The Foundation for Children with Atypical HUS

Hey everybody - we had a really good clinic today. I was nervous because it was the first time checking all Hyde's #'s after the nephrectomy and I knew from other kids that sometimes their kidneys had been doing more than they thought and they had a few hairy weeks/months trying to adjust dialysis to get back to an even keep. Well - apparently Hyde's kidney's really weren't doing that much after all (just like we though) because all his #'s were amazingly stable. BUN of 57, Creatinine of 9.6. Sodium, K, and phos OK and calcium just a little high. His hemoglobin and hematocrit are still a little low but we think this is just still recovering from surgery and all those fluids they gave him (why I don't know) but they are 10.9 and 31.9. His platelets are a CRAZY 503 - NEVER thought I'd worry about these being too high - but we'll watch this - we have never ever had platelets this high - possibly they were still being chopped up a little by his kidney's? His BP's seem to have stabilized about where they were before the surgery - but on only 2 BP meds vs. 4. This is great since we didn't think we'd have actually dropped any yet - the dr's are hopefull that this may continue to improve over the next 4-5 mths and we might even drop more meds - yippee!

Surgery wise Hyde seems good - we have the surgical follow-up Friday with Dr. Smith - we'll let you know how that goes - but all SIX of his incision sites (they are all small though) look good.

A quick tooth update - it's still there and loose (Hyde keeps trying to wiggle his teeth too and is very mad that he's not 5 and getting loose teeth too). Will update once the big event occurs - I had a big event last week (I swear it was up there with Hyde's surgery) - I registered Ruth for KINDEGARTEN - YIKES - I'm REALLY not ready for this - really not prepared, ugh ugh ugh.

Now that we've made it through the surgery AND kindegarten registratio - whew - it's time to focus on some fun stuff - the 2nd Annual Hike! We had a meeting last night and it should be bigger and better than last year (please no rain, please no rain) with even cuter t-shirts and cooler kids activities. So go on-line and sign-up today! www.hike4hyde.com

The earlier you register the better for me - and I'm nervous because we are having it only one week after spring break and worried you will all wait until you get back from spring break to register - I don't want to have a nervous breakdown about t-shirts this year ;-).

Thanks again for all the thoughts, prayers, food, calls, and love over the last few weeks - Hyde came through tough as always and it now screaming for some white powdered donuts - so better go.

Bill and Phyllis

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Comment by Donna Kolp on March 16, 2010 at 10:27pm
Phyllis~
FYI on the platelets...Jonathan is on aspirin therapy and has been for 11 years. Jonathan's platelets usually run around 380-480. They have been as high as 750. Funny how when an episode shows up, that is one of the first things to dump.
Glad to hear Hyde is doing well! Will continue to keep you guys in our prayers!
Donna :)
Comment by Cheryl Biermann on March 16, 2010 at 8:47am
Ruth will do fine, Phyllis & Bill, will be fine, Hyde will also be fine, it will be you who accumulates gray hair this year! Girls tell you everythig, so much so that you'll probably go into a trance once she starts describing the 12th girls' outfit worn for picture day! You will even know where those horrible boys sat in the lunch room and what exactly they threw at each other that accidentally got into the principal's toupe' and whether it was salted or unsalted!
It's Hyde though that will really cause you to age! When he gets to school he might tell you all about what the janitor had to mop up in their room that day, and even tell you he told the janitor that you would like some of that saw dust stuff they put down because YOU really love cleaning! But that he needs to be there with snacks on his half birthday a half hour early and that it has to be peanut-free, casein and gluten free, well, if your lucky, he might tell you the night before around 8 pm!

Let us know about those platelets, that's an interesting twist-wouldn't it just have to happen to Hyde? Could a hemolyzed sample have caused it as in the case of High K? Pulled from his line you wouldn't think it could get hemolysed, but Nathan's has for sure---just didn't affect the platelets.
Comment by Amy Swarbrick on March 15, 2010 at 10:21pm
What kind of mom are you, choosing to blog over getting your kid powdered donuts;) So glad to hear Hyde is doing so well. Now how you feel about kndergarten, Cadance starts going five days a week next year instead of three! I'm going to miss her! What are Brody and I going to do?

WELCOME - Friends, Family Members, Patients, and Researchers - JOIN US!

The Foundation for Children with Atypical HUS encourages patients and investigators to share information and explore options/resources as we work together to gain insight into this rare complement disorder. By increasing contact opportunities with researchers and medical personnel interested in helping the aHUS community, our stories foster a better understanding of atypical hemolytic uremic syndrome.

Sharing information, inspiration and support for one another, we seek to gather together people and knowledge as we strive to improve the lives of patients and families dealing with a diagnosis of aHUS.


NEW DIAGNOSIS OF aHUS?
Be proactive! Get the medical basics of aHUS, what lab values to monitor, and areas of concern...check out the "aHUS Bootcamp" and "About aHUS" tabs at the top of this page!
If your doctor has never treated a case of aHUS, please print out our 'Doc to Doc Registry' and ask him/her to contact a physician versed in the complexities of aHUS and new options for 2011 genetic testing and treatment.

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Did you know...

CFH (Serum Complement Factor H) is a regulatory protein. The secreted protein product of CFH consists of 20 repetitive units named "short consensus repeats" or SCRs (each approximately 60 amino acids). In patients with aHUS the last 5 "pearls" in the twenty pearl strand protein, SCR16 - SCR20, should bind to protect cells but do not- they are defective in one or more of the last 5 SCR locations. If they cannot bind or stick to the kidney to protect that tissue, the platelets clump into clots that affect the glomeruli of the kidney -potentially causing acute renal failure.
  
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It is estimated that there are about 300 cases of aHUS in the U.S., and it is most common with young children. The condition is life threatening and either can be chronic or can recur at intervals.
  
more factoids...

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