Part of this blog post has been running around inside my head for a long time, not exactly sure how to put it into words. Recent events in my life as a hemophiliac have allowed me to finally find the right words. This is dedicated to Vaughn Ripley, the Committee of Ten Thousand, my blood brothers & sisters with & without HIV, and all bleeder family members (moms, dads, brothers, sisters, etc.). #NeverFoldUp #BAMF
When I was about 4 or 5, I developed an inhibitor. For you clotters reading this: Inhibitors are antibodies that destroy not only the Factor VIII replacement I infuse (inject), but also the small percentage of factor (less than 1%) my body is able to produce naturally. Approximately 30% of people with Hemophilia A (Factor VIII) and 2-3% of people with Hemophilia B (Factor IX) develop inhibitors.
Obviously, an inhibitor is a very bad thing for a hemophiliac. However, developing an inhibitor is especially devastating for someone with mild (5%-40%) or moderate hemophilia (1%-5%) because the inhibitor, in effect, changes them to severe hemophilia (in comparison, you clotters have between 50% and 150% of Factor VIII in your blood). Sometime in the early 70s, I was switched from Cryoprecipitate (a frozen blood product prepared from plasma) to a new Factor concentrate, I don’t remember the name (scientists and manufacturers had developed methods for separating Factor VIII and Factor IX from pooled plasma, allowing for more accurate dosing).
Although, the frequency and amount of treatment (inhibitors typically occur within the first 50 times Factor is used) has been shown to possibly play a role, even now, 30+ years after I developed an inhibitor, what causes inhibitors is not known. Was the introduction of Factor the cause? Who knows. Looking back at it now, having done some research on inhibitors, I would say it is very likely.
Always a rebel, on October 12, 1978, during 1st grade recess, I snuck outside to play with the other kids. I don’t remember anything from that day. All I do know is that at some point during recess, I ended up at the top of the playground slide which I promptly fell off of. I sustained a basilar skull fracture and fractured clavicle. In a coma, I was airlifted from Reno, Nevada, to Sacramento, California, and admitted to the University of California, Davis Medical Center. Because my hemophilia was already complicated by an inhibitor, Dr. Charles Abildgaard started me on a daily treatment of a new clotting concentrate called Autoplex. This was an attempt to combat my inhibitor. While I did break my skull, I did not open it up. So all the blood was trapped between my skull and brain with nowhere to go. Shortly before the doctor’s were about to drill a hole in my head (like I needed another one!), the blood perforated my left ear drum and started flowing out of my ear. Claim to bleeder fame: I was 1 of the first 33 hemophiliacs in the United States to be given Autoplex, resulting in being written about in at least one medical journal (Anti-Inhibitor Coagulant Complex (Autoplex) for Treatment of Factor VIII Inhibitors in Hemophilia).
In April 1985, the 7th to be exact (don’t know why I remember the exact day), I started doing once-a-day prophylactic infusions of AlphaNine, a Factor IX concentrate. Prophylaxis is the regular infusion of clotting factor concentrates in order to prevent bleeding. Because bleeders with moderate or mild hemophilia have fewer spontaneous bleeds and experience less joint damage than us severes, doctors recommend “prophy” treatment in order to keep factor levels around 1%, significantly reducing bleeding and joint damage. My doctors hoped a daily infusion of AlphaNine would help control the bleeding, albeit with a higher infusion dosage, while also overcoming my inhibitor. Some of my generation of bleeders are pro-prophy, some are not, just depends on what side of the “Bleeder Dinner Table” they sit on.
If you’ve read any of my hemophilia-specific blog posts (you all read TheCli3nt.com every day right? RIGHT?!?!?), you know that Factor VIII or Factor IX concentrates come from thousands of plasma donors which are combined as starting material for batches of Factor. In the 80s, the pharmaceutical companies were collecting blood in prisons (Factor 8: The Arkansas Prison Blood Scandal), and in cities that had many homosexuals*, IV drug users and former prisoners. At the time, there wasn’t an accurate test for HIV; and a lot of “important” people, including the pharmaceutical companies, Alan Brownstein former Executive Director of the National Hemophilia Foundation (1981-1992), and the Food and Drug Administration encouraged me and my blood brothers & sisters to continue taking factor. They claimed the risk of joint damage was far worse than being infected with the HIV virus and that the speculations that HIV was transmitted by blood products was unsubstantiated. I have no doubt the more than 10,000 blood brothers and sisters who were murdered by tainted factor (known in the bleeding disorder community as “the hemocaust”) would much rather have joint damage.
