Hi from ward E39,

We are now a few days into the chemo and it’s going to be a long and testing time for us never mind the living hell that poor Zach is going through. His chemo schedule consists of 4, 21 day cycles. Zach gets chemo from day 0-3, 7, 14 via his wiggly in his side. He has 4 different chemo drugs to fight the Rb, one drug that stops him weeing blood, as this is one of the many side effects that he can suffer. He is also having a chemo drug put straight to the brain tumour via the Omaya reservoir in his head. He gets this on weekdays for the first two weeks of each cycle. This has to be done as there is a barrier called the brain blood barrier which stops the chemo that is being administered through his wiggly getting to his brain, this an in build safety barrier. He takes regular calpol to help with the aching that is another side effect. He gets anti emetics to stop him being sick, but these weren’t working very well on day 2 so these have been changed to a drug that makes him very sleepy, which is probably for the best as it means that he can rest and not feel rotten like he has been. He isn’t on these all the time, just when he is at his worst. For the last seven days of each cycle he has a rest from the chemo drugs then it all starts again. On top of all this he will have to have stem cells harvested from his bone marrow as the chemo drugs will “wipe out” his bone marrow. These will be put back to help his body recover and let the stem cells in his bone marrow re-generate before the next protocol starts. He also has to receive regular drugs during each cycle that stop him picking up lung infections and help the neutrophils in his blood recover.  Neutrophils help the body fight infection and are significantly reduced after chemo is given. So if you’ve got enough fingers and toes that’s about nine different drugs I’ve counted and I think I have missed one or two. Don’t worry he doesn’t get every drug every day. The chemo is broken down into ones and twos. These are often infused with large amounts of fluid so even if he isn’t eating he is still well hydrated. The down side to this is he is going through nappies like they are going out of fashion. We now have to be extra careful when changing his nappies as the contents is toxic when he is receiving chemo. When I say toxic I don’t mean they have a nasty smell, I mean skull and cross bones, this stuff will actually cause you harm. This is because chemotherapy drugs are cytotoxic, http://www.patient.co.uk/health/Chemotherapy.htm

I haven’t said much about Zach’s brain tumour so far. It is called a PNET (Primitive NeuroEctodermal Tumour), which describes the group of tumours it belongs to and specifically it is a Pineoblastoma. If you Google pineoblastoma there is some information it. It is located in the third ventricle also known as the third eye, which is in the centre of the brain (see first picture, please note this is from the internet, not Zach but it gives you an idea). It can block the flow of Cerebral Spinal Fluid (CSF), which is one of the reasons why he has an Omaya reservoir. This allows doctors to remove some of the fluid if it builds up. They can also fit a shunt which is similar to an Omaya but diverts the fluid into the abdomen. Zach doesn’t need one of these as his tumour isn’t blocking the flow of fluid. Another option which was discussed with us is to perform a ventriculostomy, which is where a Neurosurgeon makes a hole in the hypothalamus to allow CSF to drain away. This would have been done when the Omaya was being fitted and is quite routine. CSF build up is called hydrocephalus, and is quite serious. it can have symptoms that are similiar to the after effects of chemo, as we have found out.          

The ward we are on does a lot of day care so it’s busy during the week but at weekends it’s quiet, so we can spread out a bit. Livvy our clinical nurse specialist comes to see us a couple of times a day when she is working, so if we have questions she can answer them or find out. She always greets us and says bye with a hug which is reassuring. She is a very bubbly character. The prof sees us a couple of times a week, usually with results which means of late it’s been mostly bad news. He is a reserved man in our company, however I get the feeling when I see him with the nurses he could be quite witty and mischievous. They have play specialists who do as it says, play. They can take children for a wee while to give parents/carers a break to get a cuppa. They also organise activities for the kids to keep them occupied. Shaz a play specialist is a favourite of Zach’s and can get a smile out of him where most others have failed including us. Our parents have been supporting us all the way and keeping us fuelled, doing the washing and pick up any shopping we need as well as a massive shoulder to lean on when we need.

