Nursing Care for Patients with Polycythemia Vera: A Comprehensive Guide

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by the overproduction of red blood cells in the bone marrow. While conditions like anemia and sickle cell disease are marked by a deficit in red blood cells, polycythemia vera presents the opposite problem – an excess. This overabundance of red blood cells, often coupled with increased white blood cells and platelets, leads to hyperviscosity of the blood and a cascade of potential complications. Understanding the specific nursing cares for a client diagnosed with polycythemia vera is crucial for effective management and improved patient outcomes. This article delves into the essential aspects of nursing care for individuals living with polycythemia vera, aiming to provide a comprehensive resource for healthcare professionals.

Polycythemia can be categorized into two main types: primary and secondary. Primary polycythemia, or polycythemia vera (PV), is a result of a genetic mutation, most commonly in the JAK2 gene, leading to uncontrolled blood cell production in the bone marrow. Exposure to radiation or certain chemicals like benzene has also been linked to PV. In contrast, secondary polycythemia is triggered by underlying conditions that cause chronic hypoxia, such as chronic obstructive pulmonary disease (COPD), leading to the body producing more red blood cells to compensate for low oxygen levels.

Assessment of Polycythemia Vera

A thorough assessment is the cornerstone of effective nursing care. Clients with polycythemia vera can present with a wide range of symptoms affecting various body systems. Recognizing these manifestations is vital for early identification and intervention.

Physical Examination Findings

The symptoms of polycythemia vera are diverse and can impact multiple organ systems. Table 1 outlines common clinical manifestations and physical assessment findings across different body systems.

Table 1: Common Symptoms of Polycythemia Vera Across Body Systems

Hands and feet showing redness and pain due to Erythromelalgia

Figure 1: Erythromelalgia, characterized by redness and burning pain in the extremities, is a notable skin manifestation of Polycythemia Vera.

Diagnostic Evaluation

The initial step in diagnosing polycythemia vera is typically a Complete Blood Count (CBC). In PV, the CBC reveals elevated levels of red blood cells, white blood cells, and platelets. Hematocrit and hemoglobin levels are also characteristically increased. It’s important to consult the normal reference ranges for these tests, such as those found in “Appendix A – Normal Reference Ranges,” to accurately interpret CBC results.

Confirmation of polycythemia vera often involves further testing to detect the JAK2V617F mutation. This genetic mutation, present in approximately 95% of PV cases, affects the JAK2 gene on Chromosome 9, which plays a crucial role in blood cell regulation and production. In addition to genetic testing, a bone marrow aspiration and biopsy may be performed to evaluate the bone marrow and rule out other conditions.

Nursing Diagnoses for Polycythemia Vera

Nursing diagnoses for clients with polycythemia vera are individualized and derived from a comprehensive assessment of the patient’s condition, medical history, and specific needs. These diagnoses guide the development of a personalized care plan.

Common nursing diagnoses in polycythemia vera include:

• Risk for Thrombosis: Related to hyperviscosity of blood and increased platelet count.
• Impaired Gas Exchange: Related to increased blood viscosity and potential for pulmonary complications.
• Activity Intolerance: Related to fatigue and potential cardiopulmonary limitations.
• Deficient Knowledge: Related to the disease process, treatment regimen, and self-management strategies.
• Ineffective Coping: Related to the chronic nature of the illness and its impact on lifestyle and well-being.

Outcome Identification and Planning

Outcome identification involves setting both short-term and long-term goals in collaboration with the client. These goals are translated into specific, measurable, achievable, relevant, and time-bound (SMART) expected outcome statements. These statements serve as benchmarks for evaluating the effectiveness of nursing interventions.

Examples of expected outcomes for clients with polycythemia vera based on common nursing diagnoses are:

• The client will maintain adequate hydration as evidenced by moist mucous membranes, good skin turgor, and urine output within normal limits. (Addresses Risk for Thrombosis)
• The client will demonstrate understanding of prescribed phlebotomy schedule and its importance in managing PV by verbalizing the plan and keeping appointments. (Addresses Deficient Knowledge, Risk for Thrombosis)
• The client will report decreased fatigue and improved energy levels sufficient to perform desired daily activities within 1 week. (Addresses Activity Intolerance)
• The client will verbalize effective coping strategies and support systems to manage the emotional impact of living with a chronic illness by discharge. (Addresses Ineffective Coping)

Nursing Interventions for Polycythemia Vera

Nursing interventions for polycythemia vera are multifaceted, focusing on preventing complications, managing symptoms, and enhancing the client’s quality of life.

