Monday, February 21, 2011


Zander is very used to doing his infusions, although he does yell, "no neenle, no neenle (needle)" before he is poked. This was a particularly good infusion day. He stayed like this the entire time.

Thursday, February 17, 2011

Low on bricks... part 2

So ... we went in for another platelet count.   Totally recovered.  Harris is a total faker.

Sunday, February 13, 2011

Low on bricks...

At our first ever appointment with Harris' hematologist we had a short lesson on how a blood clot is formed.  Dr. R- (who is the best doctor of all time... except my mom, who is not strictly speaking a "doctor", but the smart money goes on whatever she says you've got when you're sick) - explained the clotting process as being similar to building a brick wall.  First you get yourself some bricks (platelettes) and build yourself a wall. Then something else happens (can't really remember that step), and finally your clotting factor comes in and mortars the whole thing together.  Step three has always been a bit of a problem for us (no mortar), but steps one and two were all good... until now.

Harris went in for one of his comprehensive visits last week. These are like 3 hour health care fiestas where we see the doctor, our nurses, the physical therapist, the dentist (that one was super fun) and the people in charge of data collection for whatever research etc that's going on.  They also run labs on Harris' blood to test for inhibitors and diseases and other bloody stuff.  I talked to them about how Harris has been bruising a lot more lately and we got a prescription for some anti-bacterial bum cream because he also has had some kind of angry and raging infection that my pediatrician thought was yeast, but that I was convinced was strep.  (Pediatrician - 0, Me- 1).

The next day I got a message from Dr.R, asking me to call him back... and have him paged.  (Note: having the head of pediatric hematology tell you to have him paged - never a good sign.)  Harris had some weird results in his blood work and they wanted me to bring him back in for another go.  One blood draw and a few hours later, we found out that Harris has developed another blood disorder (totally unrelated to his hemophilia) called ITP (Idiopathic thrombocytopenic purpura). (Hence the increased bruising). This is an autoimmune disease that shows up after an infection, illness or vaccination. The body's immune system is all pumped up to fight the infection but starts identifying its own platelets as foreign invaders and destroys them... sort of like those movies where the super helpful computer or monster or whatever turns on its maker and tries to take him out.

The good news- 
1.Harris's bone marrow is making new and plump platelets, so we know that this is not a bone marrow issue.
2. In 90% of the kids that develop this, it spontaneously resolves itself and never comes back.  It might take a few months, but we are hoping that Harris falls into this category.
3. We have a good treatment plan in place to make sure that he doesn't have any bleeding episodes due to the fact that we now have no mortar and we're running a little low on bricks.  Clear up that pesky infection, give more factor and we should be good. (I am double crossing my fingers behind my back).

We are ok with this, and actually find it kind of funny.  (Not funny "ha ha", but funny "sort of ha ha").  I figure that if the timing had not worked out like it did, we may have never even known about this at all- it would've come and gone and never been detected, so it doesn't seem like anything to get too fussed about.  I am glad though, that since we do know, we have such great support and access to medical care. Our little man is super lucky.  I wish that all kids with chronic issues were as lucky as he is... but that is another post all together.

ps- a warning from Dr.R... "the stuff about ITP on the internet is scarier than the stuff about hemophilia, so don't freak out"  (I paraphrased the "don't freak out" part ) ... of course this meant that I went straight to the web and read a couple of research papers for good information... and then some crazy stories that included the words cancer, inter-cranial bleeding and purple skin.  Curse you Al Gore and your internet.

Friday, February 4, 2011

sneaky bleeder

The last few days Zander has been having an issue with his left knee. Not all the time, just when he kneels down. The first time I noticed it, I didn't really think much of it. But then I saw a pattern, he kneels down and the second his knee hits the ground, he would cry and when he stands up he would limp away. But within seconds he was fine again, playing, running, no limping. We started to infuse him, a couple times a day. But after three days, he is still crying when he kneels down.
This is the first time I haven't been able to figure out what is wrong with him. He has no visible bruising (no more than usual) on his knee, it isn't swollen, hot, and he still has perfect range of motion. I broke down today and called the HTC. Basically, our fantastic nurse said we are doing everything right. We should infuse today and again tomorrow (which is a normal prophy day). If he is still having an issue we will go up to the center on Monday. She said it might be a micro-bleed into his joint...but who knows.

Time and time again Zander shows us that he likes to do things just a little different, he can't seem to just get a "normal" bleed. We love him. Hope we get whatever this is under control.