg so badly, without us knowing about it.  Our feeling is that it occurred during her (difficult) biopsy in Germany, due to the bone being weakened by the cancer as well as all of the treatment she’s had over the years.  This would explain why she wasn’t able to walk on it properly for so long after the biopsy.  It seems that the leg healed somewhat during transit and her chemo week back in Sydney, though hadn’t fully healed when she started back at school.  She was very active on her return to school, constantly running around, jumping off playground equipment and jumping on trampolines…I guess that’ll do it!  The orthopaedic consultant confirmed that the cause of the fracture was a result of the biopsy as he pointed out on the x-ray that the biopsy puncture was where the fracture was stemming from.  He wants Olivia to have a repeat x-ray in 6 weeks to check on its progress.

We returned to Sydney the following week for Olivia’s MIBG scan and bone marrow aspirate.  We were incredibly thrilled to find out that they were both clear!  This means that there was no evidence of cancer found anywhere, not even in her leg.  This proved that the chemo was working!  Technically, this means that she is in remission, though it is most likely that there are still microscopic cancer cells in her body that don’t show up on the scans.  We must continue with the planned treatment regime as it is pretty much inevitable that the disease will return if we don’t.  It could also return whilst on the current treatment so we must now make further decisions about the next course of treatment.  However, for now, we are happy to know Olivia’s disease is responding as well as we could have hoped for on this chemotherapy protocol.

When we originally planned our early return to Australia, we were expecting to stay here until late June this year.  However, just two days before we left Germany the professor sat down for a talk with us and informed us that if Olivia went into remission after the first stage (of up to 3 stages) of chemo then he would like us to return to Germany immediately for a haplo-identical stem cell transplant.  Recent developments in stem cell technology have allowed the German doctors to utilise a significant variation on the traditional transplant, using a stem cell mismatch rather than the traditional match.  As Olivia’s immune system clearly cannot fight the cancer, the idea would be to give her either James’ or my own stem cells, thereby giving her an all new immune system that hopefully can better fight off the neuroblastoma.  It was also suggested that post-transplant, we try immunotherapy again.  The theory being that with a new and different immune system, the antibodies may have a good anti-tumour effect.  However, we are reluctant to pursue this course of treatment in light of her relapse.  We are currently looking into the transplant option and seeking international opinion regarding this procedure.  We are also trying to look into other options in the US.

Unfortunately, the only options available to Olivia are in a clinical trial setting.  Most clinical trials require a degree of ‘measurable disease’ to be eligible for participation.  Olivia no longer has ‘measurable disease’ and whilst we are happy about this, it excludes her from most trials!  It’s a double edged sword – very unfair!  At this point, it is looking as though transplant is the only viable option for her.

Although we had always anticipated that if everything went well we could be heading back to Germany shortly, it is now starting to hit home.  It is a huge move and one we are not really looking forward to.  However, if this is where the greatest hope lies for Olivia then we will just have to do it.  The decision we are facing is an incredibly difficult one and hard to fathom without having walked in our shoes.  In our situation, I’m not sure there is a ‘right’ decision.  It is about weighing up the risks and benefits and quality of life and finding a balance.  Very, very difficult indeed!

We are also heartbroken at the thought of having to leave Sarah behind this time.  Sarah coped with being in Germany extremely well, making friends with the other kids and amusing herself in the playroom.  However, this time we would need to be in isolation during transplant and Olivia would be restricted from having contact with Sarah.  Sarah has been so happy since returning to Canberra and is absolutely loving school.  We feel that is so important for her to have a degree of normality in her life and for her to continue going to school and make friends in Canberra.  It looks as though our stay in Germany will be for approximately 3 months but we hope that during that time, Sarah will be able to visit us during the school holidays.  Whilst in Canberra, she will continue to stay with my parents so that she can enjoy attending school, ballet and swimming lessons. We are sure that she will be okay but it will break our hearts to leave without her!

We have tried to establish whether any haplo-identical stem cell transplants have been conducted on neuroblastoma patients in Australia.  Sydney Children’s Hospital have used haplo-identical transplants to treat leukaemia patients though not on a routine basis. Our discussions with some of the German doctors indicate to us that their transplant protocol is somewhat unique and unlike a conventional haplo transplant.  We are hoping that Olivia’s Australian oncologist will look into this procedure and the possibility of doing it here in Australia.  Unfortunately, the doctors here aren’t familiar with the German protocol and have been unable to comment on it which has been quite difficult for us in making our decision.  We are hoping that Olivia’s doctor will be speaking with the German team this week so that she will be able to inform us further about the procedure and guide us with our decision.

