Ryan was diagnosed with Stage 4 High Risk Neuroblastoma N-MYC amplified in July 2008. He was out of treatment for just over a year when in November 2010 our lives were again turned upside down when we discovered the disease had returned in his bones and bone marrow. After a year of treatment re-staging scans in November 2011 showed that he had again achieved remission.
However just 3 months later, in February 2012, end of treatment scans gave us the devastating news that the disease has returned, this time with a tumour near his spine. In March Ryan started 4 weeks of radiotherapy followed by chemotherapy which is on-going. Re-staging scans in May and August showed no evidence of disease. On the 4th September 2012 Ryan received treatment in Tubingen for a haplo-identical stem cell transplant. He is a happy, active boy who is now enjoying life to the full.
Thursday, 28 February 2013
Monday, 25 February 2013
Sunday, 24 February 2013
Monday, 18 February 2013
Thursday, 14 February 2013
They have been processed to separate as many t-cells as possible from the stem cells but anticipate that a few thousand will remain. They say that these additional t-cells will not accelerate the GvHD in their experience.
Yesterday Ryan received 4 million of these cells via his hickman line. The remaining 4 million have been cryopreserved. The purpose of the stem cell infusion is to give Ryan's bone marrow strength. His bone marrow, which has originated from my donated stem cells since the transplant in September, has been attacked by his surviving T-cells for several months leaving his counts very weak. These new stem cells will find their way to his bone marrow, engraft and develop into either platelets, HB, white blood cells etc.
This should then reduce his dependency on blood products and GCSF. However occasionally active GvHD can continue to cause thromopenia (low platelets) but if this is the case the additional cyropreserved stem cells can be used once the GvHD is controlled.
The frequency of Ryan's stool continues to be much improved - approx 4-5 times in 24 hours but the volume is still quite high. Each one being approx 400-500 of black looking water. He is very tired and weak but managing to play the x-box each day and speak to some friends
He is asking for food but his stomach cant really tolerate food yet. It is so hard telling him he cant eat when we have spent years encouraging to eat well. We are going to chat to a dietician and see what foods they suggest at this stage. I dont think spaghetti bolognese wi be one of the options though....
Ryan steroids have been reduced from 40mg daily to 30mg as these cause the most immune suppression but already his skin and liver enzymes are slightly worse and if this continues they may need to be increased again.
And lastly some very good news: the stool sample from monday is negative for adenovirus which means the virus is below the level of detection . They suspect that Ryan's t-cells have dealt with this virus as the anti-viral drug was to try and prevent the virus spreading.
Tuesday, 12 February 2013
ECP: Ryan is connected to a machine by his double lumen hickman line, blood flows out at a rate of between 15-20ml hour flows into the machine and is spun to separate white blood cells from red blood cells. The process takes between 2-3 hours depending on how the hickman is working. The red blood is returned to Ryan and the white blood cells are collected and then treated with light which is said to influence the behaviour of the t-cells. Once treated the WBC are also returned to Ryan. The treatment usually has an effect after 2 weeks, which is 4 sessions.
As usual it doesnt seem possible for us to have good news without bad. We learned yesterday that low levels of adenovirus have been found in Ryan's stool sample of last week. This is a very dangerous virus to a post transplant patient and so needs to be treated aggressively to try and prevent it spreading into the blood. Ryan blood and stool are tested twice a week to monitor the virus levels. We have been told it can stay 'dormant' in the stool and so we can only hope and pray that is the case for Ryan.
Today I had my stem cells harvested, the procedure went well with the exception of a temperamental needle what constantly needed repositioning. My white blood cell count was 62.2! (normally 1.5-5) at the start of the collection so it is expected to be a good harvest.
Sunday, 10 February 2013
I know that everyone is hoping that I will say that Ryan is a little better but in all honesty this isn’t going to improve daily, more weekly.
His mouth is showing signs of improvement in that the thick white coating of his tongue is very slightly pink in places. He has started drinking water again which is a good sign. We cant see the rest of his mouth as it hurts to open it. His skin is much the same although a new area on his head and neck can be seen. His liver enzymes are almost all within normal ranges but the diarrhoea is impacting on the liver function now too.
The GVHD of the gut causing sickness and diarrhoea remains the worst problem; the sickness is much better controlled after tweeking some of the anti-sickness medications. The diarrhoea on the other hand is relentless. We were told he was passing between 2-3 litres a day, more fluid that they can give him. The stool is watery and often red with blood. It floods out of his little body. I have to fight back the tears when I change him and clean him up; it is devastating and totally exhausting for Ryan. He weighs 2.5 kilo’s less than he did and is painfully thin. He has started to experience pain but it is intermittent and he is managing with paracetamol. The doctors are surprised that he isn’t on morphine but this is a good thing as morphine, although great for pain, brings a whole host of new side effects.
The doctors had told us that his stomach was likely to get worse before it got better – there is no quick fix for GVHD. We just have to hope that there is not a progressive deterioration.
Sleeping is obviously a problem for Ryan, until a few days ago he sat on the commode every time he needed a poo, sometimes he was only just settled when he needed to go again, rarely did he get chance to sleep more than 15 minutes at a time. He did not have one accident but it was very stressful for him. He was obviously very sleep deprived, he was almost hallucinating and waking from broken sleep with racing heart and feeling panicked. The doctors spoke to us about sedating him during the night to give him chance to sleep, this is often required for these circumstances, but this upset Ryan very much. So we talked to him about how essential sleep is and that if he didn’t sleep he would be even more poorly. We suggested he use a nappy at night and we would change it immediately – he reluctantly agreed and although it meant he wasn’t in and out of bed the sleep situation was no better. So then we asked him to try not to wake up when he needed a poo, to trust us to check his nappy and change it only when needed and to try and stay relaxed while we changed him (so hard for a proud 6 year old to have to do this but Ryan knows the alternative and is trying really hard). The nappies cannot always contain the volumes of stool, it floods out the nappy and so his bed is covered in changing pads so we don't have to keep changing the sheets. This seems to working for him and although his sleep is still broken he is getting about 8-9 hours a night which has had the desired effect and meant that he can be awake more during the day.
