Looking back at the time since our eldest son became ill with a brain tumour and reflecting on all that has happened since, is a difficult undertaking. Revisiting the emotion and trauma of that time can be just as stressful as when it originally occurred. We are, though, committed to using our experience to help the learning of others so that patients, their families and friends have better care. Any story can be told from different perspectives, and a key component of our son’s treatment was proton therapy, but it starts some time before that.
Our son had been back and forth to our GP over a period of about two years before a diagnosis was made. He suffered from headaches generally in the morning, and felt ill and very breathless after exercise. The GP suggested the headaches were due to too much homework and his love of chocolate bars. Exercise induced asthma was diagnosed so an inhaler for school sports lessons was prescribed. He became increasingly difficult to rouse in the mornings; I can remember physically lifting him out of bed to get him up for school and the associated tension this generated in the household. He was 14 at this point. In the evenings he was bright and alert and he was a high achiever at school.
In the spring of 2005 he became worse, with headaches and vomiting in the mornings in the car on the way to school. On holiday in Lincolnshire that Easter, he would become ill as we drove over a potholed track and he would vomit after exercise. His mother was convinced he had a brain tumour and had looked for neurological evidence of change in gait and vision. Despite repeated visits to the GP and a direct question about the possibility of a brain tumour, the GP was dismissive and only reluctantly and after much pressure referred him to the children’s unit of the local hospital for an outpatient appointment. He refused my wife’s request for a referral to the specialist centre insisting that they only dealt with ‘serious cases’ there. It is no exaggeration to say that at this point we were frantic with anxiety.
We decided we could not wait a couple of weeks for a local hospital appointment and we contacted the specialist children’s hospital and took our son on the same day to be seen by a paediatrician. I worked within the hospital trust and knew people who could expedite an examination. After a thorough examination and the finding of a pattern of short duration headaches and morning vomiting a CT brain scan was suggested. An urgent brain scan was performed later in the afternoon; I am sure this was expedited because I worked in the trust and we were both radiographers. The findings of the CT brain scan and the MR brain and spine scan which followed immediately continue to make me shudder. Our lives were turned upside down and changed forever. A large tumour in front of the brain stem was pushing it backwards and compressing it to 25% of normal size. The tumour was compressing the cerebellum and extended through the foramen magnum to the level of the second cervical vertebra.
The next few weeks were a blur and, even with the assistance of hindsight, remain difficult to completely disentangle. The initial view was that this tumour was a benign dermoid or epidermoid cyst although no further base of skull imaging was performed. About three weeks after scanning the operation to debulk the tumour took place at the local adult neurosurgical centre, rather than the children’s hospital which was not equipped for this type of surgery. The adult base of skull surgeon led the operation. We signed the consent form for the operation with death and stroke cited as possible consequences, along with deafness, blindness and swallowing loss. It was suggested that we discuss the possibility of ‘locked in’ syndrome with our son in case the worst happened. We were left alone to do this; it was one of the worst times in our lives and we promised him we would always be there for him whatever happened. What else could we say?
In the morning at about 9am, as we walked with our son to the anaesthetic room, he cried quietly, gripped our hands tightly; he did not want to die. We kissed him as he went off to sleep and left, both numb. Six terrible long hours later we received the news that he was out of the theatre and in the recovery room. He was alive! It was very quickly apparent, though, that all was not well. He could not speak properly or swallow, was deaf on the left side and had very severe double vision, all caused by damage during the operation to the brain stem and nerves in the base of skull. A scan subsequently showed he had suffered a stroke resulting in some of the problems. The paediatric oncologist came to see us in the intensive care unit to give us the new diagnosis which was a chordoma that would need ‘some radiation to treat it’. It was not the benign condition previously suggested, it was a malignant tumour with a five year survival in the region of 35%.
A few days later our son managed whisper to his mother, “Mum, I’m buggered”.
He left hospital after a few difficult weeks; he remained very significantly disabled, crawling around the house and up and down stairs. He had not been referred to the community nursing team and we were left to cope with all the physical and psychological effects of his neurosurgery without professional help. Unable to swallow but craving food, the family mealtime broke up as we could not eat in front of him. We would make food for him to chew and taste and then spit into a bucket. He had a naso-gastric tube for feeds which he would vomit out repeatedly due to undiagnosed hydrocephalus. His younger brother could not stand to be with him and reacted badly to his condition. His vision was poor, unable to see the television; we even bought a new one with a bigger screen. The constant hum of the suction machine that removed the saliva from his mouth as he was unable to swallow hindered his limited hearing even more. His double vision was a huge problem and it was several weeks before we met an experienced optometrist at the Eye Hospital who used opaque tape over his glasses lens so he could see without double vision. We were unprepared for this new situation and we struggled to cope individually and as a family. The fragmented medical care, and the absence of multidisciplinary team working caused significant distress and suffering to both our son and family unit.
