Hemophilia is a rare type of bleeding disorder that occurs when there is a deficiency or absence of a particular protein (clotting factor) needed for blood to clot. As a result, a person with hemophilia will experience longer bleeding after an injury because the clot formed is not strong enough to stop the bleeding. A person with hemophilia will not bleed any faster than a person without hemophilia, but he or she will bleed slower and longer. This type of bleeding disorder is inherited and occurs almost exclusively in males.

The type of clotting factor that is missing determines what kind of hemophilia you have. The two most common types are hemophilia A and hemophilia B. In hemophilia A, the missing clotting factor is factor VIII. Hemophilia A is sometimes referred to as classical hemophilia. In hemophilia B, the missing clotting factor is factor IX. Hemophilia B is sometimes referred to as Christmas disease.

The standard treatment for hemophilia is to give an infusion of factor VIII or factor IX concentrate into the veins. This is known as factor replacement therapy. Once the factor is infused, the normal clotting events can occur and a fibrin net and clot will be formed. This type of treatment is often referred to as "on-demand factor replacement therapy" and has been used for several years.

A common type of factor replacement therapy is a type of preventive care known as prophylaxis. Prophylaxis is the infusion of clotting factor at scheduled times to maintain higher factor VIII or factor IX levels in order to prevent spontaneous bleeding and decrease the number of bleeding episodes. The purpose of this therapy is to prevent bleeding that causes joint damage. This type of therapy is commonly used for children who have severe hemophilia and significantly improves the child’s quality of life because the child has fewer bleeding episodes (and possibly none) and no permanent joint damage.

Although there is no cure for hemophilia, with proper treatment, most people with hemophilia lead full, active lives.

Before surgery or dental work, people with mild hemophilia are sometimes prescribed a drug called desmopressin acetate (commonly know as DDAVP). This synthetic hormone stimulates the release of existing factor VIII stores. It can be given through a vein (DDAVP) or via a nasal spray (Stimate).

Your doctor may also prescribe a medication known as an anti-fibrinolytic agent. This drug, commonly called Amicar (aminocaprioic acid) slows the normal breakdown of blood clots and is helpful for treating oral bleeding.

People with hemophilia should avoid drugs that can exacerbate bleeding problems, such as aspirin, heparin, warfarin, and nonsteroidal anti-inflammatory drugs (NSAIDs).

No. Years ago, people with hemophilia had to rely on hospitals and clinics each time they needed clotting factor, which meant frequent absences from school and work and long hours spent in emergency rooms or clinics. Today, through the support of hematologists, hemophilia treatment centers, local and national hemophilia foundations, and specialized home care companies, people with hemophilia and their families can learn to recognize bleeding episodes early and infuse their own factor at home. This has decreased the amount of time families have had to spend in emergency rooms and clinics and has contributed to an increased quality of life. Children with hemophilia today have fewer hospitalizations, spend more time in school, are better integrated into their peer groups, and experience significantly less joint damage when compared to previous generations of hemophiliacs.

Home infusion therapy has allowed many families with hemophilia to lead a more normal lifestyle and has also improved the long-term prognosis for adult hemophilia patients, according to Hemophilia journal. Home infusion, it reported, "significantly decreased hospital admissions for bleed complications and decreased pain, dysfunction and long-term disability."

Of course, along with the freedom home infusion therapy offers, comes responsibility. For all its benefits, experts caution that hemophilia home infusion is complicated and time-consuming, and might not be for everyone.

However, by receiving ongoing education and support from your hemophilia team, home infusion therapy will put you on the frontline as your child’s caregiver and insure that your child receives the best care possible.

For the most part, living with hemophilia today is much better than it was 30 to 40 years ago. New and improved treatments allow most people with hemophilia to lead normal, active lives. Research has shown that being physically active is extremely important for a person with hemophilia because the benefits of exercise can actually reduce or prevent bleeding episodes by strengthening the muscle surrounding vulnerable joints. In fact, the National Hemophilia Foundation strongly encourages regular exercise for people with hemophilia of all ages in order to preserve joint function. As part of its national prevention program, the National Hemophilia Foundation also urges everyone with hemophilia to take these five steps ("Do the 5"):

•Get an annual comprehensive exam at a hemophilia treatment center

•Get vaccinated for hepatitis A and B

•Treat bleeds early and adequately

•Get tested regularly for blood-borne infections

•Exercise to protect the joints

Of course, there are some activities that people with hemophilia should avoid. According to a report published in Clinical Orthopaedics and Related Research, some sports such as boxing, rugby, and football are considered too high risk and should be avoided. Hematologists who specialize in hemophilia care recommend that people with severe hemophilia avoid high contact sports like football, wrestling, and other sports that are considered high risk for head or severe injury.

However, there are many different types of sports and activities that a person with hemophilia can safely participate in. Some of the most widely recommended activities include swimming, walking, golfing, and bicycling, according to the University of California at San Francisco. People with hemophilia are strongly encouraged to talk with their hemophilia team about the best activities to participate in, so that they can maintain a healthy and active lifestyle as much as possible.

References

Last updated June 29, 2009
Copyright © 2005 Consumer Health Interactive

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