Just a quick update to let people know that Olivia’s health has significantly deteriorated over the last week and that she is currently fighing for her life.

She is so strong that it wasn’t until the disease had taken over most of her body that she finally started to complain. She is currently in Clare Holland House, where they are trying to control her pain. Please pray for our beautiful girl.

Olivia

Back from the US

August 4, 2012 James 5 Comments

Hi, James here.

We are now back in Australia after six weeks in the US. The scans conducted after one cycle (28 days) of Crisotonib showed little change in her disease, although the oncologists did caveat the results by saying that she has so much disease throughout her body that it is hard to detect any small changes. Whilst we were initially very disappointed to not see any real reduction in her disease, we are well aware that the results could have been a lot worse. Olivia’s disease is very aggressive and if she had not gone on this trial, she would almost certainly have succumbed to her disease by now.

During past relapses, Olivia’s disease typically presented as isolated tumours and a degree of bone marrow involvement. Whilst we were previously always fortunate enough to be able to find some form of treatment that would fight her disease and get her back into remission, the reality of our situation is that we have not heard of anyone who has survived so many relapses. With each relapse came the realisation that this disease was not going to stay away and that it would likely come back one day in a very aggressive form. This time around, her disease has indeed been very aggressive and failed to respond at all to the MIBG treatment she had in March.

Scans conducted upon arrival in the US indicated that she had a very high degree of disease (95%) throughout her bone marrow, tumours in one of her lungs, plus many bony lesions including the one near her right eye that has caused it to partially close. Her LDH level, which is a non-specific tumour marker, had increased in just a couple of weeks from 700 to around 1500! This suggested that her disease was spreading very rapidly. We left Australia knowing that the ALK inhibitor treatment represented our last chance – if it didn’t work, we may not have been able to bring her home alive.

Although Olivia’s disease has not regressed, we are thankful that she has remained quite well and hardly experienced any pain. However, she only has a very small percentage of her bone marrow functioning and remains heavily dependent on platelet and red blood cell transfusions to stay alive. Despite this, she has on the whole been feeling well and has been her usual bubbly self.

We had initially considered staying on in the US, in case Olivia began to progress. We knew that if we returned home and Olivia progresses, then there would be very little chance of her remaining well enough to get her back to the US in time to start on a different treatment. Whilst, if we remained in the US, we might be able to commence another treatment sooner. However, the treatment that we have in mind has a lead time of almost a month and it is unlikely that she would remain well for that long. Furthermore, there would be a much higher risk that she may not remain well enough to make the return flight home.

We have searched far and wide, but to the best of our knowledge there is no other treatment option currently available for Olivia anywhere that offers a good prognosis for a child with such aggressive disease and so many relapses. After discussing the risks and benefits with our US oncologist, we decided in the end that it would be best for us to return home at our first opportunity and likely remain there should Olivia progress.

Of course, given her weakened immune system, even the trip home represented a high risk of Olivia catching a bug. On top of that, we also returned from the peak of the US summer to the middle of the Canberra winter! It was regularly over 32 degrees in Maryland and peaked at around 40 degrees and was also quite humid.

Whilst we enjoyed our time in the US, we had perhaps not been able to get out as much as we would have liked. The extreme heat somewhat limited our capacity to do sightseeing around Washington D.C. On those occasions that we did venture out, we found that the girls, in particular, quickly tired in the heat. We had originally planned some day trips to surrounding areas, but given the uncertainty with Olivia’s need for transfusions, we often found driving to air conditioned shopping malls an easier alternative – much to the girl’s delight. Thanks to the Build-A-Bear workshops, they now have the best dressed teddy bears around!

Aside from the extreme weather, Maryland is certainly a very nice place and the people are very friendly. We also enjoyed a very high level of personal care at the NIH, by very professional staff.

We were fortunate that we were staying at the Children’s Inn on the NIH Hospital campus, not just because there was generally a lot of activities going on to keep the girl’s busy, but because as a medical facility they have their own backup power generator. At the start of July, Maryland and Virginia were struck by a very fierce storm that knocked down many of the very abundant trees around this area and damaged numerous power lines, initially cutting off power to over a million homes. A week later, there were still several staff at the hospital who had no power at home – right in the middle of a heat wave. Fortunately, other than the associated traffic light chaos, we were barely effected by the storm.

