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Alice’s Arc was inspired by the ongoing journey of a little girl named Alice, diagnosed with stage 4 alveolar rhabdomyosarcoma, at the age of 3, in March 2015. She had 20 months of chemotherapy at GOSH and proton radiation in the US. Alice spent one year in remission before her cancer returned in February 2018. She underwent 6 months of relapse chemotherapy and a specialist surgical procedure with brachytherapy, known as AMORE, in Amsterdam. Alice spent almost a year clear of cancer before masses were detected in her abdomen and pelvis in July 2019. There were no options left. Alice died, aged 7, in October 2019, over 4.5 years after her diagnosis.
In her last few weeks alive, Alice made many insightful comments. She is the voice that drives our work everyday. One day, Alice, we will make sure that answers are found, so that children with rhabdomyosarcoma can be confident of growing up and leading a normal life.
I wish I had a normal life. I should be at school having fun with my friends.
I’ve got better twice before, I don’t need treatment this time, my body soldiers can fight the cancer.
I wish I could get better. Tell me all about this cancer. When will they find me a treatment?
It took about 8 weeks for Alice to receive her diagnosis and start treatment. She presented with a small lump on her neck, she was completely healthy otherwise. The GP thought it was a lymph node infection and asked us to return in a week. The lump did not go away and it seemed to be growing before our eyes. On return to the GP, we were referred to our local hospital, where they diagnosed an infection and prescribed antibiotics. It still did not clear up and we were getting concerned. We decided to access our private local hospital and they did various blood tests, ultrasounds and prescribed further antibiotics and more appointments. After another few weeks, they suggested we visit an ENT specialist in London. He believed the growing lump was a cystic hygroma but felt an MRI was necessary to confirm this. The date for the MRI was a few weeks ahead but we pushed for this to be done earlier due to our increasing anxiety. The MRI revealed a solid mass and Alice was urgently referred to the oncology department at Great Ormond Street Hospital.
The next day, after an ultrasound at Great Ormond Street, the team told us the devastating news that this was a tumour – a rhabdomyosarcoma or a lymphoma. Alice was admitted a few days later and underwent four days of investigations and procedures including a biopsy, insertion of a hickman line, PET scan, CT, lumbar puncture and blood tests. On the 4th day the full diagnosis came – stage 4, fusion positive (PAX3-FOX01), alveolar rhabdomyosarcoma with the primary tumour attached to the submandibular gland in her neck with positive lymph nodes and a tiny tumour in her lung. She was given a 50/50 chance of survival over 5 years. At this point, the tumour was enormous and was compromising her airway so Alice’s treatment began the next day.
Alice’s treatment was led by Great Ormond Street Hospital in the UK and took her to the US and Europe, in order to access the most effective treatment options.
Her frontline treatment involved 3 cycles of in-patient IVADO chemotherapy and 6 cycles of IVA in-patient chemotherapy. She responded very well after the first 3 cycles and the tumour shrunk substantially and the lung tumour disappeared. This meant Alice was approved to travel to Florida, US, to receive 29 proton radiation sessions, at the University of Florida Proton Therapy Institute. She also received 7 sessions of photon radiation to her lungs. Proton radiation is a highly-targeted form of radiotherapy – the proton beams kill the cancer cells and cause less damage to surrounding healthy tissues. This is particularly relevant to young children so that long-term damage can be limited. Her chemotherapy was given at Wolfson Children’s Hospital, Jacksonville in conjunction with the proton radiation. We spent 11 weeks in total in Florida and returned to the UK for her final few rounds of IVA chemotherapy.
Alice’s response to this treatment was very encouraging and she underwent 12 further months of out-patient maintenance chemotherapy involving the drugs cyclophosphamide and vinorelbine. In October 2016, after 20 months of treatment and aged 5, she rang the end of treatment bell.
Over the next 15 months, Alice underwent MRI scans of her head/ neck and X-rays of her lungs every 3 months and these remained stable, until a scan showed some small changes at the primary tumour in the head/neck area. At this point, it was unclear, what this meant. Could it be changes as a result of radiation or was this the tumour activating again? Alice underwent an MRI, a PET scan and a CT which appeared inconclusive. However, the PET scan revealed several areas which needed further investigation. The decision was taken to wait a month and rescan. Following another MRI and PET, Alice had surgery to remove a suspect lymph node on her neck which turned out to be non-cancerous. Alice’s team at Great Ormond Street had been concerned about doing a biopsy on her primary tumour site due to the location near an artery leading to the brain. Several options were considered due to the inconclusive MRIs and the decision was taken to biopsy the area to find out if the changes were active rhabdomyosarcoma once and for all. Sadly, the biopsy, in February 2018, revealed active rhabdomyosarcoma cells. A relapse is beyond devastating news for a family and Alice’s odds of survival dropped to 8%.
