Benson and Claire were born with a rare genetic disease in which their liver produces too much oxalate--called primary hyperoxaluria. Claire's condition has progressed much faster than Benson's. She was on dialysis since she was 3 months old and for much of that time, did dialysis 3 hours a day, 6 days a week. She receive a much needed kidney / liver transplant on December 18, 2009. It is expected Benson will eventually need similar transplants.


Wednesday, May 2, 2012

a bit about today

Claire is a disaster when it thunders. An absolute disaster. She's a very light sleeper and is easily awakened by the rumble tumbles of the sky. The only thing that can get her through a night of thunder is to play children's primary (church) music in her room. And so tonight is one of those wet, loud nights. Mabel, who hadn't gone to sleep yet either, loves the music. She stands up in her bed, flings her arms around like she's leading it, and sings like only a one-year old can! Between Claire crying and Mabel singing, hopefully they both can get to sleep sometime tonight...sooner than later, preferably. Claire told me this morning that she didn't get any sleep last night. When she heard the thunder at 12:30 am, I turned on some music for her and she told me she listened to the whole 1-hour CD! She was hoping it'd start over again after it finished, but finally gave up and went to sleep after the 'show.' Hum. Maybe we need to move to somewhere a little less wet. :) Or somehow convince her that a noise can't hurt you!

Claire had labs today and her liver numbers look soooooo much better! One is actually in the normal range and the other two have come down significantly! That's a nice sight to see. That liver likes whatever we did over the past week....probably mostly having her drain open to a bag. It hasn't been our favorite part of the week, though. It's not terrible, mostly annoying--something to lug around, worry about getting tugged on, not very sightly to gaze upon, and always the possibility of leaking bile on the furniture. Yuck. But I think the plan is to take off the bag tomorrow. I'll know for sure after we chat with her liver doctor tomorrow.

I did meet with her kidney doctor today and her kidney looks fabulous! From all the kidney tests we did a month ago, they found that she still does have oxalate coming out from the deposits all over in her body. So she needs to remain on a high fluid diet for yet a while longer. It's pretty crazy that nearly two and a half years post-transplant her body is still trying to get rid of oxalate deposits from her 'old' liver that produced too much of it. Her body isn't producing any excess of it now, but her level is still high in the urine because it's "cleaning" out the damage that was already done! How long will it take to get it all-cleared? Who knows? Likely a few more years yet. But the doctor reassured me that it wouldn't be like this forever. ((as my kids like to do.....fist pump Yesssssssssss!)) But for now, we'll keep pumping her full of fluids to keep that new kidney protected and working hard!

We went to the mall today. Claire opted out of riding in her stroller so she could push baby Annie in the stroller instead. She brought bottles, snacks, and a plastic Easter egg just for Annie. Claire couldn't wait to go potty at the mall. With diaper changing bag in hand, she took off all Annie's diaper, carefully cleaned with her own special baby wipes, and lucky Annie didn't go poop so she didn't need cream. After placing the same diaper back on Annie, she carefully put everything away and grinned. Certainly this was the highlight of her mall trip. Not the darling sun hat we found so she doesn't get cancer! :)

Being a mom really is pretty cool. I'm sure Claire would agree.

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