"I need a needle"

Those four magical words are not ones I expected my 2 1/2 year old to say. Zander woke up earlier in the day complaining about his knee hurting, I thought he napped on it funny but later that night he started to cry (randomly). He was grabbing his knee and he came over to me and said, "I need a needle!" I was very surprised: this was a first. We have had other times where I ask him, "Do you need a needle?" Sometimes he says yes and sometimes he says no. But he was adamant that he needed a needle. So, we infused him. He seemed okay and went to bed a happy camper. There were no issues to speak of the next day. I am not sure if he was having a bleed, I am not sure if he "needed" factor, but we have it and we can give it. I think it is important to show him that this is his body and his disease and if he felt like he needed a needle, then by all means a needle he will have.

Prophy (we're back)

Zander is back! It is funny as a result of his downward spiral with prophy we started some new traditions and some have stuck (ha ha no pun intended) and some have gone away as he is just laying there again. Here is a little video of one thing we do each time we infuse...it makes me smile. Let me clarify a couple of things, when I ask, "What are you going to do with your hands, legs and mouth?"...he shows me what he is NOT going to do. My favorite is what he is not going to do with his mouth...

One Anniversary and One Happy Birthday

In September we passed the one year mark for Harris' port.  A few weeks later he turned two.  The weekly trips to the HTC and the multiple needle pokes required to pin down a sneaky vein seem to be a very distant former life.  Getting the port placed in Harris' chest was undoubtedly the best decision for our family and I have been very pleased with the results.  We have experienced no break through bleeding and no joint bleeds since starting our regular prophy schedule.

One of the goals that my husband and I have is to encourage Harris' hemophilia to be a peripheral part of his life, rather than the defining center. The freedom that we and our other children have gained by being able to infuse him anywhere at anytime and with very little fuss allows us to integrate a once-problematic bleeding disorder into our lives just like other somewhat inconvenient things like soccer practices and sixth grade band concerts (seriously- who knew there could be so many different notes in "Hot Cross Buns"?)

While prophy might not be the choice that feels right for everyone, we are sure that it is the right one for us... unless you ask Harris.  His new favorite phrase - "I'm not infusey day".  Sorry buddy.  I'm pretty sure you'll thank us later.

prophy...ebb and flow

I have posted pictures of Zander in the past, just laying still while getting his prophy (or poke day/needle day as he calls it). I'm not sure if it was a mix of no longer using the numbing cream (his choice) or when we went on vacation it just through him out of his routine. But he, for the first time, struggled and fought so much with his infusions. It was a nightmare. But, we might be turning another corner (he wants cream again) and he also wants to infuse something before we infuse him. Today's patient of choice was his Woody doll, he is very serious about getting his gloves on and getting his patient his factor. He is having a bleed of some kind right now and he has been back to his better days (I keep telling myself maybe he knows it is helping him...) but it has been nice with having to poke him a couple times a day. Other things I tried (other than letting him infuse toys) to get him back to not fighting, showed him lots of pictures of him being infused and how still and happy he was, talk to him before I infuse about what I need him to do to help me make it an easy poke day (hands where they aren't over his port, legs not kicking, not screaming (although the screaming I could handle). Hope it keeps getting better.

Hemo Survey.

Who says being a hemo can't make you a few bucks? I saw a link posted on NHF's website, if you participate in the survey they send you a 75 dollar gift card. It took me maybe 35 minutes to do. They are only taking 200 participants so act fast!

Boys with arrows ... what could go wrong?

For those of you who have no family history of hemophilia, your frame of reference is a bit different than mine.  Butterfly needles, factor in the fridge and being my biology teacher's favorite example of X-linked traits were the same as shoes and socks and homework.  I have an advantage because I have seen what hemophilia was and what it is now.  (PS- it used to be lots worse).

I was reminded again of how life with this condition has changed when I read a blog post from a man named Guy Boss - it was published in HemAware online a couple of days ago.  Guy is older than my dad by a little bit but they are in the same generational cohort as far as bleeders go.  He writes a lot about his childhood and his parents efforts to raise two hemophiliac sons in a time when you didn't exactly pop a needle into your kid's port and call it good.