At this stage, we explored a trial at the Royal Marsden Hospital involving a new inhibitor drug called Regorafenib alongside chemotherapy. However, the trial was closed as it was proving very toxic for some children and the laboratories needed to revisit dosages of the drugs in combination. Therefore, we had no choice but to access the relapse chemotherapy VIT at Great Ormond Street Hospital. This combination is unlikely to be curative unless combined with another type of treatment. In order for Alice to have the best chances of a cure, Great Ormond Street suggested a procedure in Amsterdam known as AMORE. This treatment is not available on the NHS and would need to be self-funded. Amsterdam agreed to look at Alice’s case if the tumour responded to VIT chemotherapy and subject to the tumour appearing no where else in her body. Thankfully, Alice’s rhabdomyosarcoma responded very well and the cancer was localised so she was approved for AMORE in Amsterdam.
AMORE was undertaken at Emma’s Children’s Hospital in Amsterdam. This was prior to the children’s cancer unit being moved to the Princess Maxima Centre in Utrecht, which is dedicated to treating children’s cancers. The procedure involved a major surgery to remove Alice’s tumour which was highly complex and risky due to it’s difficult location. She had rods inserted in to her neck in order to receive brachytherapy radiation. Upon completion of this surgery, Alice had a day to recover before brachytherapy commenced. This was undertaken every two hours for 15 minutes over three days and nights. This was gruelling and we weren’t allowed to be in the room when this was administered. During this time Alice contracted an infection and had to be treated with antibiotics. She remained slightly sedated during this part of the procedure as she was unable to move from her bed. Alice was then allowed a few days to recover before her next surgery where the rods would be removed and her face reconstructed, if necessary. She was very lucky and the Amsterdam team were able to close up her face successfully with no need for reconstruction. Alice remained in hospital, in Amsterdam, for a further two weeks to recover. We stayed in the hospital for just over three weeks and then returned to the UK, where Alice completed two further cycles of VIT chemotherapy. We felt elated when scans in October 2018 showed no evidence of cancer and Alice proudly rang the end of treatment bell for a second time.
She then entered a second phase of scanning every three months in the same way as the first time. Everyone was overjoyed with the results of VIT and AMORE and there was huge celebration with each set of clear MRI’s. Following a clear MRI of her face and neck in July 2019, we initiated a discussion about our concerns relating to Alice’s abdomen which had started a few weeks before. It appeared to be increasingly distended and we asked for an ultrasound. We were delighted about her clear scans of her face and neck and hoped this ultrasound was just a formality and a ‘normal’ reason could be found for her larger than usual tummy.
Unfortunately, the ultrasound found a large mass (size) in her abdomen, with masses forming around her bladder and pelvis too. This was impossible news to hear and for the next few days Alice found herself having a biopsy and MRI, PET and CT scans. Sadly, these showed extensive rhabdomyosarcoma (we had hoped it might be a different type of cancer since Alice had never had disease detected in this area – funny what you wish for) in the abdomen and pelvis areas which they would not be able to conduct surgery on. Blow after blow. In disbelief, we were introduced to the palliative care team and Alice was given weeks to months to live.
We started researching treatment options around the world and sought a second opinion in the US where an option involving surgery and HIPEC chemotherapy was a possibility. However, Alice was not going to be accepted for this due to the location and extent of the disease. Alice was becoming increasingly unwell as her stomach grew with fluid build up as a result of the tumours creating blockages. We were offered non-curative chemotherapy but Alice was adamant that she wanted no more chemotherapy and no more hospital. Experts believed that treating her at such an advanced stage was not in her best interests.
And so, we took Alice home in August 2019, under the care of the out-patient palliative team at Great Ormond Street Hospital, where she peacefully died on the 8th October 2019.
A rollercoaster ‘highs and lows’, beyond words, a learning experience, life-changing, shocking, exhausting and emotional. In some ways a beautiful insight in to how special life is and how important it is to seize the day and live a ‘full’ life experience.
