It’s been a while!  

Firstly, apologies for not updating the blog for sometime. Zach is very much developing into the 3 year old that he nearly is. He has been bum shuffling since spring. It would appear the penny has finally dropped and getting things yourself is far easier than gesturing at everything in the hope it will be pasted to you. The appetite is being helped by the need for calories to fuel the movement. He is eating a lot better than he had been. For a little while he wasn’t keen on eating and would fight tooth and nail, causing his mother and I quite a lot of grief. As you’ll see in the pictures all of Zach’s hair has grown back. You would never know he had been ill looking  
  at him now. He did get some treatment in the form of Retinoic Acid (Vitamin A) which will hopefully mature any cancerous cells that linger post chemo. Zach had this for two weeks in four. He’s had four cycles of this. He was due to have six but Prof Grundy decided that four cycles was enough as Zach was starting to develop other medical problems which may have been associated with the toxicity of the drug. Needless to say once he had stopped the Retinoic Acid, Zach recovered and has had no further problems other than what he picks up from nursery. Zach is now in the process of having all his immunisations repeated as the high dose Chemotherapy and subsequent stem cell implant have knocked out his previous immune system. All of his check ups at Birmingham so far have come back with very good results. His eye tumour is showing signs of shrinkage when compared with all his previous images, but there is a patch that needs to be monitored as it could flare up. If it shows no sign of doing so after the next 2-3 check ups the doctors are happy to start lengthening the gap between visits. Zach is having four monthly brain scans, which are coming back as showing no change to his tumour, which is good. It remains 7mm in diameter, which compared with the 17mm it was at diagnosis, is quite a change and all that shrinkage occurred in the initial treatment he had. 
 

We are now very much gearing ourselves at getting Zach back on track with his development post treatment. He now has a team of child health and development specialists who are looking at what support he needs now and making sure that he gets it. We attend regular meetings with them all where we discuss which stage he is at with medical treatment and how he is developing, what needs to happen next and how we will facilitate that. His nursery has played a massive part in getting all of this started. When we left hospital after his high dose chemo, we were pretty much left with no idea of what was happening next. We knew what was planned for him medically, but developmentally we didn’t have a clue what to expect or where to get help. It would appear that the health visitors should have been all over this but Zach had unfortunately not seen a health visitor since he was eight months old. We now have a new health visitor who heard about all of this and she is keen to try and help, although it would now appear sometimes that she too is out of her depth.  

Zach’s nursery has very much gone above and beyond making sure that Zach gets all the help he needs. They have secured funding so that he can have one to one help around times in the day when Zach needs more help than the other children. They are also looking to invest in new equipment for Zach again through funding they have access to. He will be moving up a class in September so will become a “busy bee”. This move will hopefully trigger a new phase in his development and he’ll come up against staff that can push him that little bit more. It was felt the ladies who look after Zach in his current class, maybe give way to his demands a bit too easily as they know his history, which is understandable, but Zach now requires a firmer approach. 

Zach is very mischievous now which is brilliant. He is quite an independent child, and will only want help if he gets stuck or needs a bit of encouragement. He does get very frustrated which is funny to watch as he’ll scream and shout to try and get you to do whatever it he wants but he’s getting there.  Hopefully it won't be long until Zach is able to walk and talk, then there will be no stopping him.  

 

We have received a lot of support from family and friends and want to thank everyone for donating to Zach's just giving pages.  We have recently ordered our first cot for PICU and will hopefully see that delivered soon. Many of you will know Granny Sinclair ran the London Marathon for CHECT and Nana Adcock baked cakes to raise money for the Children's Brain Tumour Research just giving page we have. Big hugs to both of them x

High Dose Chemotherapy…DONE!

As the title suggests, the last part of Zach’s in-patient treatment is complete YYYYYEEEEEESSSSSSSS!!! It didn’t go entirely without hindrance; on the last day that Zach received chemotherapy he developed a nasty infection in the vicinity of hisHickman line. Normally this would mean that his line would be removed fairly swiftly and he would be put on a course of antibiotics to treat the infection, however nothing is ever that simple with Zach. He needed to have his stem cells implanted on the second day after his last dose of chemo also known as day “0”. This meant that they had to leave his line in place so it be used on day “0” and run the risk of the infection colonising the line. The implications of this are quite severe. A Hickman line is tunnelled under the skin, up one side of the chest and passes into one of the major veins in the neck. The ends of the line sit just above the heart. As you can imagine if the line gets infected it can pass easily to the heart!!! It doesn’t end there either. While all this was going, Zach’s bowels were trying to set new speed records and the results was his potassium levels had started to drop dangerously low. Potassium plays a part in the way electrical signals pass through the heart and if they drop too low you can run into serious difficulties. Zach came to the point where if his potassium levels had dropped any more he would have to transferred to the high dependency unit. Well within a few hours the drugs started to work and disaster was avoided. 