Due to the extreme heat during the day and very late sunset, we sometimes took the girls out to play on the basketball court after dinner. As Olivia doesn’t really have the required upper body strength to shoot the basketball through the adult height hoop, she preferred to play with the ice hockey stick and puck. Whilst she generally wasn’t able to hit the puck with any great force, she seemed to enjoy giving it a go. Unfortunately for Kirsty, there was one particular occasion when she did take a big swing – only to miss the puck and connect with Kirsty’s nose on the follow through! The amount of blood and obvious pain that Kirsty was in suggested that this was not just a light injury. It was quite a chaotic scene, as Olivia was also very upset about the whole incident.

As the NIH is a trial medical facility, there is no public Emergency facility. Thankfully, we had a car and was able to get her to the local hospital just down the road. We had to wait a while, but they eventually advised that Kirsty had broken her nose in two places. Luckily, aside from the swelling and a couple of cuts, her nose looked still quite normal. The girls were remarkably well behaved that night at the hospital, considering that we didn’t get back to the Inn until around 1am.

We weren’t initially sure whether Kirsty would still be able to fly home on the planned date, so had to delay booking our return flights until she had been cleared to fly. Luckily, our oncologist was able to make some phone calls and get Kirsty in to see an ENT specialist the next afternoon. The specialist subsequently advised that Kirsty was cleared to fly.

Once the decision had been made to leave, we thought that we would try to take the girls to Hershey Park in Philadelphia. Both our girls have always loved going to theme parks, though we were worried that Olivia’s love of fast rides would not be tolerated by her platelet deficient body and she would risk bruising and internal bleeding. However, with the support of the NIH medical team, she was topped up with platelets and red blood cells and given a possible 2 day window. We were greatly concerned by Olivia having intermittent nose bleeds and a swollen abdomen on the day we arrived. We debated whether to take her to hospital, but decided that we would rather monitor her and avoid the faster rides. Thankfully, her bleeding nose slowly got better and we eventually succumbed to Olivia’s constant requests to go on the faster rides. Needless to say the girls had a great time, despite the heat – though Kirsty didn’t really enjoy having to sit out all the rides because of her broken nose.

We arrived back in Maryland two days prior to our departure. The doctors looked at her swollen stomach, though couldn’t find anything obviously wrong – and there was nothing apparent in her scans just a week earlier. They once again transfused Olivia to prepare her for the flight back home.

Given that in some instances Olivia now cannot last more than two days without a platelet transfusion, we decided to minimise our stopover in LA and keep travelling back to Australia ASAP. Furthermore, we had to take back 6 bottles of Olivia’s medication (about $20,000 worth), that had to be kept refrigerated the whole time.

The return flight was certainly more tedious that the departing flight (when we fortunate to receive Business Class upgrades). This wasn’t helped by the fact that Sydney was fogged out, forcing us to divert to refuel in Brisbane. We eventually landed some four hours late and then spent another hour waiting for the girl’s suitcase that didn’t turn up until the next day. It was a very long and tiring trip, though Olivia held up quite well. We ended up staying overnight in Sydney as Kirsty was able to get in to have her broken nose fixed the next morning.

We are happy to be back in our own home and being around family and friends (and our dog Ellie). We are definitely enjoying the extra space and not all having to live and sleep in the same room!

Whilst it is great to be home, we are somewhat anxious as we feel as though the safety net of being at the NIH has been removed. We have only just started to experience the logistic problems of being back home and having to take Olivia across town to Hospital for transfusions every second or third day. It was comparatively so much easier to just walk 200m up the hill to the hospital in the US. However, we are lucky that an arrangement has been made to have a nurse come to our home every few days to take Olivia’s blood samples. Even so, her blood levels can be somewhat unpredictable, and we have already had three urgent trips to hospital with bleeding nose or gums (caused by low platelets). Olivia’s gums always bleed when she is in need of a platelet transfusion, though usually stop bleeding straight after being transfused. She woke up a few weeks ago with particularly bad bleeding, though she wanted to watch the conclusion of the dance-a-thon before taking going to hospital. She was transfused Sunday afternoon prior to returning home after the transfusion. However, Sunday night her gums were still bleeding quite heavily so we had to return for an overnight stay in the hospital. There was an anomaly with her blood test indicating that her blood was not coagulating properly. Furthermore she had some blood in her urine. After bleeding for several hours, she was given a dose of vitamin K to help her blood clot and the bleeding slowly stopped.

