Keep Focused and Stay Strong
A Life Changing Diagnosis
Brooke was a typical 16-year-old, enjoying life, working part time as a waitress in a local restaurant and had recently participated in the Duke of Edinburgh Award Scheme on an overnight expedition. She just had her Year 11 Prom and was in the middle of her GCSE exams at High School.
She had been experiencing some dull low back pain for quite a few weeks, but the doctors had dismissed this as growing pains and advised her to take pain relief when necessary. They gave us instructions to return if her symptoms changed. Quite frequently during the night she awoke in great pain and it became very upsetting that she was experiencing this. We felt we were being ‘fobbed off’.
After one particular shift at the restaurant, Brooke called home to ask for a lift as she had started to feel some numbness / tingling in her right leg and that her foot felt quite strange, as if she had a great big snow boot on it. She also felt that something was wrong when she went to the bathroom, as she was suddenly unable to control her bladder.
That evening her dad, Pete, took her to our local Accident & Emergency room where they examined her and found that she had large amounts of protein in her urine. They also wanted her to be booked in for an urgent MRI scan, however, she was to be called back in the morning.
The morning after Brooke was adamant she was going to take her GCSE History exam. She has studied so hard and was insistent to go to school. She was in quite a lot of pain, so we dosed her up with Paracetamol and as she found it more comfortable standing up, the school arranged for her to take the exam standing up against a high table rather than sitting at an exam table.
Shortly after the exam, the hospital called, and we took her for the MRI. Upon initial examination, they requested another MRI with contrast dye, which at the time did not mean anything to us. She was away for what seemed like a long time. We were becoming quite worried at this time that there was something very wrong. After the MRI, we were sent to another hospital so that the specialist neurologists could examine the scan and then report back to us.
Treatment, Recovery, and Moving Forward
Brooke was then admitted to Salford Royal Neuro Hospital in Manchester and we were left anxiously waiting for someone to come and see us. When they did, we were in total shock. Their initial comment was that there was a lesion along her spine. When we asked what that meant, they just said that it was something that shouldn’t be there! At this point we were all very scared. How did this happen? What did this mean? What could they do? The lead neurologist came to speak to us and performed a full examination on Brooke. They then dropped the bombshell that she wanted to operate on her the following day since she was slowly becoming paralysed as her spinal cord was being compressed by the tumour and that tingling and numbness she was experiencing was because of this tumour. We were in total shock! They wanted to perform a T4-T10 laminectomy to try to remove as much of the tumour as possible and warned us the operation could take in excess of 6-8 hours!
Brooke's operation was actually 15 hours long! The wait from 3pm in the afternoon was excruciating. After six hours, we were informed that she was doing well, and that the surgeons wanted to operate very slowly and carefully so as not to damage her spinal cord. They told us it could be another four hours or so. The lead neurosurgeon called in about 4am in the morning to speak with us to say that she was pleased with the progress and that they had in fact removed about 95% of the tumour which is more than what they initially thought they could do (although they didn’t tell us that!). The spinal surgeon was then left to place her spine back together with several small titanium rods. We were shortly told she had an Ependymoma Grade III tumour!
Brooke spent 5 weeks recovering on the Neurology Ward at Salford Royal and was then transferred to The Christie Young Oncology Unit where she was an inpatient for a week. She was being prepped for Radiotherapy there and had daily physiotherapy to learn to walk again. Brooke had to start wearing a back brace for at least the next six months. She was also confined to a wheelchair for the time she was having her treatment there, as she was so weak, and in so much nerve pain.
She endured 30 fractions of 35Gy craniospinal radiotherapy with a boost to 54gy which finished in September 2015. The treatment made her very weak, very sickly and she lost quite a lot of weight.
Shortly after finishing her treatment, we were given the fantastic news that the tumour was gone, and that no more treatment was necessary. We were so relieved! She was cancer free and we could finally put the past four months behind us! She could at last plan her future, starting with A Levels at college and learning to drive. She had her life back, and we could all breathe a sigh of relief that she was finally pain free and could carry on with her life!
Brooke broke free from her wheelchair and used a walking stick for a short while. She also learned to drive, quickly passed her test, and studied for two years at a local Sixth Form College gaining 3 great A Levels in Art, Business and Accountancy. Brooke thrived in education. She was successful in a Level 3 Business Apprenticeship in Payroll, where she spent a year gaining experience in the workplace.
Shortly afterwards she accepted a place at Manchester University and began to study for a Degree in Teaching.
The Devastating Recurrence
Brooke continued to have MRI scans every six months and then in December 2018 we were given the devastating news that the cancer had returned in her spine at T 7/8. She had no symptoms at all and so it came as a massive shock to us. She completely broke down and we were all just so upset. We had plans to travel to Poland for Christmas and her Oncologist told us that we must still go, and that he would liaise again with the neurosurgeons at Salford Royal for yet another treatment plan. We were determined to enjoy our family time away and we thoroughly did just that!