The Stem Cells have arrived



Stem cells thawing

Day “0” arrived and we were blue lighted to Birmingham Children’s Hospital so Zach’s stem cells could be implanted. It is a straight forward procedure. The stem cells arrive in a vat of liquid nitrogen, they are defrosted in a water bath which takes about 5 min,  they are drawn up into a syringe and delivered through his Hickman line over a space of 4 mins which is timed. This was repeated again as Zach had two batches of cells. The preservative the cells are kept in can cause nausea and poor Zach was retching and vomiting for the entire 8 minutes of his cell implant. Another side effect of the preservative is it gives off a smell of tinned sweet corn when it is being excreted through the skin. So for 4 days, Zach’s isolation room smelt like a tin of green giant. The stem cells help the bone marrow recover after high dose treatment, and by day 12 the only support that Zach needed was platelet transfusion, which are still ongoing, as these are the last to recover. He will need transfusions of blood and platelets for up to six months. Zach will have further treatment for his tumours but this will be done through day care. He will be having more intrathecal chemotherapy and a course of retinoic acid, which causes cancer cells to differentiate or mature. A cancer cell is immature i.e. it is not an eye cell or a brain cell. This course of acid should force any remaining cancer cells to change and no longer be a threat. It is used in a wide range of cancers and there is good evidence to support it. This is administered orally so we can give it to him at home. Again it comes with side effects. It can cause the skin to become dry and the lips and mouth in particular. Regular vitamin E cream does help to reduce these.

We are now at home enjoying the nice weather we have been having. Looking back at the journey we have been on it hardly seems real now to look at Zach playing. He is a little behind in his development but this is understandable considering where he has come from. We have been through the extreme highs and lows of paediatric oncology and unfortunately some of the kids we have met along the way are no longer with us, but the outlook for Zach is much better. It was hard to be overtly positive about the future, but now we are looking forward to a typical British summer with some days away, the occasional barbeque and dodging the rain. For some this is something to whinge about, but for us it is a dream come true.

 

On the fundraising front it has be a feast of accomplishment. “Nae farrer” Zach’s Granda (Graham) managed to set a new and first pb at the Baker Hughes 10k in Aberdeen by completing the course in under an hour in 59mins 24 secs. His granny (Margaret) and great Aunt (Carol) also completed the course in 58 mins 31secs and 1hr 7 mins respectively so well done to all. 5 (AC) Sqn have continued their summer of sport. Personnel who are currently deployed managed to complete the journey back to the UK in the Gym. A distance of over 7500kms in 49 days. Thanks to all those who took part in this epic show of physical prowess! Kaf Whiteman tackled the tour of the Midlands ,cycling his Raleigh chopper from Skegness to Nottingham, a distance of 98 miles in a respectable 10hrs. 2hrs of this were stops ,so taking that into account along with a couple of wrong turns and he had to replace the card board that was wedged in his brake callipers, giving it that distinct motorbike sound so he did very well indeed, Thanks kaf.  There is a further programme of events to come so watch this space.

Hello again

well since we last spoke, which has been some time...sorry, Zach has managed to get his appetite back. he is drinking lots of milk but isn't really into eating solids yet. he did manage a few weeks without his nasal gastric tube as some of you might have seen in the pictures on facebook, the mountain of drugs he was on after getting out of PICU has been reduced to a quarter of what he was having.

so whats been happening with Zach's treatment? we were in Birmingham on the 24th March for a stem cell harvest, which is basically a bone marrow harvest. Zach was on a drug called lenograstim  (GCSF) which encourages your body to produce stem cells. normally you are on the drug for up to two weeks before you have enough to harvest. Zach being Zach set a new record of nineteen days on GCSF before he had enough cells to harvest, but they got a good amount. 2 would be the minimum required and they got 2.5 so it will help him even more after his high dose chemotherapy. Harvesting is a painless task which involves Zach being hooked up to a machine via his Hickman line. his blood is passed through the machine which spins out the cells in a centrifuge and puts back everything else. it took 5 hours to do.

as I write this we are back in hospital and Zach has had his first dose of High dose Chemo. it takes six days to get through the high dose protocol then he has two days to recover before an ambulance transfer to Birmingham to get his stem cells back, then we have to wait for the stem cells to re-establish themselves in his bone marrow and his blood count to recover. this can take six weeks in total but as we have been hearing the stem cells significantly reduce that to about three weeks so we will have to wait and see.

the fundraising is still going strong, our justgiving page has gone over £5,500 thanks to some large donations from friends and family. My squadron completed the Lincoln 10k and they have raised £3,250 so far. they have other events planned for the future so watch this space. A big thanks to RAF Wittering for raising over £700 in cake sales. Defending the realm is hungry work, thanks lads and ladies.

