Hemophilia is a genetic bleeding disorder characterized by the body’s inability to form blood clots effectively due to insufficient clotting factors. These proteins are essential for the coagulation process, working with platelets to stop bleeding. Individuals with hemophilia can experience prolonged bleeding even from minor injuries, or spontaneous bleeding without an obvious cause.
Managing External Bleeding
For visible external bleeds, such as cuts or nosebleeds, prompt first aid helps control the situation. Apply direct, firm, continuous pressure to the wound using a clean cloth or sterile gauze. If blood soaks through, place another layer on top and maintain pressure without lifting the original dressing. Sustain pressure for at least 5 to 10 minutes to allow a clot to begin forming.
Elevating the injured body part above the heart, if possible, helps slow bleeding by reducing blood flow. Applying a wrapped ice pack to the affected site can constrict blood vessels, which also helps to slow bleeding and reduce swelling. Apply ice for short durations, such as 10 minutes every two hours, and never directly on the skin. For small cuts and scrapes, these measures are often sufficient, but if bleeding persists, medical attention may be necessary.
Identifying Internal Bleeding
Internal bleeding is often less obvious than external bleeding but poses a greater risk, requiring careful monitoring and prompt intervention. Joint bleeds are common, frequently affecting the knees, elbows, and ankles, but also occurring in shoulders, wrists, and hips. Initial signs may include a tingling sensation, stiffness, or discomfort in the joint, often before visible swelling appears. As bleeding continues, the joint may become warm to the touch, swollen, painful, and exhibit a reduced range of motion.
Muscle bleeds are another frequent internal bleeding site, sometimes presenting as deep bruising, tightness, or pain. Large muscles, such as those in the thighs or groin (iliopsoas muscle), can accumulate significant amounts of blood, potentially leading to nerve compression and severe complications if untreated. Symptoms like hip flexion difficulty, groin pain, or numbness/tingling in the thigh can indicate an iliopsoas bleed.
More serious internal bleeds include those in the head or abdomen, which are considered emergencies. Signs of a head bleed can include a persistent or worsening headache, repeated vomiting, confusion, drowsiness, changes in vision, or seizures. Abdominal bleeds may manifest as abdominal pain, distension, vomiting blood, or blood in the stool, which might appear black and tarry. Any suspicion of these severe internal bleeds warrants immediate medical evaluation.
Factor Replacement Therapy and Other Medications
Factor replacement therapy is the primary medical intervention for managing bleeding episodes in individuals with hemophilia. This treatment involves infusing the specific clotting factor that is deficient or missing, typically Factor VIII for Hemophilia A or Factor IX for Hemophilia B. These infusions provide the necessary proteins to enable proper blood clot formation, stopping the bleed. Factor concentrates can be derived from human plasma or produced synthetically (recombinant factors), with recombinant options carrying a lower risk of infection.
Individuals with hemophilia or their caregivers are often trained to administer factor concentrates at home for prompt treatment. This immediate administration can reduce pain, recovery time, and potential long-term damage. Other medications can also support clot stability. Antifibrinolytic agents, such as tranexamic acid or epsilon-aminocaproic acid, help to prevent the breakdown of existing clots, which is particularly useful for bleeds in mucous membranes like the mouth or nose. For some individuals with mild Hemophilia A, desmopressin (DDAVP), a synthetic hormone, can be used to stimulate the release of stored Factor VIII, temporarily increasing clotting factor levels.
When to Contact Medical Professionals
Seek professional medical attention if a bleeding episode does not stop with initial home treatment measures, such as sustained pressure and ice. Any suspected internal bleeding, particularly in sensitive areas like the head, neck, or abdomen, requires urgent care due to the potential for serious complications. Signs such as severe pain, new neurological symptoms, or difficulty breathing are indicators for immediate medical attention.
Contact medical professionals if factor concentrate is unavailable or if the individual is unable to administer it. Have an emergency plan in place, including contact information for a hematologist or hemophilia treatment center, to facilitate timely and appropriate care. Even seemingly minor injuries or symptoms, if they cause concern or persist, should prompt communication with healthcare providers.