Editor: following is part one of a series of articles written by one of our hemophilia community members. It is a history of his life, his struggle with hemophilia, HIV and Hepatitis C, along with the complications that go along with those diagnoses. We hope this will be an encouraging series and that it will be helpful to all.
“Please allow me to introduce myself. I am a 53-year-old guy who ‘suffers’ from hemophilia A, clotting factor VIII deficiency. I have lived through some tough experiences in my journey with hemophilia and this journal will attempt to document that journey. I am an individual with my own story, and I don’t intend to imply that other stories are vastly different from mine, but no two stories are alike, either. Perhaps it will help you as much as it helps me if I tell my story and talk about my struggles and how I have attempted to deal with them.
“My story starts in February of 1968. I was six months old. My parents were worried because their fifth child, me, was bruising quite easily, most notably just from resting on his forearms in his bassinette. They took me to their family doctor and his nurse was visibly shaken. ‘Who handles this child?’, she demanded. The doctor had birthed four other children for this family and knew that abuse was the last thing they would need to worry about. He had an idea though and ran some tests. The results came back. The baby had hemophilia. My mother asked, ‘What’s hemophilia?’ This is a very typical response even today. Perhaps sometime in the relatively near future people will ask that same question, but because hemophilia has been snipped from the gene pool by genetic engineering, not because they are simply unaware of it. We aren’t there yet, but perhaps it is on the horizon.
“Hemophilia was tough on a child in the 1960’s. The most effective treatment at that time was cryoprecipitate, which came from the thawing of plasma and was high in factor VIII. This was still not very concentrated and so I would bleed every time I tried to walk. By the age of five I was wheelchair bound. At the age of five we moved from one state to another and changed hemophilia treatment centers. This coincided with the availability of freeze-dried powder formula of factor VIII. This was a life changer. My mother asked the doctors to help me to walk. They were, I gather, a little taken aback. But she and they devised a plan. She would bring me to the center five days a week. They would treat me with the clotting factor. They would let me splash around in a whirlpool for a bit, soaking all of the nurses in the process. This was a bit of fun for me prior to the painful next step. That was physical therapy. Grueling physical therapy that I loathed. But it worked. In three months I was cheating my way out of the wheelchair every chance I got and I would walk a few steps. I moved to crutches and we stopped the daily therapy. I started school three months late and on crutches. I was ecstatic. All of the other kids were disappointed. They had been prepared for a child in a wheelchair to join them. Within a couple of years I was running, playing baseball in the neighborhood and generally making a pain of myself, primarily for my mother.
“Now these clotting factors, as life-saving as they were, were not the perfect solution for the hemophilia community. It wasn’t clearly understood how to best use this new tool, so it was generally given in reaction to bleeding, not to prevent bleeding. My mother would use it prophylactically if I was interested in a potentially dangerous activity, such as wrestling, which I did one time. I pinned the guy and was completely happy to hang up my wrestling boots for life. But prophylactic treatment of bleeding disorders was still decades away, so we bled frequently and treated to get rid of the bleeds. This led to joint damage over the long haul. In addition these products were manufactured from the pooled blood of thousands of donors, so infections could enter the supply and infect many people from just one donor. This became a real problem when HIV surfaced, as well as Hepatitis C.
“In my junior year of high school I contracted what was then known as non-A, non-B hepatitis. This was in fact Hepatitis C. I was hospitalized for a couple of weeks and never thought too much about it for quite a long time. It was in the spring of my senior year of high school that I was notified of my positive HIV test, which I wasn’t even aware was taken. They tested my blood, which had been stored for research purposes, and it tested positive as early as 1981. Apropos of nothing, I was notified of my HIV status via letter, which just goes to show how little HIV was understood at the time. In this day and age such a notification would be considered cruel and pains would be taken to have a personal conversation with each affected individual.
