Living with Hemophilia:
A Personal Story
By Kevin Irvine
I am a bleeder and have been for all of my 40 years.
Please don’t be alarmed: It isn’t nearly as messy or freaky as it
sounds. If it were, I probably wouldn’t have been married for eight years
with a 3-year-old daughter.
Technically, I have severe Hemophilia B, but it really
comes down to bleeding and clotting. Apparently, the most common mental image
for the average person who learns about hemophilia is of a minor cut that
won’t stop bleeding. In reality, those are as insignificant for us as for
anybody else. What we really worry about are more serious injuries or trauma,
both external and internal (such as in joints or muscles). People living with
most forms of hemophilia bleed for longer periods of time (not harder or
faster) than someone without a bleeding disorder. Granted, I do tend to bruise
easily, but that comes with the territory.
As with many disabilities and medical conditions,
treatments have changed radically over the last 50 years, and those have
greatly improved the quality of life for many people with hemophilia. In the
United States, medical treatment of hemophilia is very expensive but often
subsidized by the government and, therefore, readily available. One of the
arguably preventable hazards of the advancement in treatment was contamination
of our blood-based products by viruses, especially HIV and hepatitis. I did not
escape unscathed. My experiences with blood, both mine and that of others, have
been as complex as any significant long-term relationship.
My hemophilia experience began shortly after my birth in
1969, when my parents had me circumcised. I bled for a prolonged period of
time, which turned a routine procedure into a scramble for a diagnosis.
Hemophilia is usually an inherited disorder, but I was one of the 30% to
experience a spontaneous gene mutation. In the U.S., hemophilia occurs in about
one in 5,000 males. In rare cases, females are diagnosed with hemophilia (von
Willebrand disease is a different bleeding disorder that affects men and women
equally).
As with joke-telling, timing is everything, and I greatly
benefited from being born at the end of a decade in which a revolutionary
discovery completely changed the treatment landscape. To understand the
evolution of treatment, it helps to understand why people with hemophilia have
such difficulty forming clots. In the 1940s, researchers discovered that the
bodies of people with hemophilia failed to produce adequate amounts of a
protein necessary for blood to clot. These are called “clotting
factors,” and most people with hemophilia are missing “factor
VIII.” I belong to the group of 10% of people with hemophilia that
doesn’t produce “factor IX.” In the 1940s, the standard
treatment involved icing joints and transfusions with whole blood, neither of
which was very effective. Most people with severe forms of hemophilia
didn’t live past 30.
In 1965, Dr. Judith Graham Pool discovered a process to
extract clotting proteins from plasma through a form of freeze-drying. Beyond
being able to effectively treat bleeding episodes, this discovery meant that
people with hemophilia could have emergency surgery and elective procedures
that had been avoided before. It also meant that kids like me would not wind up
spending the majority of our lives shuttling in and out of hospitals. In many
ways, my childhood unfolded in a manner not too dissimilar from those of my
peers, although I did get involved in a few public blood-drive campaigns. My
parents were advised to keep me out of contact sports, which wasn’t a big
deal for me because I was more of an artsy type anyway. When I had a bleeding
episode (we just called it “a bleed”), my mom took me to the local
children’s hospital for treatment, and then we went home. Eventually, my
mom was trained to give me an intravenous injection, and I was able to remain
at home in many cases.
As the experiences of people with hemophilia started
shifting from just surviving to living, in the 1970s, joint damage became our
biggest problem. Guys with hemophilia typically have problems from repeated
bleeding into high-usage joints, such as knees, ankles, shoulders and elbows.
When I first met other people with hemophilia, a lot of guys used wheelchairs
or crutches at least some of the time. My life changed when I had a major bleed
in my left knee as a toddler. Being a typically adaptive and creative kid, I
wasn’t fazed when I couldn’t walk one day. I just wriggled into the
other room and announced to my parents that I was “a snake!”
Following that episode, I got into a cycle of bleeding
into my left knee, which gradually began destroying the joint. I wore a leg
brace for a year, but it didn’t help much. As I got older, I began using
my right leg more, and the muscles around my left knee atrophied. That left it
more vulnerable to bleeds, a truly vicious cycle. Besides my left knee, or
“target joint” in the medical parlance, I bleed into various joints
and muscles throughout my body. Sometimes it happens spontaneously and
sometimes because I bang into a table or chair. Blood flows into the affected
area, which swells and quickly becomes painful. Injecting clotting factor into
my vein allows my blood to clot, and the blood is gradually reabsorbed into my
body. In a day or two, the clotting factor has cycled out of my body, and
I’m a bleeder again.
I have always had an abundance of confidence and
self-esteem, so I was out and proud as a kid with hemophilia in elementary
school. The law that became the Individuals with Disabilities Education Act
went into effect just as I began kindergarten, so I was never segregated from
non-disabled peers. I experienced some mild teasing but recall general
acceptance, although I’m sure I fielded the usual questions that anyone
would have about something unusual like hemophilia. I also had a fantastic
experience when I attended a camp for kids with bleeding disorders at the age
of 11. Many of the counselors were also guys with hemophilia who had gone to
camp themselves. A standard experience for many kids at camp was learning how
to self-infuse with clotting factor for the first time, with the guidance and
support of the medical staff plus the encouragement of older peers. I
didn’t have the courage at that time but did learn a couple of years
later, when my dad volunteered his own arm for my first practice jab into a
vein with a butterfly needle!
