Four More Personal Stories Of Hemophilia

Older hemophiliacs often have an opportunity to help their doctors learn how to treat the disease. One such hemophiliac was a sixty-year-old German man who prefers to be identified simply as Klaus.

Teaching Doctors How to Treat Hemophilia

Sixty-year-old "Klaus," a lifelong resident of Hamburg, Germany, had been diagnosed with hemophilia in 1957. Klaus had the milder form of the disease, called hemophilia A, in which the body retains the ability to make small amounts of some of the clotting factors and bleeding usually is not severe. Klaus only received blood products when doctors knew bleeding was likely.

Before a routine colonoscopy, Klaus received anti-bleeding product known as FVIII, or Factor 8. Two weeks later, he fell and bruised his hip, so he got another injection of FVIII. Another two weeks later, however, Klaus started bleeding spontaneously from his bowel. Klaus, as sometimes happens in older hemophiliacs, had developed resistance to the drug that had successfully been used to treat him for many years.

What Klaus did that was different was to ask his doctors to try a combination therapy, steroids (prednisolone) plus more of the anti-clotting factor. Although steroids also have serious side effects, the combination worked, and steroid drugs are becoming increasingly accepted as part of the necessary treatment of hemophilia in older persons.

The Challenges of Being a Woman with Hemophilia

Karen, not her real name, is one of the 5 to 10 percent of hemophiliacs who are women. Karen's symptoms did not appear at birth, or in childhood, but became quite severe when she had her first period. Karen had not just normal menstrual bleeding but gushes of blood that made normal activities impossible. Her periods sometimes last 7 days or more, and, she reports, it was necessary to change pads every two hours for up to a week.

Karen was one of the first women to take the birth control pill Lybrel, advertised for its ability to limit a woman's period to once a year. Although she had some success in stopping her periods with other brands of the Pill, Karen reports that for the first time in her life she is free of one of the most difficult problems in daily life for women of reproductive age who have hemophilia.

A Christmas Baby Born with Christmas Disease

Now six years old, Scott was a Christmas Day surprise (probably more so because his parents are Jewish), born on December 25. The parents were concerned, however, when the heel stick to test Scott's blood for phenylketonuria (PKU) bled an unusually long time. When Scott was circumcised eight days later, the rabbi was unable to stop the bleeding, and after a trip to the emergency room, Scott's parents were told that he had an especially severe form of hemophilia B also known as Christmas disease.

Fortunately, Scott was born in the twenty-first century, when treatments for Christmas disease are readily available. Scott's parents have been diligent about his weekly treatment, but Scott has had a happy and otherwise healthy childhood without drama or complications. Scott's life isn't "normal," since he can't participate in rough and tumble children's activities, but at just the age of six he has gone to Hemophilia Camp and learned how to infuse himself with the medications he needs each week to survive.

A Cop with Hemophilia

The New York Times also reported the story of John Vieke (his real name) of Anderson, Indiana, a hemophiliac who decided to go into law enforcement because people told him he couldn't and, as he told the New York Times, "I didn't want to be the guy with hemophilia who played it safe."

"If you treat it early," Vieke says, "you can prevent the bleeding in the joints from damaging the tissues in the joints. So now, going into my adulthood, I don't many of the problems many hemophiliacs have with their joints."

The stories of these people who live with hemophilia show personal courage and resilience. They also illustrate the power of medical advances, and the role that patients themselves can play in making them come about. Hemophilia remains a serious and life-threatening disease, but it is no longer a death sentence, and great strides have been made. Living with hemophilia has been safer than ever before, which in turn has a great positive impact on patients' quality of life.