Everything You Need to Know About Hemophilia
We lead such busy lives that there are times when we don’t even notice when our skin has been scratched. We get paper cuts, animal scratch marks, knee scrapes, and more throughout the year. Many don’t realize how lucky they are not to have the worry or fear of irregular bleeding. To commemorate World Hemophilia Day, Healthcare Solutions is spreading awareness of this rare and incurable disease.
What Is a Bleeding Disorder?
Bleeding disorders are also called coagulopathy, abnormal bleeding, and clotting disorders. It is a general term that refers to poor clotting, which results in continual bleeding that can happen both internally and externally.
Bleeding disorders occur when there is a low platelet count, or when the platelets do not function properly. Most disorders are inherited and, in the case of hemophilia, affect mostly men.
When someone has a bleeding disorder, their quality of life will be affected, since performing everyday tasks or participating in group activities can lead to serious consequences. Unfortunately, many patients don’t realize that playing sports from a young age helps strengthen their muscles and joints, which can actually help prevent spontaneous bleeding.
What Is Hemophilia?
Hemophilia is a condition in which the body does not make blood clots to staunch blood flow because the ability to produce the necessary protein called fibrin has been compromised. There are two versions of hemophilia:
- Hemophilia A: There is a deficiency of the clotting factor VIII. Eighty percent of men have this version.
- Hemophilia B: There is a deficiency of the clotting factor IX. Twenty percent of men have this version.
In rare instances, someone can acquire hemophilia as an adult, but this form of the disease is curable.
Hemophilia is a rare bleeding disorder; only 1 in 10,000 people have it. Men inherit the disorder while women are the main gene carriers. The severity of hemophilia can range from mild to severe, and women rarely experience severe cases of hemophilia.
- People with severe hemophilia bleed frequently and spontaneously, usually into their muscles or joints, which makes early detection essential.
- Moderate hemophilia affects patients after a major injury, surgery, or dental work. Spontaneous bleeding is not as common.
- Cases of mild hemophilia lead to less frequent cases of heavy bleeding; people with mild hemophilia may never experience a heavy bleeding episode.
Signs and Symptoms of Hemophilia
People with hemophilia A and B experience the same symptoms:
- Spontaneous bleeding internally or externally
- Big, dark bruises
- Excessive or prolonged bleeding after an injury, surgery, and dental work
- Aching, stiffness, and swelling due to bleeding in muscles and joints
Hemophilia in History
Perhaps the most famous case of hemophilia belongs to the last of the Russian royal family, the Romanovs. Prince Alexei Nikolaevich, the only son of the Romanov family and heir apparent to the throne, was afflicted from a very young age with what was known then as the Royal Disease—so named because of the prevalence of this affliction amongst other European royal families. The cause for this spread was the practice of disciplined breeding to secure the purity of the royal bloodlines. The source of hemophilia: Queen Victoria of England.
Queen Victoria’s great-great-grandson, Prince Alexei, suffered from hemophilia B, and as a result, he was in poor health throughout his short life. His mother, Empress Alexandra Romanov resorted to desperate acts to find a cure for her son’s affliction, which led to her infamous affiliation (and some say romantic affair) with the mystic healer, Grigori Rasputin. It was partially the close friendship between Rasputin and the Romanovs that led to the Bolshevik uprising of 1917—all because a desperate mother and father were trying to find a cure for their son’s disease that had yet to be identified.
World Hemophilia Day 2019
Every April 17, we celebrate World Hemophilia Day, which was chosen in 1989 to commemorate the birthday of the man who founded the World Federation of Hemophilia: Frank Schnabel. The purpose of WHD is to spread awareness and reach out to people with a bleeding disorder who are looking for resources and a sense of community and fellowship.
Living with Hemophilia
Hemophilia A and B are incurable; however, treatment is available to help the body produce clotting factor concentrates with prophylaxis. At the moment, prophylaxis is only effective for people with mild or moderate cases of hemophilia. People with hemophilia should avoid Aspirin and other non-steroidal anti-inflammatory drugs, as these blood-thinning medications slow down the blood’s ability to clot.
How Healthcare Solutions Can Help
Our online and onsite Edmonton store offers high-quality dressings and first aid supplies to help hemophiliacs properly treat and care for their wounds. You’ll find surgical dressings, skin protectants, hemostatic dressings, and more.
Shop online, or come and visit one of our Edmonton locations today.