I will be updating more as I will have some spare time now we are inpatients again. It has been great to get home for a few weeks and do some of the normal things like getting back to work, running and doing a bit of DIY. Hayley managed to celebrate her birthday at home which was good and we got out for a meal with friends and we were away from the hustle and bustle of hospital.

speak to you again soon

Hi again from Ancaster

That’s right we’ve been home!!! We managed to get a couple of weeks at home while we waited for Prof Grundy to get information from Birmingham Children’s hospital on Zach’s current health. We went to Birmingham on 12 - 14 Feb. We were there to get a bit of an MOT on Zach, and to get his retinoblastoma re-assessed by the team. The results were that the tumour in his eye is pretty much dead and any viable tissue they think is present can be treated with a laser. Which just leaves the brain tumour…After the MOT, Zach was assessed as fit enough to be put back on treatment and high dose chemotherapy?.

We returned to the QMC on the 23rd of Feb to restart Zach’s treatment but his lymphocytes were too low and we were sent home again on the Wednesday while we waited to hear from an immunologist. These are the white blood cells which fight viruses. These have been low in Zach since November, and Prof Grundy isn’t sure if it is a hangover from the spell in intensive care, so we should find out soon. When we get the nod, Zach will be put on an 80% dose of his previous chemo treatment and what happens next will depend on how he reacts.

We were called back in on the 4th of Mar to start treatment and fortunately Zach has tolerated the 80% dose. We are back at home waiting to go to Birmingham so Zach can have his stem cell harvest. He is due to have another MRI at the end of March to assess what effect this treatment has had. What we have to remember is that in the 34 days Zach was in PICU, he didn’t have any chemo and his tumours didn’t get any worse or spread. They are reactive so we just have to keep everything crossed.

My squadron (5 Army Co-operation Sqn) have been putting a lot of effort into organising fund raising events to try and raise a bit of cash for CLIC Sargent. This is the charity that run Billy’s house across from the Queens Medical Centre. We have used it extensively whilst we have been in patients for our own use and for family. It helped alot particularly when Zach was in PICU as we had family coming from all over to see us. In there first event, there is a team running the Lincoln 10k; they have a just giving page https://www.justgiving.com/teams/SentinelEngineeringCharities2014 and have set a target of £1000.They also very kindly took Hayley and I out for a curry and a few drinks in Lincoln. It was a much needed evening of respite and it was good to see everyone again and for Hayley to put some faces to the names she has been hearing about.

Hayley’s WO was also able to meet up with us. RAF Coningsby Personnel Management Squadron is in the process of raising funds for the PICU in the Queens Medical Centre. The aim is to raise enough money to help buy a new bed for the unit. While Zach was in intensive care, he was put on a trial bed. It has four movable plates which mean that rather than try and man handle him every few hours to stop him getting sores or help with ventilation nurses and doctors just need to push buttons and the bed does the rest. It also has clear plastic sides instead of the horrible bars on a standard NHS cot. It has lots more benefits but you get the gist. they will be holding events in the near future so watch this space!!!

Well a massive thanks to everyone for all the support we have been getting, be it running 10k's, replies to the blog, donations to just giving pages, or simple texts or emails asking how we are getting on. We aren’t religious people but some of you have been lighting candles for Zach, which we find very touching thinking that they are burning for Zach somewhere. 

Speak to you all again soon.

Hi again from Nottingham,

We are now back on the ward. This is our second attempt at moving down the scale of treatment. We were initially moved back to the ward from the high dependency unit, on Friday 24^th Jan but by the early hours of Monday morning Zach’s oxygen requirement had gone up and the nurses couldn’t keep a close enough eye on him. On a normal ward its 4 patients between 1 nurse. On the high dependency ward it is 1 nurse between 2 patients. Whilst we were in intensive care cardiologist’s found a hole in Zach’s heart which could have been caused by the lung disease. It means that blood “shunts” across the top of his heart. Blood that has come from the body, deoxygenated can pass through the hole and mix with oxygenated blood that has come from the lungs, and vice versa. This means that Zach’s normal oxygen saturation level can be lower.

