I am back!

It has been a long time since Endre or I have written on this blog. Life gets busy. But recent events (I'll get to it) caused me to reflect on the boys early experience with hemophilia.

Zander is now six, he is starting the first grade soon. I am still amazed at how fast this time has gone. Zander hasn't had any major issues at school (pre-k and kinder) and I anticipate we will continue on that same path. He attends a small charter school and the teachers are aware of his bleeding disorder and they called me a couple of times last year to ask questions but I never had to go to the school for infusions.

Zander is the same as he was when he was little (because six is big??), he still bleeds a lot. His port really is our life saver. He gets break through bleeds all the time (at least a couple times a month, sometimes more, never less) It still takes us a couple double doses a couple times a day for a couple days to get bleeds under control. This is our normal. Usually, at his yearly comprehensive visit, we up his dose. Sometimes we see that higher dose cut down on the amount of spontaneous bleeds, but that is like a honeymoon phase and it ends quickly, then we are back to lots of bleeds.

Zander takes this all in stride. He doesn't complain (about needing infusions), he knows when he is having a bleed, as I looked back on the blog, I was amazed that at age 2 1/2  he was asking for a needle day. This is Zander's normal.

Much to our surprise at our comprehensive appointment earlier this week, I saw one of Zander's original nurses, Cyndi. She was there when Zander started prophy (9 months old) she was there when he was diagnosed with his inhibitor, when he bled out during his PICC line placement, when he had his port placed, she removed that same PICC line. She taught us to use his port and was a huge part of our early journey with hemophilia. I was so sad to see her go, although happy she was having a baby and she was able to be at home with her daughter. At that early stage we needed strong nurses, we needed these women who helped us navigate our way. Then, after Zander had his port, things changed. We didn't need to go to the center, except one time a year for a comp visit. We didn't call and ask questions. We just did what we needed to. Needless to say when I saw Cyndi, I was confused and then excited and then really excited. She looked through my list of Zander's bleeds for the year and commented about how he is still bleeding the same as he did as a baby. She was not happy with this. She was not willing to accept this as normal. She had several ideas, first of which was to do a recovery study to see how well his factor was working. She didn't just want to increase his dose, she wanted answers. I can't say it enough, I love her. I didn't realize I had become so complacent about Zander's ability to bleed. I really had accepted it as life, I know which activities will cause bleeds (the beach without shoes), again this has always been Zander's pattern of bleeding.

We are going in next week to do a long recover study, longer than we have ever done. Our last recovery study was 24 hours. We saw that Zander, as he always has, metabolizes his factor really fast. Interestingly enough, when they did a blood draw this week, she ran a level on his factor (which we gave between 3-4 hours earlier) he was at just 49 percent. We were both amazed at how low he was. So, with the recovery study we will see if his 100 percent dose is even getting him up to 100 percent. We will see how long he is protected (I was always amazed he could get a bleed on a prophy day, but it happens not infrequently), we will run another inhibitor test. Then after the 36 hour recovery study, we will discuss our next steps. Do we want to switch products, do we want to use a plasma based product (research shows it is more effective in children).

I am excited to get answers. I am excited Cyndi pushed me to not accept his bleeding as normal. We can do better. Zander deserves better.  I will post updates as we receive them, Even if no one reads the blog, I am glad we have it. I was able to go back and read about Zander's early journey and see that he really has had a pattern that he has always followed. It is good to write things down. I am glad that I keep records as closely as I do. I can track bleeds and compare old records with current records. I am glad that even though Zander does every other day prophy and a lot of infusing in between, that he doesn't know it isn't normal. He is happy. He is healthy. He is "all boy." It takes a lot to slow him down, we learned recently that he can even go pretty fast on crutches.

walk shirts

Endre, mother to Harris is VERY talented. We had a new name for our hemo walk team this year and she came up with this idea....LOVE IT! What do you think? There is still time to sponsor us!

The Clot Thickens.

This is just our second year having a hemophilia walk in Oregon and we are getting our walking shoes ready to go. We will be walking June 28th. I made a video this year of Zander and Harris (ages five and four) talking about what hemophilia means to them. You can also see they are very typical boys, they have likes and dislikes and one thing is for sure, hemophilia doesn't define who they are. It isn't who they are, it is just one part of what makes them, them. If you can donate that would be great CLICK HERE. Enjoy the video.

First set of stitches

Notice how I said first, I am sure we will be back for more (knowing Zander).

A couple weeks ago we did a quick trip to Elko, Nevada for my grandma's funeral. After driving all day it was finally time to relax, wishful thinking.

