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Sunday, January 24, 2016

Not Today

One of the youngest members of our Fibrinogen Free community recently had a conversation with his cousin that went like this.

Cousin: "Do you have Hemophilia?"

Response: "Umm, not today."

I think I know how he feels. I began this blog six years ago, and I haven’t posted in almost two years. Each time I think about posting I think to myself, “Umm, not today.” At 66 years of age I actually feel like I don’t have a bleeding disorder except for the few hours I spend each week being infused with Factor I.

The biggest change is that fibrinogen concentrate is now available and cleansed of known viruses. The standard of care is to use this concentrate on a prophylactic basis so that it prevents the deterioration of joint tissue and life threatening bleeding. And, gene therapy is in the works. Then there will be no more concern about what undetectable hazard has entered the blood pool. I believe it will not be long before Factor I deficiency is a thing of the past.

When I began this blog I hoped to find some more people who either had a fibrinogen deficiency or had someone who was a family member with little or no fibrinogen. We are a small group yet we know there are more. We learn from each other, support one another, and advocate together. I am grateful that folks younger than I are using social media to reach out and say, “Hello, you are not alone.” 

Scars from bleeds in the past now limit my walking. I don’t climb stairs with ease. Knees, ankles, shoulders, and back aches remind me daily that being born with no fibrinogen has taken a toll. My brain does not function the way it did before a hemorrhagic stroke in 2002.

The more we know about preventing unnecessary bleeding incidents the healthier we will be. Because I am currently the oldest member of our online community I remember the bad old days viscerally. My body and mind signal the reminders of insufficient treatments or delayed medical attention. There are still many people who experience this needlessly. There are still doctors who do not properly diagnose or treat those of us with Factor I deficiency.

We all deserve to have more days when we don’t feel like we have hemophilia.

Tuesday, April 22, 2014

Miner's Canary

When I get to a mirror I see my yellow face and blood shot eyes staring back at me, looking frightened. I go back to bed and sleep another two hours.

The doctor said these new medications were safe, minor side effects, perhaps some rash or slight headache. I took a leap of faith and agreed to go down the mine.

People with bleeding disorders are like the canaries miners used. The canaries were crude measurement of the air quality. If toxic gasses leaked into the air in the mine, the canaries died, but the miners might still have time to get out of the mine alive.

People with bleeding disorders who regularly use blood products to survive are like those canaries. We were among the first to die of HIV in the 1980’s. We were also among the first to contract Hepatitis C. So when I learned that I had Hepatitis C, I was not surprised.

As I learned more about this slow-moving deadly virus, I decided to try the self-injected pegylated interferon and Ribavirin pills. The results were devastating and I came closer to dying than I had ever been before. That was twelve years ago.

Late in 2013 I learned there was a new drug available for Hepatitis C, a second had been approved by the FDA; it was due to be released in a few days, and yet another was in the final stages of testing and would most likely be available in about a year. I could use the two pills by themselves, without need of interferon or Ribavirin.

By mid March, I was taking the new pills, Sovaldi and Olysio. By the first week of April, I had experienced the known side effects. The doctor had advised large doses of antihistamines.

A few days later I received a frantic call from the hematologist. “The lab results show your other clotting factors are abnormal and your bilirubin is elevated.” Since I was only half awake, I couldn’t make sense of what he was saying. I went back to sleep.

“Are you jaundiced?” the doctor asked.

“Yes,” I answered.

I managed to stay awake long enough to get a liver ultrasound and more lab tests drawn. So, now at the bottom of my birdcage, the doctor’s email message reads, “Stop taking the Sovaldi and Olysio.” Then he adds, “"This has never happened before.... I am so sorry... Another medication may get released by the FDA in October, we can try that one next.”

I’m not so sure I have the courage to go into the mine again. I'm using my time singing my song.

“A bird doesn't sing because it has an answer, it sings because it has a song.”
Maya Angelou