We have also contacted another hospital in the German town of Tubingen which is near Stuttgard.  They have performed approximately 30 haplo-identical transplants on relapsed neuroblastoma patients (but conduct 50 to 60 haplo transplants a year all up).  The team in Tubingen are extremely experienced in this procedure which opens up the possibility of travelling to Tubingen instead of Greifswald for the transplant.  The team at Tubingen conduct more haplo-identical stem cell transplants than anywhere else in the world.

We were very surprised to hear that the doctor suggested that there may already have been one haplo-identical stem cell transplant conducted in Australia, though he could not remember the doctor or hospital involved.  This certainly gives us something else to look into as a matter of urgency.

Olivia completed her 5^th round of chemo two weeks ago.   On the whole, she has tolerated the chemo fairly well, though she does get quite nauseous and has a tendency to vomit.  She has only required one transfusion during the entire treatment.  Sadly, a lot of her beautiful curly hair has fallen out, though she has accepted this very well, as she does with most things.  One of the biggest challenges we’ve had is trying to get Olivia to swallow the massive chemo capsules every night while we’re in Sydney.  At the beginning of her treatment, she did really well and swallowed five capsules every night without much fuss at all.  However, swallowing the capsules has become increasingly difficult for her.  Sometimes she just feels too nauseas to take them but she has also developed a phobia which results in her becoming very upset and anxious to the point of vomiting when it comes time to taking the capsules.  We have tried everything to gently encourage her to take the medication but it can be a very frustrating experience for us all involved.

We will keep going with the current chemo regime whilst we wait for Olivia’s leg to heal and finalise our treatment plan.  Our original intension was to treat her leg with local radiation once the fracture fully healed, however, the radiation oncologist has advised us to wait for her leg to fully heal before embarking on radiation as it may cause more problems for her leg.  Whilst we are a little anxious about the possibility of the tumour returning to that site, we are fortunate that there is currently no evidence of disease, so we at least have the option of leaving it for now.

We have finally just received the results of Olivia’s biopsy.  We had hoped that her tumour would be slower growing, differentiated disease.  Unfortunately, this is not the case – she has a highly aggressive, very active disease.  We were devastated by this latest news, however, it does not change our treatment plan and options.

We are fortunate that we have been able to get Olivia into remission, though are now faced with the most difficult decision of our lives.  Sadly, there is no known cure for relapsed neuroblastoma so we cannot be sure what treatment will ultimately be the most beneficial for Olivia.  If we do nothing, then she will surely relapse again.  We have resolved to keep on fighting.  We will never give up on our beautiful girl.  There is always hope…hope is what gets us through each day.  Olivia is in remission and she still has a lot of fight left in her.  However, we need to make our decision very soon as this disease will not give us time.

The most frustrating thing about this whole experience is having to do all of the research myself without much guidance from specialists.  Whilst Olivia’s doctor has been supportive and understanding, we despair that with the number of clinical trials options available around the world (none of which we perfectly well understand carry any guarantees of survival) that the burden of adjudicating is left to be shouldered by the parents.  It’s probably too much to expect but it would certainly be a valuable resource to have an independent specialist who has a grasp of all open clinical trials and is prepared to say…’in Olivia’s case, on balance this is the trial that I recommend you should pursue’.

We are incredibly grateful to have our beautiful Olivia in our lives and are so proud of the way she accepts everything thrown her way.  In spite of her battles, she continues to amaze us with her incredible fighting spirit.  She doesn’t let anything get her down and continues to dance around and celebrate life despite the all of the treatment and a fractured femur!  We love you more than anything beautiful girl!!

I would also like to take this opportunity to pass on our deepest heartfelt thanks to our family and friends.  My parents have been unwavering in their support for our family.  They have taken such wonderful care of Sarah in our absence and have taken in our family whilst we’ve been in Canberra.  We don’t know what we would do without their incredible support. My dad has worked tirelessly on the Olivia Lambert Foundation and has continued the fundraising efforts to help support Olivia’s ongoing medical expenses.  He is also working towards changing public policy so that children like Olivia have access to financial support to participate in clinical trials overseas.  I should also mention that he has organised a charity Golf Day on Thursday, April 7 at Federal Golf Course.  A huge thanks goes to the main sponsor, Classic Constructions, Mix 106.3 and to all of the other sponsors of this event.

We are so fortunate to have such wonderful friends who have always been there for us.  We would like to extend our gratitude to the Burgmann staff and community, as well as to the general public for their continuing support.  We feel very lucky to be surrounded by such genuinely caring and compassionate people.  Thank you so much!