The hole is present in all babies at birth as a short cut for blood because the lungs aren’t used in the womb and will close normally. Sometimes it doesn’t close or is reopened as a result of illness. It was probably caused by the pulmonary hypertension which was caused by the “stiffness” of Zach’s lungs. Pulmonary hypertension is when the pulmonary artery becomes narrow meaning the right side of the heart has to work harder. Zach is now on sildenafil which some of you who probably wouldn’t admit; know it as the gentleman’s friend…Viagra. This increases blood flow which is helping with the blood oxygenation and captopril which helps his heart pumps better. Zach is still being weaned off his sedatives. This means that he can get quite upset when he is due his next dose, as you would expect with someone who is addicted to drugs.

Zach will be going to Birmingham children’s hospital for a MOT in a couple of weeks. Along with this we will hopefully get some time at home together. We haven’t been home since the 6^th Dec so it would be nice to get acquainted with home again. I think we have now spent more time in hospital than we have in our new house.

That’s about all our news thankfully; boring is good as we’ve found out. We would like to apologies to our French followers, who were unfortunately missed on our list of countries the blog is read in, in our last post. Do you know the difference between the French and toast… you can make soldiers out of toast.

On that note we will bid you fare well, till the next time au revoir!

Zach is out of intensive care!!!!!

After 34 days Zach has finally made it off the ventilator and out of the paediatric intensive care unit. It has been a long slog, but we are glad to be on the high dependency unit. It is a well needed change of scenery and a big step down. Now Zach has to share a nurse with another patient, and we get to step in and start taking up some of the responsibility for his care. The constant noise of machines and the horrendous atmosphere are a distant memory now.  As he has been sedated for such a long time with strong drugs, (morphine for pain relief and sedation, midazolam for sedation, rocoronium as a paralysing drug during invasive ventilation, fentanyl to replace morphine as it’s stronger and is used for sedation, clonidine which helps with withdrawal and is used as sedation), Zach has to be weaned slowly to stop him going cold turkey. He does suffer a little with withdrawal, he sweats at times, is irritable, and his sleep pattern is completely shot to pieces. He spends quite a lot of time just staring, but does follow you if you step into his line of sight. It’s just a matter of time before they can wean him safely and he returns to his normal smiley self. The doctors are sure that he doesn’t have brain damage as a result of the ventilation, Zach has had an MRI scan and the Neuro radiologist couldn’t find any evidence of brain damage and it showed that the tumour in his brain had shrunk slightly again. He will be receiving some chemotherapy this week, but it will only be the chemo that is injected via his Omaya reservoir in his head as it isn’t toxic to the rest of his body. The rest of his therapy is still a bit up in the air at the minute. Ira Dunkel who is an Oncologist in the states, gave our professor Zach’s original chemotherapy protocol. He is now helping to work out where to go next. Zach will receive some systemic chemotherapy but the amount is still to be decided and will be very much guided by Zach’s state of health. They are sure that Zach was just unfortunate to get a bug whilst he was immuno-supressed, but none of us want to end up back in intensive care if we can help it. To gauge how much ventilation a patient is on; doctors use a system called the “oxygen index”. A normally ventilated person should be between 10 and 20, with a threshold index of 40. That is the point when doctors get nervous. Zach’s oxygen index at his worst was 70, which gives you an idea of the severity of the disease in his lungs and the aggressiveness of the ventilation he needed to get gaseous exchange to happen. Today as I am writing this he has a nasal prongs on which delivers about 0.5l/min of oxygen with no pressure. He did have a full face mask which allowed him to stay on a ventilator without a tube being inserted into his windpipe. The doctors could give him pressurised oxygen enriched air, and he didn’t like it being removed as he was still dependent on the pressure to help his breathing, but on Saturday all that changed. We took off his mask at about 1030 to give his face a wipe and stop him getting sores. He didn’t panic in the slightest, so it was decided to keep it off and instead waft oxygen across his nose and mouth and it worked. Four hours later he was moved along with a mountain of drugs to the high dependency unit, which is only next door. It is a very different beast. We have our own bay, privacy, no more beeping machines. We can have a cup of tea by his bed. We have a TV in his bay so we can relax with him. There is more of a day and night routine, not that Zach is interested in that yet so will no doubt take him a few days to get back into the swing of things. We can’t sleep on the ward with him but we are welcome any time of the day or night. We will have to try to rest as much as we can as once Zach is feeling better he won’t want to be lying in bed all day!

