This month is bleeding disorders month and we are doing our part to raise awareness so we can learn the symptoms and implications of these rare diseases.
More than three million Americans have a bleeding disorder which are conditions that prevent the blood from clotting in a normal manner. Hemophilia and von Willebrand disease are two blood disorders which can lead to prolonged bleeding after an injury, surgery or other physical trauma.
Hemophilia is rare and mostly affects males. 1 in 5,000 males born in the United States are born with hemophilia each year. Only about 20,000 people are living with a form of hemophilia in the U.S. Hemophilia A, B, and C are all genetic disorders and are treated differently depending on various levels of protein in the blood. Though extremely rare, a person can develop hemophilia as a result of illness, medications, or pregnancy. Unlike other forms of hemophilia, Acquired Hemophilia usually resolves itself with proper diagnosis and treatment.
Symptoms of hemophilia include:
- Bleeding into the joints and muscles
- Nose bleeds
- Prolonged bleeding from minor cuts
- Bleeding that stops and resumes after stopping for only a short time
- Blood in the urine
- Blood in the stool
- Large bruises
- Easy bruising (unexplained bruising)
- Excessive bleeding with dental work or tooth extraction
- Heavy periods and/or periods lasting more than 7 days
The most common complication of hemophilia is joint disease caused by bleeding into the joints and muscles. The pressure of blood filling the joint cavity causes severe pain and can lead to chronic swelling and deformity. Repeated bleeding into the same joint can cause joint damage and is most common in the elbows, ankles and knees.
For those with hemophilia A, Novo Nordisk recently announced ESPEROCT® [antihemophilic factor (recombinant), glycopegylated-exei] is now available in the U.S. for the treatment of adults and children with hemophilia A. ESPEROCT® is a recombinant extended half-life factor VIII replacement therapy used to prevent or reduce the number of bleeding episodes, to treat and control bleeding, and to manage bleeding during surgery in people with hemophilia A.
Von Willebrand disease (VWD) is the most common bleeding disorder affecting 3.3 million people in the U.S. Men and women are equally likely to have VWD, but women are more likely to notice the symptoms because of heavy or abnormal bleeding during their menstrual cycle or after childbirth.
Blood contains many proteins that help the body stop bleeding. One of these proteins is called von Willebrand factor (VWF). People with VWD either have a low level of VWF in their blood or the VWF protein doesn’t work the way it should and platelets do not form clots as they should. Although rare, bleeding can be severe enough to damage joints or internal organs, or even be life-threatening.
VWD is almost always passed down from a parent to a child, though it is possible for a person to get it without a family history of the disease. Rarely, a person who is not born with VWD can acquire it later in life. This typically happens when a person’s own immune system destroys his or her VWF, often as a result of a medication or another disease. If acquired, it cannot be passed along to future generations.
VWD Symptoms include:
- Frequent or hard-to-stop nosebleeds lasting more than 10 minutes
- Easy bruising, often with very little or no injury or trauma
- Heavy menstrual bleeding with clots larger than the size of a quarter are passed. This could lead to anemia.
- Longer than normal bleeding after injury, surgery, childbirth, or dental work. Bleeding can last more than five minutes, or may stop and start again hours or days later.
- Blood in the stool from bleeding into the stomach or intestines
- Blood in the urine from bleeding into the kidneys or bladder
- Bleeding into joints or internal organs in severe cases (Type 3)
Common treatments include:
Desmopressin Acetate Injection: (DDAVP®) is injected into a vein to treat people with milder forms of VWD (mainly type 1). It works by making the body release more VWF into the blood. It helps increase the level of factor VIII in the blood as well.
Desmopressin Acetate Nasal Spray: This high-strength nasal spray (Stimate®) is used to treat people with milder forms of VWD. It works by making the body release more VWF into the blood.
Factor Replacement Therapy: Recombinant VWF (such as Vonvendi®) and medicines rich in VWF and factor VIII (for example, Humate P®, Wilate®, Alphanate®, or Koate DVI®) are used to treat people with more severe forms of VWD or people with milder forms of VWD who do not respond well to the nasal spray. These medicines are injected into a vein in the arm to replace the missing factor in the blood.
Antifibrinolytic Drugs: These drugs (for example, Amicar®, Lysteda®) are either injected or taken orally to help slow or prevent the breakdown of blood clots.
Birth control pills can increase the levels of VWF and factor VIII in the blood and reduce menstrual blood loss. A doctor can prescribe these pills for women who have heavy menstrual bleeding.
If you think you have a bleeding disorder, seek medical attention. Your doctor will ask questions about personal and family histories of bleeding. They will also check for unusual bruising or other signs of recent bleeding.Because certain medications can cause bleeding, even among people without a bleeding disorder, the doctor will ask about recent or routine medications taken that could cause bleeding or make bleeding symptoms worse.
Bleeding disorders are treatable and most people live long healthy lives as long as they get the treatment needed.