In this particular post he talks about wanting to be a cub scout.  After much anticipation and confusion about why the other boys were hauling their super rad scout uniforms on over to their den meetings, he  found out that he was not being allowed to join in the scouting fun.  The scout master gave many reasons, none of which I can repeat because I don't say things like that - even in quotes.

Fast forward the hemo time clock 60 or so years and there I stand (as a chaperone) with my son Henderson (not a hemo), my nephew Jack (hemo) and his twin Grady (not a hemo) at cub scout day camp... where they encourage 8 year old boys to shoot real arrows and real b.b. guns under the careful supervision of ... 16 year old boys.  There is hiking and full-contact-capture-the-flag and lots of "boy humor" the primary focus of which is underwear, butts and poop.  Clearly, this is what goes on when girls (and moms) are not around.  All of our scouts had a whole week's worth of scouting fun... a tiny act of cosmic redemption for my father, Guy and every man of their generation.  It is a tender mercy to be able to offer these experience to all my sons and I breathe a sigh of relief that I am me and not my grandmother when it comes to caring for our little (and not so little anymore) hemos.

*The picture at the top is Jack on "the jumping rock".  I did not take pictures at cub camp... I was too busy covering my eyes.

FYI: Factor Recall

Hey, saw this on Hemophilia.org site. Thought I would post it just in case it affected anyone out there that reads on our site.

Medical Advisory #413: Baxter Voluntarily Recalls One Lot of Recombinate Baxter BioScience has announced that it is voluntarily recalling one lot of Recombinate Antihemophilic Factor (Recombinant). Baxter states that this recall is being taken as a precautionary measure after a retrospective review of its manufacturing process revealed a breach in aseptic processing. This lot did meet all in-process and final container specifications, which include sterility and pyrogenicity. There have been no adverse events reported regarding use of this lot of Recombinate to date. This action is being taken with the knowledge of the U.S. Food and Drug Administration (FDA).

Recalled Lot: Lot Number – TRA09834AB
AHF IU/vial – 1060
Expiration Date 1-28-2012

If you have any of this product in your possession, please contact Baxter Customer Service at: 1-800-423-2090 for instructions on how to return the product and obtain a replacement.

PHYSICIANS: Please distribute this information to all providers in your area who treat patients with hemophilia.

CHAPTERS: Please distribute this information to your membership.

Please sign up for the Patient Notification System (PNS) to be notified directly about the latest recall or withdrawal of recombinant and plasma products. The System is confidential and time sensitive. It is administered by an independent third-party organization and is free of charge.
To enroll in the PNS, please call (888) UPDATE-U or go online at http://www.patientnotificationsystem.org.
This material is provided for your general information only. NHF does not give medical advice or engage in the practice of medicine. NHF under no circumstances recommends treatment for specific individuals and in all cases recommends that you consult your physician or local hemophilia treatment center before pursuing any course of treatment.

high flyin' hemo

Zander is wondering if being a trapeze artist is on the "do not participate in list" for Hemos...If so he may just have to break the rules, look at his form, his technique, his smile. He is a thrill seeker!

You Want Grady to do What?

So I have made the executive decision that Grady (Twin brother to Jack) will learn how to infuse him this summer...yes that's right I am giving permission for Jack's brother to to stick him with a needle! Jack thinks I am totally nuts...and maybe I am, but after the Valentines Day fiasco I need to make sure someone has Jack's back. Jack isn't too keen on Grady trying the needle, so we are starting slow for now. Grady set up the kit, and pushed the syringes. Jack's expression in the picture say's it all. I think Grady knowing how to infuse Jack will bring everyone more piece of mind. These boy's are young....but they are mighty!!

Get yourself a girl

I knew a woman several years ago, who came from an affluent family in Peru.  She always used to tell me that I needed a girl for stuff (laundry, housekeeping etc).  Apparently "sexism" means something different in Peru than in the US.  However... sometimes, when I'm feeling overwhelmed with the many tasks of motherhood, I do find myself muttering "I need a girl".

