Hemophilia is a genetic bleeding disorder that is characterized by a deficiency of clotting protein. Patients with hemophilia experience longer bleeding time than others because their blood clots much slower. The amount of clotting protein available in the blood determines the severity of hemophilia and categorizes it into three levels: mild, moderate and severe. Complications of the disease include bleeding into the joints, hemorrhage into the central nervous system or vital organs, and aspiration from bleeding into the airways.
Hemophilia is genetically (X) linked with females being the carriers, and males being affected by the disease. There are two types of hemophilia. Type A (classic) is caused by a deficiency of Factor VIII (8) clotting protein and is the most common, affecting 1 in every 10,000 males. Type B (Christmas disease) is caused by a deficiency of Factor IX (9) and affects 1 in every 30,000 males.
Patient will be free from bleeding complications; patient will be educated on how to prevent injury and bleeding; patient will have optimal physical mobility
Assess patient for signs of bleeding; cuts, scrapes, bruises, swollen joints
Patients often experience deep bruising from minimal contact or minor injuries. The deep bruising may lead to bleeding into joint spaces and vital organs.
Assess patient for evidence of pain (non-verbal cues)
Patients may report painful joints or aching muscles; younger children may not know how to express pain and exhibit symptoms through guarding or irritability and fussiness.
Provide passive ROM exercises
This is not advised during acute phase or with active bleeding, but encouraged when patient’s condition is stable to maintain or improve joint and muscle mobility
Provide assistive devices as required
Physical deformity of joints may occur due to bleeds into joint, which may cause limited mobility. Provide assistance and devices as necessary.
Initiate bleeding precautions per facility protocol
Small and simple injuries may cause excessive bleeding. Client should avoid blade razors (use electric razors), use soft-bristle toothbrush, and avoid blowing nose during nosebleeds
Monitor coagulation tests; hemoglobin and hematocrit levels
Monitor for effectiveness of interventions and therapeutic levels of medication and supplements
Expect bleeding times to be reduced from the client’s baseline
Anticipate need and administer blood products as required
Excessive blood loss may require transfusions; have O-neg blood immediately available in case of hemorrhage
Avoid unnecessary invasive tests and techniques
Avoid heel or finger sticks and IM injections if possible
Be prepared for excessive bleeding with administration of vaccines (opt for subcutaneous routes if available).
Apply pressure to site for several minutes to allow time to clot
Administer medications orally as available; administer replacement clotting factors as necessary
Replacement of clotting factors is the primary treatment for hemophilia; other supplements, antibodies and antifibrinolytics may be required
Educate patient and caregivers on safety and prevention of injury
Prevention of injury is most important; patient should avoid physical contact sports and high risk activities; use soft toothbrush, give appropriate (not sharp-edged) toys, use helmets and padding for sports, supervise playtimes
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