“Treatment for HIV developed. The first drugs were extremely hard to take. They had side effects that made you want to scream. I ran the gamut of treatment options, often ending with allergic or other reactions that just made them impossible to take. Eventually, in the 2000s the drugs became much more easy to take. My current regiment is one pill each day. I never think about my HIV any more. Never. Except when I’m writing something like this. However, back in 1994 I suffered from an opportunistic infection, Cryptococcal Meningitis, that redefined pain for me. I was in the hospital for 16 days and spent the next 12 years recovering from that illness. That is a topic for another day.
“Also in the 2000s they developed recombinant factor products that didn’t contain any human or animal plasma. Allergic reactions were greatly reduced and the risk of transmission of infectious diseases was eliminated. Subsequently they developed extended half-life products that cut the frequency of treatment in half or even more. I was a fairly early adopter of both of these tech advances.
“By 2015 I was still experiencing some spontaneous bleeding and thought this was perfectly natural. After all, we hadn’t eliminated the hemophilia, just introduced some controls on it. This, I think, is a common thought. Let me tell you, it doesn’t have to be that way. Especially if your joints are relatively healthy. In late January of 2015 I experienced a spontaneous subdural hematoma. This was a life changing experience. I am unaware of the next 6 weeks of my life. I have very little memory of the next year. However, they did some PK studies at that time and discovered that I was going through factor significantly faster than the average hemophiliac. Not extremely so, but enough that my prior prophylactic treatments were inadequate to keep me above the level where spontaneous bleeding is common. They adjusted my factor levels and I didn’t have a major joint bleed for about three years after that. We now try to keep my factor levels above 5% at all times. Some suggest that 10% should be the target. My doctors and I agreed that keeping above 5% should get us where we need to be. I have experienced some bleeding in recent years, but it’s still pretty rare. I have some joint damage that is beginning to aggravate the joint in such a way that bleeding is more likely even when I am sufficiently covered.
“Last spring, another old friend came to play. We had eventually been able to eliminate the Hepatitis C from my system. It had taken three attempts, with everything failing until the protease inhibitors for HCV became available. When I joined a study in 2013 I cleared the virus from my system in one week. That’s from a hundred million or more copies per ml of blood to undetectable in just one week. Those results stayed and I was declared cleared of HCV in the fall of 2014. However, thirty years of infection into that liver had done some serious damage and last spring, that is spring of 2020, I was informed that a lump they had found in the liver a couple of years ago had suddenly changed dramatically. It had burst it’s capsule and invaded a blood vessel. This was proof that it was hepatocellular carcinoma, liver cancer. We did a radio-embolization process that successfully killed the tumor and left only dead and dying tissue around the site. The latest news is not great. They have found six new tumors of varying size in the liver. These will be treated individually with chemo-embolization on January 12th. These two treatment options are relatively new and offer hope to control and even possibly eradicate the cancer in the liver.
“So this is the overview of my life with hemophilia and many of the complications you can face as you deal with a chronic condition. Over the next month I will attempt to write a number of focused articles on chronic illnesses, focusing particularly on my experiences with this topic. I will attempt to explain some of what it has meant to me to go through these experiences, what I have learned along the way, and how I deal with life-threatening situations. I would like to discuss several things along the way: dealing with illnesses; dealing with disclosure; and dealing with relationships. I don’t pretend to know all there is to know about these topics. I don’t even pretend to have the answers that are key to getting through these situations. What I have is a survivor’s mentality that helps me get through and perhaps I can share a bit of that with you.
“Stay with me if you can! This will be quite a trip!”
We aren’t on a strict schedule for this story, but we hope to accomplish our goals in about a month.
- A History of Hemophilia: A Bleeder’s Story (Part 1), Overview of life with hemophilia and it’s complications.
- A History of Hemophilia: A Bleeder’s Story (Part 2), Hemophilia, bleeding and it’s treatment.
- A History of Hemophilia A: A Bleeder’s Story (Part 3), HIV infection and it’s complications.
- A History of Hemophilia A: A Bleeder’s Story (Part 4), Hepatitis C infection and it’s complications.