Alice has left us with a mission. We want to see more new, curative treatment options for children at relapse. We want to see improvements in frontline treatments that can diminish the chances of relapse. We want to see kinder, better treatments that do not leave survivors with the devastating life-long side effects that we see now.
After chemotherapy blood counts drop dramatically making a child neutropenic. This means they have no immune system to fight off infections. If Alice’s temperature hit 38 degrees she went to our local hospital. Blood is taken to see if an infection is present. If the blood test is positive for an infection a long stay in hospital to recover and administer antibiotics is required. For example, chickenpox needs 10 days of treatment with a specific antiviral drug. Antibiotics are given through the Hickman line and usually infused 2/3 times every 24 hours depending on the infection type. If no infection is detected a stay of 48 hours is required to receive standard antibiotics. No temperature must be present for at least 24 hours before the child is sent home.
At such a young age Alice could not be expected to lie still for the multiple scans, procedures and radiation she received during the cancer treatment. Alice had a general anaesthetic each time she had imaging to assess the tumour e.g., Magnetic Resonance Imaging (MRI) and Computerised Tomography (CT), had a clinical procedure such as a biopsy, lumbar puncture to assess the cerebrospinal fluid, Hickman or Peripherally Inserted Central Catheter (PICC) line insertion (devices needed to allow infusion of chemotherapy to larger vessels in the body) and radiation treatment. With every anaesthetic there are certain risks to the child.
Alice had a Hickman line as it is less traumatic to receive treatment and have regular bloods taken through this method rather than inserting a cannula each time. Hickman lines are inserted in to a large blood vessel just above the heart. This enables chemo and antibiotics to be pumped around the body. Alice had two lumens coming out of her chest that were protected in a small bag that she wore. Having a Hickman line meant that Alice was at a greater risk of infections entering the body so they had to be cleaned regularly and you are unable to get the site of the insertions or the lumens wet. Therefore, Alice was unable to go swimming for 20 months or take a proper bath. Alice also got several Hickman line infections which resulted in long stays in hospital to treat them. Community nurses took bloods using the Hickman line twice a week throughout Alice’s treatment to monitor blood counts. This helps to assess whether Alice was well enough for chemotherapy and when to be extra careful going to public places where infections could be caught.
VIT chemo comprises Vincristine, Irinotecan and Temozolomide and is given for 5 days consecutively every 21 cycles. Vincristine is administered as a push, irinotecan as an infusion and temozolomide in tablet form. It was very difficult to convince Alice to take the tablets and have the infusion of irinotecan as she was aware that they made her sick.
This involved 4 chemo drugs called Vincristine, Actinomycin D, Ifosfamide and Doxorubicin. The first two drugs are administered via an injection in to the Hickman line. The second two are infused in to the Hickman line over a few days and nights. At the same time hydration is also infused to flush the toxic drugs out of the system. This means the child is hooked up to a machine for a number of days unable to move or play properly and also feeling very ill as the drugs take effect. All the hydration makes the child need the loo very frequently so they are unable to sleep well. The drugs were given for 8 months every 21 days over the course of 4 days and 3 nights.
Alice went to Jacksonville, Florida’s UF Health Proton Therapy Institute for 11 weeks to receive this treatment as it isn’t available in the UK yet. This was funded by the NHS as part of her treatment protocol. Over the course of 6 weeks Alice received proton radiation every week day. Anaesthetics are required as it is vital that the child is completely still to ensure the precision of the proton beam. Alice received chemotherapy alongside this at Wolfson Hospital. Proton radiation is preferable for children with some brain tumours or cancers located in the head, neck or spinal areas as it is highly targeted and causes less damage to healthy tissues than conventional radiation.
Chemotherapy makes blood counts drop dangerously low. Drops in red blood cells and haemoglobin cause extreme feelings of fatigue and lethargy. If it drops below a certain level a blood transfusion is conducted using the Hickman line. This typically takes 4 hours. Similarly if platelets drop below a certain level a platelet transfusion takes place. It is very common for children receiving chemotherapy to receive these transfusions frequently.
This procedure involved an 8 hour surgical procedure in which the tumour was removed and rods for brachytherapy were inserted. The next stage involved 3 days of brachytherapy administered every 2 hours for 8 minutes each time. During this time the parent had to leave the room. She then underwent another surgical procedure to remove the brachytherapy rods and reconstruct the area where the first surgery had taken place. Alice was unable to eat and kept sedated for some of this procedure.
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