Upon return she had a complete resection. Again, we breathed another sigh of relief.
However later in the same year, after complaining of more aches and pains, she was diagnosed with metastatic spinal recurrence in September 2019 and so began Cisplatin/Cyclophosphamide Chemotherapy from October 2019 to June 2020, converting to Carboplatin/Cyclophosphamide due to hearing loss and tinnitus. She responded very well to the chemotherapy, however, it was stopped early due to prolonged myelosuppression. She had no treatment then for three months until the next MRI scan showed further progression with two small tumour nodules in her posterior fossa and an extra-axial nodule adjacent to C 1/2. She began another course of chemotherapy straight away, which was Oral Etoposide from January 2021 to April 2021. Unfortunately, a further MRI scan showed one cerebellar lesion at C 7. This chemo regimen was once again halted, and Brooke was referred back to the MDT at Salford Royal / The Christie, where they discussed her case again in detail. An operation was ruled out due the many risk factors, however, a Cerebellar lesion biopsy was carried out in May 2021 for the purposes of molecular profiling at a BioBank in Luxembourg as part of a “Specta” Study. This is a collaborative European platform that delivers high-quality molecular and pathological screening across tumour types to aid patient selection into clinical trials. The biopsy was sent over to Europe. We were however informed that this may take several months.
Clinical Trials, Advocacy, and Community
In the meantime Pete and I spent many hours researching clinical trials, not only in the UK but worldwide too. We felt that Brooke should be still on some form of treatment, if only in the meantime, since we knew how fast these tumours grow. We were not happy to be told that there was no other treatment for her at this time. Pete had read about the Phase II study of dose dense Temozolomide and Lapatinib called CERN 0802 on recurrent low grade and anaplastic spinal cord Ependymoma and had previously been in touch with Dr Mark Gilbert at the NIH through email for his perspective.
We researched in great depth for any other possible treatments for Brooke and would have gone to the end of the earth for her. We happened to read in great depth about the CERN Foundation and how this prospective phase II clinical trial could target the HER2 proteins common in ependymoma tumours. We discovered that this treatment, with demonstrated clinical activity with objective responses and prolonged disease control associated with disease-related symptom improvement, could be actually an option for adult patients with recurrent ependymoma. We knew that the trial had been carried out and had been completed and that it could stand as a standard regime for recurrent ependymoma. We immediately contacted Brooke’s oncologist at The Christie and asked for his approval to try this new treatment.
A few days later, he called us back to inform us that Lapatinib was not routinely offered on our NHS, and that we may have to pay privately for it. We were devastated on hearing this, but we were not going to let this setback stop us from attempting to get Brooke the treatment she so deserved. We were then referred to a private consultant, but in the meantime, her oncologist mentioned that he could possibly request funding from GSK on compassionate grounds since there is no other alternative treatment for Brooke, her diagnosis could be life threatening, or it could become seriously depilating, and that there was sufficient evidence to believe that the potential benefit to Brooke justifies the risk. A few days later we were overjoyed to be told that we had been granted compassionate use for the Lapatinib. It was then just a case of waiting for The Christie to approve this drug.
Brooke has now been on Lapatinib and Temozolomide now for almost 3 months, and after a rocky start, after developing an all over body rash and after a couple of infections (pausing treatment) she is now more settled with the week on/week off regime and her recent MRI has shown stable responses from her previous MRI scan in April 2021.
Currently Brooke is not suffering from many side effects and is generally quite well. She decided to leave University and concentrate on being an artist. She draws pencil portraits, as she is a fabulous artist with a keen eye for detail and a unique creative flair. She has more recently also become interested in tattooing. She enjoys spending time with her friends as they are very important to her, and so is her family. She has bought lots of tattooing items and is planning on becoming a Tattoo artist.
Brooke’s advice to others is to live life to the full, always be happy, keep your hopes up, stay focused and positive. Brooke is definitely a positive person and always looks on the bright side. She focuses on what is good in her life and finds joy in the simplest of things. She has a fabulous attitude to life and excels in everything that she does. Nothing seems to get her down. Even on her ‘off’ days, she tries to smile. I am sure that Brooke feels fear sometimes but she does not let it stop her from moving forward. She is the strongest person I know, and she is definitely my “hero”.
To conclude, we are grateful to Dr Mark Gilbert and his team for trialing this new treatment for recurrent ependymoma and feel very lucky to have researched this, in that our daughter is on the very same treatment here in the UK even though the Lapatinib is not routinely offered in the UK. It seems that Brooke’s consultants were struggling to find any alternative treatment and so we were delighted to pass on the outcome of the US trial as a hopeful treatment for her, to at the very least, stabilize her tumours to control the disease.