The blog is becoming ever more popular. It has had over 9500 views since we started it. It is viewed in America, Canada, Afghanistan, Switzerland, Germany, Cyprus, Norway, Australia, Denmark, Spain and even Aberdeenshire.  We would also like to thank you all for your thoughts and prayers over this dreadful time. They have all helped in some way as Zach is now known as the “Christmas Miracle” in the intensive care unit.

Keep reading and we will speak to you again soon.

Happy New Year!!!!!

 

As you’re about to read, ours wasn’t very happy and Christmas wasn’t very merry either. The second week of December was pretty normal. Zach was on a very small amount of oxygen as the doctors thought that he had a virus which he would get over himself and the oxygen was there just to help with the oxygen saturation (sats) in his blood which was lower as a result of the virus. On the evening of the 15^th Dec Hayley and I sat down to a Chinese from one of the local takeaways and put Zach to bed. During the night Hayley noticed that Zach was making a noise when he was breathing, which is usually a sign that he’s finding it a bit more difficult. The doctors that were on that night came and had a look, and weren’t too concerned although they did take a chest x-ray. Zach was supposed to be going to Birmingham Children’s Hospital for a stem cell harvest that day and they were happy for him to still go. A quick chat with nurses soon changed that and after a phone call to one of Zach’s consultants and they all decided not to send him, just as well. Around the same time, Hayley started complaining of not feeling too well. She ended up having to go and speak to hew down the big white telephone. Sickness had been doing the rounds on the ward and it would appear that poor Hayley was its next victim. Well morning came and Zach wasn’t feeling too well, neither was Hayley, so she was sent home by the ward to tough it out and left Zach and I to stay on the ward, this being the first time Hayley had had to leave Zach. Professor Grundy came to see Zach and had a look at his x-ray. He diagnosed bi-lateral pneumonia. Zach would have some samples taken from his lungs the next day to help further diagnoses and treatment. As the day went on Zach grew more tired and uninterested. Then at about 1730ish the doctor was called again to take another look at Zach. His oxygen requirement was increasing and he was struggling to maintain his oxygen saturation level. She had a look and an oxygen mask was put on him which he didn’t like too much. Then he started to really fight and breathing was becoming more and more difficult. They took another chest x-ray and his condition had worsened dramatically. It showed a lot of fluid build-up. An Anaesthetist was called and after a little hesitation from the on call doctors, he decided that Zach needed to be intubated, which is when a tube is put down in order to maintain his airway. This would reduce the stress he was going through and stabilise him. As this was being done, the doctors called the Paediatric intensive care unit (PICU) and it was decided that Zach would be transferred. Once the ball was rolling it was a very slick operation although something no parent ever wants to see. Zach was anesthetised, intubated and moved in the space of about 40 mins. When he was anaesthetised his lungs collapsed quickly and it took a while for them to “re-recruit” or inflate the collapsed parts.

 

