The symptoms of hemophilia may not develop for many years and they vary in severity depending on how deficient clotting factors are. However, the main symptom is prolonged bleeding after an injury.
Children who have mild hemophilia often do not present with symptoms until a particular event arouses suspicion that a bleeding disorder is present. However, most cases of hemophilia are severe and symptoms may be noticed in the following situations:
- In cases of assisted delivery such as vacuum extraction or forceps delivery, a baby with hemophilia is usually born with extensive head bruising or hematomas. Severe bleeding within the brain may also occur, which is referred to as intracranial hemorrhage.
- Early circumcision during the newborn period may also lead to suspicion of hemophilia if the wound fails to stop bleeding.
- In girls, heavy periods can occur as a result of the disease. This can happen when girls are only carriers of the condition.
- Among children with mild hemophilia, the condition may only become apparent after a significant injury, surgical procedure or tooth extraction, for example. Mild hemophilia is defined as a factor VIII level of between 5% and 40%.
- Among those with moderate hemophilia, injury or surgery may cause prolonged bleeding but these children will also bruise easily and be prone to joint bleeds if they knock themselves or fall. Moderate hemophilia is defined as a factor VIII level of between 1% and 5%.
- In cases of severe hemophilia, external and internal bleeding can occur spontaneously. Bleeding may take the form of nosebleeds, joint bleeds, muscle bleeding or bleeding gums, for example. If hemophilia is poorly controlled, internal bleeding can occur around the joints and muscles leading to pain and stiffness. Eventually, bleeding in the joints can damage cartilage, which is the soft tissue that surrounds joints and acts as a shock absorber. The synovium, a tissue layer inside the joint can also become damaged. Severe hemophilia is defined as a factor VIII level of less than 1%.
Reviewed by Sally Robertson, BSc