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.

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...

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".

Hmmm.

Klingon

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!

Bloody paperwork

Even though infusions can be stressful (especially the first few), you need to remember to keep a record of each one.  The information that is recorded on these logs can be an invaluable tool for dealing with insurance companies, doctors, and factor producers, as well as tracking bleed patterns. (Or so I'm told).


You can find all different kinds of infusion logs online.  Here are examples from Coram and Hemophilia Navigator.  I actually have no idea who the heck either of those are, but they came up high in the google search... so there you go.  I actually do like the format of both of these forms.  I think that these are designed to be printed out.


I decided to keep both paper and online infusion logs.  I did a spreadsheet in gmail (free and easy) because it is web based and I can access it from any computer, not just my own. ( I'm keeping paper ones as well, because I'm pretty sure that some kind of meteorite or the swine flu or Al Gore will eventually kill the internet.)  


On my sister's advice, along with all pertinent information about the infusion, I also included insurance information on my spreadsheet.  (Cost, amount covered by insurance, amount owed, etc.)  Apparently, some insurance companies get all uppity about paying their bills.


You can also get free infusion logs from makers of factor products and all of the associated gear.  Ask your local treatment center if there's any good stuff that they've got lying around or if there are any resources that might be available.  I have a really cool little one that comes in it's own little spiral binder... now if only I can find that thing.

Bloody Bling

It's hard to get a medicalert bracelet around a cankle, but it can be done.  Oh yes, it can be done.

Bleed Shmeed also known as I'm Not Afraid Of No Stinkin' Needles also known as the First Infusion

This is what happens when you think that it looks like fun to scale the leg of a piano at your church, but then you think "hey, I'm 6 months old.  I'm pretty sure I'm not supposed to try the whole walking thing yet."  Then, because your confidence has been shaken, you lose your grip and try to spin around like a tortoise flipped onto his back, but instead you land on the foot/leg/edge of the piano... and you think to yourself  "well at least I led with my head... oh wait... umm... a little ice here?"


Then, because emergency rooms are good times on Wednesday evenings, and because you don't fancy walking around with what appears to be half a $.25 gumball sticking out of your forehead... you book yourself a room on over there for your first infusion.

Nurse... nurse... doctor.... nurse.... insurance checker lady... nurse...nurse... needle.  (You knew she looked like trouble... with her purple gloves and beeping thermometers and fancy "oh, you're so cute" talk...)

It's all okay though...because your mom is there, with the singing and the whispering in your ear and her boob exposed to half of OHSU because she really believes that anything can be cured if you just put a little breast milk on it ... and if she can nurse on an airplane while sitting between a lesbian whose partner is expecting their first child and is super chatty about it and a 275 pound man who clearly didn't know that that's what those things are for... then she can nurse you on the exam table while being pumped full of clotting factor... especially since she's been saying over and over "This is not scary.  I can do this" all the way from your house to the hospital... where you got the medicine that makes your head look like this... which is a lot better then how it looked when you got there.


So it's all good ...but all the same... if you ever see that nurse again... you're gonna give her one of these...