Day Plus 18

The last few days have been tough. She currently has the mild symptoms of graft versus host which is what we have been expecting. She has developed a rash, her bum is really sore and she has developed mucasistis. For the last couple of days she has been waking up and throwing up a combination of mucus and blood, not very pleasant at all. She had been waking up through the night in pain and during the day it was clear she was suffering from discomfort inside. The specialists are on the case though, and when symptoms have manifested they have been treated. In addition to the regular doctors and nurses we have had visits from Pain Management Specialists, Tissue Viability Specialists and X ray Specialists. The morphine has been increased so she now has a continual background feed with an additional push from the nurses. She has been given oxygen whilst sleeping as her SAT levels were low. A chest x ray was carried out as a precaution and all found to be good.  She has had a couple of temperature spikes and paracetamol has been administered. She has now had 3 blood transfusions and 3 platelet transfusions. Her Neutrophils however are showing signs of rising. Yesterday they were 0.12 and today they are at 0.14. As her Neutrophils rise we are expecting to see the illness’s slowly decline (touch wood). These are usual symptons for someone who has undergone a stem cell transfusion so altough it is not pleasant it could be a hell of a lot worse.   Even with  a swollen face and doped up with morphine she still likes to sing and perform the actions to head, shoulders, kness and toes.

It is now BMT Plus 14 and things have been going well. At Day Plus 10 Anabelle’s Neutrophils finally hit zero which meant that she now no longer had an immune system.  Her Neutrophils should start rising at about BMT Plus 21 days, which would then show that the engraftment is working. (Fingers, toes and everything else crossed).

She has now developed Mucasistis and is suffering from a sore mouth and it is also affecting her digestive tract right down to having a sore bum. The experts from Tissue Viability came up and provided some silicone based cream to apply to her bum instead of the 50/50 we have been using. She is also now on morphine to help with the pain and it is doing a great job at keeping it at bay. (She’s not sharing it though!!!). Although we had shaved her head it actually started growing back and we were actually wondering whether we were premature in doing this. However it is now falling out quite quickly with the evidence all over her bed.

We have been having a few visitors to come up and help relieve the boredom.  Through a mixture of the play specialist, nurses and volunteers we have been able to get out for a bit. The Play Specialist (who is invaluable) got Anabelle a puppet and they are now inseparable, she has it with her all the time except at Bath time. Bath times have drastically improved with her and she no longer screams the room down when being bathed but likes to sit there and splash around with her ducks and leaving the room in a wet state.  Her general temperament has improved the longer we have been here and when she’s not sleeping she likes to cruise around the cot dancing with the radio on. We make idiots of ourselves by dancing around with her!!! When people walk past the window she gives them a massive smile and brightens up their day.  Some people actually walk past just to get a smile out of her. Even when the nurses need to carry out their observations she now holds her arm out waiting for her blood pressure to be taken!! The only thing she isn’t a massive fan of is the mouth swabs and we have to pin her down to carry those out. We have also had visits from the Music Therapist and Speech Therapist. Her signing has developed and would put Mr Tumble to shame!!!

Her levels are being constantly measured and a couple of times her blood and platelet counts have dropped. She therefore has had to have additional transfusions to counteract this. Again nothing to be concerned as the experts here have got it under control. The Doctors and Specialists are very pleased with her progress.  


Two hoodies together!!

The other day we had a bit of a scare as we had been advised that her test (she is constantly being tested for bugs and nasties as she has no immune system) had come back positive for Paraflu. The Analyst wasn’t happy with the sample though and feared it may have been contaminated.  So a further test was done. As a matter of precaution whilst we were waiting for the second results to come back anybody that would come into our room had to wear a mask. We felt as though a red cross had been painted over our door!!! If the second test had come back positive it would have meant additional treatment, moving rooms and Anabelle having to sit in a tent for 6 hours. Fortunately it came back negative. We breathed a sigh of relief at that one but it also made us realise that things can change quite quickly when she is in this fragile state. We are however constantly amazed as to how well she is coping and very rarely crying throughout this journey which is more than most people have to face in a lifetime.



Today is Day Plus 4.

We have to say so far so good. She is no longer taking in her 200ml of water she was allowed per day and she has a bit of a sore bum but currently nothing really bad to report. She is generally very cheerful and is enjoying her playtime although after about half an hour she tires and needs her nap. Her HB levels were low so she had a blood transfusion the other night and they have picked back up.

For those that were asking this is a rough outline of her treatment as I see it.