Once through on PICU he was put on a type of ventilator called an oscillator. This works the lungs differently from a conventional ventilator by inflating the lungs and keeping them inflated, and just panting them rather than doing breathes as you would normally. It is gentler but looks disturbing as it makes you breathe at about 300 breathes per minute. This would help to clear the fluid from the lungs and reduce the build-up of CO2 that had occurred. We were sat down with the PICU consultant and one of Zach’s consultants and they explained that he was critically ill and the next 24hrs would be difficult. Through the night Zach had made some progress and they managed to switch Zach on to a conventional ventilator. The next day things looked like they were heading in the right direction. The PICU doctors weren’t entirely happy with progress that Zach had made but he was more stable. They diagnosed him with RSV (Respiratory Syncytial Virus). This is a common virus and would make adults feel a bit rough with a cough and runny nose, but often puts healthy infants and babies in intensive care. As Zach was immune-supressed it was hitting him hard. He made very slow progress and had to be put back on the oscillator a few days later, as his CO2 was starting to build up again. The progress he had made then started slipping. The doctors then started looking into ECMO. This is a lung bypass machine, which is only available in four hospitals in the UK. It is a very specialised piece of equipment which would oxygenate Zach’s blood for him, allowing his lungs to rest and repair. It is very high risk as it involves putting one or two large cannulas into a vein in the neck. The blood is thinned to allow it to pass through the machine. The criteria that children have to meet are very specific because of the risks involved. 30% of children put on ECMO end up with brain damage as a result of bleeding in the brain. Some children don’t survive due to other complications. Well the first time the ECMO service was asked, they refused Zach. It would now appear after further discussions with the Zach’s doctors that certain staff within the ECMO service think that all children with cancer that are put onto ECMO die. So they had to find another way of getting Zach better. They tried different drugs, putting him in different positions, giving him physiotherapy to try to bring up the secretions in Zach’s lungs. Zach made some progress then stopped again. The ECMO service were contacted again, more tests were carried out and they came back….no. then Zach made some more progress and the ECMO service were asked again….no. this time they thought that he would be more at risk being put on ECMO than if he was treated conventionally. So Zach’s doctors carried on and then he started slipping backwards. One evening ended with a doctor running down the ward towards us saying that she thought Zach may be giving up as his sats had dropped significantly during a session of physio and they were struggling to get them back up, and that if we wanted to come and stand by his bed if we thought it would help. This is the sort of news that you normally get before you wake up in a pool of sweat, but this was real and happening. We stood there as Zach slowly recovered but it highlighted to everyone how ill Zach was. The ECMO service was contacted again…..no. Zach then started to slip again. We were sat down and told that they would try three more drugs on Zach. The third would be surfactant, which is a fluid used to treat ante-natal babies with under developed lungs. It has to be squirted down his ventilation tube. Given Zach’s situation and that he was on the maximum amount of ventilation the doctors said that if he deteriorated to the point where he would need resuscitation then they could not help him any longer, and they would preserve his dignity, unplug him from all his machines and drips and pass him to us, so that we could cuddle him. This is the most disturbing thing that we’ll ever probably be told. The surfactant would be the last thing they tried. By about 1 am it was time to try the surfactant. They squirted it down, it was quite disturbing as you can imagine swallowing something and it goes the wrong way. Despite his sedation poor Zach was coughing and retching, but after he settled his sats came up a bit. It takes a long time for surfactant to work. It did seem to have a bit of an effect but then he started to slip again. We were advised to call our families. This we did and by the evening of the 27^th Dec everyone was here by Zach’s bed. Come the early hours of the 28^th the nurse looking after Zach woke us up as Zach was starting to loose colour and she feared that this maybe him letting go. Everyone rushed to his bed side and we hoped and prayed and cried. His sats were dropping, along with his blood pressure and heart rate. Then they stopped dropping, and started climbing very slowly. By midday everything was looking a bit more normal but he was still critically ill. The doctors were starting to run out of ideas.

 

ECMO were contacted again….this time Glasgow said they would consider him. He needed more tests first. They scanned his heart and found a small hole. This is quite normal as Zach’s heart was working hard trying to pump blood around his lungs. After further consideration they came back…….no. He was too high risk as he had been on a ventilator too long. The ECMO option was now out. Zach would have to do this on his own. In the meantime he had been put on a gas called Nitric Oxide which occurs naturally in the lungs and helps be improving gaseous exchange. The amount he was on had gone up and up and up. The Professor in the PICU had managed to speak to a doctor in Los Angeles, who is a leading expert in RSV. He suggested putting Zach back on to a conventional ventilator, starting a 28 day course of steroids, along with sildenafil, which is found in Viagra, and would help by increasing blood flow around his lungs and give him time. The junior doctors had become fixated with Zach’s CO2 levels during his treatment and in order to treat it had been turning up the pressures on his oscillator, but because Zach’s lungs were in such a poor condition they had been chasing figures which were unachievable in his condition and the hole in his heart made this worse. So the PICU Professor, who is also a respiratory consultant, sat them all down and drew up a plan of how Zach’s treatment should go. Zach was put on a conventional ventilator and his new drugs started. The results were almost immediate.

 

Zach has now recovered to the point where they will be able to take him off his ventilator and wake him up, which is great considering he was on four drips and eight syringe drivers, all of which were running almost continuously as well as all the machines he was connected to. It has been a complete rollercoaster of emotions. It is something that you often see on TV or read about but never expect to happen to you. It makes you take a good look at everything in your life and puts a whole new perspective on it. The last three weeks have flown by despite the longs hours of waiting for tests and results. You completely lose track of the time and the days. Our next milestone is still to get to Birmingham for Zach’s stem cell harvest and examination under anaesthetic (EUA) of his eye before his chemotherapy starts again and to get him a MRI scan to see how his brain tumour has been getting on without chemo. They have drawn some of his cerebral spinal fluid (CSF) and didn’t find any cancerous cells which is good as it means it hasn’t spread during this episode.

 

Well keep reading and thanks again for all the support be it emails or donations to the justgiving page which never fails to amaze us.

Speak to you all again soon.

