Symptoms began with feelings of nausea on the morning of Friday 26 July 2013, followed by a perfectly normal day. This progressed to a single vomit in the morning, followed by a perfectly normal day. After ruling out a stomach bug and other common ailments, the GP was the next port of call. He was diagnosed with reflux, and prescribed antacid medication.
With no significant improvement, we went back to the GP, where he was prescribed stronger antacid medication with advice that an immature digestive system, in particular the sphincter muscle at the entrance to the stomach, can sometimes cause this problem. Again, there was no improvement and a further GP appointment was made. He was referred for blood tests, which were taken on the 20th August, and they came back indicating high potassium. He was recalled to hospital for further tests, which proved to be normal. We tried late night and early morning feeds to rule ketotic hypoglycemia, again no effect.
By this stage because of regular vomiting, he had become a little lethargic from a lack of regular nutrition. On each of the days over the Bank Holiday, 24th to 26th August, he vomited three times. So on Tuesday 27th August, it was back to the GP, where we forced a paediatric appointment at the John Radcliffe Hospital. At 5pm on the 27th, I received the call from my wife at the hospital to inform me that Skye had a brain tumour, and I was to get to the hospital as soon as I could for a meeting with the Neurosurgeons.
A CT scan had indicated a significant mass sitting between the Cerebellum and the Brain Stem, and a few simple tests confirmed that his balance and co-ordination was starting to be affected. Just a week earlier, family and friends had witnessed him tearing about the garden with absolutely no indication of anything untoward, apart from nagging nausea each morning.
On Wednesday the 28th August, he underwent an MRI scan under GA to obtain a much clearer picture and to assist the planning for surgery. The decision to proceed with surgery was an easy one, in relative terms: "if we don't operate, this will kill him" are words that still resonate through my mind on a regular basis, and every time I 'hear' them I well up.
Surgery is the frontline treatment. An incision down the back of the head and along the line of the cervical spine and removal of a section of the base of the skull, allows access to the cerebellum, the portion of the brain that controls co-ordination and movement. The surgeons gently lift the cerebellum to access the fluid filled fossa between the cerebellum and the brainstem. They remove as much of the tumour as they safely can, with as little disturbance to the brain as possible. They then stitch everything back into place.
The analysis of the tissue confirmed that the tumour was a medulloblastoma. It is the most common of childhood brain tumours. It is much easier to treat if it is confined to its origin, but it becomes more complicated if it has become metastatic. Unfortunately, in Skye's case, it had become metastatic and nodules were evident not only elsewhere around the brain, but in the spinal cord as well.
The standard for treating High Risk Medulloblastomas in the UK was known as the Milan Protocol. As a result of some very positive outcomes in a clinical trial in Milan, the Children's Cancer and Leukaemia Group (CCLG) proposed that the UK adopt this protocol of treatment in the absence of any better form of treatment.
The Milan Protocol was a sandwich treatment, which operates on the principal that chemotherapy immediately post surgery will act to reduce the size of any residual tumour, giving the subsequent radiotherapy a better chance of 'mopping up' the rest of the malignant cells. Radiotherapy, although seen as the gold standard in terms of its efficacy in treating cancer, also has devastating long term consequences for the developing child.
Radiotherapy is then followed up by further chemotherapy to consolidate the work done by the radiotherapy. This comes in one of two forms, either maintenance chemotherapy or high dose chemotherapy. The course of action is dependent on the results of an MRI scan taken prior to the start of Radiotherapy. If there is no sign of residual tumour (complete remission), then maintenance chemo is adopted, but if there is evidence of stable or progressive disease following upfront chemotherapy, then myeloablative high dose chemotherapy is adopted, followed by stem cell rescue.
Withdrawal of the Milan Protocol
In May 2014, the Milan Protocol was suspended due to the emergence of severe neurotoxicity. Children continue to be treated with a combination of Radiotherapy and Chemotherapy, but under a different protocol.