A few weeks ago Zander had hives. We were really tracking everything he ate to see what could be causing them. We started to notice a pattern, on the days we infused his hives would be worse. This concerned me, I looked at the side effects of his factor and hives is one of them. Then Endre pointed out to me maybe it is the cream we use to numb his port. So, for the first time we infused him without cream. When I stuck the needle in he seemed generally surprised and he fussed a little. That day he had no hives (the first time in a week). The next time we infused we used cream to see if the hives would come back (they didn't come back so maybe the hives were related to something else). Travis and I infused Zander with the cream and then a couple times without the cream. This morning when I infused him (no cream) he was frantic. He has NEVER acted that way with his infusions. So, it may not be right for everyone but for Zander we are going to keep using the cream. Maybe in a few months we will try again. I figure if it makes it more comfortable for him then we need to do that for him.
(the picture has nothing to do with the post. It makes me smile and that is what Zander is doing... pretty bad right).
PS... This is Endre and I am adding to Nika's post without her knowledge. I just want to point out how amazingly bruise free he looks and how great his port is healing. That's it...
My baby has cavities. There. I said it. 4 cavities right in the front of his mouth.
Figuring out how to get them filled was stressful and I let it linger on longer than it needed to because dealing with the insurance company was overwhelming. We opted for a dental maintenance organization during our last open enrollment period assuming that the our family's dental needs were going to be fairly standard. The rates in a DMO are good, but the choice of dentists is very limited, and the choice of specialists is even more so.
The advice we had received was to fill the cavities without anesthesia. I'm no dentist, but that plan sounded like a pretty bad one to me. I insisted that due to my son's age and his bleeding disorder (which somehow was documented as a breathing disorder with our insurer) that he needed to be sedated. This required me to find a dentist with access to on site anesthesia services - again made difficult by the limited number of dentists covered by our plan.
I found a dentist with a contracted anesthesiologist... who wanted a $600 deposit before booking my appointment. I looked right at them and said "no." After some negotiation and the repeated use of my favorite phrase when dealing with people who want my money ("no") - I was able to get the insurance company to confirm with the doctor that because we had met our out of pocket maximum that they would indeed get paid. Then the anesthesiologist found out about the bleeding disorder and that, as they say, was a deal breaker.
So it was back to finding a pediatric dentist within my DMO who also had hospital privilege so that the anesthesia could be done in hospital (paid for by our medical insurance) and the cavities filled (paid for by our dental insurance). It was setting itself up to be a paperwork disaster. I was already planning my appeal. I was writing down people's names and everything. After much calling around and a 40 minute drive, I met with the new dentist (who is also a professor of pediatric dentistry at the local dental school which is located on the same campus as our treatment center.) She told me that she would love to fix my son's teeth ...without sedation... right then. Which is what happened and the whole "procedure" took about 5 minutes
for 4 teeth. It was a little stressful to watch, and I was totally on the alert for bleeding gums, but it was fine.
I learned something important from this. Fear doesn't solve any problems. When the first dentist told me that she was not going to sedate my son to fill his cavities, I panicked. I pictured them holding him down and hollowing out his tiny little teeth with something similar to a Black and Decker power drill. I was pretty sure that they would poke him multiple times on account of the flailing and of course there would be a lot of blood. Blood everywhere. Had I taken a breath and just asked some simple clarifying questions like "how often do you treat infants with these types of cavities?", "how do children typically react to having their cavities filled", "what exactly does filling a cavity entail", "how long will this procedure take" etc. I would've saved myself a lot of time, headache, cell phone minutes and gasoline money... and really, what if his mouth had bled? Hello? Port, factor, infusion... does any of this ring a bell? It would've been ok, is all I'm saying.
In the end the whole thing ended up being a big, fat, so what, and I have recommitted to keep my panic waiting in line until I've gathered the information I need to make appropriate care choices for my son.
Here is a video of me doing Zander's infusion. Of course, the day I set up the video camera (sorry of the constant shot of my leg, it was on a tripod) Zander was a little fussier than usual. Also, I needed to push my needle down a little more in order to get a blood return. We are getting more and more comfortable with the infusion process. Zander is a real trooper, even if we miss and have to try again he doesn't complain that much. Sorry, Awny did a better job of explaining what she was doing (I can't talk and infuse...give me a few years).