Anabelle is missing the alpha-L iduronidase enzyme due to genes passed down by myself and her mother. She has had the stem cell transplant in the hope that with the new stem cells she will be able generate this particular enzyme.

As part of the conditioning for her body she has had chemotherapy which will destroy her cells in order for the new cells to grow. In addition the chemotherapy lowers the immune system so that the body does not fight against the newly installed stem cells. The chemotherapy drugs given to Anabelle to achieve this were Rabbit ATG (this is actually derived from rabbits by the way!!), Bulsulphan and Fludarabine. All these drugs have particular side effects so she has been given a whole host of other drugs to prevent and minimise these. Before the transplant her Bulsulphan levels were measured and found to be a little too low there a higher dose was given. As the cells came from an umbilical cord she needs approximately 3 weeks of gut rest so is on TPN (total parental nutrition) which is basically liquid food given intravenously over a 24 hour period.

What we are currently waiting for is her Neutrophils and platelets to drop down.  Her Neutrolphils were 3.95 at the start of the treatment and are as of today’s date at 0.33. Her platelets were 470 and are now at 208. These two items help form the white blood cells. Neutrophils fight infections and Platelets help with clotting. Once they have dropped down then the donors cells will engraft and these should then rise back up. Our biggest concern currently is infection as she now has no immune system and is at risk from all sorts of nasties, hence the isolation.  Other issues include Mucositis. This is where cold sores / ulcers form all the way from the mouth right through the system to the bottom. This obviously is very painful and uncomfortable for them. Her bowel movements are not very pleasant and are very similar to a hot cup of Bovril. (I never liked that drink anyway!!!). Another issue to be wary of is Graft Versus Host Disease. This is where the new cells recognise Anabelle's body as foreign and send out lymphocytes to attack the body. This can be mild, moderate or severe. She is taking Ciclosporin as a preventative for this. Another potential complication is Veno Occlusive Disease, which basically involves the liver with the blood vessels to the liver becoming blocked. Normally there is less than 10 per cent chance this happening however as she had to have additional bulsulphan Anabelle’s chances of getting this have increased. The closer towards day zero these illness surface the more severe they are so we will feel a lot better at somewhere around Day Plus 14.

Day Zero

Full of goodness !!

Today Anabelle had her Stem Cell Transplant. This was basically a 3 hour infusion of stem cells harvested from an umbilical cord from Slovakia. The cord is from a male donor and shares the same blood type as Anabelle. It also has a high amount of stem cell content. One thing no one as really mentioned is the smell. Throughout the whole process and even after it Anabelle has a blood smell about her, similar to that smell you get when you open a sealed joint of beef from the supermarket!!!. A nurse had to sit throughout the process and take observations before during and after to ensure that there was no adverse reaction. We are pleased to say there is no adverse reaction. She is still on her concoction of anti viral drugs administered via infusion. She no longer wants her 200 ml of water which we have been told is expected. Her neutrofils and Platelets have steadiliy been decreasing  (Neutrofils are one type of blood cell that help fight infection and plaelets are blood cells that assist with clotting and help to stop bleeding).
Her temperament has improved over the last couple of days and it has been good to see her smile and play again. Now we have gone passed day Minus 2 we have to gown up upon entering our room. At least we are able to get out unlike Anabelle who is stuck in the room.


Yesterday we had additional support with people looking after Anabelle so we took the opportunity to walk up to Camden Market for an afternoon out. It was good to get out and sample some decent street food!!!.

Passing the time!!!



Fatty at Camden!!!

It is now Day Minus Two.
She is on her last day of her lovely concoction of Fludarabine and Bulsulfan before her rest day of day Minus 1. However after checking her Bulsulfan levels she needs an extra infusion on the rest day. The last couple of day have been really hard. She has not been a happy little bunny at all. She wakes up- she cries, she gets changed - she cries, she has a bath - she cries (and she used to love her baths). To top it all we decided to cut all her hair off before it fell out. this was probably the hardest thing we have ever had to do but we know it is for the best long run. We are just hoping that it grows back the same way and not dark and curly as we have heard it can go after a bone marrow transplant (no offence to the people with dark curly hair).
Anabelle's moods seem to have started at about the same time as Ciclosporin commencing as per her protocol. (thanks to Teresa's comments for us to notice the connection) She is not drawing her legs to her stomach to indicate stomach pain but apparently it is also known to bring on head aches. Its frustrating because she cant tell us!!! Her codeine has been upped and the Ciclosporin has been diluted. This seems to have a positive effect.