So infusing during this time was akin to playing Russian roulette. As a result, I didn’t do as many infusions as I needed. Put yourself in the shoes of a 10- or 11-year-old boy living during the hemocaust and having to stick a needle in your arm every single day! Imagine going to hemophilia camp (summer camp for bleeders) and year after year finding out one of your brothers or sisters had passed away from AIDS. Believe me, I may hate that I can no longer run, ride a bike, or go skiing, but the alternative could be a lot worse. I say “could” because some of my brothers and sisters to this day, are defying the odds and winning the fight against HIV. I got “lucky.” Despite pushing a lot of factor down the drain and lying to my mom about it, I still managed to infuse with tainted factor around 1986. Luckily, I never contracted HIV. Unfortunately, a little bleeder friend, who’s Alphanate was from the same lot (i.e. batch) and lived 45 minutes up the road near Seattle contracted HIV from one single dose of his Alphanate and died.
Thus, not doing my prophylactic infusions kept my inhibitor around a lot longer than it should have and I have a LOT of joint damage. Arthritis in every single joint of my body EXCEPT my right knee. Ankle fusion in 1997 followed by a knee replacement in 2004, knee scar tissue removal in 2009, and a broken hip pinned together in 2012.
Anyway, to the point of this post. Over the past 3 months I have been more responsible with my infusions (even today at 43-years-old I find every excuse I can to get out of doing it). Despite following my doctor’s schedule, my bleeding seems to have gotten worse, almost like something is killing the Factor VIII before it can stop my bleeds. My depression has kicked into overdrive, “oh shit, do I have an inhibitor again?”
After an unbelievable number of ankle bleeds (turning my ankle into a “target joint”) as a kid, I had my ankle fused. I went almost 20 years without an ankle bleed. That is until about 3 years ago. Though the bleeds have been very infrequent, they would happen. Over the past year, they have become more frequent: twenty-three just since July 16 (I can’t walk to the mailbox without running the risk of a bleed)!!
Well, finally I got scheduled to see an orthopedic surgeon. And not just any surgeon, I get to see one from the Coughlin Clinic, one of the leaders in ankles and ankle replacements. After taking some x-rays of my ankle, it was determined the screws used to fuse my ankle had shifted, with one of them pushing through to the heel joint and it is the tip of the screw dragging along the middle of the joint when I walk that is causing the bleeding.
Presented with two options, A) pull the screws, heal and evaluate or B) pull the screws, fuse the heel joint and hope for the best, I decided on option A. No sense further limiting my mobility if not needed. So, surgery has been scheduled for November 18, less than two weeks away!!
But what about my excessive bleeding? Did I have an inhibitor? What is going on? Why is my factor not working? Well, after three separate blood draws (my hematologist is amazing, his phlebotomists are also awesome, following my instructions to the letter and never making my elbows bleed; however, I cannot say the same thing about his lab. They screwed up the first blood draw by testing my Factor V levels (Factor V Leiden is an increased tendency to form abnormal blood clots that can block blood vessels). Say What? Then they said the second blood draw didn’t have enough blood (apparently they didn’t like the results and couldn’t run it a second time?); and then yesterday’s blood draw, I am happy to report, I am INHIBITOR FREE!!!!
As long as I don’t need to give more blood (crossing my fingers), I should get the results of my PTT (partial thromboplastin time is a blood test that measures the time it takes your blood to clot) test back Monday or Tuesday. When my blood was drawn this past Monday (draw #2), my Factor VIII levels were so low (my infusion before the draw was on Friday), the lab thought the results were bad (apparently less than 0.25% of Factor VIII). So yesterday morning, I gave my normal Friday dosage a few hours before the blood draw. Hopefully the blood work will come back and I’ll find out my body is just metabolizing (using) the Factor VIII really fast (hey, it could happen. I have the same 29″ waist today that I had when I was 21, no matter what or how much I eat).
Barring any weird complications (that too could happen: my body did cure itself of Hepatitis C, but that’s another story), I will head into the hospital Wednesday, November 18 at 7:00 AM and have these damn screws pulled (I’m thinking about asking if I can have them…maybe make a necklace or something) and be up and walking normally by Christmas. Well, as normal as most of my bleeder generation walks.
Pimpin’ ain’t easy, but my hemo-limp and cane help keep me looking fresh!
* Note from The Cli3nt: I am not blaming, nor am I trying to disparage LGBT people. The blame and responsibility for the spread of HIV through the hemophilia community rests squarely on the shoulders of executives at Alpha Therapeutic Corporation, Institut Mérieux (which then became Rhone-Poulenc Rorer Inc., and is now part of Sanofi), Bayer Corporation and its Cutter Biological division, Baxter International and its Hyland Pharmaceutical division, along with Alan Brownstein, Executive Director (1981 – 1992), National Hemophilia Foundation, Dr. Louis Aledort, Medical Co-director, National Hemophilia Foundation and many other “important” people.