My relationship with openness experienced a seismic shift
when two events hit me like a landslide in the early 1980s: middle school and
AIDS. In many ways, my experience with middle school was the universal one in
that it was bad for everyone but in a unique way. As I entered adolescence, I
wanted to fit in, be cool and be different, but it soon dawned on me that my
“difference” wasn’t cool (or at least that’s how it felt at
the time). My decision to stop talking about hemophilia was strongly reinforced
when doctors began diagnosing cases of AIDS among people with hemophilia.
Because of our dependence on a treatment made with blood, people with
hemophilia had already accepted exposure to blood-borne hepatitis as a
cost-of-living expense – I had a bout with hepatitis B as an infant. But
hepatitis didn’t ignite a community’s fears in the same way that AIDS
would. A Time magazine cover story from July 1983 sealed the deal by
bringing the link between AIDS and hemophilia into living rooms and waiting
rooms all over the country. I went underground, and by high school I had begun
developing an arsenal of excuses to explain why I was limping, using crutches
or missing school.
My “official” HIV diagnosis came on January
20th, 1987, during my senior year of high school, but I had already concluded
that I had it because the odds were not in my favor. From the late-1970s until
the mid-1980s, when a process to kill viruses in blood products was developed,
I injected clotting factor into my veins about once a week. A compounding
factor was the fact that the blood-products industry pooled the plasma from
thousands of people together while making our product. More than 50% of people
with hemophilia would eventually acquire HIV through blood products, while more
than 90% of people with severe hemophilia did. My experiences with HIV/AIDS,
and later, hepatitis C, have had profound effects on my life, but they are
quite complex. It will suffice to say that I’m neither dead nor dying, am
extremely fortunate and have many reasons to expect to be around for a long
time to come.
Having survived high school, I moved from California to
New York to attend college. By 1990, I came to terms with the reality of my
situation and began living openly as a person with hemophilia again (and as a
person with HIV for the first time.) Coming out in college by writing an
article in the school newspaper was a daunting but ultimately fantastic
experience because it allowed me to better define the way in which my
disabilities were perceived by others. I also began developing my disability
rights perspective and understanding of disability culture by getting involved
in both the HIV/AIDS and hemophilia communities, including returning to
hemophilia camp, this time as a counselor.
I returned to California after college and got a job
teaching community-living skills to adults with developmental disabilities.
Fortunately, my new (low-paying) job had an HMO plan because I couldn’t
remain on my stepmom’s health insurance as I was no longer a full-time
student. After a year of working that included many hours on my feet every day,
the pain in my knee increased significantly. A visit with an orthopedic surgeon
led me to conclude that I needed to schedule a total knee replacement, shortly
before my 23rd birthday. The surgery was possible with large-scale
infusions of clotting factor before, during and after surgery. I was very
fortunate to have an employer that was very supportive: I got medical leave and
short-term disability benefits, and I was back at work full time three months
following surgery.
One of the most eye-opening experiences of my surgery was
when I received a copy of the bill and realized how much the blood industry
charged for its products: nearly $200,000 for the week of my surgery just for
clotting factor. The HMO paid for it all. The clotting factor is still
expensive: about $1 per unit. I use 2,000 to 3,000 units for each infusion,
every four to six days. It is not uncommon for people with severe hemophilia to
reach lifetime insurance benefit caps of $1,000,000 when blood products are
counted as medical care and not as pharmaceuticals. It is one of the reasons
that the hemophilia community has pushed for the elimination of lifetime caps
as part of health care reform. On the flip side of this issue, however, is that
pharmaceutical home care companies serving the hemophilia community compete for
our business. I get birthday and holiday cards from both my current and former
home care providers.
From 1994 to 1997, when I worked at a center for
independent living in Albuquerque, N.M., -- the only time I had a job that did
not provide health insurance -- I was able to convert my insurance from my
previous job into an individual plan and pay for it out of pocket. My monthly
payment of $325 was a drop in the bucket compared to what I received in
benefits. In 2009, such an arrangement might still be possible, but it is
unlikely that the monthly payment would be affordable. Luckily for me, my move
to Chicago in 1997 followed by only six months the effective date of the Health
Insurance Portability and Accountability Act (HIPAA), which allowed me to
circumvent a one-year pre-existing condition exclusion policy on my new
employer’s health insurance plan.
Since then, life as a bleeder has been relatively smooth,
with a few problems along the way, such as the week that I was bleeding into my
gastrointestinal system and didn’t realize it until I was passing out from
blood loss. I also had to get my knee replaced again in 2004 when the cement
holding the first one in place came loose.
I met my wife, Karen, through our work in the disability
rights movement, and we adopted our daughter, Dominika, in 2006. At that time,
I left my job as a disability rights trainer and transportation advocate to be
a full-time, stay-at-home dad. Dominika doesn’t really understand about
hemophilia, but she is fascinated by my self-infusing (and all the
paraphernalia that goes with that) and always claps for me when I’m done.
For more information about hemophilia and other bleeding
disorders, go to:
http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.
aspx?menuid=0&contentid=1,
http://hemophiliafed.org/ ,
http://www.cott1.org/about.html or
http://www.time.com/time/magazine/
article/0,9171,950937,00.html.
Kevin Irvine is a stay-at-home father and former
disability-rights educator and advocate for the transportation rights of people
with disabilities. He lives with his wife and daughter in Chicago, Ill.
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