Her stomach also remains a major concern, as her liver seems to be constantly growing bigger and her stomach is now quite enlarged. As much as we really want to know what is causing this, from a trail perspective, we didn’t want to know. We have avoided bringing forward her scans to diagnose the cause of this problem, as the most likely answer is cancer progression. However, that would still come as a shock to us, as we first noticed the symptoms less than a week after her last scans. Unfortunately, if that turns out to be the case, it would mean that she would be immediately taken off the trial – which appears to be otherwise controlling her disease. Furthermore, there is nothing more that can be done to help her.

We have been very careful with Olivia attending school, as her immune system is barely functional, and she is a very real risk of contracting some bug that could possibly be fatal for her. Incredibly, despite all her treatment over the years, Olivia has barely had so much as a cold. In fact, she has always been an amazingly healthy person for someone whose body has suffered from so much cancer. However, this time around, the risk to Olivia health is very real. As her bone marrow is not producing sufficient neutrophils to strengthen her immune system, she is most likely unable to fight off any significant infection. In cases such as hers, it is often an infection that proves to be fatal. However, we cannot simply seal her away in a bubble, so just have to take some precautions and pray that she does not contract anything.

Alyssa Bloomby is an amazing person who briefly taught Olivia dance when she was only three. She has always maintained a keen interest in Olivia’s progress and recently volunteered to organise a 24 hour event called Dance4Olivia to raise funds for our family and the Olivia Lambert Foundation. The event was held over the weekend three weeks ago and was a great success, with lots of dancers and many well respected instructors from Canberra and Sydney who volunteered their time. Olivia, Sarah and Olivia’s best friend Ella had a great time, participating in classes most of Saturday afternoon. We were amazed to see that there were 8 people who kept going all night and attended every class over the 24 hours! We can’t thank Alyssa, her helpers, the instructors and the participants enough for making the event a great success.

Jacqui Ion is another amazing person, who even from South Australia has supported our family over the years. Whilst we were away in the US, she organised a successful Women in Business lunch and donated the proceeds to our family. Thank you so much for your support.

Finally, we would like to pass on our deepest sympathy for the family of Olivia Downey, who sadly lost her battle with neuroblastoma a few weeks ago. We are glad that they were able to stabilise her enough to get her back home from Mexico, so that she could be surrounded by her family and friends. Although every parent of a relapsed neuroblastoma child has to live with the nightmare of knowing that they will most likely one day lose their child to this awful disease, we cannot fathom the heartbreak that they must be going through right now.

We will post an update in the next few days, but since drafting this post, Olivia’s health has started to decline.

Olivia

ALK Treatment in the US

June 27, 2012 James 1 Comment

Hi James here,

After such a chaotic lead up to our departure, it was a relief to actually walk onto the plane. Whilst we had initially been devastated by Pfizer denying us access to the drug in Australia, it was surreal that the option of free treatment in the USA arose that same day. There were so many hurdles to overcome to actually get to the USA, and we knew that we would still face further hurdles prior to actually being accepted onto the trial. Then there is no guarantee that the treatment will actually work. However as we left Canberra, we knew that we were doing everything we possibly could to try to save our gorgeous girl.

Of course, given our recent luck, even getting to the airport wasn’t without drama. Luckily, I went into the garage early in the morning, as I was greeted by the sight of the Kluger leaning heavily on a completely flat front tyre! Of course, our spare was also partially flat, so it took me almost an hour to get the car sorted. Thankfully, we still made it to the airport with time to spare.

We only had a short stopover in Sydney, where my best friend Tony and our friend Deanna (whose wedding we unfortunately will now miss) came to see us off. A special thanks to Tony for picking up copies of all of Olivia’s previous scan results from Sydney Children’s Hospital for us, which we were requested to bring with us to the US.

A special thanks to Qantas who gave us an amazing deal with our US airfares, considering that they were only booked less that 2 weeks before we departed. Furthermore, Natasha, the mother of another neuroblastoma child who works for Qantas, advised us that our Sydney to LA flight had many vacant seats. Kirsty thought that she had nothing to lose in asking for an upgrade at the counter, and after quite a lengthy wait was blown away to be told that we were being given complimentary upgrades to Business Class!

