Hemophilia is a rare inherited x-linked genetic blood-clotting disorder which means it is passed from the parents to their children through their genes.
Since it is an x-linked genetic disorder this most usually affects males. Female cases are tremendously rare. The disease condition occurs due to a defect in the blood coagulation process that enables blood to transform from liquid to a solid state.
The root cause is that Haemophiliacs are born with minimal or the lack of any clotting factors which are proteins that work in tandem with platelets to help the blood in its clotting mechanism.
Four types of Hemophilia
| Form of Hemophilia | Etiology |
| Hemophilia A (Classic) | factor VIII deficiency |
| Hemophilia B (Christmas D.) | factor IX deficiency |
| Hemophilia C | factor XI deficiency |
| Von Willebrand’s disease | factor VIII deficiency Von Willebrand factor deficiency |
Clinical Manifestations:
Even as children some haemophiliacs are already diagnosed of the condition and these manifestations may go on if not properly managed
• When a baby is able to crawl or cruise, bruises usually appear on some parts of the body.
• Incidentally diagnosed during a circumcision or tooth extraction wherein the kid bleeds excessively.
• Bleeding on other mucous membranes (usually nosebleeds)
• Heemorrhaging that occurs in areas of the body. (Most frequently at the patient’s joints elbows, shoulders, and wrist but especially on the hips and knees.)
• Hematomas
• Pain, swelling and decreased mobility of the joint.
• GI bleeding
• Hematuria
Medical Management:
• Clotting factor concentrates
• Aminocaproic acid─ antihemorrhagic/antifibrinolytic
• Desmopressin ─increases plasma levels of factor VIII
• Analgesics─ alleviate pain.
Nursing management:
1) Encourage the patient to be independent by giving him the discretion to stay away from activities that may create trauma or injury that may cause him to have bleeding episodes.
2) Provide health teaching about limitations to activities and proper care measures to minimize the chance of haemorrhage. The nurse should put SAFETY as a priority either at home or in the workplace.
3) Instruct to not take in any agents that interfere with platelet aggregation, such as aspirin, NSAIDs, herbs, nutritional supplements, and alcohol.
4) Encourage haemophilia patient to wear a medical identification band or card.
5) If possible injections or any other invasive procedures should not be done but if necessary it should be done right after the appropriate clotting factor replacement.
6) Nurses should always assess the patient’s surgical site for any bleeding right after undergoing a surgery. Monitoring of the vital signs should be done to ensure that there is no covert postoperative bleeding.
7) Hemophilia is not a treatable disease but it isn’t enough reason to sulk and be hopeless because with good management and proper self care living a full life with the condition can be attainable.