Zander and Ayda were chasing each other around and Zander ran down the hall, giant bouncy ball in tow, he ran smack into the partially opened bathroom door and split his forehead open. Travis grabbed Zander and I went to get his factor ready and that is when I saw the blood. A lot of blood. It was the first time Zander has ever had an injury like that. It took just a second to wrap my head around the fact that I wouldn't be able to just infuse and be done. We did a double dose of factor and we were off to the OHSU emergency room.

I have said it before and I will say it again, an ER with valet parking is pretty good to me. By the time we got to the ER Zander was pretty calm. We still had a bandage on his head and an ice pack. We went back and Zander talked everyone's ear off, never leaving one detail out of how he hurt his head. Zander has only been to the ER once before (when he was one...pre-port) and now that he is old enough to understand I talked to him about everything they were doing. I told him they would put numbing cream in his cut and it is just like the cream we use on his port. When they asked if we wanted to do nasal versed or nitrous to keep him calm during the procedure, I asked Zander what he wanted. I am not sure if the nurse thought I was nuts but I figure it is his body and his choice.

Although it was pretty funny to hear his reasoning for choosing the nitrous. I explained the versed would be sprayed up his nose, he launched into a story about how he picks his nose and how it is okay to pick your nose but you shouldn't put other things in your nose....so he picked the nitrous because he would get to wear a mask and superhero's wear a mask. I laughed.

When they went to clean out the wound the doctor was telling him he needed to spray it with special liquid, to which I said, he is cleaning it with saline, just like we put saline into your port. The doctor told him he couldn't touch his special cloth, I simply said that is the doctor's sterile field. He knew not to touch it.  The nurse commented how well I did explaining stuff to Zander, I guess there is no need to talk to him like a child when he knows as much as he does about different medical terminology.

The stitches went in, five or six in total. Zander did great. He rocked an awesome black eye and has a cool little scar.

Come one come all...walk for hemophilia with us!

This is the first time our Hemophilia Foundation has sponsored a walk and we are excited to participate. We are always on board with any chance to raise awareness about hemophilia. Here is a little video we made about our bleeder crew!! We really do have a bloody good life. If you have any questions about our bloody good disease let us know and we can answer them.  If you are able please click the link below and sponsor our team!!

Click here and then click "sponsor this team"...

Alive and kicking

We are alive over here at The Bloody Good Life!! Here are a couple pictures of the world's cutest hemo's.
Zander is now doing a pretty large dose of Advate every other day to help keep his joint bleeds under control. Nothing slows either of them down!!

Swimming is good for the joints!

He is a master at holding his breath!

Hemo pull...

The next generation of hemo's (Jack, Zander and Harris)

Jack was a good sport to run the boys down the hill....many times.

...they'll never take our FREEDOM!

 

Love this picture!

Zander and Harris, they could be the poster children for hemo cowboys!

The upside... aka - free stuff

It's that time of year again. That's right people, spring means - FAFSA and scholarship season.  If you will be attending a college or training program, or have a child, sibling, etc, etc. who will be attending a college or training program in 2012-2013 you must check out the scholarship page on the HFA's website.  There are funding resources available for many types of education and all types of people within the bleeding disorders community including care givers, siblings and carriers.

Education is the key to expanding life choices.  Visit the link above to explore some ways to help finance your's or your child's schooling.

A needle day song...

Zander doesn't mind getting his needle day and I wish I could say it is solely because it makes his body feel so much better. But sometimes it is just because he wants to watch a show. I know this because sometimes he wants to watch a show and I say no, then he sneakily responds, "Oh, ow, I need a needle..." I can't remember what started this song, but it cracked me up.

In the calm of night...

I just finished reading a book, "Dr. Guilt?" (about many families from Oregon, affected with hemophilia, good read, made me cry a lot). I read something in the book that made me feel like I am not crazy when it comes to Zander's bleeds. He will be active all day (boarder line crazy) and when we get in bed and he falls asleep, he will wake up screaming and crying and holding which ever body part is hurting (usually his right knee). He tells us he needs his needle day. A couple of the boys in the book had this same thing happen. Apparently, sometimes being up and going you feel fine, but when your body relaxes, the bleeding into the joint can be felt. I know this wont happen to everyone, but it is good to know Zander isn't alone.

One of these kids is not like the other

Here are our family twins.  One is a hemo, one is not.  Time for our favorite game (actually, I just made it up) - Spot The Bleeder.

Box on boys.  Box on.

ps- I love this picture of my nephews.  I found it on my sister's facebook page... and stole it... and reposted it on our site.  Is that stalking?

"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.