Mixed Day

Today's results came very late in the day and were mixed.  Her hemoglobin (part of the red cells above) went down again, this time from 7.4 to 7.1 (normal ranges are listed in the last post).  This keeps her on the border for needing a transfusion however she tires easily and will need the boost with school staring again next week.  Thus we'll be spending another Friday at Children's getting transfused.  On the up side she was able to make some platelets this past week and those numbers went from 6,000 to 11,000 (there are high error margins with these numbers as they just count a random sample of cells, like those above, from a sample of her blood).  Nonetheless we're always happy to see any evidence that her body hasn't just stopped producing blood cells all together.  Additionally, her ANC (absolute neutrophil count, the infection fighting part of her white cells) also went up, this time from 590 to 700.  That is always good as she needs that to be out in the germy world.  We have one more blood test before our meeting with her doctor on March 2nd.  I'm guessing that will be a significant meeting discussing a treatment plan but everything else has taken longer than I think so who knows.  As always much thanks for the support!

CBC

We've settled into a bit of a routine with having blood draws Thursday mornings and appointments for transfusions on Fridays.  If the blood counts aren't low enough for a transfusion then we get to skip the Friday appointment.  Today's blood counts made a solid drop but not quite enough to need a transfusion tomorrow.  A CBC or complete blood count is reported on a sheet like the one above.  I WISH this was a CBC for Annie, but her major values are way below the reference ranges above.  One group of cells that are still in the normal range are the neutrophils.  These are the part of the white blood cells that fight infection.  If these get too low she has to stay home so as to not be around sick people.  So, as long as we can keep her safe, we'll be out and about until next week's CBC.  Thanks for the well wishes and prayers!

Salsa dancing polka dots

Today's blood test showed Annie's platelets did not drop and her hemoglobin went up from 8.1-8.2 (normal for children is 11-13).  This is promising as we'd expect it to drop as the transfusion wears off.  Hopefully this indicates that by increasing her cyclosporine (immunosuppressive medicine) back up again we can keep her from needing transfusions while a treatment plan is being explored.

Thus Annie gets a green light for another week at school.  One of her teachers recommended practicing visualization techniques and Annie had her first success with it this morning.  Last week the phlebotomist had trouble tapping into a vein for Annie and it was a weepy experience for both of them.  This week, Annie visualized her blood moving to the draw site and things went perfectly.  Then she spent the car ride to school brainstorming visualizations for the proliferation of her blood cells and their happily sticking around.  Her final choice was of polka dots popping up all over and then doing a kind of vibrating salsa dance ... I'm pretty sure it's just a matter of time before this procedure gets published in the New England Journal of Medicine.

What's going on in there?

The results of Annie's biopsy came in and, fortunately, she still only has one serious condition ... instead of three.  Things can always be worse and we are grateful there was no evidence of MDS or leukemia in her bone marrow.  Since we're on the topic of bone marrow here is a short primer on what aplastic anemia looks like on the inside.  First, the name.  "aplastic" means empty and "anemia" refers to blood cells.  Thus Annie's bone marrow is "empty of blood cells."  When they look at a slide of her bone marrow under a microscope it looks like the bottom picture above.  Mostly a lot of empty, white space.  Someone with normal blood counts would have bone marrow that looks like the top picture.  Bone marrow filled with cells.  Now if a cure were only so simple :)

Platform 9 3/4

Annie is home and happily recuperating from today's biopsy.  This was her fifth bone marrow biopsy but her first at Children's.  She was off food since dinner and liquid since 6 a.m.  Although she was scheduled to arrive at 8 a.m. the procedure was delayed and didn't begin until 10:30 a.m.  Nonetheless she did fine with just a little anxiety about being in a new setting and having gas anesthesia versus through an IV line.  She was also happy to have a special friend along - a former student and babysitter who recently graduated from nursing school.  Nothing like having your own nurse on the team!

It was interesting to note the differences from her last hospital.  First, being a children's facility, they explained and showed pictures of the procedure and rooms so she knew what to expect.  Second, they had a team of six nurses and one doctor versus one nurse and two doctors.  Third, it was in an operating room and parents weren't allowed to stay for the procedure (but she was asleep so it didn't seem too bad).  But a really interesting difference was that while you wait during the surgery you watch a large board like at a train station.  Each child has a number and you see which room they are in and how many minutes they have been "in pre-op," then how many "in op," then when they move to "post-op" and "recovery."  While it's no eurorail, it does ease some of the tension.

Now we wait and transfuse until the results from the biopsy and marrow search come in.  The next meeting with her doctor is March 2nd so it will be a bit of a wait.  One thing that helps in the meantime is that she was prescribed aminocapoic acid for any "bleeding events."  Since they don't want to transfuse platelets and they continue to drop (9,000 vs normal at 150,000-400,000) this will hopefully help in an emergency or when she gets a bloody nose.  Her last one required pinching her nose for 1 1/2 hours in the middle of the night so that is a welcome help.