I was raised by a very strong mother, who taught me a magical phrase that seems to be helping me out a lot while dealing with Hemophilia. Repeat it with me, "Everyone has a boss and I want to talk to yours." I have adapted this a little to fit my situation, since Zander started receiving treatment I have written more letters than ever before. I never just write a letter complaining about service (although that is included in the letter) but I always offer suggestions on how to make the situation better. If you were to peak into my medical folder on my computer you would see a lot of saved documents that start this way, "letter re Zander's _____". I must admit this has seemed to work really well for me. I have had two bills paid that were originally denied. I have the pharmacy implementing a training on factor (or so they say...). I just thought I would share my mother's wisdom. If you aren't happy do something about it, make a call, write a letter, stand up for your child (self) and help make some changes.
Today was a BIG day for us, we accessed Zander's port for the first time. I was pretty nervous and my hands were shaking really bad but I took a couple of deep breaths and calmed myself down. I got my sterile field all ready to go, with all the necessary supplies. Travis helped Zander stay calm, he asked him lots of questions about who was giving him his factor. It was then time for the maiden voyage of the port. I poked the needle in and said, "I didn't get it in all the way." Our wonderful nurse C said, "it will stick up a little, just check for a blood return." And boom, just like that, blood. Blood is a good thing, it means the needle is in a good place and it is okay to give him his factor. And as quick as we started it was all over, I pulled the needle out and we stuck a band aid on. Zander cried a little but not that much. We will do two more times at the center and hopefully next week pull his picc out. Then one of our wonderful nurses will come to our house and make sure we are ready to do it at home, then we will be free!! The plan is for me to do one more try and then Travis will do a couple tries. We both want to be able to do it, especially once his picc is gone.
(here is the port, the redness will go away soon.)
Here is Zander waiting in the waiting area today, he pulled out the purple plastic couch and made himself at home.
Harris turned one a couple of weeks ago. I keep trying for a baby that will stay a baby, but they keep sending this fast growing variety. I am totally sending a complaint to their shipping department.
Derek and I were talking about how much our plans changed over the course of Harris' first 12 months. If you would have told me that by the time he turned one I would be infusing my baby on our coffee table three times a week, I would have asked you to hand over your keys.
It may seem counter intuitive, but I cannot express how lucky I think my son is. This morning I was able to access his port, infuse him to some of his favorite music while his dad held him and then pop him right into a warm bubble bath. His crying and escape attempts are subsiding substantially - he hardly fussed at all today, and I have every hope that the routine will be less and less dramatic with each infusion.
As I was wrapping him up in a big old bath towel, I thought about how amazed my father would be at the advances in care for those with this condition. If he were alive, my dad would've been 60 years old in May. There was not even an effective treatment when he was a child. He did not have the luxury of good joint health, or instant relief from the pain of an active bleed. In the end, the miracle treatment led to an early death. (Which I'm still a little pissed about.)
I do not have a memory of him that doesn't include a wheelchair (although I've seen pictures with him on crutches. It's a little weird to me.), but it has never crossed my mind that my son might someday lose the use of his legs due to joint damage. 60 years can make all the difference in the world, I guess.
I have learned that in hemophilia, and in life, the choices you get aren't always the choices you pictured yourself having to make. Sometimes that means choosing the least crummy of all the crummy options... and sometimes you can end up believing that the option that scared you the most has become the biggest blessing of your life.
I mentioned Zander had to stay an extra day in the hospital because his levels were all over the place. We went in today and they did another recovery study and his levels are still pretty low. We will stick with infusions three times a day. We are quickly learning Zander doesn't want to be like just any hemo, he wants to be in a bleeding class all his own! No one is sure why his numbers are so low, since he is getting a lot of factor. We may be fighting that stinking sneaky inhibitor (still....again?!?)
Zander is now a proud owner of a Port. His surgery went really well and on the outside Zander appeared to be perfect....but that is not what the labs were showing. Zander's factor levels were getting down pretty low, we started with doing factor every eight hours. When his levels stayed low it meant we had to stay an extra night in the hospital. We also went to doing factor every six hours, which meant he was getting about 2000 units of factor a day. To give you an idea of how much that is, our nephew Jack gets around 1500 units of factor for his prophy and he is eight years old. We aren't sure if this means Zander's sneaky inhibitor is still lurking around or if Zander just metabolizes factor that fast. When Zander's levels started being a little more consistent we were able to go home. We are still doing factor three times a day and will do another recovery study on Monday to see if we can drop that down to two.
The top picture is before his surgery (bright and early). This is Zander after his surgery.
Here Zander is in the push car, we put A LOT of miles on this car.
cheese!
Here he is making a break for it...
playing on the playground at the hospital (same day as his surgery).
As I typed that I laughed, port land...get it we live in Portland. Okay, tomorrow Zander goes in for his port surgery. I would be a big fat liar if I said I wasn't a little nervous. I know it will be okay just the thought lingers that something might go wrong. Endre keeps telling me that this is the best thing and I will just love Zander's port. I will update tomorrow...wish us luck.
