Hemophilia is an inherited bleeding disorder where the blood fails to clot. Hemophilia is a lifelong bleeding disorder that currently does not have a cure.
- There are low levels of clotting proteins in the blood.
- It is seen mostly in boys (rarely in girls).
- Very few people have it.
- There are about 400 babies with hemophilia born in the U.S. each year.
- About one in every 20,000 men in the U.S. have hemophilia.
- About 80% of those with hemophilia have hemophilia A (factor 8 deficiency) and 20% have hemophilia B (factor 9 deficiency).
- Hemophilia occurs in all races and social groups.
- Women may carry the gene that is passed on to her children.
- People with hemophilia are born with the disorder.
How can medications help?
- They can help prevent or stop bleeding.
- By using medicine and visiting a hematologist regularly, a person with hemophilia can expect to live a long and healthy life.
What happens when someone with hemophilia has an injury?
- The injured blood vessel gets smaller (vasoconstricts) to let less blood through.
- Platelets rush to the site and stick together to form a platelet plug.
- Clotting factor proteins in the blood work together to make threads of fibrin (a protein produced by the body). The fibrin weaves itself into a clot over the platelet plug. This makes a strong seal.
How are injuries different when someone has hemophilia?
- People with hemophilia can’t make a fibrin clot.
- A person with hemophilia has problems when a fibrin clot is needed to stop the bleeding. People with hemophilia don’t have enough of certain clotting factors.
- The fibrin clot is not made or is so thin that the bleeding continues.
- Someone with hemophilia does not bleed faster than someone without hemophilia. However, the person with hemophilia will bleed longer.
Why is this a problem?
- Bleeding inside the body is more of a problem for people with hemophilia than bleeding on the outside from a cut or scrape.
- Inside the body, the blood can go into spaces in joints, muscles, and organs.
- Over time, this can cause great damage, especially if the bleeding is not treated or happens often.
For educational purposes only. For specific medical advice, diagnosis and treatment, consult your doctor.
For more information, visit Hemophilia and Bleeding Disorders Birmingham, Alabama (AL) – Children’s of Alabama (childrensal.org).