Hemophilia Medication Plan: All About Hemophilia
Hemophilia is not a single condition, but rather an inherited group of bleeding disorders in which normal blood clotting is impaired. Hemophilia is a treatable, but lifelong disorder. Doctors typically prescribe a hemophilia medication plan, which includes what is known as replacement therapy to help manage the symptoms of hemophilia.
How Many People have Hemophilia?
A rare disorder, hemophilia A, occurs in about one in five thousand live male births. Hemophilia A is roughly four times more common than hemophilia B, which occurs in about one in fifty thousand live male births.
Because certain clotting factors are absent, patients with hemophilia can experience excessive or exaggerated bleeding (internally or externally), even from minor injuries such as small cuts.
Can Girls Develop Hemophilia?
Although girls can develop hemophilia, it is extremely rare in females. Females do not usually develop symptoms of the disease, but they can carry the specific genetic mutation that causes hemophilia. Some, called symptomatic carriers, do experience what are usually milder symptoms of hemophilia.
The genetic mutation that causes hemophilia is passed down on the X chromosome. (Females typically have two X chromosomes. Males usually have and X and a Y chromosome.) If a mother carries the gene for hemophilia, any sons she gives birth to will have a 50% chance of developing the disorder. Additionally, any daughters she may have will also have 50% chance of being a carrier themselves.
What are the Missing Blood Clotting Factors in Hemophilia?
There are several missing blood factors that can cause bleeding disorders such as hemophilia. These include factors: I, II, V, VII, VIII X, XI, XIII. In rare cases, people who do not have hemophilia can develop it.
Known as acquired hemophilia, it is usually related to the development of certain antibodies to clotting factors. This form of hemophilia can develop during later stages of pregnancy or after giving birth.
How Does Blood Clot?
Normal blood has 13 clotting factors which work together to stop bleeding when the body experiences damage to a blood vessel. If a cut or other damage occurs, the body reacts very quickly to minimize blood loss, triggering what is known as a clotting cascade.
In healthy people, the clotting process begins with small specialized blood cells, called platelets. They rush to the site, clumping together to form a plug that quickly seals the leak. Within a matter minutes, other clotting components, circulating with the bloodstream are attracted by the newly formed clot. Very soon afterwards they begin to form a much stronger clot using a specialized blood protein called fibrogen.
How is Hemophilia Diagnosed?
Hemophilia is typically diagnosed with lab tests that measure the level of certain clotting factors in the blood. Hemophilia has a strong genetic component, so you should advise your doctor if you have a family history of the disorder.
Depending on which factor is missing or low, the doctor will diagnose the specific form of hemophilia. Hemophilia can range from mild to severe forms. The exact hemophilia medication plan your doctor recommends will depend on the form and severity of the disease.
If an expectant mother is a known carrier, tests can be done prior to the baby’s birth. These include a testing method known as chorionic villus sampling for early pregnancy (9-11 weeks) or by taking fetal blood samples if the pregnancy is at a later state (18 or more weeks) of gestation.
If a child is diagnosed with hemophilia, a hemophilia medication plan is usually developed to help lessen the impact of the disease. This often includes prophylaxis (preventative) infusion therapy prior to dental work, surgery, or other activities. Other supportive medications may also be required.
People with hemophilia may experience spontaneous bleeds within the body in areas such as joints or muscle groups. People with hemophilia can bleed internally or externally.
Bleeding often occurs inside the body including muscles and joints. Common internal muscle bleed areas include: biceps, calves, groin, thigh, ankles, knees, elbows, and shoulders.
Because repeated internal bleeds can damage joints and tissues, patients with hemophilia may have trouble walking, or performing other daily activities. Symptoms for both hemophilia A and hemophilia B typically include:
• Large bruises
• Bruising Easily
• Prolonged bleeding
• Pain and stiffness in joints or difficulty moving joints or muscles
• In females, hemophilia can cause excessive vaginal bleeding, with or without, clots
What is Replacement Therapy for Hemophilia?
Hemophilia replacement therapy is designed to artificially replace the appropriate missing clotting factors with synthetically manufactured ones, or with concentrates derived from donated human plasma. Depending on the specific form of the disorder, treatment typically includes infusions of Factor VIII (for hemophilia A), or Factor IX (for hemophilia B).
Administered via iv infusion, hemophilia clotting factors can be given as an on-demand therapy to help replace blood and control further bleeding after an incident. They can also be used as a preventative therapy to help reduce the chance of future bleeding events.
Replacement therapy may be given in a doctor’s office, or at home. Because clotting factors are eliminated from the body within hours to days (depending on the specific clotting factor), they must be replaced on a regular basis.
Common Hemophilia Medications
Hemophilia medication plans commonly include medications designed to improve blood clotting. Other supportive drugs may also be used to minimize symptoms related to the disease. These may include:
Hemophilia Medication Plans and Support
At ASP Cares, we understand the challenges that come from living with hemophilia and other clotting disorders. Our specialty trained pharmacists are uniquely qualified to help patients thrive and enhance their quality of life. Click here
This content does not represent medical advice. It is not meant to diagnose or treat any condition. Nor is intended as a substitute for the advice of a qualified medical provider.