Today we did our first solo infusion via Harris port. We did so good. Seriously. Derek was able to hold/restrain him by himself and I don't even flinch anymore when I stick him. (Kinda morbid, but there you have it.) Harris still cries when we hold him down, but he cries when I hold him down for a diaper change too, so...
Upon the advise of our hemo nurse, we played some of Harris' favorite music during the infusion to see if it would help him stay calm. Once we got the needle in and he realized that it was not so bad, he stopped crying and let the Nigerian rhythms fill him up. Mormon Tabernacle Choir:1 Harris:0.
Monday we're rocking it old school. Rad.
Good luck little Harris. May all of your infusions be fast and all of your factor be clean.
-Mom
I have conquered the Huber needle and infused my son for the first time today. It was better than last time. I put the numbing cream on at our house before we headed to the center. (I didn't have any of those big, clear bandage things that they use to hold the cream in place, so I used Saran Wrap and tape. Not pretty, but effective nonetheless.) It seems to have worked well. Harris cried the whole time that we had him pinned to the table, but he didn't cry any worse when I stuck him, so I call that victory.
I am, as always, so grateful for our amazing HTC team. Our staff is the best, best, best ever. Their support is the reason that this journey to independence is moving forward in a (mostly) smooth way.
After 2 weeks of healing today was the big maiden voyage for accessing Harris' port.
I want to do the infusions myself as soon as possible. I figure, I can learn now, or I can learn later, but either way, I've got to learn. I have been practicing my sterile technique and infusion skills at my house... on an orange. This is good practice. If I could've tracked down an orange that tries to put his tongue on the chloraprep swabby thing, touch my sterile field with his fat little fingers, scream, kick and buck like a chaffed donkey when I stuck it with the needle, I'd have been all set.
Too bad for me, my orange just layed there all quiet and unannoyed. Stupid orange.
One the upside... my sterile technique was quite good (if I do say so myself), but when I did the actual needle sticking part... well, that was the downside. Either I got it in the wrong place, or got the needle wonky, or didn't get it in far enough. but there was no blood return... which means we try again. We pulled the needle and started over, this time with the nurse holding the needle. We know now that the port works at least, and two sticks into a port is way better than 4 sticks in the arm, so we are still ahead in my opinion, but I am disappointed that I couldn't get it to work. I hate it when things are bumpier than I expected. Typically I am properly pessimistic enough to prepare me for the worst, but with Harris' hemophilia I can't help but be annoyingly optimistic. Maybe it's just a kinder form of denial. Oh well. Better luck next time.
So, it's at least another week with the picc line and two more trips to the center this week to perfect my technique.
On Monday we had a port placed in Harris' chest. We came to this decision gradually and jointly with our care team. I am a big believer in looking at the entire picture when deciding what type of medical care is appropriate at any given time. I believe this is true in all aspects of health care, not just hemophilia.
We considered a lot of different factors before settling on the port. Among other things...
1. Harris' body has the ability to get him to places that his mind doesn't know to warn him about... there's a fair amount of scaling of tables and surfing on rocking chairs at our house. Plus, with three older siblings there's a lot of keeping up to do... and that can be hard on the joints.
2. His "rubber ball" veins (ie- good veins that bounce around when you try to stick 'em). This makes for a very sweaty, hold-the-hemo/wrestlemania extravaganza, where I usually end up laying across my baby attempting to distract him with his favorite food source, (me), while he screams his bloody head off.
3. The potential for turning out bitter siblings who tell their therapists all about how their mom made them wait for hours in the hospital waiting room with a baggie of brownish apples, some stale pretzels, and the choice to either watch the waiting room movie and like it, or watch the waiting room movie and not like it.
4. To this girl, self-reliance is always better than dependence... even though we will miss seeing our nurses on a weekly basis. We really love them and they have become like members of our family.
After discussing the risks of the port, (infection, migration (another word for moving around), out growing it, clotting (are you kidding me?)), and the surgery (punctured lung - rareish, bleeding into the heart and chest cavity- really rare, oozing (another word for bleeding) - pretty common) we decided to go ahead.
The surgery was short, maybe 30 minutes and we got to go back into recovery with him while he was still coming out of the anesthesia. He didn't have any problems with the meds, but he was "a wild man" while leaving their influence, so the recovery nurse asked us to come be with him. I think the mark of an excellent care provider is knowing their limitations. I am sure that J. is an amazing recovery nurse, but she is not my baby's mom, and she realized that "mom" is what he needed. I have so much respect for doctors and nurses who ask for help when they need it, or say "I don't know" when they don't. They are really the ones I trust the most.