We were initially seated in a two plus two configuration, with the two girls next to each other, behind Kirsty and myself. After take-off, Kirsty and I each chose a movie, put the headphones on and sat down to enjoy a peaceful relaxing flight. Then reality hit home, when approximately every ten minutes one of the girls would yell out MUM……MUM…….MUM! or DAD……..DAD……..DAD! Because the reclining seats are capsules, it’s not possible to turn around and see the seats behind, so we had to pause our movie, take the headphones off, get up and walk over to where the girls were to see what they wanted. After a few hours of this calling out, much to the amusement of (or was it annoyance to) other passengers, Kirsty and Olivia swapped places. It was a real treat to have beautiful meals, great service, all the extra space and a bed to lie on.

We stopped overnight in LA and stayed near Santa Monica. To our surprise, it was very windy and really quite cold and we were just wearing shorts and T-shirts. The girls really enjoyed going on some rides at the pier, though we ended up having to drag them away as Kirsty in particular was really feeling the cold.

After a bit of a rough night’s sleep, we headed off to LAX in two taxis, as we had so much luggage and our booked van didn’t turn up! It was around 5 hours flight time across to Washington D.C., so with the time difference, we didn’t actually arrive until around 6pm and were all feeling pretty exhausted. Departing the plane, we were shocked by the change in climate between LA and Washington D.C. – It was very hot and quite humid.

Whilst waiting at the car rental depot, we noticed that the inside area near Olivia’s right eye seemed to be developing a bruise and also appeared partially swollen, consistent with a tumour growing in that area. We wondered if that was being caused by a new tumour or the one in her sinus (that had been previously irradiated) coming back?

We had pre-booked a car for six weeks at a discounted rate through my work, but the guy at the counter was hell bent on selling us an upgrade. It took almost an hour of being mucked around before we finally got our original car – not a pleasant experience at all! By this time, we are all very tired and frustrated.

We arrived at our new home at The Children’s Inn at the NIH (National Institutes of Health) at Bethesda, Maryland around 8pm. However, it took us a while to get through the incredibly tight security, including being issued passes and having our car and luggage searched. The Inn is huge, about four times the size of Ronald McDonald House in Randwick. Our room is like a typical 3 star hotel room with 2 double beds. Unlike our room in Germany, there is no separate bedroom, which means that we can’t watch TV or make any noise until the girls go to sleep, which has been taking them ages. This is partly due to jet lag and partly due to them sleeping together in a double bed. My feet hang about a foot over the end and I can’t really move without disturbing Kirsty – we really miss our King bed! Thankfully, we only expect to be here six weeks.

The Inn is very conveniently located only 200m from NIH where Olivia is being treated. However, it is up a steep hill and we have had some scorching hot days here. The hospital itself is huge and quite modern.

I think that when the staff at NIH first met Olivia they were very surprised at how good she looked and how much energy she had. Obviously, her scans and blood test results made her out to be a very sick girl, though the only visible sign of her disease was her eye. Just prior to leaving Australia, Olivia’s LDH, a non-specific tumour marker, had risen to a very high level, indicating rapid disease progression. Also, a couple of her liver enzymes were abnormally high before we left so she had to take some medication to help protect her kidneys.

They have only previously had one ALK positive patient like Olivia on the ALK Inhibitor trial at the NIH, and unfortunately that child didn’t respond to the drug. However, they were quick to point out that they have two patients on the trial in CHOP in Philadelphia who are currently doing well on the drug.

Although Olivia theoretically qualified for the trial based on her test results in Australia a week prior, she was still required to undergo three days of re-testing here to determine her current eligibility. Needless to say this was a very anxious period for Kirsty and I.

On the first day of testing, one of the nurses came in to advise us that her blood test indicated that she was neutropenic (meaning that she was very susceptible to infection) so we needed to make her wear a face mask and keep her away from other people as much as possible. More importantly, her neutrophil count at 110 was significantly below the required level of 750 to enter the trial. We had struggled so much to get her here and travelled so far, only to be likely turned around – this was turning into our worst nightmare! Such low counts would also mean that the flight home would present a very high risk of infection for her.

