Back from the US

Sisterly Love

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. 

5 thoughts on “Back from the US”

  1. Have followed Olivia’s journey for a few years now. You have done so much to help your beautiful girl. Please know you are in our thoughts always.

  2. Kirsty & James, you are NEVER, EVER far from our thoughts. Our hearts are breaking for what you have all, especially Oli, had to endure unrelentlessly for the past 6 years. You have been the most amazing parents/ family who have done absolutely EVERYTHING you possibly could to help Oli. You gave her the extra years that you were told she wouldn’t have, you never gave up. I have always been in awe of your strength & determination. Sending SO much love your way, I wish with all my heart I could do more. Love & hugs to you all xxxxx

  3. Thank you so much for taking the time to update. It is very much appreciated. I am sending strength, thoughts and prayers to Olivia and all who love her.

  4. Dear Olivia

    You are such a brave couragoeus little girl who has fought so hard along with your amazing family that helped carry you thru the hard days….from my family we are sending you hugs kisses and so much love ♥

    Your story touched my heart when I saw your story on 60 minutes so much and although I have never met you sweetheart my family has so much love for you ♥

    love always ♥

  5. Thank you for posting up date…

    And know that this FANTASTIC family are in our thoughts and prayers.

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