*Help for Holly* from Bone Marrow Failure

In June 2017, Holly a usually active, outgoing and super healthy 9 year old kid, was struck down with a sickness that just wouldn't improve. Although Holly has always been very well, she did have a history of very easy bruising. After many blood tests it was evident that Holly was/is suffering from a rare form of Bone Marrow Failure called "Aplastic Anemia" - only 1-2 per million people are diagnosed with this disease. Basically it means Holly doesn't produce her own red blood cells, white blood cells or platelets - this leaves her dependent on transfusions for her red cells and platelets, and also very immune-compromised as she has very few white cells to fight infection.

Holly and her family, Mum, Stepdad and Three younger sisters Belle(6) Willow (3) and Frankie (2 mths)temporarily relocated to Auckland Ronald McDonald House so Holly could receive a Bone Marrow Transplant. She was fortunate enough that her younger sister Belle(6)was a match, so in August 2017 she received a bone marrow transplant in Starship Bone Marrow unit. This was very hard on her physically, but after 4 weeks in isolation it appeared her transplant was successful. Unfortunately at 70 days post transplant her marrow failed again. Her blood levels got lower and lower, until she was completely transfusion dependent again. After some attempts to save the graft with marrow stimulating drugs, she was left with no option but to undergo another Transplant this time using stem cells rather than marrow. She is still in isolation in Starship at present. She has undergone a significant amount of immunosuppression in order to ensure this transplant will engraft properly and she is starting to show the initial signs of engraftment.

Meanwhile back in ChCh, their earthquake damaged home has not had the rerepairs needed to provide the safe sterile environment needed to help Holly once she is finally able to return home.

Holly has a long road ahead back to health which will require extreme care as she is and will be very susceptible to infection for quite some time.

Thanks for taking the time to learn about our Holly, a wonderful wee girl with a quirky sense of humour who has maintained a positive attitude through it all.

Update from Holly's Mum Debbie 27/11/18

Holly has had two bone marrow transplants in an attempt to cure her from aplastic anemia - which is a rare form of bone marrow failure. She does not make her own blood, and relies on blood donors to provide her red blood and platelets (the part of blood which stops bleeding). It’s also means she is severely immunocompromised as her white cells are low.

Holly and her family have been living away from home at the Ronald McDonald House for 15 months, so holly can receive treatment.

Holly’s case has the doctors baffled as she has not responded to treatment as usually expected, and will likely require a very seldom performed third bone marrow transplant in the new year.

Holly’s younger sister donated bone marrow and stem cells for Holly’s last two transplants, however the next transplant will be using an unrelated donor from America, which increases the risks of developing graft vs host disease. Transplant is a very tough process and requires chemotherapeutic and immunosuppressive conditioning to eradicate any of Holly’s own defective marrow and replace with donor marrow. This leaves her completely open to infection and requires she live in protective isolation for the duration.

At present Holly is trialing some drugs in attempt to increase her marrow production - these have had no effect so far and she continues to receive very frequent transfusions. She also requires a continual IV infusion to reduce her iron levels which are extremely high due to continual transfusions. This is can be very hard on organs and requires reduction before a third transplant.

Holly is one of the most funny kids you’d ever meet, so full of personality and tenacity. Despite having to deal with things most kids have no idea about, she continues to make everyone around her laugh !