Hi,

Last night we received the results of Zach’s MRI scan from that day and it’s great to be able to share with you our GOOD NEWS!!!!! His brain tumour has reduced in size by half and the residual disease around his brain has gone. The tumour in his eye looks like it may have disappeared too. We will still need to have that checked out by an ophthalmologist, but it was clearly visible in his first scan, so that is very promising.

The next milestone now is to have Zach’s bone marrow stem cells harvested and implanted prior to his “Mega therapy” protocol starting. The harvest will be done in Birmingham which will be done through Zach’s Hickman line therefore not causing him any discomfort. There is also a Retinoblastoma clinic there which just happened to have been set up by our Consultant, Professor Grundy. At the Clinic in Birmingham they have a “Retcam” so they can take pictures of the retina and compare it to previous pictures.  

Zach is now starting to keep down his feeds that he receives through his nasal line. This helps in two ways. Firstly it means that he will get nourishment from his food and help him recover quicker and secondly it will protect him from the fluid that he has been receiving. If you don’t have enough protein in your system, your blood vessels start to leak fluid. So by giving him feeds it means that he will get rid of the fluid in a more conventional way other than it leaking out into his body. The doctors think that this may have contributed to the respiratory problems he had the other week.  Another advantage is that we are also able to give Zach his medication through his nasal tube, which is less upsetting for Zach.

Speak to you all again soon.

Hi Everyone,

We finally managed to make it home on Thursday, only 24hrs after we were expected to depart, due to a bit of a cock up with Zach’s drugs. After a rush hour trip home we got in the door to a cold house, which didn’t surprise me given no one had been in the house for a few days. As I opened the cupboard to the hot water tank and looked at the controller, I was met with a blank screen, after pressing every button and switching everything off and on, there was still nothing. Frustration soon turned to anger, as I thought “is there anything else that can go wrong”. I managed to get an engineer out to look at it the next day but he couldn’t help either as the part it needs, he doesn’t carry so we spent the weekend living with the hum of fan heaters. At least the immersion heater still worked so we could still get hot water.

The force is strong Zach!!!

We are now into cycle three. The fluids Zach receives during his treatment have been recalculated to avoid the bloating and other side effects of having too much fluid going around his system. He is still tolerating the feeds through his nasal gastric tube and even managed to eat some solids over the weekend which is great. His personality is starting to show a bit more, especially his temper. We often get a grunt or scream if things aren’t quite going his way which makes us laugh, unless it’s three in the morning and he’s had enough of sleeping in his cot, then I think it’s his turn to laugh.

While Zach has been in hospital, he has been collecting “beads of courage”. These come from America, and you are given them as a way of recording your stay in hospital. You get one for pretty much everything that happens to you in hospital from having an overnight stay to getting general anaesthetic, injections, treatment or dressings changed. As you can imagine Zach has quite a collection of beads now. Apparently the record for the ward is 15m, and that is a record we definitely don’t want to break. At the moment his beads are about 1m long. 

Zach is due to have another MRI scan on Wednesday to see how the treatment is affecting the brain tumour. We will write another post with the results so keep everything crossed.

Speak to you all soon.

Hi again,

May we just firstly say thank you for all the messages of support we are getting through the blog. They are helping us when we need a “pick me up”.

The just giving page is going well. As I write this the total is at £1,332.39 which is fantastic so a big thank you to everyone who has donated.

Well it's week six of cycle two and things aren't getting any easier I’m afraid. Zach has been picking up bugs left right and centre because he is neutropenic. This means he has no way of fighting infections or virus'. These usually manifest themselves with a temperature of 38 and above. When this happens we have to take him straight back to hospital. He has had a few more transfusions of blood and platelets and plenty of anti-biotics. Zach has pretty much stopped eating now and isn't drinking much either. Has to be topped up with intra venous fluids, this is a double edged sword though as he doesn't develop a thirst when he is getting fluid so won't drink, and because he doesn't drink they have to keep putting him back on fluids. I think the fact that he isn't feeling very well doesn't help either with the eating or drinking. Zach has had a nasal gastric tube put down so that we can feed him. It looks worse than it actually is and apart from when they put it down initially, he hasn’t been bothered by it in the slightest. A lot of the kids on the ward have them so it's quite normal here. Zach also developed a virus in his lungs over the weekend so has had to have yet more anti biotic and a nebuliser along with some oxygen to help his saturation levels as they were dropping quite a lot. This looked quite scary watching a little boy panting and groan with every out breath but the staff didn't seem too disturbed by it, which is reassuring in a way. They kept telling us this happens a lot as well. It has now transpired that Zach may have had his system overloaded with fluid. He was re admitted on Friday after about six hours off the ward with dehydration. He was given a fluid bola which is saline syringed straight into him via his Hickman line. This was done twice then he was put straight on to continuous IV fluid. This then gathered in his lungs and looked like an infection/virus on the x-ray, but his rate of recovery has been too quick for it to be a virus/infection. He is now having a week off the chemotherapy as he hasn't had a break since the treatment started, and he is exhausted so it will definitely do him some good.

