Nursing Care Plan for Hemophilia

Pathophysiology

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.

Etiology

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.

Desired Outcome

Patient will be free from bleeding complications; patient will be educated on how to prevent injury and bleeding; patient will have optimal physical mobility

Hemophilia Nursing Care Plan

Subjective Data:

  • Irritability (infants)
  • Pain or tightness in the joints
  • Sleepiness or lethargy
  • Double vision

Objective Data:

  • Large or deep bruises
  • Unexplained nosebleeds
  • Blood in urine or stool
  • Excessive bleeding from cuts or after dental work

Nursing Interventions and Rationales

  1. Assess patient for signs of bleeding; cuts, scrapes, bruises, swollen joints

  2. Patients often experience deep bruising from minimal contact or minor injuries. The deep bruising may lead to bleeding into joint spaces and vital organs.

  3. Assess patient for evidence of pain (non-verbal cues)

  4. 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.

  5. Provide passive ROM exercises

  6. 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

  7. Provide assistive devices as required

  8. Physical deformity of joints may occur due to bleeds into joint, which  may cause limited mobility. Provide assistance and devices as necessary.

  9. Initiate bleeding precautions per facility protocol

  10. 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

  11. Monitor coagulation tests; hemoglobin and hematocrit levels

  12. Monitor for effectiveness of interventions and therapeutic levels of medication and supplements

    Expect bleeding times to be reduced from the client’s baseline

  13. Anticipate need and administer blood products as required

  14. Excessive blood loss may require transfusions; have O-neg blood immediately available in case of hemorrhage

  15. Avoid unnecessary invasive tests and techniques

  16. 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

  17. Administer medications orally as available; administer replacement clotting factors as necessary

  18. Replacement of clotting factors is the primary treatment for hemophilia; other supplements, antibodies and antifibrinolytics may be required

  19. Educate patient and caregivers on safety and prevention of injury

  20. 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


References

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