He was seen at the local oncology unit where conventional radiotherapy was prescribed for the remaining tumour. The clearance operation had achieved about 80% removal which was good; however a significant amount of residual tumour was wrapped around the brain stem. The clinical oncologist talked of ‘control’, but we had expected a more positive approach. After the pathological diagnosis we started to research clival chordoma largely via the internet, but also accessing research papers from the university medical library.
The general consensus from Europe and the United States indicated that proton therapy was the best way to manage this base of skull tumour. The tumour, chordoma, is of medium radiosensitivity requiring a large dose of radiation to control it. The limiting factor to successful treatment is the amount of radiation the tumour can be given whilst adequately sparing surrounding brain tissue. Proton therapy, with its absence of radiation beyond the target area can, as a result, deliver higher doses to the tumour whilst sparing the critical structures such as the brain stem.
At that time there was evidence from France and the United States to show that the 30% five year survival had been increased to the range 50%-80% via the use of proton therapy. We put this to the clinical oncologist at our first meeting; he dismissed it immediately and would not discuss this option further. We were told “to stop doing our own research”, “it would be best for you and your family to stay in here and get on with it” and “the doctors and nurses are in charge”.
It was only after our son’s treatment that we learnt that in the year 2000 the Proton Therapy Working Party published in Clinical Radiology “…patients have been referred to the Harvard cyclotron, and at Orsay, Paris. They will continue to require referral abroad for proton therapy for base of skull tumours.”
We felt we had no choice, but to continue with the programme we were offered.
Our son went for a treatment shell fitting in the treatment planning phase of the conventional radiotherapy process. The mask made a few days earlier would not fit, because his operation site in the right parietal region had started to swell. The undiagnosed hydrocephalus had resulted in the formation of a meningocele which needed treatment. By this time he had also had a PEG fitted to enable gastric feeding rather than via the troublesome NG tube but, as the wound was still healing and sore, he was unable to lie prone for any period. It was planned that he would lie prone for the radiotherapy as he could not swallow and needed to allow his saliva to dribble out of his mouth during his treatment sessions. At this point all planning had to stop until his post operative condition became stable. We had not stopped our research and this fortunate break in the treatment planning gave us another opportunity to address the proton therapy option. We knew we had to fight to achieve what we thought was the best option for our son’s survival.
We had sought independent medical advice from the Samantha Dickson Brain Tumour Trust: the opinion from a respected clinical oncologist in the UK who had referred patients to the Paris team for proton therapy was that proton therapy may well be advantageous but was dependant on the exact nature of the tumour. He kindly offered to give a second opinion if he could have access to our son’s case-notes and scans. This offer was angrily refused by the local clinical oncologist and, after a heated argument with us, he agreed to refer directly to Paris for a second opinion. It was his view that this was an untested modality and would not employ a higher treatment dose. Our sense of relief was enormous. We knew, whatever the outcome, we had done the absolute best for our son. All decisions made with a sick child will live with you for the rest of your life; there is a weight of responsibility which, if tinged with regret, would be impossible to endure should that child die.
Two things happened then. Scans and medical details were forwarded to Paris for an opinion as to whether proton therapy was possible and would be of benefit. A diagnosis of hydrocephalus was made and further admissions and neurosurgery to treat this were required. Preparation for conventional radiotherapy was put on hold. An initial opinion from Paris came back quite quickly that our son’s tumour was suitable for proton therapy and that this would be of greater benefit than conventional radiotherapy, conferring a treatment dose of 72G rather than the conventional 52G. This was a significant difference, a point conceded by our local oncologist. However, the multidisciplinary team in Paris had not yet discussed the case and neurosurgical opinion was important. At this point our local oncologist contacted our local Primary Care Trust to seek approval to formally refer our son to Paris for treatment and to access funding.
The French multidisciplinary team’s report subsequently came back from Paris stating that the residual tumour was too close to the anterior aspect of the brain stem; a further clearance operation would be required to provide at least 5mm clearance to allow adequate space for the exit dose fall off. Remarkably, at this time, a neurosurgeon from our local centre was on a training programme in Paris and working with the French base of skull surgeon on the MDT. Internationally, there were few surgeons who had the expertise to remove tumour in this location close to the brainstem and no UK expertise. We are forever grateful that the French base of skull surgeon travelled from Paris to our local centre to perform the further clearance required. Once again our son had to face the risk of death, stoke and further cranial nerve palsies and, once again, we had to face losing our precious son. Remarkably, the surgery was uneventful and adequate clearance was achieved without any complications or worsening of his disabilities.
We were now in early November 2005. The local PCT, about two months after requesting, had confirmed funding in October although its requests for various breakdowns of cost from France had delayed the response.
