Tuesday, April 21, 2009

Making Lemonade

We're making lemonade here in St. Louis.

We're here to investigate the newly discovered familial hypophosphatasia. (HPP is an exceedingly rare bone disease that causes soft bones and early tooth loss). It's not what we'd choose to do for vacation, or even instead of regular life, but we're making the most of it. It almost feels like a break, but not quite.

The kids are undergoing all kinds of testing -- I don't even know the names of all the tests. I do know they took 7 tubes of blood this morning. (They only got 1.5 from Ruby because she kicked and cried at full volume for over half an hour.) The boys had pulmonary function tests at 9pm last night, all the kids have had a series of x-rays, and we're all doing 24 hour urine catches (even GrandMary and Grandpa). The boys are peeing into jugs, and Mary, Ruby and I are peeing into "hats" decorated with flowers by the imaginative nurses. We take our receptacles with us in conspicuous red bags everywhere we go.

Today we were escorted to see Dr. S., the dentist with the most HPP experience anywhere, in a Shriner's van. His office had big console video games and colorful giant-sized paintings of sports heroes and princesses in every room. Spiderman was hanging from the ceiling and Superman was punching through a wall. Dr. S. called Ruby "princess" and I've never seen her sit so still in the dentist chair. Usually she screams. He told Abe to only worry about brushing the teeth he wants to keep. Abe's gums are in terrible shape and no amount of nagging gets through, but I think he heard Dr. S. (It helps that he is friends with Albert Pujols and that one full wall is devoted to the Cardinals.) I'd almost fly here every three months. He's that good.

The kids' food is all painstakingly prepared by the research kitchen and is weighed before and after they eat. They want to make sure they know exactly how much calcium each is consuming when they analyze their blood and urine specimens. But there are a lot more things they are testing, things that I've never even heard of, despite having pored over all the technical research papers many times.

I don't like not knowing everything, I don't like feeling like the overly persuasive mom, I don't like it when the doctor calls me "Mrs. Hill" and refuses to call me "Mary", even when I ask. She's a wonderful doctor, though. She got Abe to laugh and also earned his respect by knowing before he said it that he wanted to be a baseball player when he grows up. She also predicted, based on the position of his cowlick, that he could be a switch hitter. (She made him promise to give her credit if he makes it to the big leagues on that information.) Ruby flirted and giggled with her, and Oscar was not anxious. I couldn't ask for better. And she did agree to call me "Hey You!", an excellent compromise and a big step up from "Mrs. Hill" in my opinion.

I've asked for tests that they will not do:
I want Oscar's scoliosis x-rays taken both in and out of the brace. Denied.
I want them to try to draw Ruby's blood again. Denied.
I want them to evaluate Oscar for orthotics. Denied.
I want them to x-ray Abe's arm to check if his fracture has completely healed. Denied.
This is a research hospital and we are part of a study. I need to remember that.
I also need to remember that this is all free. Yes, FREE!

But we are getting starting to get some answers. We think Ruby might "only" have the odonto (teeth) form of the disease which will hopefully be very very mild. We think Abraham and possibly Oscar might just be carriers. (So we'll just have to test everyone they date more than once to make sure any potential offspring would only be passed one mutation and not two, which could be lethal).

We learned that Oscar's scoliosis curve is "very small". (What does THAT mean? I can't believe they didn't give me a number yet! I've taken that kid for x-rays every 6 months since he was two and have charted his progress and regressions. I need a number!). But, they are not recommending surgery at this point and apparently that is all I need to know.

The big conference with all of the doctors is tomorrow so we'll hopefully learn more then.

We've also laughed, a lot more than you would expect in a 4-day hospital stay. And the nurses and therapists have been fantastic. Fantastic! They took the kids to the special toy closet after the big blood draw. Abe chose a cardinals hat (he left his at home and really wanted one for the Cardinals game we hope to go to on Thursday or Friday). Ruby chose a doll which she promptly named "Luna" and carried with her all day. And for Oscar they had a set of six plastic animals, the very kinds he collects. The smile was precious but I didn't have the camera at that moment.

And tonight, the evening nurses agreed to watch the kids while Paul, his parents, and I enjoyed a nice dinner out. I could post pictures of us all laughing so hard we had to wipe the tears away with our napkins. We found many things to laugh about and to be grateful for (like getting to sleep in a hospital bed instead of on a fold out chair). We felt like escapees. We were escapees.

The kids are having fun along the way too. Abe is playing guitar and reading a book with Paul. He's also discovered a new Wii game down in RT (recreational therapy). He's not working on his research report but that is okay. Ruby is coloring, and playing, and making friends in RT. Oscar is making friends too, and reading, doing sand art, and painting. They are getting exposed to and befriending kids with all kinds of orthopedic challenges, using all sorts of assistive devices and starting to see beyond the visible disabilities to the children within.

And I'm learning how to not be in charge, at least not all the time.
We are making a lot of lemonade here in St. Louis.


  1. Thinking of you! My sister lives in St. Louis -- I wish I knew where you were staying and I would have sent over some food. Hope that all goes well and that you learn something. And your family is adorable -- there's something so special, albeit weird, to see the whole family in a hospital room, hunkering down.

  2. That's some good lemonade, Mary! : )

  3. Mary - Good to hear of your stay in St. Louis. We should talk about the switch-hitting some time but suffice it to say that you are in the hands of some awesome professionals who know just what TO say to put kids at ease (rather than saying things they should NOT say as you/Abe had to endure not so long ago). Can't wait to see all of you back in the Village.