Which brings me to - our daughter, who is deep in tweenhood and quickly approaching teenhood.  She is taking on more and more responsibilities in our house (although she is certainly not utilized as a domestic servant- although she might disagree) and this week, she helped me do Harris' infusion.  She held, I stuck.  She did really well - especially considering the weight to length ratio of her brother.  I am so glad that we have another helper around and I am so glad that our kids can be raised with such a broad idea of what makes a normal life... especially since she has a chance of having a little bleeder of her own one day.

He's a big boy now

Yesterday morning I had to leave the house early and didn't get back until the afternoon. Travis needed to go to work when I got home. We were just going to infuse Zander that evening but I kept having this nagging feeling that I should just get it done. So, I braved the infusion process alone.

Zander is really good to just lay there but I worried a little about him reaching into my sterile field. I told him that I would turn on Buzz (Toy Story--his current obsession) and explained he needed to keep his hands behind his head. I got my sterile field ready, laid him down, put my gloves on (that is the point of no return) and did it. He didn't move, fuss or try to run away.

I felt very proud of him (and me). This is really great because it is an added measure of freedom. I don't have to worry about Zander having an incident and needing another set of hands to infuse. Yay for MORE independence.

Is this a sign of obesity?

Does anyone out there ever look in those celebrity magazines, they have the sections labeled something like "stars are just like us" then show the celebs taking out their trash or walking a dog. Well this video could be labeled, "hemos they are just like us." Oh, and I don't read those magazines either, I have just heard about them from a friend.

ps-If Zander continues to eat in his sleep, we will need to make sure he gets some extra play time so we can avoid having a "hearty" kid with bad joints.

Sometimes you have to be the giver

I love me a good mom catch phrase.  I am certain that at some point it was fun and games until someone got their eye put out, that someone's face did stick like that, and that some fool actually did jump off a cliff because their friend did it first.  When my kids tell me I'm the meanest mom ever, I say "Good.  I love being the best at stuff".  Also, I think I'm going to teach them that the Constitution of the United States has a "because I said so" clause.

One saying I use with my kids a lot is "sometimes you have to be the giver."  I made that one up myself and it is good ammo when someone in the family is whining about doing something that doesn't make them the center of the world.  I always want our children to realize that in relationships and in life, sometimes, you have to be the one who helps meet another's needs.  I think this is especially true when families deal with conditions like bleeding disorders that can easily ooze over every part of life sucking attention continually toward hemophilia.

I want my kids to know that whether a person has been dealt a lousy hand or a great one, the obligation to serve others is the same.  My son is not exempt from giving to others just because others will spend time giving to him.  I want him and his siblings to know that there are lots of people in the world, with lots of different problems.  Some problems you can see in bruises, some you cannot.  Our problems are not more significant than those dealt with by the billions of other people that live on this planet.

That is why every year, we help raise funds for the Cystic Fibrosis Foundation.  I have two nieces with this disease and I want a cure.  We also participate in the annual CF walk with our family and friends to support the foundation and our girls... and because the walk begins and ends at an amusement park, there's never any complaining about being the giver.

Warming up for the big walk

Our family baby... diagnosed with CF just after birth

Harris getting bossed

Harris with cousin Arden (diagnosed with CF at 4 months) and her soccer team

Harris and Derek along with uncle Matt and cousin Maisy on
"Big Pink"  (I have been lobbying for a name change for
that slide for 4 years now)

... post Big Pink

Harris and Henderson piloting their plane in opposite directions

The Frog Hopper part 1... yep, my kid's wearing shorts and rainboots.  Classy.

Harris and Zander cruisin' in their sparkly ride...

...with a chaperone.

Frogger part 2... 

Zander remains unimpressed

The Frog Hopper story... way better than the actual Frog Hopper

Hmmm... does this mirror make my head look big?

brothers on a coaster... in the front of course

Walk the plank

I was in the kitchen when I heard my 8 year old son say "OK.  Walk the plank Harris". Huh? (Clearly, this is what the inventor of pillows intended...)

The plank... (aka- the chair mom's been about to reupholster for 5 years.)

Man overboard

... and again

and again (nobody panic, Hurley did not land on the baby)

look out below...

...a dive with a twist.

why are my hands as big as my brother's face?

I am your captain.

