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.