He is talking more and interested in watching his favourite YouTube video’s again on his beloved ipad. He has even requested that we bring in his x-box and has played on-line with a couple of his friends. Obviously he is easily tired, which he finds frustrating, but at least he is doing something that he loves which makes him feel slightly normal.
Gareth and I are taking it in turns sleeping in hospital with Ryan and sleeping at the parent's house. In hospital we get a total of an hours sleep on a bad night (if you add up the ten minutes here and there) and a total of 3-4 hours on a good night. The feeling of panic I felt has gone and we are both coping ok. Gareth has always said he will sleep when he's dead and so he even struggles to sleep at the parents house, and on my nights at the house I try not to think, but the sounds of machines alarming fill my head even though they arent there and I have to let the tears that I hold back all day fall silently onto my pillow.
Ryan has a superb medical team looking after, he is also visited regularly by the doctors from the day clinic and gets regular pop in's from the director of the children's hospital. Everything is being done to support him through this period and get him back to good health.
Wednesday, 6 February 2013
Skin: overall his skin is improving, the only remaining signs are on his feet, hands and groin. The itching is all but gone - so one piece of positive news.
Mouth: Ryan mouth started to detiorate last Tuesday and has progressively got worse, the roof of his mouth is raw and his tongue is completely white so are his gums in places - Ryan says his mouth is very very dry and you can see his gums stick to his teeth when he talks. Now a diagnosis is confirmed and viral and bacterial infections have been ruled out Ryan has been started on a steroid mouthwash which should gradually allow his mouth to repair.
Liver: the liver enzymes are slowly reducing and, although they are still high, the doctors say they are happy with this improvement which they attribute to steroids.
Gut: the biggest problem for Ryan, causing watery diarrhoea often 2-4 times an hour, the longest interval being about an hour, but only occasionally. There is occasionally blood and lately mucus and gut lining being passed too which is very upsetting to see. He has also had persistent nausea and vomiting although this has improved in the last 24 hours as we are tweaking the numerous anti-sickness medicines. He has lost approx 2 kilos in weight. He is totally exhausted.
Today he has been started on an antibody called inflixamab, which will target the gut GVHD. The steroids and immune suppressant medication that has helped the liver may also start to impact on the gut as the therapeutic doses are reached. Tomorrow he will also start a treatment called ECP which I believe is a form of light therapy which has shown good results in treating both liver and gut GVHD. they are also considering Mesenchymal Stem Cell therapy (MSC) which is way above may head and I can't begin to explain how that works but again they have seen good results with this treatment. This involves stem cells being extracted directly from the bone marrow and used as a targeted therapy. They will select either Gareth or myself as the donor for this procedure. Gareth has volunteered but we will go with however is the best candidate.
The purpose of these three therapies is to, hopefully, speed up the recovery process (which even then is likely to be months rather than weeks) and reduce the amount of time that steroids are used. The biggest problem with using steroids is that they suppress the whole immune system and so increase the risk of infection.
Infection is the biggest threat to Ryan. We have been told that now is the time to be paranoid about being around other people who might have coughs, colds etc. Then today we learn that swine flu is confirmed on one of the wards, effecting oncology children and the nursing team! But already the whole hospital is now being told that face masks are mandatory, we should not eat in the communal ward kitchen but bring the food back to the outer part of Ryan's room and eat there. We are really happy to follow these rules and hope that all other visitors to the hospital do too.
Vanessa Riddle, who also had a transplant a few weeks before Ryan, had arrived to start her 4th cycle of antibody therapy but when the family have very wisely decided to delay treatment until the swine flu is gone from the ward - hopefully when Vanessa returns Ryan will be feeling a little better.
Tuesday, 5 February 2013
This afternoon the doctor on the ward has confirmed there are no evidence of bacterial or viral infection and that by tomorrow the analysis would be complete and a diagnosis of GVHD is expected. This gives him the ability to add some medication to try and reduce the frequency of the bowel movements and to try and retain fluid in the blood which will be passed as urine and not passed through the bowel.
Ryan has so many side effects of both the GVHD condition and the numerous medications that he is on that I cannot write about them all, it is not expected that his condition will improve short term, this will be a gradual process.
Monday, 4 February 2013
Sunday, 3 February 2013
The tests have slowly come back negative to infection and so the Dr's think it is most probable that his symptoms are caused by GVHD, which could now be effecting the gut and mouth in addition to the liver. His mouth lining has turned white and started to break down in places (although he says it isn't painful just dry). The liver biopsy has been requested and should be done early next week to give a conclusive diagnosis.
He hasn't eaten since Tuesday, has been exhausted and sleeping on and off through the day. As he had been drinking well and was ok clinically (based on blood results) they said he could be treated as a day patient and not admitted. We have been going into hospital each day for iv medication.
However by yesterday we became increasingly concerned, as his diarrhoea worsened and he slept almost all day and so he was admitted to hospital at our request.
He has not been this poorly in a long while, even through this most recent transplant. It has also come as such a shock as only days before he had been ski-ing and having such well deserved fun. We are totally devastated but remaining positive that the Dr's will do all they can to improve Ryan's condition as soon as possible.
My stem cell harvest has been delayed by one week due to lab time for processing of the cells.