Our recovery has been surprisingly smooth. I expected Harris to be in a lot of pain, but with the help of appropriate medications, he was up and walking (sort of) and climbing (sort of) the same day and was off everything except Tylenol by the end of day 1.
We went in for post op on day 4 and he looked "the best that we have ever seen". Hardly any bruising, no oozing and the incision looks immaculate.
We are glad we made this decision and are keeping our fingers crossed that all goes smoothly from here on out.
So our school re-districted its boundries, which means we were moved to a new school...which means, educating a new principal, new teachers and a new bus drivers. I went in to this meeting very well prepared...not to pat myself on the back but I have done this before, like every year since Jack started pre-school. This year I went in with my typical handouts from our htc as well as my own instructions, definitions, and handouts. I like to educate with facts, and personal experience. I like the teachers to know that I want Jack to use his Hemophilia to his advantage, but not use it as an excuse or a crutch. The staff, to my surprise was very inquisitive and the principal and supporting staff, actually wanted to know how to infuse Jack. I was like great...I am sure there are some of you thinking scary Awny, very scary,...but I think about it like this. If the emergancy response team can't get there fast enough, and I can't get there fast enough, In a pinch....I would love to have the principal know how to give an infusion. If I had to give three tips on how to give a successfull school presentation, they are as follows:
1. Be prepared. Know you material, so you aren't reading off of a paper
2. Don't use medical jargin...we all know how smart you are...use everyday people language,,,its sticks to their brains more.
3. Keep it short! no one likes a blabber mouth....!
Jack - I love you, and I know it scares your teachers to death that I don't put limits on your activity...I just want you to love life, and enjoy it...you know your limits.....
Take an active role in getting your school educated about hemophilia. The nurses here in ohio will come to the school and give the information to the teachers directly....but, well, .... I like the way we do it and it works for us...do what ever works for you....
I recently received a hemoish question via email from one of our readers. She wanted to know how to help a family who was having a hard time finding daycare for their child with an inhibitor. Apparently no one in their area would take the child into their care.
Ok. First of all, mean. Second, ignorant.
Having gotten that out there- here is my advice: I have no idea what this family should do. I have not had to find childcare for my son (thankfully) and my sisters all stay home with their kids too, so this is definitely not my area of expertise.
However, I know that my 8 year old nephew (Jack - you'll know him from this blog) got a nosebleed on the bus the first day of school... which was a huge emergency... to the bus driver lady. Apparently, she pulled over and refused to take the kids to school until my sister came and retrieved her child. Too bad for her my sister doesn't like being bossed around by the likes of scaredy cat bus driver ladies.
In the end, my sister got her to take a breath and drive already, but the kids ended up being 45 minutes late to the first day of school... thus making my nephew a hemo hero to all those tardy souls who share his bus ride. (In the future, his plan is to carry lots of tissues and keep the bus driver in the dark. Wise.)
Having told that sort -of-but-not-really- related story, I actually do have one suggestion... which I stole from my other sister. I would call the Bleeding Disorders Legal Hotline and ask for some advice. I don't know if childcare is covered by the Americans with Disabilities Act, but it seems to me that refusing to enroll a child because of a medical condition that affects no one but him/her is a bit sketchy. The hotline is free and there is a link to it in the "bloody good helpers" section of our blog.
Here is the number, just in case.
Bleeding Disorders Legal Hotline: 1-800-520-6154
If anyone else has dealt with this and would like to share their experience or advice that actually feels like advice, feel free to leave a comment.
We got GREAT news today, Zander's inhibitor seems to be under control. His actual inhibitor level was undetectable. The recovery study showed his factor levels starting out at less than one percent (which means even with double doses of factor he is still burning through it fast) but after the thirty minutes his levels went up to 167 percent!! The team was hoping for at least 134 percent, look at us going above and beyond. So, the plan is now to drop down to regular doses (not two times the amount we should be taking) and go to maintenance therapy, which is three times a week (not everyday). We will also get to start talking about port placement, which I look forward to. Looks like we beat this inhibitor!!
After Harris' last tangle with his weekly infusion and subsequent bleeding episode in his upper arm (rubber tourniquet: 1, Harris: 0) we set the process in motion to have a port placed in his chest. The first step for us is the placement of a picc line.
The procedure went smoothly and reinforced to us how incredibly lucky we are to live where we live and have access to the kind of care that we do. Every nurse and doctor with whom we have worked has been stellar. The nurses in pediatric sedation and the piccu nurse were no exception. They demonstrated a lot of sensitivity for Harris' past experiences with people trying to hold down his arms and legs and along with the pediatric anesthesiologist came up with a plan that would be the least traumatizing for our son. I also appreciated that they listened to me when I told them that I was not a fan of the rubber tourniquet and that I did not want one used on my baby... ever again.
