There are times when this job is hard, there are times when this job is really hard, and then there are times when it’s all worthwhile.

Back in late October, I was on the hematology consult service, and was called down to the emergency room to see a four year-old little girl (we’ll call her M) who came in with easy bruising. On checking a set of blood counts, the ER found that her platelet count was low (below 10, with a normal count being more than 150); otherwise, though, M was active, alert, and completely well-appearing (aside from the head-to-toe bruises, that is). As a hematologist, that kind of kid just screams idiopathic thrombocytopenic purpura, and that’s what we told her family. We elected to observe M, but when she came back in a week later with nosebleeds and an even lower platelet count, we gave her WinRho. Her platelet count didn’t budge, and when it remained rock-bottom even after intravenous antibody treatment, M bought herself a visit to the heme clinic for a bone marrow biopsy to figure out what was going on. In ITP, you’d expect to see the precursor platelet cells all revved up in an attempt to fix the problem, but in M’s case, we saw a whole lot of nothing; literally, all the precursor cells — for platelets, red blood cells, and white blood cells — were vanishingly rare. And that meant that we were looking at aplastic anemia.

Aplastic anemia is a shitty disease; once your bone marrow shuts down, it doesn’t turn back on again without massively serious help. There has been some success with throwing profound immunosuppressants at it, but in the current state of medicine, the gold standard for curing aplastic anemia remains a fully-matched sibling bone marrow transplant. In less technical terms, that means that if a patient with aplastic anemia has a brother or sister whose bone marrow is a perfect match, the treatment with the best outcome is to do a bone marrow transplant from that sibling. In any given family, there’s a 25% chance that a sibling fits that bill, and in M’s case, it turned out that her little sister was a perfect match. And with that, in the period of a week, we went from reassuring a family that their daughter had a transient condition to telling them that she had a lethal condition, and that in order to cure M, they should let us give her lethal doses of chemotherapy and then rescue her with bone marrow from her fifteen month-old sister. And in a way that I can only admire (mostly because that’d be the perfect recipe for making me totally lose my shit), they jumped on board almost instantly.

M’s transplant course was pretty rocky. Given what we’re doing to them, we like to bring patients into transplant in a pretty controlled fashion, but within a few days of being diagnosed, M spiked a high fever and was found to have a bad infection at the site of her bone marrow biopsy. She then got hit hard with conditioning chemo, lost all of her hair (but shaved her dad’s and grandfather’s as a deal), developed another infection, got bad mouth sores, couldn’t tolerate anything to eat or drink for a two or three weeks, and around thirty days into the transplant, her white blood cell count still hadn’t come back in. We were at the point of talking to M’s family about possibly needing to get more marrow from her sister, but then, everything just fell into place. Normally, even after bone marrow transplant patients recover their white cell counts, they still need transfusions of platelets and red blood cells for a while longer; when M’s new marrow came back in last week, it clearly decided that it was done fucking around, and came in strong enough to support all of her own blood cell needs. And that meant that yesterday, when she was eating well, we decided that it was time for her to see someplace she hadn’t seen in two and a half months — home.

Today, I saw M in clinic for her first visit as an outpatient, and she looked fantastic. Her counts were phenomenal, her electrolytes were perfect, her appetite was unbelievable, and her dad said that she had spent over an hour yesterday evening just playing outside with her cousins, like a normal kid. On her way out the door, I have her a little rub on the head; feeling the faint peachfuzz peeking through, I realized why I do what I do.

Comments and TrackBacks

jason, you don’t know how much it warms my heart to read that story and to see you wrote it, yesterday, on the day of my brother-in-law’s 16th ‘birthday’. xo

• Posted by: lisaann on Jan 6, 2004, 12:17 PM…

• Pinged by anil dash's daily links on Jan 6, 2004, 5:03 PM

I had itp when I was 10. You’re story brought back many memmories, including the one where I promised myself if it wasn’t serious, if I didn’t die, I would go on to be a doctor - that never happened. Your story is one reason why. The determination to see her treatment through, to not waver over the impossible - those traits you have - are very rare and precious. Good job!

• Posted by: DQ on Jan 7, 2004, 1:17 AM

A very nice first-person account by a pediatrics resident

• Pinged by Tom McMahon on Jan 14, 2004, 1:07 AM

I just found out my 35 yr. old wife has aplastic anemia. Her sister will hopefully be the marrow donor. What are we in for. This is fairly terrifying

• Posted by: Rob on Jan 27, 2004, 1:50 AM

A colleague of mine - age 39 with 2 young children just diagnosed with aplastic anemia. Her sister is a match so far on 4 important things they are looking for and so it looks like BMT will be scheduled soon. Your story is reassuring

• Posted by: dn on Jan 29, 2004, 11:33 AM

A colleague of mine - age 39 with 2 young children just diagnosed with aplastic anemia. Her sister is a match so far on 4 important things they are looking for and so it looks like BMT will be scheduled soon. Your story is reassuring

• Posted by: dn on Jan 29, 2004, 11:33 AM

In a little town in the middle of the Pacific Ocean, in the house next door to me, a 5 year old girl - cute as a button(& My daughters classmate)lays, waiting for the Doctors on a neighboring island to come up with something. She was diagnosed with Aplastic Anemia about 3 weeks ago, and just recently asked her grandmother whether or not she would go back to school soon. This story is reassuring for her future and lightens my heart strings. I wish there was something more I could do to help them out.

• Posted by: MFO on Feb 2, 2004, 7:38 PM
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