Another massive thank you to all of our family and friends for the messages of support whether they be posted on the blog or written in a card. We are somewhat amazed at how many times the blog has been looked at. Keep the messages coming and look out for the next post. 

Hello again,

Thanks for all the kind words of support in your posts. They are all helping to keep our chins up while we wait, wait and wait some more. We’re all starting to get a bit fed up now, but we know it’s the means to an end and writing this is helping to keep some of the boredom at bay.

We have included a pic taken of Zach in the multi-sensory room. This is in the complementary therapy unit within the ward. It has some lava lamp type cylinders that have ambient lighting within and fibre optics which Zach is sat amongst. It gives us some time away from the hustle and bustle of ward life, although once it’s all up and running and the music is on, you do find yourself choking up as your mind wonders. Zach now has a U-shaped scar on the right hand side of his head, which you can see in the picture. This is where the Omaya reservoir is situated. It doesn’t bother him too much as the Neuro surgeons have severed the nerves to that area, so we are assured he shouldn’t feel anything. This will also help when it comes to accessing the port with needles for his treatment. The scar is behind the hair line so once the hair grows back you shouldn’t be able to see it.

We have attached another picture of Zach which was taken around the time he was diagnosed with Retinoblastoma (Rb). Some of you may have not noticed at first but his left eye has no red reflex or “red eye”. This is one way of spotting Rb, although it is very rare with only 40-50 children a year being diagnosed in the UK. If you would like to find out more about the signs and symptoms of Rb then go to the childhood eye cancer trust web site http://www.chect.org.uk/cms/index.php/signs-and-symptoms. Fortunately we don’t think Zach was displaying any of the signs or symptoms of his brain tumour when he was diagnosed but there is a website for the research centre that Prof Grundy works in, its www.cbtrc.org. There is a picture of Prof Grundy there just in case anyone wondered what the Professor looks like. If you go to http://www.headsmart.org.uk/ they have a good guide to recognising signs and symptoms of brain tumours in children and young people.

Zach has now started his chemotherapy. It will be a “fairly intense course followed by a very intense course” as the Prof described. The protocol for his treatment came from the children’s oncology group which is in the states, as the UK children cancer group doesn’t have a protocol for the combination of two types of tumour that Zach has, which goes to prove how rare his combination of cancers are. The chemo he is receiving hasn’t be given at this hospital before but the Prof has used this protocol before. It will make Zach very poorly; most kids including Zach spend a lot of time sleeping during their chemo, which is best for them. He will most likely lose his curls, and he will probably lose weight. He may end up with a nasogastric tube to help feed him if he is off his food. Having spent a wee bit of time around other children who have cancer, suffering the symptoms above doesn’t seem to affect them too badly, there is still screaming kids running up and down the wards, the play room is used a lot by them, they still smile and get up to mischief. They are a lot better with just getting on with it, than us adults. Even Zach who was a bit sick today still managed to pick his head up from the rim of a sick bowl and smile at the nurse as she walked past and he had some food at supper time as well which goes to prove he must be a Sinclair!!!!

I hope that you are all doing fine and this is managing to keep you all up to date. Please keep spreading the word on how Zach is getting on and look out for the next post.  

This blog has been set up to keep all of Zach’s friends and family up to date with his treatment. We have tried to give as much info as possible whilst trying not to overload you with the medical jargon and give Zach some dignity. Some of it may not be nice and some of you may find what you are reading upsetting, but remember that all of the Doctors and staff at the Queens Medical Centre are world leading experts in their fields and are always looking to cure Zach.