Derek and I were able to be with him as he went to sleep (with the help of the anesthesia - Derek actually held him and helped with the mask) and we were able to be with him when he woke up. He was hungry and thirsty as he had restrictions on when he could eat, nurse and drink before the procedure. I nursed him right on the table while they drew blood for his panels, infused him and dressed his picc line.
This is the first time that Derek has met Team Harris. I have taken the lead on his treatments and do the interfacing with our medical staff because I am comfortable working with doctors and nurses (a result of my mother and my training as a doula), I know this disease and its lingo, and I stay home with our kids. Derek works during the week and is therefore, not really available to go to our HTC like I am. I think that it was very helpful for him to be there with our son. It was good for his peace of mind as a father and as someone relatively new to dealing with hemophilia to see the procedure go well with no major complications like those experienced by my nephew a few weeks ago. I am glad that having a picc and then a port will mean more independence for our family, and will also allow Derek to be more involved with the workings of Harris' day to day care.
Now... which sock would you like to wear over your line today sir? ps - keep it clean, because it's sponge baths for the next 4 weeks buddy. Sad.
healing up
picc line dressing
What happens when he doesn't get what he wants... like the camera.
This post started out as an update on how Harris' prophy is shaping up. Here is the abridged version- if we are measuring how effective we've been at protecting his joints we get an A+. We are the valedictorians of joint protection. If we are measuring how effective we've been at protecting his quality of life, then basically we are the borderline-highschool-drop-out guy that Judd Nelson plays in "The Breakfast Club".
Here's what I learned this week:
If you are interested in being a perfect parent, sign up for a different health condition. I re-realized that I will not always know what is going on with my baby after he cried for about 6 sleepless hours because he was suffering a muscle bleed that I mistook for an ear infection. As you might imagine, the next morning's appointment with the pediatrician didn't do a whole lot to solve the problem of the arm that had (by that time) swollen to something that looked like it belonged to a sumo wrestler who is retaining water. (When I finally got a hold of my husband to tell him that I was headed in for what promised to be an awesome morning at the hospital... there were tears of guilt.)
I am so sorry for my Grandmother. It must have been so hard for her when her baby was suffering because of a bleed and there was not a thing she could do about it. No therapy to offer. No treatment. No doctor to ease her child's pain. I cried for her not being able to help her baby like I could help mine.
My sisters and I have chosen sides in our ongoing debate about whether hemophilia is a disease or a condition. I say that it's not a disease unless it can kill you. My sisters say that I'm full of it. Wikipedia agrees with my sisters. My hematologist ( who knows everything) agrees with me.
(Unless it's an emergency) a 4 stick infusion just ain't worth it. You'll probably cause more harm than good. (see bullet point number 1).
I hate rubber tourniquets. (see bullet point number 4). Seriously. I mean even my dad used velcro and that was back in the day. Maybe I'll have a prophy shirt made that says "Keep your rubbers to yourself".
As I was yelling into the speaker phone that was wedged underneath the shoulder strap of the seat belt so that I wouldn't get a ticket for talking on my cell, I thought how good it is to have sisters that you can call and tell what the treatment center said... so that they can tell you to call back and "make sure you ask for..." and "tell them you want..." and "have them find Dr. R". I can say without hesitation that it is the first time in my life that I did every single thing that my little sister told me to do. I think she likes bossing me.
Calling your treatment center to tell them that somewhere between the clinic and the pharmacy you've lost the home infusion supplies that they spent an hour assembling for you, does not inspire your medical team to have a whole lot of confidence in your ability to help your bleeder child. (In my defense, I had to walk a long way across that hospital campus dragging my 4 year old who had to sit through 3 hours of doctory stuff with an uncharged Game Boy/DS, had not eaten lunch and needed to stop into three bathrooms on the way.)
IV = sponge bath
Morphine is better than children's Tylenol.
Factor is my favorite. That stuff works really quickly. Harris went from not being able to bend his arm to put his shirt back on at the pediatrician's to playing with his dad and sleeping it off in just a few hours.
Plans change. We have decided that we are done poking around in chubby little hands... and arms and feet. We have ordered a consult with a surgeon and are having a port placed in Harris' chest that will give us 24/7 access until he gets old enough to learn how to self infuse via his veins. I am a bit nervous about the surgery, but I think that this is the reasonable course of action for our child. His picc line goes in next week and then the port after school starts and our schedule calms down a bit.