After about half an hour contemplating the ramifications of this bad news, the Dr came in to see us and indicated that something didn’t add up with the blood test results so he rang the lab to check. It turns out that of all the many numbers in the blood test report, there was a typo on the one that mattered the most to us. Her neutrophil could was actually 1100, not 110, meaning that she was now back in consideration for the trial! This was just so typical of the emotional rollercoaster ride that we have been on over the last month.

Olivia underwent several different scans, some taking hours. She was a champion and lay so still for each one. They did have trouble finding a vein to inject contrast for her head MRI, which caused her a lot grief. Once she recovered and dried her tears, she actually apologised to us for making such a fuss. This, practically brought Kirsty and I to tears. It is so unfair that she has had to deal with this for most of her life!

We went in with Olivia for her MIBG scan, which we knew would show extensive disease. They indicated that they do one full body scan and then do a spinning scan of one or two of the worst areas. After the first scan, the technician indicated that she was going to consult with the radiographer about which area to re-scan. She was gone for ages. By this time we were becoming very concerned that there was so much disease that they couldn’t determine which parts to re-scan! Finally the technician came back in, apologised for the delay and advised us that there were no particular issues. We never actually saw the results of the scan, though were advised that she had very extensive bone marrow disease. Whilst that is obviously not good news, it was not unexpected.

They did note some spots on one of her lungs from the CT, though this was not confirmed on the MIBG scan. It is possible that there are tumours starting to develop there, which could become very dangerous. The MIBG scan revealed a small tumour near her eye, which explains the bruising and swelling, as well as a couple of bony lesions but it mostly conveyed extensive bone marrow disease. Most importantly, her brain remained free of tumours and she qualified for the trial. The biggest risk to Olivia’s health remains the disease suppressing her blood counts and associated problems such as anaemia, internal bleeding and infection risk.

Olivia qualified for the trial and commenced taking the trial drug crizotinib on the Friday. She is required to take an oral solution of the drug (she refuses to take the capsule after some bad experiences in the past) and needs to take it twice a day, 12 hours apart. Although she clearly hates it, she always eventually takes it. She didn’t stomach the first dose of the medication very well, throwing it back up 15 minutes later. Apparently, this is not uncommon as the nurse later told us that every patient had vomited the first dose. Since that first dose, we have given her an anti-nausea drug half an hour beforehand. According to Olivia, the medication is a very sweet but foul tasting syrup and continues to make her feel a little nauseous. She rinses her mouth out afterwards with water and then has a mouthful of something nice to counter the bad taste. We have been giving her ice cream, but unfortunately, she is now turned off ice cream. We can add that to the list of foods including apple sauce, yoghurt and juice, that she has been put off by taking them with different treatments over the years. We don’t normally let her have soft drinks back home, but we have just tried giving her lemonade instead. She may end up being turned off soft drinks too, which is probably a good thing, as ‘soda’ is everywhere over here and is hard to avoid.

The whole time we have been here, we have all been going to bed quite late and sleeping in. This has been mostly caused by Olivia having to take her medication quite late at night. Olivia seems to take a long time to get to sleep each night, which may be combination of a possible side effect of the medication and the fact that she usually sleeps in the afternoon when receiving a transfusion as she receives pre-medication that makes her very drowsy.

Other than that one huge mistake with the blood count mentioned above, we have been very happy with the level of care over here. The doctor/nurse to patient ratio is so much higher than in the public system back home and they are always willing to sit down and discuss anything relating to her treatment and arrange any further testing/investigations necessary.

In our second week here, Olivia started to experience tooth pain, due to one of her adult teeth pushing in front of her baby tooth, in a similar fashion to what happened several months ago. Olivia’s oncologist booked her in at short notice to see the dentist. They explained that provided the pain doesn’t get any worse the tooth could stay in to see if it will fall out on its own accord. As part of the investigation, they conducted a panoramic mouth x-ray. We were surprised to be told that Olivia has not developed her adult molars for whatever reason. It is unclear if this is due to a treatment side effect or just an abnormality that Olivia was born with. It just means that she will have to live with her baby molars for life and will probably require extensive dental work. This is merely one of many issues that we will happily deal with in the future if we can cure her of this dreaded disease.
We were warned by the doctors that one of the potential side effects of the ALK inhibitor was light visual disturbances. After only a few days on the crizotinib (the trial drug), Olivia started experiencing slight blurring vision at night, when in darkness. The doctors decided that it would be best to get her eyes checked out. Her vision was okay, but the test indicated that she has swollen optic nerves. This was initially a concern as this can often indicate pressure in the brain. However, her recent head MRI didn’t show anything. We had a follow up appointment with the ophthalmologist/neurologist, who confirmed that both optic nerves were swollen though could not find an immediate cause. He ordered another MRI just to be sure, which also came back clear. It was suggested that one possible cause is her low haemoglobin levels. We just need to ensure that she is monitored carefully as this can lead to further eye problems.

