29 St Barnabas Road, Cambridge CB1 2BU Telephone 01223 321581
28 October 1995
Dear friends and relations,
6th Dan bulletin
Our news: some scans, more gloomy conversations, 5th chemotherapy, Rosie broke her arm (and Thunderbirds are still GO).
The fourth chemotherapy admission got off to a bad start with an all-morning wait, thirsty and anxious before Dan’s intra-thecal treatment. My eye was on the clock wanting to leave for Katherine Hallidie Smith’s funeral, but in the event all the well-laid-plans were wrecked. It made me want to wave the Patient’s Charter at the system....but it also made me realise how little respect we have for the preciousness of patient’s time. After the aggro, the admission went fairly smoothly, two nights in hospital, terrible vomiting as usual in the first 8 hours but off to Burger King by the last evening.
Two weeks later Dan went in on the Wednesday to have a CT and a bone scan. Fortunately someone had taken a lot of trouble with the timing of the bookings and had found a very helpful anaesthetist who kept Dan under good sedation for whole time while showing David all his gizmos. I saw the CT scan roll up and to my inexpert eye, it looked amazingly good. However, we heard nothing formally until after the weekend and David particularly took the silence as ominous. We were due to meet the oncologist on Monday, but by then she was laid-low by some virus and the meeting was delayed for another week, though by now we knew that the news was not bad.
When we eventually met, we were able to compare the ‘before and after’ bone and CT scans which were quite remarkably different. On the bone scan all the hot spots suggestive of secondaries in his spine, ribs and skull had gone, with the possible exception of the main area representing the erosion from the ethmoid sinus mass. The tumour crawling into the orbit of Dan’s good eye was not visible on the new CT scan and all but possibly a tiny area of the main tumour had gone.
However, I think it is fair to say that we took all this ‘good news’ quite badly. We had seen the amazing response to the first treatment when the tumour mass shrunk dramatically over about 3 days, so the confirmation of the good response was not really a surprise. Even with this ‘best case scenario’ the chances of surviving 5 years are less than 15%. It was also a reminder that we are already on ‘borrowed time’ - if we had not opted for chemotherapy or if the treatment had not worked Dan would almost certainly be dead by now. Bart’s (who have a virtual monopoly of retinoblastoma UK) have never seen metastatic RB like Dan’s. In recent years they have only twice encountered locally invasive tumours; both responded to initially chemotherapy but both children died within a couple of years. Sorry about this gloom - but puncturing the smiles after I tell people that the scan was clear had been the hardest thing for me. We don’t have any illusions that there is no tumour left.
When we eventually met, our oncologist handled us very well. David particularly is quite pressing about the 'evidence base' of any treatment suggestions and she is ready for all the questions. At the beginning of treatment, we opted for scans at this stage in case they gave us information that might make us want to stop chemotherapy (eg that the disease had advanced). That isn’t the case, so the decision to go on was not difficult - more treatment should mean more time before recurrence. The difficult decision floating around at the moment is whether to intensify things.
The most similar (and commoner) childhood malignancy to retinoblastoma is neuroblastoma, the ‘biology’ of which is probably quite like Dan’s problem. Stage IV neuroblastoma (ie with widespread secondaries) if it responds to conventional chemotherapy is sometimes subsequently treated with a blast of such intensive chemotherapy that the patient’s bone marrow is wiped out. In advance of this blast of treatment, ‘stem cells’ from the patient’s own bone marrow are harvested using fancy biology and technology. After the blast, these stem cells are returned to the patient’s blood to ‘rescue’ the situation. After a time lag the stem cells start dividing and producing red and white cells. Because these are the patient’s own bone marrow cells, rejection issues do not arise - the risks are smaller than for a bone marrow transplant coming from someone else but there is no doubt that the ordeal is still great.
In neuroblastoma, this aggressive treatment increases the cure rate usefully (perhaps to around 30%) and does seem to increase the survival time even if the patients later go on to die. Dan would be in hospital for about three or four weeks (big confidence intervals here!) feeling absolutely rotten much of the time. The drug they use (Melphalan) has a lot of unpleasant side effects (patients can need morphine for their mouth ulcers and some need intravenous nutrition) and there is no knowing what bugs can get picked up while the patient has no white cells. But the risks of death during treatment seem low and once the ‘self graft’ has taken, things apparently normalise quite quickly, the Hickman line comes out and the quality of life is good until any recurrence happens. After that only palliative treatment would be appropriate.
So that is the decision we are going to have to make in the next couple of weeks. If we go ahead, it won’t be until the New Year, conventional treatment carrying on until then.
The good news? Rosie broke her arm - definitely good news. She fell off the monkey bars at school, four teachers come to help and she fetched up in Addenbrokes. Greenstick fracture, left arm. David met the party in casualty; Dan was there - delighted to be in his hospital but not the centre of attention. Rosie got a nice back-slab and lots of sympathy. The next day she acquired a very flashy bright blue fibreglass plaster and went back to school for her moment of glory. We also had a lovely weekend on a narrowboat, Dan in very good form, grand-dad there to do all the difficult steering for the locks. The day after we got back, Dan had his 5th chemotherapy which went fairly smoothly. Even so, it was hard to watch him leaning on the table with his head on his arm and the sick bowl on his lap after his enthusiasm at the weekend. Still, he puts it behind him very quickly once he is home.
Thunderbirds are an ongoing problem, starting at 6.30 most mornings. Parker, Brains and Lady P arrived from Chesham, the Hood from Suffolk, a huge poster and other loot from Kent along with occasional little notes of sympathy “we had Thunderbirds a couple of years ago, so I know what you are going through”.
Those of you who have not seen Dan for a while may think of him as an invalid but he amazes us and everyone with his vitality and exuberance. School is his own world (photo enclosed). He has gently been pulled into the circle by the wonderful Mrs Macbeth and this has given his (and our) confidence a big boost. He really is growing up and it is the ordinary business of food, dressing, school, books videos, play and bedtimes of a three year old that fills his days.
Best wishes to everyone
David and Kate