Here is our last couple of days... in pictures:
...after two infusions there are creases in his arm again
... swelling in right arm and shoulder (don't you want to kiss his belly?)
smiles again with dad
sleep. finally. (the swelling has not moved to his lips. he sucks his tongue.cute.)
I volunteered to be on an advisory committee for our local HTC. The committee's goal is to work with our HTC staff, and make sure our needs, as clients, are being met. I have not had any issues with our HTC, in fact, I feel like the doctor's, nurses and every other person at OHSU has bent over backwards for Zander's care. Here is one example, while Zander was going through the process of getting his picc line in and subsequently had other complications, Dr. R (who is not even Zander's hematologist) was on vacation but still was in contact regarding Zander's care. I don't know many people who are so invested in their clients to give up personal time to make sure their needs are met. I can't compare this center to others because this is all I know, but from what I gather, it is a pretty great HTC. I am not sure if there are any readers who use our center here in Portland, but if you have any suggestions I can pass along, don't be shy. Let's make this a great experience for our kids and for us!
I am writing this post about the opportunity Jack and I had to go to Berkley to visit the Bayer plant. We had some really great discussion on the first day, and we were able to meet alot of great people. I am always amazed at the wide range of experiences that other people have had, and continue to have with their hemophilia. I can't discuss the topics that were presented to us, because of waivers we had to sign, but I can talk about the tour we got to take through the plant.
Before i write about the tour i want to thank all of the Bayer reps that ran things and Meg who was with the westfield group who set everything up for us.
Jack was unable to attend the tour because they didn't have a gown small enough for him. You have to go through this crazy gowning process before you can enter the facility. This is to help protect the factor from us, not the other way around. The Reps from Bayer were great and gave jack his own private tour. I have some great pictures of him. Jack and I both learned so much during this process its hard to relay. There was so much information to take in, that at times it was overwhelming. I loved seeing how the factor was made. I never imagined how complex this process would be. I think i majored in the wrong thing in college. I am fascinated by the science of it all. We were able to see a bag of factor before it was concentrated. It looked like an IV bag filled with orange soda.
I challenged Jack to lean 3 new things the first day, and 3 the second day. He learned 5 the second day, and while not everything he learned had to deal with the process or hemophilia, i am glad he enjoyed himself. Everyone loved Jack. He did an amazing job sitting through these feedback sessions that were 3 and 1/2 hours plus. It was fun, and would love to attend more information sessions to increase our awareness and knowledge.
Just a quick update on where we are with Zander's inhibitor. When we did his last recovery study (take blood, test for factor levels, give factor, take blood 30 minutes later and check levels again) for a severe hemo his levels should be almost 70 percent but could be higher (that is good, considering he is less than one percent usually). Well, he was at 32 percent. Not great, but could be worse. His inhibitor level is really low but we learned that some inhibitors CANNOT be detected by the test they do. But, because his factor level tops out at a measly 32 percent tells us he still has an inhibitor. So, we will continue doing SUPER doses of factor. We can't think about port surgery until we get his levels up to that 70 percent range. No surgeon wants to get a call that they have to do surgery on a hemo whose levels are still so low...As far as his picc line it is great, we get the dressing changed weekly. We are regulars at the pharmacy picking up boxes and boxes of factor to keep up with his daily infusions. Life is good. note: we keep a sock on his arm to hide the picc (not from the world, mostly from his busy hands)
Recently I heard a brief discussion on NPR about whether or not the standards for donating blood should be revised to include men who have had sexual contact with other men in the potential donor pool. The FDA has recently upheld the current standard of declining to accept the blood of people who fall into this category.
As I listened to the guest talk, I must admit to struggling with a schizophrenic battle between the sickening panic in my stomach and the logic in my head. The idea of any kind of lowered safety threshold makes me deeply concerned. The consequences of contaminated blood is not theoretical for those of us in the bleeding disorders community. It is not a debate over ideologies or value systems or religious beliefs or civil rights. We have good reason to support strict controls on the level of risk we are willing to accept when it comes to blood born pathogens. By the end of the 1980s 10,000 hemophiliacs in the US had contracted HIV and 15,000 were infected with hepatitis as a result of FDA-approved tainted blood product. (To help understand the magnitude of that number you must know that there are currently around 17,000 people living with hemophilia nationwide.) It is well documented that both the government and the pharmaceutical companies knowingly allowed tainted blood clotting product to be marketed to and used by the hemophilia community. Exacerbated by poor blood screening/treatment mechanisms and high risk donors being allowed to contribute to the blood supply, the result was, what I consider, a generational genocide...and the reason my mother was widowed with 4 children before she turned 40.