After funding was approved we were left to make all the arrangements for our son’s treatment ourselves. The local healthcare system had no mechanism for or interest in providing any assistance. The next task was to get to Paris; arranging flights with an airline that would take a sick child who was wheelchair bound and required portable suction equipment. The low cost carriers would not fly a post operative disabled passenger and the only airlines that would consider this were British Airways or Air France after medical screening. We went through the medical screening process with British Airways; they required a medical report and scan prior to the flights as our son had a further shunt revision only one week before flying. We arranged hotel accommodation close to the hospital(s) where possible. We had several outpatient journeys to and from Paris for treatment planning and mask preparation. There was one inpatient stay at the Necker Children’s Hospital for the insertion of radiotherapy pins. On this occasion when our son was an inpatient, the hospital offered accommodation for one parent. I was able to stay in the room with my son and his mum stayed in a local convent, courtesy of arrangements through an aunt who is a nun. We attempted to keep the costs for accommodation and flights as low as possible as we only realised late before travelling that the PCT approval was for costs of treatment; the terms had not been explicit and we found we were expected to fund all flights and accommodation ourselves.
A further difficulty was the requirement to obtain a signed, stamped E112 form from DH prior to travel which French medical authorities would accept as a certificate for them to claim back the costs. We required one form for every visit and, initially, these took several weeks to generate through our local PCT. This process was unfamiliar to the PCT staff as they had not been through it before, further adding to confusion. We left for Paris on more than one occasion either without the form, and had to pay direct costs to the hospitals, or with faxed copies which the French authorities would not accept. They would only act on the original signed and stamped forms.
The stress of this bureaucratic nightmare whilst caring for a very sick child eventually overwhelmed us. We asked our local MP for assistance and the process was revised to accept one E112 form for the whole of the care at each institution. We were very grateful for her intervention.
We went to Paris for a prolonged stay from early December 2005 to early February 2006. Treatment started at the Institut Gustaf Roussay just to the south of Paris for 25 daily sessions of conventional radiotherapy. The proton therapy was delivered in Orsay, outside Paris, for a further 25 daily sessions. An early reaction of sickness and vomiting and sinusitis was treated with steroids. We were, after a little while, able to get permission to stay with our son in the local Ronald McDonald House. We were given a family room that allowed his brother and other relatives to visit and stay. We found this of huge benefit. The cost was nominal, but to be with our son and in the company of families in a similar situation eventually allowed us to be able to relax there in a way that we had not been able to before.
We returned in spring 2006 to a very confused set of follow-up arrangements. The local oncologist refused to see our son on his return from France because he did not have experience of proton therapy follow up, even though he was the referring clinician. Our son was receiving care from seven different institutions with no clinical lead or coordination centre. Essentially, we have been coordinating our son’s care, we have had to fight for follow up scans, hearing aids, speech therapy, physiotherapy and rehabilitation. We have had to fund some of these services ourselves. We were left with £9000 of debt on our return from France, due to funding accommodation in one of the world’s capital cities for almost four months and paying for flights. After lengthy negotiation the local PCT eventually agreed to reimburse half these costs.
There has been little or no communication with the French team and we have continued to liaise with them independently, sending copies of follow up brain scans. Today, four years on, our son remains well with regular follow up scans, and the remaining tumour is stable. He has recovered from his stroke and regained his ability to swallow and eat normally. Compared to 2005, his brain scans remain hugely improved. More recently, hormone support for pituitary problems following high doses of radiation has been started and will continue. We continue to live from scan to scan and will continue to do so every six months until five years post treatment.
There are a number of key features of our experience that we hope will improve:
• We have found a need to improve openness about choices available for patients in difficult circumstances, rather than simply ‘doctor knows best.’ Regrettably, in our experience, there remains an element of paternalism in some specialties.
• Multidisciplinary team working is essential for good patient care along the patient pathway. A need for good communication amongst the whole clinical team along with patients and their carers is essential. There should be a clinical lead providing clear direction.
• A case manager/key worker should be in place to facilitate seamless care through the whole clinical pathway whilst addressing the social support needs of the family and patient.
• Liaison and shared care with the local DGH and GP would enhance seamless pathways of care and provide quality care.
• The difficulties of treatment and funding/accommodation need to be recognised and resolved.
• A greater understanding of, and using contributions from charities and the voluntary sector, should be encouraged.
• Properly coordinated follow up is essential for patient care and international research.
• Substantial education programmes are required to raise the profile of proton therapy across all specialities.
• Concise information on which categories of patients would benefit from proton therapy with detail on how to access proton treatment pathways must be produced.
During the very difficult time of our son’s acute illness these were very real issues where, in our view, the NHS had substantial failings. Work has started on some areas in recent years, such as the formation of the National Proton Reference Panel. Coordination of travel and accommodation arrangements and funding have been addressed, however much remains to be done.
We have recently moved our son’s medical care to a new multidisciplinary team in another area of the country. The difference in a coordinated team approach is remarkable and, at last, he is receiving quality healthcare that should be available to all cancer patients. This way of working has huge benefits to patients and carers and we have found a new confidence in the NHS.
At the time of writing this article our son is awaiting his A level results, having won a place to read Natural Sciences at Cambridge University. In confronting his own illness he took MR scans (after treatment) of his head and converted them into a series of prints, one of which is shown here. He has endured twenty one episodes of surgery. He is a remarkable young man.
Both our son and our family were handed a life we did not want and the medical care he received in some areas, regrettably, made it so much harder to bear.
It really need not be that way at all.
Written by Di and Luke Readman
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