Hemophilia

Hemophilia is an inherited bleeding disorder.

Causes

Hemophilia is caused by a genetic defect. The genetic defect causes the body to produce too little of substances called clotting factors. Hemophilia is considered an x-linked genetic disease. This means that it is passed by a mother to her son. The most common types of hemophilia are: Hemophilia A (also called classic hemophilia) - caused by too little factor VIII Hemophilia B (also called Christmas disease) - caused by too little factor IX There are other types of hemophilia due to decreased amounts of other factors.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.
  • Family members with hemophilia
  • Sex: Male
  • Race: Ashkenzai Jewish ancestry (for factor XI hemophilia)

Symptoms

Symptoms may include:
  • Hot, swollen, sore, stiff, and/or deformed joints
  • Bleeding into muscles
  • Bleeding into the intestinal tract
  • Bloody stools
  • Bloody urine
  • Difficulty stopping bleeding after minor cuts or bumps
  • Heavy bleeding even after simple surgical or dental procedures

Diagnosis

The doctor will ask about your symptoms and medical history, and perform a physical exam.
Tests may include:
  • Tests of bleeding time
  • Blood tests to measure the amounts of clotting factors present

Treatment

Treatment includes:
Treatment with the Appropriate Factor
If you have hemophilia, you can be given blood factor concentrate through an IV. You'll be tested frequently to monitor your factor levels. When your factor levels drop too low, you'll be given a dose of factor concentrate.
Desmopressin

This medication can be given through an IV. It helps increase factor levels. It is often given before minor surgeries, to help decrease the chance of excess bleeding.
Aminocaproic Acid

This medication may be given if you are bleeding and neither factor concentrates nor desmopressin have been helpful.
Avoiding Certain Medications
Don't take products like aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) that might decrease your clotting factors and make you prone to bleeding.
Avoiding Contact Sports

Rough activities can put you at risk of serious bleeding.
Orthopedic Treatments
If youve had a lot of bleeding into your joints, you may need special treatment by an orthopedic specialist

Prevention

Because Hemophilia is a genetic disease, there is no known way to prevent it.