The NPR guest did a lot of talking about the right to give blood - which, first of all, I'm not so sure is actually a right. Secondly, I do not think it is discrimination to say "Hey, you make risky choices... which may be ok with you, but not so much with us. Thanks, but no thanks." I think it's smart. I don't think it's smart, however, to have guidelines that make people think that they are more safe, while actually doing nothing to improve safety.
The question then becomes, does the ban on accepting blood donations from homosexual men in general improve the overall safety of the collective blood supply. In deciding where I come down on this issue I asked myself: "If my son needed a transfusion (like my 14 month old nephew just received) or a human-derived clotting product, would I consider using the blood of a gay man who has sex exclusively with one person more risky than using the blood of a straight man who engages in sexual activity with multiple partners? Not taking into consideration the infinitely improved screening and treating processes, I would choose the blood of the monogamous gay man over the promiscuous straight man.
When making changes to the screening mechanism, I would prefer that donors be excluded based on the number of partners with whom they've had sex over a given period of time. Quite frankly I don't want my son exposed to the diseases that accompany risky sexual practices, whether those practices are with men or women. It seems to me that this criteria would appropriately identify risky behaviors practiced across all potential blood donors, while allowing those who engage in more responsible sexual behaviors to contribute to the much relied upon blood supply.
In the end, no one can truly police blood donors. It's not as though a list of questions is going to stop someone from donating blood that is considered high risk if they want to donate blood. This is why I am grateful for improved screening, treating and monitoring protocols, as well as recombinant factor that is not derived from human blood product. Constant vigilance is vital and I want to support policies that both actually keep our families safe and demand that those who are in a position of guardianship over that blood supply take their responsibility seriously this time... not just look like they do.
NOTE: Bad Blood is a documentary that was released to limited markets about a week ago. It tells the story of the devastating consequences borne by those in the bleeding disorders community when they were knowingly exposed to diseased blood product. I have not yet seen this documentary, but when it is released on DVD in December, I intend to purchase it. (I am in no way connected to the makers of the film, but am grateful that this chapter of our history to finally be told. Loudly.)
Our new routine for infusing Zander (via his picc line) is quick and easy. We usually try to do it while he is sleeping (before Travis leaves for work). Even if he wakes up he is a pretty happy camper (as long as he is involved). Here are some pictures from this mornings infusion.
check the line, flush it with saline, check for blood return.
My daughters have done a fabulous job of creating this blog for those who have the courage to follow it. While I have kept up with the blog, I have been somewhat reluctant to post, but the past week has reminded me of my own journey that began in 1973 when I married the Grand Pooba Hemophiliac (also known as Grandpa Rod). At that time in Montana there was no doctor or medical facility personnel that really knew anything about this crazy bleeding disorder, so it was on the family and more specifically Grandpa Rod to learn about and manage this condition. The treatment product and approach to treatment was okay for the time. We carried our own factor to the ER on many occasions before we could infuse at home. Every bleed was a trip to the ER where we mixed the factor, administered the factor, helped ourselves to a few rolls of paper tape, got a shot of morphine and headed home. Lots of memories of trying to get home before the full effect of the morphine set in. Imagine me in Montana in the middle of winter trying to get a drug induced, wheelchair ridin' husband through 30 inches of snow, into a van with no power lift to a safe place before he started singing "Dead Skunk". The nurses put us in a room and left us alone, because they knew nothing about the process. I knew we were in trouble when I saw two or three nurses gathered with the factor "directions" trying to figure out what to do with the two little bottles of "stuff". This is not an indictment of the nursing profession, because we had our share of terrific nurses, but rather an acknowledgment that hemophilia was not often seen or treated, so Grandpa Rod became the teacher of many.
The HTC at Doernbecher provides a different perspective on the treatment for hemophilia. The personnel is trained and the doctors are knowledgeable, fabulous and caring. The treatment for my 3 grandsons is superior to the treatment of my husband's era. He used to say, "someday, kids with hemophilia will probably just take a pill or something instead of all the infusions". Clearly, research hasn't progressed quite that far, but with the regular infusions (prophy), these kids are light years ahead of their grandpa.
Bright future aside, it was a little eery to be in the hospital room with one of my sweet grandbabies and his parents last week. Mom and dad were just trying to soak up the required learning to assist their son--asking every question and filling their parent library with information that will be recalled for future events. There is a little twinge of sadness, as I would prefer my family not have this experience. I suppose it would have been much easier not to have had children -- that would have been the solution for some -- but we would have missed out on the fantastic journey that has come with four daughters and subsequently 13 grandchildren. I would have been a total loner in that scenario. I mean, I'm real real fun, but even I would have grown weary of being around me!