We'll report after next Thursday's hospital visit and hope we have no "events" before then!

This is how we do it

Hopefully most of the people reading this (especially Annie's classmates) have never seen what a blood transfusion looks like, so here it is.  The pole to the right has a bag of packed red blood cells hanging at the top.  These go through a pump that determines the drip rate (Annie likes a slow drip or it gets uncomfortable).  You can just see the tube carrying the blood against the pillow on her lap.  This connects to a needle sticking out of her arm.  I know that part doesn't sound so nice but it doesn't bother her.  The pillow on her arm actually contains a warm pack that helps the blood flow more smoothly.  Today she only received one bag of blood so it took three hours for the transfusion part.  Although this seems unbelievable, we calculated that Annie has had over fifty transfusions and around 300 needle pokes so far.

For me, going through the third treatment round in six years feels like when you read the same book at different points in your life.  While the plot remains the same, your experience with the book changes as you've changed.  First it's a lot easier as I have already worked through so many layers of the experience and both girls are much more independent.  For example, Annie can now read and write and stay by herself without getting scared.  In fact she asks me to leave the room so I can get her a treat from the cafe and she can sneak in a video.

The preliminary bone marrow registry search turned up 159 potential matches at the six-out-of-six level.  However we know from earlier searches that it is unlikely any of these will be a perfect match as she has one very rare allele.  Nonetheless they want to retype her blood since they now match for twelve alleles.  This blood was collected today.  I have been asked how to get involved with the marrow registry.  The website is http://marrow.org and they'll send you a kit where you swab the inside of your cheek and mail it back.

In the meantime the plan is to increase her immunosuppressive medication dose in hopes that she can avoid more transfusions.  She has only received whole blood transfusions as the body develops an immunity to platelet transfusions so the doctor only wants her to have one if she is actively bleeding.  Thus, I warned Annie, she is like a walking bull in a walking china shop.  Her body is very fragile yet she's all jacked up on blood (good thing vampires are still cool).

She is scheduled for a bone marrow biopsy next Friday and no news is good news until then.  Thanks for the continued prayers and support!

Fuel gauge

Unlike cars, unfortunately, we don't have little fuel gauges on our t-shirts so we didn't realize Annie was going to run out of gas at school this morning.  (Special thanks to our miracle-working neighbor!) Although her blood levels are checked weekly, it is best for her to have as few transfusions as possible so it's always a guess as to if she can make it another week.  She's hanging out on the couch taking it easy now and all is ready at the hospital to refuel her tomorrow.  So as not to paint the wrong picture of her status, let it be known she is looking forward to playing guitar and singing in two numbers and doing a tap routine in a third at the school talent show on Friday.  I'm not kidding when I say this is like a roller-coaster, she can go from 0 to 100 in a matter of hours! 

Here we go again!

Greetings faithful friends!  It has been over six years since Annie was diagnosed with aplastic anemia in preschool and we started keeping everyone updated on our roller-coaster ride.  I can't say we have gotten off the ride, it's just we've been on a kind of long, flat section.  However our little cart is picking up speed again hence we're firing up the blog.

To recap...Annie was diagnosed with aplastic anemia in November, 2005 when she developed an unusual number of large black-and-blues.  She began almost weekly blood or platelet transfusions as well as large numbers of blood draws and medical procedures.  In February, 2006 she had a bone marrow transplant with her own stem cells saved from the chord blood at her birth.  The transplant was a success and she began kindergarten.  One year and a half later her blood counts dropped again and she needed treatment.  In August, 2007 she had the immunosuppressive therapy called ATG.  She has been on immunosuppressive medicine ever since but with a relatively low response.

Since that time we learned that our home had been improperly treated with a pesticide called chlordane decades ago yet the levels were still unsafely high and her body was contaminated with the substance.  Chronic exposure to chlordane can cause aplastic anemia and since Annie has tested negative for every genetic form it's our best guess as the cause. We moved her out, cleaned up the house and bought a new house but the condition remains.

We moved her care to another hospital and tapered her off her immunosuppressive medicine to see how she would respond.  Her blood counts dropped again and we are now deciding what treatment path to follow from here.  There will be a search of the bone marrow registry to see if she now has a perfect match (she did not in 2009) and she will have a bone marrow biopsy to check for any unknown developments.  In the mean time she has weekly blood tests and transfusions as needed.  She has been able to stay in school and is a happy fifth grader who enjoys school, friends and performing in musical theater.  Her next blood test/transfusion date is this Thursday and I'll update after that.

Many thanks for all the support and prayers!