Tuesday, February 28, 2012

Roller Coaster

The word roller coaster can be used to describe an amusement park ride with tight turns, steep slopes and high speeds. That word can also be used figuratively to describe something that goes through wild and unpredictable changes. I think the word roller coaster fittingly describes how my life has been living with hemophilia.

I was born on November 28, 1968, the youngest of four children: three boys and one girl. My maternal grandfather had hemophilia. My oldest brother, who was nearly 16 when I was born, was born with Hemophilia A. My other older brother was not. I have two male cousins who weren’t born with hemophilia and one male cousin that was born with it. Given the family history, the doctors knew to test me immediately at birth and sure enough I was also born with hemophilia.

Back in the late 1960s and through the 1970s, the common treatment was to receive Factor VIII or Cryoprecipitate transfusions, which were derived from human-donated blood. My older brother received Factor VIII and my doctors chose to give me Cryoprecipitate.

My family lived in rural Fauquier County, Virginia, about 70 miles west of Washington, DC. Our very small community hospital was not prepared for treating a hemophiliac, so I had to go to Georgetown University Medical Center each time I had a bleeding episode.

I’m very lucky to have grown up on a 56-acre horse farm. My mom was a pro at handling hemophilia related issues, as she’d gone through all of that for 15 years with my older brother. My parents pretty much let me go and do things on our farm, like riding my bike or horses sometimes. Inevitably, I would fall and get a bleed or get bleeds for no reason. Each time I had a bleed, my mom would get everything ready and we’d get in the car for the long haul to DC. It would end up taking several hours to get there with all of the rush hour traffic. They had to thaw out the frozen Cryoprecipitate and wouldn't start doing that until we arrived.

As if this all weren't enough, when I was about four I had a reaction to the Cryoprecipitate and broke out in a severe rash and hives and had trouble breathing. From then on, I was given a large dose of Benadryl, which would completely knock me out. We’d have to wait hours for them to do this, then it would be another few hours to infuse it, then another few hours drive home. I couldn’t begin to tell you the hundreds and hundreds of times we did that while I was growing up.

As I got into my teens, my bleeding problems persisted, but for different reasons. I was told I couldn’t participate in Physical Education class or play any sports whatsoever. My mom told me not to worry, as she knew I’d find something else to interest me instead. My parents raised me with the mindset that I wouldn’t let hemophilia define me.  If I found something that caused me to bleed, I simply found another activity to take its place.

Since I couldn’t play sports, I learned to play the flute and went on to be the first-chair flutist through junior high and high school. I played semi-professionally for a while after high school.  Instead of taking gym class, I took art class and learned all about art, which I still love today.

When I learned to drive a car, I learned to drive cars with both manual and automatic transmissions. After driving a car with a manual transmission, I got a major bleed in my left hip joint. This caused my left hip joint to bleed every time I’d drive one. When I joined the marching band, which I loved, both knees and one of my ankles got repeated bleeds until I had to stop marching. I became the manager instead and played with the band in the stands.

Growing up with hemophilia, we didn’t have the hemophilia associations there are today. I never went to hemophilia camp. Aside from relatives, I never met any other hemophiliacs. When home infusion became an option, my mom wasn’t comfortable with learning to do it or me doing it at home. She had our routine down and was most comfortable doing that. After all, she’d been doing her routine since 1952 between my brother and me.

When the HIV infection hit the blood supply, I don’t remember anyone ever really talking to us about the risks of what could be in the product I was getting. I was in high school at this point and still receiving Cryoprecipitate numerous times a year. My oldest brother was somewhat of a rebel, a biker, musician and tattoo artist. He led a rough life at times and as a result, had many, many bleeding episodes. He was receiving Factor VIII numerous times per week, whereas I was getting infusions numerous times per year.

I remember us finding out around 1986 that my brother Billy had tested positive for HIV. My parents didn’t understand anything about HIV or what that diagnosis meant. None of us did. The doctors immediately began testing me and I was negative for HIV. I’m convinced that my doctor’s decision to give me Cryoprecipitate saved my life. I remain HIV negative today.

Sadly, my brother died in 1991 from a hemophilia-related bleeding problem. He was also experiencing full-blown AIDS at that point, so he wouldn’t have survived but maybe another year.

I lived the next 20 years wondering why I was spared when so many hemophiliacs were taken because of HIV and I was spared. I soon had my answer.

After high school I went to work in the corporate world for nearly 20 years, eventually ending up in senior management level positions. In 2005, my mother was diagnosed with terminal lung cancer. My dad had already battled two types of cancer and wasn’t doing well either. My mom died in 2005. The day after her funeral, I received a call from my boss in California, where I was living and working, telling me that my job had been eliminated and I’d been laid off. I’d just taken this job managing 600 employees and moved from New Orleans three months earlier (1 year before Hurricane Katrina).

My dad wasn’t doing well, so I moved home to Virginia to help him. After a few months of sending out resumes all over the country, I suddenly had an epiphany. I realized that my true calling was to be a nurse. I thought about this when I was graduating from high school; however, in 1987 men were not widely accepted as nurses. My parents reminded me “being a nurse is a woman’s job.” Back then it was.

In the summer of 2005, I made the decision to go to nursing school. I decided to become a Licensed Practical Nurse first. I completed the accelerated program in one year and passed my state board examination on the first attempt. After graduating nursing school, I went to work at a local hospital near my home and worked as a LPN Staff Nurse on a Medical/Oncology in-patient unit.

Due to the physical demands of being a staff nurse, I began to have more and more bleeding problems each year. In 2009, I had nearly 40 spontaneous bleeds. All along, I’d also been going to school working towards becoming a Registered Nurse. Sadly, I had to leave my nursing career because of the bleeding problems and degenerative damage to my joints. I’m now on disability and trying to stay active by working with projects related to the hemophilia community.

So, yes, I can say that my life living with hemophilia has been much like a roller coaster. There have been many ups and downs, twists and turns, screams and thrills. I wouldn’t have it any other way! It’s made me a strong, resilient, compassionate and caring person.

Monday, February 13, 2012

Hemophilia: It's A Family Affair

My name is Amber and I am a 30-year-old mother of four kids: Dillinger (10), Emma Coalesce (9), Brandston (7), and Elise Mae (3). My boys have severe hemophilia A with inhibitors. About two years ago Dillinger's inhibitor was tolerized. Thank God! Brandston is currently on his fourth round of Immune Tolerance Therapy (ITT) and we just found out that his inhibitor is on the rise again (not exactly the results we have been hoping for). Emma is considered a symptomatic carrier, but we recently learned, through a session at NHF’s annual meeting, that her 27 percent factor level actually classifies her as a mild hemophiliac. My youngest, Elise, is the lucky one of the bunch. She is just a carrier.

Dealing with multiple children with special medical needs has definitely taken its toll. Throughout the years it has molded each of us into the people we are today. I'm sure it will continue to change and mold us in the years to come, in both positive and negative ways. It's not easy, but I believe everything happens for a reason and how we choose to deal with it is what matters the most.

I am excited about the opportunity to share my family's experiences and I hope that it provides helpful insights to other families affected by bleeding disorders. Please post comments and ask questions. I'd love to hear from you.