The other patients or should I say their parents haven’t been helping the situation either. When Zach had gotten over his last infection he was moved out of his side room back onto the ward. That night at 0045 peppa pig could be heard on repeat coming from the next bed, when a nurse went to turn it off, the little cherub went mental so it was turned down. Mum then decides to watch a film till 4 am. Needless to say words were had with staff the next day and rules were loosely enforced but poor Zach, who was exhausted anyway, was set back by this episode and I think it made him a little bit worse. It is difficult living with people from all parts of the social spectrum. 

Well we are all looking forward to getting home and sleeping in our own beds for a few nights before the next cycle starts on Sunday. Keep reading the blog and we’ll speak to you all soon.

Hi Everyone, 

We have set up a just giving page http://www.justgiving.com/Zachary-Sinclair so that people can donate money to charity in aid of Zach. People have wanted to donate money to a charity but didn't know who to donate to. The page has been set up for the Children's Brain Tumour Research Centre, who are based at the Nottingham Queens Medical Centre, where Zach is receiving treatment. Prof Richard Grundy helped establish the centre along with Prof David Walker. Richard is Zach's consultant and David has helped treat Zach on several occasions. 

 Whether it is a donation or a few words of support on the blog they do make a difference to us knowing that people are thinking of us.

Speak to you all soon and thank you 

HAPPY BIRTHDAY ZACH!!!!!

First of all, apologies for the delay in updating the blog. We finally managed to make it home for a few days before and after Zach’s birthday but unfortunately Zach spent his 1st birthday on the ward. We went home on the weekend of the 18^th Oct and we were coming in on day care over the weekend so Zach could receive medication to help his system recover from the chemo. On the Sunday we woke to find Zach just wasn’t interested in anything and was really lethargic. Immediately we started to think something wasn’t right. He didn’t have a temperature at that time but when we arrived at hospital and explained what was happening, the nurse took his temperature and it was 37.8 degrees Celsius. He had an infection; we were readmitted there and then.  The nurses’ monitored Zach and he was put on antibiotics once he had a temperature of 38. He slept for the rest of the day and night. They took lots of samples to try and find out where the infection was. He also had a CT scan as they were concerned that he might have pressure on the brain (hydrocephalus). The sight where his Omaya reservoir is had swollen but thankfully all the results were negative. A doctor explained later that a lot of these “infections” never produce any positive results but each time he has a temperature they have to treat it with antibiotics. The problem with this approach is he can start to become resistant to the antibiotics. Unfortunately when Zach is neutropenic, (he has no neutrophils to fight infection) they have to fight it for him; otherwise it could make him seriously ill. He also had to have a blood transfusion with platelets which sounds drastic but is entirely normal at this stage, as is the infections. It’s not uncommon to see kids riding a drip stand with a bag of blood on it, whilst mum or dad are pushing. He got another infection a week or so later but this time they think it may have been one of his scars that had become infected. Never the less we got home the day after his birthday and had the rest of the week at home (apart from the day care visits) which was entirely uneventful…….GREAT!!!

Zach was completely spoilt rotten on the 28^th by the staff, family and friends alike. He now has more toys than Santa’s grotto. We’ve had to order another toy box to squeeze them all into the house. As Zach was being treated for an infection, we were put into a single room, so we managed to decorate it with banners and balloons, much to Zach’s delight. His behaviour has returned back to normal and he is eating and drinking, although he doesn’t care much for savoury food or anything with lumps or too much texture. He has started to lose his hair, and the curls have gone, but the cheeky chap is still very much present.

Get your aprons and gloves at the ready; we are now into cycle two. He has managed to start his next cycle on time as his blood count was sufficient. Finally we have some good news, the last sample of CSF (Cerebral Spinal Fluid) came back showing no cancerous cells and the bone marrow samples he had taken at the start have come back showing no evidence of disease. The chemo is working although it is early days. The lack of cells in the CSF means that the chance of it spreading within his central nervous system is significantly reduced. The next major mile stone now is having a bone marrow stem cell harvest which should happen before cycle three.

Speak to you soon.

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.