Happy World Hemophilia Day

I am not sure how to celebrate today, it never came in my Hemo handbook when Zander was born. But to all you out there...Happy World Hemophilia Day!! Don't get a bleed and party it up! Zander suggests celebrating with ice cream! (ps this pic is a couple weeks old...his bruise is looking much better now)

bloody lips and other mouth bleeds

When Zander started walking his hematologist prescribed him Amicar. We had Amicar before we had factor. Amicar is for mouth bleeds. I have had that bottle sitting in the fridge (no it doesn't have to be refrigerated but I choose too) and used it for the first time Saturday. We were at the park and he hit his mouth while climbing. We used the Amicar and it seemed to work pretty well. That got me thinking....what is it that makes the Amicar work. It isn't factor so what stops the bleeding. I got on the Internet and found the answer to my question, I thought it was interesting so I thought I would post. Amicar or aminocaproic acid is an important medication for anyone with a bleeding disorder, it works by preventing enzymes in the saliva from breaking down a newly formed clot. The medication allows the tissue under the clot to heal and prevents re-bleeding. *Info from ihtc.org

Not all bleeders are created equal

Since Zander's amazing technicolor dream face has been having such a fine run on our blog, I thought I'd throw a couple of auntie observations in there... because that's who I am.  (PS - my momtuition tells me we never really got the real story about what went on between the two 5 year olds, the 2 year old and the trusty retro rocket express).

You would think that since Nika and I have the same mother and father, and therefore, carry the same versions of the bleeder gene, that our children (whose genetic soup also has that same version of the bleeder gene) would have similar bleeding patterns.  Not so much.  Not long after Zander's wild ride, Harris pulled a dining room chair over on his head. Nothing.  
... Today I was changing his shirt  because it was covered in peanut butter and we were going into George's Giant Hamburgers (they got me successfully through 3 of my 4 pregnancies) and I was too lazy to unbutton that last tiny little button on the collar of his polo... which meant his head barely made it through... which meant he had to sort of snap his head forward to break free of his peanut butter prison.  Smacked his head hard, right on the little stair step thing on the side of the car.  Nothing.
...Fell in the shower because he thinks he needs to wash his stinky little toes with his dad's man soap while standing on one foot instead of just sitting and making a mess with the water like a normal toddler. Nothing.
... Tackled continuously.  I mean continuously people, by his 5 year old brother.  Nothing.
We do prophy 3 times a week and have had almost no break through bleeding (that we know of) in over a year, even with the bumps and bruises of toddlerhood.

My point is this - no two bleeders are the same, even when logic says they should be.  What works for one kid, may not work for another, even when their genes came from the same grandpa.  My sister does what she feels in her mom gut is best for her boy and I do what is best for mine... and we will both hide the retro rocket from now on.

record keeping

There are several good reasons to keep records of infusions and bleeding events. I felt like we were infusing a lot for this head bleed but looking back we are at about the same amount of infusions that we have done for other episodes like this. Without my records I wouldn't have known that. Here is a new picture of what our little bruiser looks like today.

ps-when events like this happen I cannot imagine trying to infuse him several times through a vein. I love his PORT!!

the retro rockets wild ride

You take a fun retro rockets like this

and the pushing power of your 4 year old sister, add in one door jam and this is what you end up with.

The Legend of Ol' Shale Tooth

For the last six month we have been waging a battle against tiny, little bacteria warriors for Harris' front teeth.  This week, Ol' Shale Tooth lost his battle... and his teeth.  Or at least a part of them.  After trying an alternative treatment to help stop the advancing decay (twice) and watching his teeth break apart piece by piece, we went to a new pediatric dentist and pulled out the big drill.  (Actually, I don't know if the drill was big or not, but it is really big in my mind).

Due to the extent of the decay and his age we chose to have him totally out for the procedure.  The in-office anesthesiologist did not feel comfortable treating him because of his hemophilia.  We ended up at one of the area  hospitals where our dentist has privileges and had the treatment done there.

Harris has had no complications.  We upped his infusions a bit and we're using Amicar (spelliing?), but other than being a little grouchy and clingy.  He seems all good.

Goodbye Ol' Shale Tooth... may you wander far away from us forever.

Pretty sick of me trying to get a cute picture of his teeth.

Chillin'

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.

Low on bricks... part 2

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

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.

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.