Hemophilia is an inherited bleeding disorder characterized by the body’s inability to form blood clots properly. This nursing diagnosis focuses on preventing bleeding episodes, managing acute bleeds, and promoting optimal quality of life for patients with hemophilia.
Causes (Related to)
Hemophilia can affect patients in various ways, with several factors influencing bleeding risk and disease management:
- Genetic mutations affecting clotting factors VIII (Hemophilia A) or IX (Hemophilia B)
- Family history of bleeding disorders
- Severity level (mild, moderate, or severe)
- Risk factors such as:
- Physical activity/trauma
- Surgery
- Dental procedures
- Poor adherence to prophylaxis
- Contributing factors including:
- Development of inhibitors
- Joint damage from repeated bleeds
- Limited access to treatment
- Poor understanding of the condition
Signs and Symptoms (As evidenced by)
Hemophilia presents with characteristic signs and symptoms that nurses must recognize for proper assessment and intervention.
Subjective: (Patient reports)
- Joint pain and stiffness
- Muscle pain
- History of easy bruising
- Prolonged bleeding from minor cuts
- Frequent nosebleeds
- Heavy menstrual bleeding in carriers
- Family history of bleeding disorders
Objective: (Nurse assesses)
- Visible bruising
- Swollen joints
- Limited range of motion
- Hematomas
- Prolonged bleeding time
- Abnormal coagulation test results
- Decreased hemoglobin/hematocrit
- Joint deformities in chronic cases
Expected Outcomes
The following outcomes indicate successful management of hemophilia:
- The patient will remain free from serious bleeding episodes
- The patient will demonstrate proper self-administration of factor replacement
- The patient will maintain joint function and mobility
- The patient will avoid complications
- The patient will participate in appropriate physical activities safely
- The patient will demonstrate knowledge of prevention strategies
- The patient will maintain a regular prophylaxis schedule if prescribed
Nursing Assessment
Monitor Bleeding Risk
- Assess for signs of active bleeding
- Monitor vital signs
- Check the skin for bruising
- Evaluate joint function
- Document bleeding episodes
Evaluate Treatment Adherence
- Review factor replacement schedule
- Check supply inventory
- Assess self-administration technique
- Monitor prophylaxis compliance
- Document factor levels
Assess Knowledge Level
- Evaluate understanding of the condition
- Check recognition of bleeding signs
- Review emergency protocols
- Assess the family support system
- Document educational needs
Nursing Care Plans
Nursing Care Plan 1: Risk for Bleeding
Nursing Diagnosis Statement:
Risk for Bleeding related to coagulation disorder as evidenced by deficient clotting factors and a history of prolonged bleeding.
Related Factors:
- Inherited coagulation defect
- Inadequate factor levels
- Physical activity risks
- History of trauma
Nursing Interventions and Rationales:
- Monitor for bleeding signs\
Rationale: Early detection allows prompt intervention - Administer factor replacement as prescribed\
Rationale: Maintains therapeutic factor levels - Teach injury prevention\
Rationale: Reduces bleeding risk
Desired Outcomes:
- The patient will remain free from serious bleeding episodes
- The patient will demonstrate the proper factor replacement technique
- The patient will identify early signs of bleeding
Nursing Care Plan 2: Impaired Physical Mobility
Nursing Diagnosis Statement:
Impaired Physical Mobility related to joint damage as evidenced by decreased range of motion and joint pain.
Related Factors:
- Chronic joint bleeds
- Joint inflammation
- Pain
- Fear of injury
Nursing Interventions and Rationales:
- Assess joint function regularly\
Rationale: Monitors disease progression - Implement a safe exercise program\
Rationale: Maintains joint function - Teach proper body mechanics\
Rationale: Prevents injury
Desired Outcomes:
- The patient will maintain optimal joint function
- The patient will participate in appropriate physical activities
- The patient will demonstrate proper body mechanics
Nursing Care Plan 3: Knowledge Deficit
Nursing Diagnosis Statement:
Knowledge Deficit related to complex treatment regimen as evidenced by verbalized confusion about management protocols.
Related Factors:
- Complex medical condition
- New diagnosis
- Limited exposure to information
- Language barriers
Nursing Interventions and Rationales:
- Provide comprehensive education\
Rationale: Improves self-management - Demonstrate treatment procedures\
Rationale: Ensures proper technique - Review emergency protocols\
Rationale: Prepares for complications
Desired Outcomes:
- The patient will verbalize understanding of the condition
- The patient will demonstrate proper treatment techniques
- The patient will identify emergencies
Nursing Care Plan 4: Risk for Ineffective Health Maintenance
Nursing Diagnosis Statement:
Risk for Ineffective Health Maintenance related to demanding treatment regimen as evidenced by missed prophylaxis doses.
Related Factors:
- Complex treatment schedule
- Cost concerns
- Limited support system
- Treatment fatigue
Nursing Interventions and Rationales:
- Develop treatment schedule\
Rationale: Promotes adherence - Connect with support resources\
Rationale: Provides assistance - Monitor compliance\
Rationale: Ensures treatment effectiveness
Desired Outcomes:
- The patient will maintain the treatment schedule
- The patient will utilize support resources
- The patient will demonstrate improved compliance
Nursing Care Plan 5: Anxiety
Nursing Diagnosis Statement:
Anxiety related to chronic condition management as evidenced by expressed concerns about bleeding risks.
Related Factors:
- Unpredictable disease course
- Fear of bleeding
- Activity restrictions
- Financial concerns
Nursing Interventions and Rationales:
- Provide emotional support\
Rationale: Reduces anxiety - Teach coping strategies\
Rationale: Improves stress management - Connect with support groups\
Rationale: Builds support network
Desired Outcomes:
- The patient will express decreased anxiety
- The patient will utilize coping strategies
- The patient will participate in support activities
References
- Ackley, B. J., Ladwig, G. B., Makic, M. B., Martinez-Kratz, M. R., & Zanotti, M. (2020). Nursing diagnoses handbook: An evidence-based guide to planning care. St. Louis, MO: Elsevier.
- Batorova, A., Boban, A., Brinza, M., Lissitchkov, T., Nemes, L., Preložnik, I. Z., Smejkal, P., Zozulya, N., & Windyga, J. (2022). Expert opinion on current and future prophylaxis therapies aimed at improving protection for people with hemophilia A. Journal of Medicine and Life, 15(4), 570. https://doi.org/10.25122/jml-2022-0103
- Berntorp E, Fischer K, Hart DP, Mancuso ME, Stephensen D, Shapiro AD, Blanchette V. Haemophilia. Nat Rev Dis Primers. 2021 Jun 24;7(1):45. doi: 10.1038/s41572-021-00278-x. PMID: 34168126.
- Dolan G, Benson G, Bowyer A, Eichler H, Hermans C, Jiménez-Yuste V, Ljung R, Pollard D, Santagostino E, Šalek SZ. Principles of care for acquired hemophilia. Eur J Haematol. 2021 Jun;106(6):762-773. doi: 10.1111/ejh.13592. Epub 2021 Mar 18. PMID: 33527471; PMCID: PMC8252574.
- Silvestri, L. A. (2020). Saunders comprehensive review for the NCLEX-RN examination. St. Louis, MO: Elsevier.
