Tag: factor IX

Let it be a dance we do

~ Linda Wright

This morning I awoke earlier than usual, jittery, and craving sweet foods. It is the day after an infusion of clotting factor. Years ago I began having allergic reactions, which included hives and shortness of breath. Now I receive pre-treatments that control these reactions, however one of the pre-treatments is a corticosteroid. It makes me hyperactive for at least 24 hours before I crash. It is a familiar feeling to me now and I have learned to adjust.

In the 60th year of my life, I changed my approach to dealing with my bleeding disorder once again.

I realize that I am part renegade and part coward when it comes to treating my bleeding disorder. In my childhood, the advice from my hematologist was to infuse with Factor I (fibrinogen) only when I had a life threatening bleed. Blood products were dangerous and not to be used freely.

Frequently long days of rest, elevation and ice would slow a bruise enough for it to be managed without and infusion. There were topical solutions like Gelfoam sponge™ and Blood Stop™ that could be used on surface wounds.

When I was a teenager one physician who did not believe that I could be a girl and have a “real” bleeding disorder carried this advice to an extreme. During those difficult growth spurt years I suffered unnecessarily from bleeds into my ankles. The effect of the untreated bleeds has been permanent arthritic damage.

For a period of time in the 70’s, I took the other road and joined the flock. The mantra then was “when in doubt, infuse.” I was on the Board of the National Hemophilia Foundation when HIV began to infect the flock. We were once again the canaries in the mineshaft. Many of the flock already had Hepatitis C.

I abruptly left that heavily trafficked road, to follow “the road less traveled.” It felt familiar and safer although more challenging. Like Robert Frost described, it has “made all the difference.” Frost never said it was a better road. Most people like to think that is what he meant. I would like to think that my choice of roads brought me to the age of 60, but I don’t say that anymore. I do believe it was just the road that made all the difference in my life.

So now that I am over 60, I have chosen still another path. Each month I go into the outpatient hematology/oncology clinic and get a dose of cryoprecipitate. It brings me up to about 50% of normal clotting. The half-life is long and even though the research does not back me up on this one, I believe I can notice an effect for at least two weeks.

It’s a matter of choice. Even if my body can no longer dance, I feel like it gives me some time for my spirit to dance.

How long have you had hemophilia?

~ Linda Wright

My friend Bill and I used to joke about the questions health care providers asked us. Bill had Hemophilia B, a factor IX deficiency. Although our bleeding disorders were different, our experiences with doctors had been much the same. Medical professionals rarely wrote our answers down or consulted our previous medical records for information. Not surprising to us the questions didn’t make any improvement in our medical care. What was startling though was how the questions we had been asked so routinely were identical. By the time we were in our twenties we had answered the same questions countless times. More than once I had been asked. “How long have you had congenital afibrinogenemia?” I felt as if I should carry a dictionary with me and open it up to congenital: adjective (esp. of a disease or physical abnormality) present from birth.

Often two or three different doctors would ask the same questions in one day. Once the doctor had finished asking his or her questions, they showed little or no interest in us. It felt like we were rare birds in a zoo, not people who had gone to a hospital for treatment. It took years for us to understand that we were viewed as subjects for research. We were offering a short cut for doctors who didn’t want to use the medical library.

Bill started responding with “Are the answers to your questions going to help you treat me or are you asking me because you need to learn?” He didn’t say this with a sarcastic tone of voice; he simply wanted the inquisitive doctor to be honest.

Like many people we wanted to be of help in educating doctors. We weren’t acknowledged for providing a service; instead we were expected to respond to questions that seemed irrelevant, even foolish, before we could receive medical attention. It seemed as if the priorities were upside down.