New, seeking to learn more about our daughter's condition

[Franx]

Greetings from Eastern Oregon.

Our 16yo daughter has been struggling with low blood counts for the past 3 years. We started with started with just low WBC (0.8 - 1.5) 3 years ago, and now her condition involves all 3 cell lines.

Currently her dx is pancytopenia with unknown origin. She receives 2 units of PRBC every 4-6 weeks. Typical CBCs are WBC (0.8-1.0) with ANC (400-600), Hg will fall from 8.0 to below 7.0 between x-fus, platelets (50-70) and lab notes usually describe RBC as hypochromasia with both micro/macrocytosis.

The most recent bone marrow biop was the first of December at Doernbecher. It revealed hypocellularity, displastic cells, and a lot of descriptions beyond our comprehension. they have ruled out viruses, gene abnormalities, autoimmune suppression, and environmental causes.

We hope to have another bone marrow biop in another week or so at Seattle Children's Hosp. The goal will be to identify if her condition is progressing toward AA or MDS.

So, why are we here? We hope to learn more about bone marrow disorders, how to support our daughter during her teen years, and how to maintain "normal" household activities with 2 very busy younger sisters.

Thank you

[NLJabbari]

Hello Franx,

Pancytopenia and Aplastic Anemia do sound an awful lot alike, but I know there is a difference. Is it possible that with Pancytopenia, your daughter's counts might spontaneously revert to normal? Just wondering what the doctors say about this.

Well, I hope that your daughter is doing well and that she's coping well with the low Hgb. My son who was dx'ed with SAA back in 2004; his Hgb runs at about 8.0 and he still has more energy than I do.

[ssdavi71416]

The link below is from Neal Young at the NIH. It addresses many questions. The Title is "How I treat acquired aplastic anemia" by

Phillip Scheinberg and Neal S. Young

http://bloodjournal.hematologylibrar...1185.full.html

Have the Drs discussed BMT and if your daughter has siblings have they been tested for a marrow match? With the need for transfusions and the low ANC it might be worth discussing BMT with the Dr.

Good luck with everything.

[Heather8773]

Ssdavi

Thank you for posting that link!

[evansmom]

Hi Franx,
Sorry you've had to come here but I can tell you first hand that this is a wonderful, informative and supportive community. Everyone here really helped me get my then 10 year old son from AA diagnosis, through unsuccessful ATG and then through a very successful unrelated BMT. He's 15 and a half now and the picture of complete health.

It's very good that you are taking your daughter to Seattle Children's to seek expert opinion and you are doing everything right in terms of finding and using the resources available to you to be your daughter's best advocate. Most specialists in the field of bone marrow diseases are very good and very thorough but never forget that it is not they who will lose sleep over your daughter's illness, only you and bottom line - squeeky wheel does get the grease. Don't let yourself be intimidated by anyone; you have every right to ask any and all questions until you are comfortable with the answers. You have every right to protect your daughter from infections by speaking up if you witness any member of any medical team not following best practice, like washing hands and wearing masks if appropriate.

A diagnosis and treatment plan is needed ASAP as you want to minimize the number of red cell and platelet transfusions your daughter receives and any time her neutrophil count is below 500, she is at risk of becoming overwhelmed with a fungal, viral or bacterial infection. My biggest concern when we were at that point was keeping my son away from every potential source of fungus/mold such as damp, dark places like barns, rooms or buildings that had old dank window air conditioners (big source of aspergillus) etc. Evan was home schooled during this time.

I believe it is easier for a child to cope with all this than it is for a teenager. They are just starting to have some autonomy and freedom and make choices for themselves. The best approach might be to emphasize 'short term pain for long term gain'. Following the medical team's instructions, being cooperative and taking her situation seriously will only help to ensure she returns to a normal healthy life in the hopefully not too distant future.

She could make her own account here on Marrowforums and seek to chat with other teens who've been there, like my son.

As far as what's going on with her marrow, the description 'dysplastic cells' makes me lean more towards MDS. With AA, there is simply a lack of cells but usually the cells that are there are normal, not dysplastic.

Seattle will be of tremendous help I am sure. Keep us updated and best wishes for a speedy diagnosis and treatment plan.

Evan's journey is described HERE on Marrowforums, including a chapter written by him to other kids going through a similar journey plus his CaringBridge site is linked below which describes in detail his entire journey.


P.S. Is your daughter on any routine medications such as anti-epileptics or other meds that could have, over time, damaged her marrow?