I love this family and let's face it, for some the challenges are not health related, but all have their own challenges to bear. At least, we were somewhat prepared for this potential and thanks to research and medical advances the path is much more sophisticated than it was in 1950 (that sounds like a real long time ago and I don't want anyone to infer my age based on that number) and I look forward to the role of grandparent with this generation.
We went to the fountains. Maybe you are thinking "A nubie walking hemophiliac trying to keep his balance on wet pavement. Don't do it lady. Don't do it."
Harris did a downward dog right onto his forehead (actually, that might be a totally different yoga position). Contrary to my previous assertions about "fountains are better than pools because no one can drown", he nearly drowned because downward dog and standing water apparently aren't good friends. Also, he scratched up his head. Nothing happened.
Same fountain, different day, he did what my family refers to as a "belly whomp" on the concrete and then sort of rolled up onto one shoulder and the side of his face. He looked like one of those slow-mo shots that they use on that show "Wipe Out". (Just for the record - I hate that show, and only watch it when my husband has Hulu held hostage. I mean how many times can you watch people get hurled into the mud by a Wylie Coyote inspired punching arm and still think it's funny? Seriously people.) Nothing happened.
We have been through a fall down the stairs (it was a slow roll), immunizations, falling, tripping, banging, pulling chairs onto our chests, being lifted by our 4 year old brother in a rather unconventional fashion, an ingrown toenail and a chest x-ray because Harris may or may not have swallowed a Lego guy's head. (Waste of an x-ray, as it turned out) and we've only had one trauma related infusion so far. Piano leg - one, Harris' head- zero.
Mostly, so far, "nothing" happens a lot more than "something"... which would make for a bloody boring episode of Wipeout, but a pretty bloody good life.
We are home from the hospital!! We went in yesterday morning to get Zander'spicc line placed. When we got there the doctor and nurses were a little concerned at the swelling in his right arm. They told us that he might need to be admitted, so he could receive proper care. I could only think about one thing at a time and first I wanted to get the picc line in him and then we could worry about his right arm bleed.
The actual procedure was so quick, maybe 30 minutes. When we went back to see him he was still out (probably getting the best sleep of his life). They did a chest x-ray to check the picc and it was in a perfect spot.
Then the excitement started. The picc nurse went to change the dressing and it was soaked with blood. The hematologist came in and said just make sure it is getting lots of pressure. I started to get worried as I watched them try and get the bleeding to stop, I mean it was like watching a movie, this didn't work, let's try this....this went on for a while. Many doctors and nurses were coming and going and there was a lot of quiet talking. By the time Zander's bleeding was under control he had lost a lot of blood, when the doctor said, "he needs a blood transfusion" I got sick.
Here is what happened, even with the massive doses of factor before and after the procedure, the inhibitor was doing what an inhibitor does, it eats up the factor. Further more, he is having a pretty good bleed in his arm. There is only so much the factor can do, there were just too many issues going on. Zander was admitted to the hospital and was getting his special factor (Novo 7) every two hours. At one point they also gave him a HUGE does of his regular factor(Advate), trying to trick that stinking inhibitor.
This morning his numbers all were looking better (the blood transfusion did its job) and we drew blood (ironic right) for more tests. The tests came back that the inhibitor was down, and his clotting factor was up. WE DID IT! We fooled the inhibitor.
Now, we are doing that massive doses of Advate daily. With the picc line we are able to do that at home. We still will have to go to the center to have the dressing changed and to have a lot of labs done to monitor factor level and the inhibitor.
Zander was back to himself this morning, getting into everything in his hospital room and was thrilled to leave. His arm that started all this is covered in a pretty impressive bruise, reminiscent of my dad's bruises.
A few weeks ago Zander had hives. We were really tracking everything he ate to see what could be causing them. We started to notice a pattern, on the days we infused his hives would be worse. This concerned me, I looked at the side effects of his factor and hives is one of them. Then Endre pointed out to me maybe it is the cream we use to numb his port. So, for the first time we infused him without cream. When I stuck the needle in he seemed generally surprised and he fussed a little. That day he had no hives (the first time in a week). The next time we infused we used cream to see if the hives would come back (they didn't come back so maybe the hives were related to something else). Travis and I infused Zander with the cream and then a couple times without the cream. This morning when I infused him (no cream) he was frantic. He has NEVER acted that way with his infusions. So, it may not be right for everyone but for Zander we are going to keep using the cream. Maybe in a few months we will try again. I figure if it makes it more comfortable for him then we need to do that for him.