Overall, Olivia remains quite well. We were initially hopeful that the treatment is working. Given that it has been four weeks since we left Australia, we expect that she would be much more unwell if the treatment wasn’t doing anything. There seems to be several contradictory signs with her blood test results, for example, the tumour marker, LDH, has been fluctuating when we were hoping that it would just continue to decrease. Most of Olivia’s blood levels are still quite abnormal as well as some of her liver enzymes, suggesting that the cancer is still significantly affecting her bone marrow. As such, we are not under any illusion that the treatment is rapidly killing off most of her disease. It may be doing something, but at this point, we can’t really be sure how effective it is.

The bruising and swelling around her right eye seemed to subside within a week of starting the treatment, filling us with hope that the treatment is working. In fact, the bruising had completely healed. However, she has since developed a new bruise and her eye is again swollen, appearing quite misshapen.

Olivia was originally needing red blood cell transfusions once a week, though her last one lasted her just over two weeks. However, she still requires frequent platelet transfusions, averaging about every three days. If we were back in Australia, we might be able to boost her platelets by giving her back some of her own stem cells (that we recently collected), however, we can’t do this whilst she is on this trial.

Thankfully, it is pretty easy to tell when Olivia needs platelets, as she normally wakes up with bleeding gums. The hospital still has to do a full blood count and await for the results before transfusing her. She also requires prophylactic pre-medication as she can have a severe allergic reaction to platelets so, all up, it can take around 5 or 6 hours. We are hopeful that her platelets will start to last longer as the treatment continues, so she won’t need to spend much time in hospital. Other than transfusions and scans, the treatment itself actually requires very little hospital time.

Whilst we can walk to hospital from the Inn, we are so glad that we have a car, as the nearest supermarket is about a ten minute drive. Given that we only have one shelf in the shared refrigerator, we find ourselves going to the shops every second or third day. We have also done a bit of exploring Bethesda and downtown DC, though the traffic here can get pretty congested. From what we have seen so far, this is a really nice part of the US. We had originally planned one or two overnight trips to surrounding attractions, though this will have to wait until Olivia’s blood levels stabilise some more, so that we can be sure that she won’t need a transfusion (which we would have to pay for) whilst we are interstate. We also have been told that she should not be going on any rollercoaster rides or the like that could possibly cause internal bleeding. Olivia was not happy at all to hear that. Hopefully, if everything goes well, we would love to take the girls to DisneyWorld on one of our future trips when we return for scans.

Olivia has three days of scans and procedures this week, which will show us if the treatment is working. On the one hand, we are scared to see how much disease she has, but on the other we are so hopeful that there is a significant reduction and that we can return home with the medication knowing that we have bought our gorgeous girl some more time.

We should mention that there is another treatment option available to Olivia at the NIH, should the current ALK inhibitor not work and Olivia remains well enough. It is a customised treatment, based on testing of her disease’s response to numerous different agents in the laboratory. This seems like a logical approach, given that different types of neuroblastoma respond to different treatments. This will require taking a tumour sample for testing and can take a minimum of three weeks to analyse. Unfortunately, as Olivia’s disease is in her bones so it has never been easy to obtain tumour samples.

When we were in Germany, we became friends with a Scottish family who also have a daughter called Olivia, who is one year younger than our Olivia. In what was a shocking week for all of us in Jan 2011, both Olivias relapsed within a few days of each other. Unfortunately, she has been constantly fighting the disease ever since. In a desperate attempt to help her, they recently travelled to Mexico for a some alternative treatment. Unfortunately, Olivia’s health has been deteriorating and they are now desperately trying to get her back to Scotland so that she can be at home. We pray that her condition will improve enough to allow her to make the journey home and that they can raise the required funds to make it possible.

Our Beautiful Girl