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.)