It all started on the 9^th Sept 2013 when we took Zach to his GP with a bobbling left eye. It would bobble or twitch very slightly up and down. It wouldn’t do it all the time. Mostly when he was tired or grumpy, and if you know Zach well, he isn’t grumpy very often. There was no evidence of loss of vision and he was developing normally. The GP very quickly realised that there was something wrong and referred Zach straight to the children’s ward at Grantham Hospital. He was then sent to the Royle Eye Clinic at the Pilgrim Hospital in Boston, Lincolnshire, but they could not conclusively diagnose what they could see in Zach’s eye. A week later after much chasing around, we were seen at the Retinoblastoma Clinic, the Royal London Hospital, where Zach was examined under general anaesthetic. They diagnosed Zach that day as having unilateral Retinoblastoma (Rb) of the left eye. We were given a prognosis that Zach had a 50/50 chance of losing the eye.  If that was the case they would perform an Enucleation, where the eye is replaced with a prosthesis which are matched very accurately and do look convincing, but we were assured that they were aiming to save the eye with Chemotherapy (Chemo). This type of cancer doesn’t normally kill children and has a 98% cure rate. This type of Rb, it being unilateral, only spreads in very rare cases and the oncologist assured us that she wouldn’t be looking to conduct any more tests to see if it had spread as Hayley and I didn’t have any history of Rb and although Hayley’s grandfather did have a brain tumour they weren’t concerned that the type of Rb was genetic rather they thought that it is sporadic i.e. a freak one off event. This did ease the pain slightly, but when you have had a long tiresome day with a child who has been starved and poked and prodded, in a ward with many sick children, some of which bear the scars of previous battles won against cancer, and the fact that up to this point we had no inkling that Zach had cancer, the enormity of the journey ahead weighed heavy.

After much more chasing around again, we eventually got to the Queens Medical Centre (QMC), Nottingham where would have Prof Grundy looking after us. He is Professor of Paediatric Neuro Oncology and Cancer biology. He would be co-ordinating Zach’s chemo. On our initial consultation with the Prof was slightly concerned that London hadn’t carried out any more tests to see if the cancer had spread.  He mentioned that only in very rare cases does a unilateral Rb spread and that London had mentioned that since the tumour wasn’t near the optic nerve that the chances if it spreading were even slimmer. Never the less he wanted to be completely sure that he had covered all eventualities before going ahead with the planned chemo. A lumber puncture (LP) was carried out along with the fitment of a Hickman line or wiggly as it is more affectionately known. The wiggly would allow staff to give chemo and take blood without having to stick needles into poor Zach. We were all confident that nothing more would be wrong and pretty much forgot about the LP results and came back into hospital on Monday 30^th September ready to start the fight back and get the chemo started. How wrong could we have been?

 When the Prof came to see us that morning I was feeding Zach, he sat down and said the fateful words, “I’m afraid they have picked up some suspicious cells in his CSF (cerebral spinal fluid).” It sort of took a minute for the words to register. Once more that sinking feeling comes and the uncertainty which had subsided over the preceding days hit you like a sledgehammer. This now meant that Zach would have to go through more tests, but this time he would be having another LP with a bone marrow sample being taken and a MRI to try and find the source of the anxiety. Again you hope and pray and hope some more that there will be nothing found, and that it may just be the Rb leaking cells into the CSF but on that Friday ours fears were confirmed. Zach had a brain tumour. It was sighted in a part of the brain known as the third eye and is linked to sight. It is in the middle of the brain, which would mean that a complete removal of the tumour would not be possible and oncologist don’t like to partially remove them as it makes gauging how the tumour is reacting to the treatment. When we asked if he had ever seen this before, he said that he had only ever seen this combination in one other child. When we asked about a prognosis he suggested that we concentrate on curing Zach and that putting a figure on it might lead to more stress and anxiety, but that they were looking to not just treat Zach but cure him and that he and his team would do their absolute best to achieve this. Zach would now have to have another op but this time he would have to have an Omaya reservoir fitted. This would allow doctors to put chemotherapy drugs straight to the tumour. It would also allow them to drain some CSF out as it looked like the ventricle where the tumour was sighted, was blocking the flow of fluid.  A biopsy of the tumour would also be taken to ascertain what type of tumour it is and that way tailor the chemo accordingly and a bone core sample would be taken to see if there was bone marrow involvement. Since then Zach has recovered from his op with no ill effect other than a few stitches and a lump where the Omaya reservoir is.