[MGirl]

HI-

I'm the Mom of a very young girl who recently underwent a BMT (bone marrow derived) for SAA (diagnosed early 2010) in the setting of Dyskeratosis Congenita (just diagnosed one month before transplant--strangely, nearly a year after her SAA diagnosis: 2x ATG cycles, cyclosporine for the entire year--which didn't work).

She is at day +81, and her counts are still so low, although she herself is very well and hasn't had any fevers. Her most recent counts were: WBC 2.0, ANC 0.26, platelets 5, Hbg low 60s, and a reticulocyte count that is 'abnormally normal' at 50. Her specialist keeps telling us not to worry, "to wait....it will come,...just wait". Another less-experienced doctor who we don't usually see, but was filling in one day, said "oh ya, she isn't doing spectacular, is she...". We feel very confused, but with a bad feeling in our guts on what is going on.

Her unrelated bone marrow donor was apparently "great" and there was a really good cell count in the harvest. They are both CMV neg and pre-BMT screening otherwise was normal. It was a perfect 10/10 HLA match. She had a "reduced intensity" conditioning regimen prior to transplant of Campath, Fludarabine, and 1 day of Melphalan. She was extremely slow to show any engraftment, despite starting GCSF injections on day+7 that continued daily for almost 4 weeks. At that point, her highest WBC was 1.46, and so they discontinued the GCSF after 3 days at this count (around Day+40). Immediately after, and ever since, her ANC has deteriorated and has barely got above 0.2-0.3. Despite those counts, we were sent home to "home-isolation" on day +49 and are going to the out-patient clinic twice per week. The only other complication she had in hospital was that she picked up adenovirus while there at around day +12 (other kids in the unit were in full bloom with it at the time), and was treated with cidovofir with marginal results at the time (per PCR testing of blood, urine, stool), although she was not very symptomatic from that itself.

What are we missing here?
Our BMT 'team' refuses to state that engraftment has failed, as she "still is producing some cells", but we know she is at a very delayed state of blood count recovery at this point in time. We are getting tired of being told "just wait a bit longer" week after week, with nothing happening. She is still fully transfusion-dependent (platelets now once weekly, and RBCs every few weeks). Are we just paranoid parents? Or is our little girl made out of kryptonite and doesn't really even need blood? HELP!