Hemophilia is a rare, inherited disorder that prevents the blood from clotting normally.
Hemophilia patients may bleed for a longer time than others after an injury. They may also experience internal bleeding, especially in the knees, ankles and elbows, which can damage organs and tissue.
People born with hemophilia have little or no clotting factor, a protein needed for normal blood clotting. There are different types of the protein. To clot, the blood needs the proteins to work with platelets, small blood cell fragments that form in bone marrow.
There are two main types of hemophilia: A and B. If you have hemophilia A, you’re missing, or have low levels of, clotting factor VIII. About 80 percent of patients have type A hemophilia. If you have hemophilia B, you’re missing, or have low levels of, clotting factor IX.
On rare occasions, hemophilia occurs without being passed on through genes. This happens if our bodies form antibodies that attack clotting factors in our bloodstream.
Hemophilia can be mild, moderate or severe, depending on how much clotting factor is in our blood. About 70 percent of hemophilia A patients have a severe form of the disease.
Hemophilia occurs more often in men. About 1,500 American men are born with hemophilia annually, according to the National Heart, Lung and Blood Institute.
Signs and Symptoms of Hemophilia
The major signs of hemophilia are excessive bleeding and easy bruising. The extent of bleeding depends on how severe the disease is.
Children with mild hemophilia may not show signs unless they experience excessive bleeding from a dental procedure, an accident or surgery.
Signs of external bleeding may include:
- Bleeding in the mouth from a cut or bite or from cutting or losing a tooth.
- Nosebleeds for no obvious reason.
- Heavy bleeding from a small cut.
- Bleeding from a cut that resumes after stopping for a short period.
Signs of internal bleeding may include:
- Blood in the urine, from bleeding in the kidneys or bladder.
- Blood in the stool, from bleeding in the intestines or stomach.
- Large bruises, from bleeding in the large muscles of the body.
- Bleeding in the joints, which at first causes tightness in the joint without pain or visible signs of bleeding. The joint can become swollen, hot and painful to bend. Swelling can continue as the bleeding continues. A person with bleeding in a joint can eventually lose movement temporarily, and experience severe pain and joint damage.
- Bleeding in the brain after a bump on the head or a more serious injury. It lead to long-lasting, painful headaches or neck pain, repeated vomiting, sleepiness or mood changes, sudden weakness or clumsiness in arms or legs, problems walking, double vision, and convulsions or seizures.
When to see a doctor
With hemophilia, there’s a slight risk of bleeding inside the skull, known as intracranial hemorrhage. It’s estimated that 3 percent of moderate-to-severe hemophilia patients will experience an intracranial hemorrhage. It can result from a head injury or, rarely, can occur spontaneously.
In this case, you should treat the hemorrhage as a medical emergency get help immediately. Call 911 or a medical emergency number as soon as possible.
Symptoms of an intracranial hemorrhage include:
- Severe headache.
- Stiff neck.
- Change in mental state, such as feeling confused.
- Difficulty speaking, such as slurred speech.
- Changes in vision, such as double vision.
- Loss of co-ordination and balance.
- Paralysis of some, or all, facial muscles.