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.