Friday, May 28, 2010


I have learned a thing or two about immunizations since Zander was born. These are things I never had to worry about with Ayda. First things first, when you are giving a baby/kid/adult (with Hemophilia) an immunization it needs to be done subcutaneously (just under the skin and NOT into the muscle). That is the really important part, if you give an injection into the muscle it can cause a muscle bleed.
So far, we have had really good luck with his immunizations but our luck ran out on Zander's birthday (I know who schedules shots for a birthday...I guess I got that from my mom). Zander had five shots on Wednesday (two in each thigh and one in his arm). I noticed some swelling in his right leg on Wednesday evening but he seemed to be doing okay. By Wednesday night he wouldn't walk or put any weight on that leg. We called up to the on call Hematologist and he told us to bring him into the ER.
This is our first ER visit and I will say the emergency room at OHSU is NOT your Mama's ER. Complete with valet parking and a separate area for pediatrics. There were video games and tv's with movies, books and puzzles. None of that really mattered to Zander but it was nice for Ayda. The other magical thing, Zander was crying during the initial exam and like angels, two women came through the doors with bubbles. They did a great job keeping Zander's mind off of what was happening and it was nice for Ayda too.
One thing people don't realize is not all doctor's or nurses are familiar with Hemophilia, that can make it interesting while getting care. I have heard my Mom and Grandma tell stories about nurses who took care of my dad, who refused to listen to my Mom and it resulted in incorrect treatment for my dad. I did NOT have this experience. The nurses were great, I mixed Zander's factor, told them which vein is best to hit, helped hold him down and they did a great job.
Unfortunately, Zander needed more than just that one infusion to stop the bleeding into his thigh (imagine it kind of looks like a cartoon drumstick). So, we are all set up with an IV and I will infuse him for the weekend.

One of the nurses at the HTC did give me a couple good pieces of advice, one: schedule prophy for the same day as your immunizations and two: ask the nurse giving the shots to use a shorter needle (less chances to hit the muscle.).

Tuesday, May 25, 2010

The Power of Prophy, part 2.

Lots of people ask questions about exactly what treatment for Harris means.  I think they imagine it to be lots worse than it is...

Prophy day (we have a standing appointment at our center for once a week treatments:)

  • 7:30 wake up.  Have a nurse.  Have a bath.

  • Daily bruise inventory? Check.

  • Get dressed in short sleeves for easy access.  Go for a layered look with a sweatshirt to keep the veins warm and plumpy...and stylish.

  • Electronic distraction for 4 year old brother? Check.

  • Factor? Check.  Extra box of Factor?  Check.

  • Arrive at treatment center a little early for parking...

  • ... and playing

  • Favorite exam room?  Check.

  • Purple gloves.  You know you've got trouble when they pull out the purple gloves.

  • Pick a vein... any vein.

  • Mmmm.  Hot packs.  Warm veins are happy veins.

  • Mix the factor.

  • Cross your fingers for one good stick.

  • Let the good stuff flow.

  • Voila!  

  • Get big love from the amazing team.  Seriously.  They are like prophy Ninjas.

  • Get big love from Mom.

Done and done.  Now, what's for lunch?

Saturday, May 22, 2010

bloody good time

Today we had a seminar put on by the HFO we learned about joints and joint health. The keynote speaker was a physical therapist, he gave a lot of really great advice. Both the PT and the host (from Bayer) are Hemophiliacs. It is still so odd to me to see grown men, with Hemophilia, looking so healthy and being so "normal". The seminar was held at a rock climbing gym and anyone who wanted to could use the rock climbing wall. Ayda was so brave and did so well. Travis gave it a whirl and Avery showed us all up. She said she didn't want to go but within two seconds she had scurried to the top and was bounding back down.

Monday, May 17, 2010

The Power of Prophy

Last week was our 7 month comprehensive appointment for Harris.  (It was supposed to happen at 6 months, but I sort of didn't schedule it.  Oh well.)  He has had 2 infusions so far... both within the last month... and he is trying to walk.  In case you missed that... he is 7 months old and trying to walk.

The title of "Earliest Walker" is currently held by a cousin of ours... 8 months- full on walking.  Zander, who you will also know from this blog, walked at somewhere between 9 and 10 months.  Harris currently holds the family record for "Biggest Baby" (9lbs, 10 oz) and "Longest Gestation" (42 weeks, 2 days - hence the biggest baby thing).  At this point I'd like to go for the hat trick just so that we can get past the crawling... which is basically a phase where your kid becomes a human vacuum cleaner/Lego detector.

Between the family bleeding history (namely all the hemos are covered in bruises all the time) and the shaky standing/walking... we have made the decision to start Harris on a "step wise" prophy protocol. (Prophy is short for prophylaxis... which is short for "we're going to just go ahead and put that clotting factor back in your veins before you start that pesky bleeding.")  He will go into the center once a week to get his factor on... until he needs to go in twice a week to get his factor on... until he needs to go in three times a week to get his factor on.  (Factor is - in our case- clotting factor 8. Which is what Harris' blood does not have, but what his medicine does.)

It might be the first long-term plan I've ever had.

For me this decision came down to one basic question.  Is Harris' risk of developing an inhibitor (an auto immune response that is sort of like having an immunity to the clotting protein), by starting prophy early greater than the risk of him sustaining irreversible joint damage by putting it off?  We're going with "protect the joints".  The research study we discussed with our hematologist shows that the children (who they followed from birth to age 6) who only infuse when there is an "episode" already showed signs of joint damage at age 6.  Even kids who never had a documented joint bleed followed this pattern.  The kids who infused with prophy - no joint damage at age 6.  Unfortunately, the study did not ask or answer the questions "when is the best time to start prophy" and "what is the best way to administer prophy treatments".

We know, based on family history and genotyping that Harris has a low risk of developing an inhibitor.  We also know, based on watching him  fall on his bum, knees, head, elbows, hands and wrists that he does not have a low risk for developing a joint bleed.  I also know what joint damage looks like (courtesy of my father) and it ain't pretty people.

SO... this week we are putting on the power of prophy... if they can find a vein in Harris', um, "healthy" little arms.  Which is another post all together.

PS - Here's what Harris thinks of all this...

Sunday, May 9, 2010

To call or not to call...

One of the things that is still slightly hazy for me is exactly what kinds of injuries should have me rushing to the phone in search of help... or an appointment at our treatment center.

They keep telling me "we don't infuse for bruises", but when I took Harris up there last week because his forearm looked like a Popeye after a bar fight, I heard "yeah, this is bad... wow, he's really bruised up... we might be looking at prophy a lot sooner than we thought".


Monday, May 3, 2010


Zander took a door to his face on Friday. The door won. He got an IV in his hand and four infusions later he looks a little less like a Klingon and more like a boy. This picture was from Saturday as of today he is sporting the starts of two black eyes!