In 1854, the son of Queen Victoria, Prince Leopold, died following minor fall. Leopold had hemophilia: a hereditary bleeding disorder in which the blood does not clot properly. In the 19th century, hemophilia was a misunderstood and devastating disease, and contributed to the deaths of people in all levels of society, including princes. But with his prominence in society as a member of the Royal Family, Prince Leopold’s death led to more attention to the disease and more research for a cure. Today, hemophilia can largely be controlled, and patients’ quality of life can remain high.
What is hemophilia?
Hemophilia is a bleeding disorder that prevents blood from coagulating properly. It’s usually inherited, and it can lead to both spontaneous bleeding and bleeding following major surgeries or injuries.
Blood contains many components, including proteins called clotting factors that help to stop bleeding. People with hemophilia show low levels of two blood factors: factor VIII or factor IX. Hemophilia presents itself in many different types, but is mostly commonly classified as either hemophilia A or hemophilia B. Hemophilia A, or classic hemophilia, is caused by a total lack or decrease of clotting factor VIII. On the other hand, hemophilia B, or Christmas Disease, is caused by total lack or decrease of clotting factor IX.
Symptoms of hemophilia
Signs and symptoms of hemophilia are best encompassed by spontaneous instances of internal and external bleeding. For example, bleeding into the joints and bleeding into the skin are key signs of the disorder. Bleeding into the joints typically affects the knees, elbows, and ankles, while bleeding into the skin typically creates a hematoma: a build-up of blood in a certain area. Furthermore, bleeding of the mouth and gums or bleeding after surgeries and vaccinations are warning signs that hemophilia might be present.
Hemophilia primarily affects males, but it can occur in females. Hemophilia occurs in about one out of every 5,000 male births and can affect people from all racial and ethnic groups.
Hemophilia treatments
The best way to treat hemophilia is to replace the missing blood clotting factor so that the blood can clot properly. This is done by infusing the patient with pre-prepared factor concentrates. Eventually, patients can perform the infusions themselves so that they can stop bleeding episodes as they occur or prevent most bleeding episodes with regular injections. The best choice of action is to visit a comprehensive Hemophilia Treatment Center as they can provide care and health education to increase hemophilia sufferers’ quality of life.
However, about 15-20 percent of people with hemophilia have an antibody that prevents the infusion treatment from working. Essentially, the antibody blocks the infused clotting factors from clotting the blood, making it much more difficult and costly to treat. Patients with this mutation often experience more joint disease and other complications.
How to prevent hemophilia from developing
The National Hemophilia Foundation’s Prevention Program suggests five tips for healthy living to prevent the development of hemophilia. First, get an annual, comprehensive checkup at a hemophilia treatment center. Second, get vaccinated against hepatitis A and B. Third, treat bleeding early and adequately. Fourth, exercise and maintain a healthy weight to protect your joints. Finally, get tested regularly for blood-borne infections.
Although hemophilia is a serious disease, this condition often resolves with the appropriate treatment and quality of life can remain high with a consistent therapeutic regimen.