Medical and Collaborative Interventions

While medical interventions are primarily physician-directed, nurses play a critical role in implementing, monitoring, and reinforcing these treatments. Common medical interventions include:

• Therapeutic Phlebotomy with or without Plasmapheresis: Phlebotomy is the cornerstone of PV management. Regularly scheduled phlebotomy involves removing a unit of blood to reduce red blood cell mass and blood viscosity. Plasmapheresis, which removes red blood cells and returns plasma to the client, may be used in some cases. Nurses are responsible for preparing the client for phlebotomy, monitoring vital signs during and after the procedure, and educating the client about the importance of adherence to the phlebotomy schedule.
• Low-Dose Aspirin Therapy: Low-dose aspirin is often prescribed to reduce the risk of thrombotic events by inhibiting platelet aggregation. Nurses administer aspirin as ordered, monitor for signs of bleeding, and educate clients about the importance of taking aspirin as prescribed and reporting any unusual bleeding or bruising.
• Cytoreductive Medications: For clients at higher risk of thrombosis or those with significant symptoms, cytoreductive therapy may be initiated. Hydroxyurea is a commonly used medication to suppress bone marrow overproduction of blood cells. Nurses administer these medications, monitor for side effects (such as myelosuppression, mucositis, and skin changes), and educate clients on potential side effects and the importance of regular blood count monitoring.
• JAK2 Inhibitors: For patients with the JAK2V617F mutation, JAK2 inhibitors like ruxolitinib may be used to target the underlying genetic abnormality and control abnormal cell growth. Nurses administer these medications, monitor for specific side effects (including thrombocytopenia, anemia, and infections), and provide education about medication management and potential adverse reactions.
• Anticoagulation Therapy: In clients with a history of thrombosis or at high risk, anticoagulant medications such as warfarin or heparin may be prescribed. Nurses are responsible for the safe administration of anticoagulants, close monitoring of coagulation studies (e.g., INR, aPTT), assessment for signs of bleeding or bruising, and client education regarding anticoagulant therapy and safety precautions.

Nursing-Specific Interventions

In addition to supporting medical treatments, specific nursing interventions are essential for holistic care:

• Preventing Thrombosis:

  • Promote Hydration: Encourage adequate fluid intake (2-3 liters per day, unless contraindicated) to reduce blood viscosity.
  • Encourage Mobility: Promote regular ambulation and leg exercises, especially for hospitalized or immobile clients, to prevent venous stasis.
  • Administer Anticoagulation as Prescribed: Ensure timely and accurate administration of prescribed anticoagulants and monitor for therapeutic and adverse effects.
  • Educate on Signs and Symptoms of Thrombosis: Teach clients to recognize and report signs of deep vein thrombosis (DVT), such as leg pain, swelling, and redness, and pulmonary embolism (PE), such as sudden shortness of breath, chest pain, and cough.

• Ensuring Adequate Oxygenation:

  • Monitor Respiratory Status: Regularly assess respiratory rate, depth, and oxygen saturation. Auscultate lung sounds for adventitious sounds.
  • Administer Supplemental Oxygen as Needed: Provide oxygen therapy as prescribed to maintain adequate oxygen saturation levels, particularly if the client experiences dyspnea or decreased oxygen saturation.
  • Elevate Head of Bed: Position the client in a semi-Fowler’s or Fowler’s position to promote optimal lung expansion.
  • Encourage Deep Breathing and Coughing Exercises: Teach and encourage these exercises to prevent atelectasis and promote airway clearance.

• Assisting with Energy Management:

  • Balance Activity and Rest: Help clients plan and prioritize activities, incorporating rest periods to minimize fatigue.
  • Promote Adequate Sleep: Create a conducive sleep environment and implement strategies to improve sleep quality.
  • Encourage Regular Exercise: Advise on appropriate exercise within tolerance levels to improve energy and overall well-being.
  • Address Factors Contributing to Fatigue: Assess and address other potential contributing factors to fatigue, such as anemia, pain, and psychological distress.

• Managing Pruritus:

  • Skin Care: Advise on gentle skin care practices, including using mild, non-drying soaps, avoiding hot baths or showers, and patting skin dry rather than rubbing.
  • Emollients: Recommend frequent application of moisturizing lotions or creams to relieve dry skin and itching.
  • Cool Compresses: Suggest cool compresses or cool baths to soothe itchy skin.
  • Pharmacological Interventions: Administer antihistamines or other antipruritic medications as prescribed.

• Providing Health Teaching and Health Promotion:

  • Disease Education: Provide comprehensive education about polycythemia vera, including its pathophysiology, symptoms, treatment options, and potential complications.
  • Medication Management: Educate clients about prescribed medications, including dosage, administration, potential side effects, and the importance of adherence.
  • Importance of Phlebotomy: Emphasize the critical role of routine phlebotomy in managing PV and preventing complications.
  • Lifestyle Modifications: Discuss lifestyle modifications that can support health, such as smoking cessation, weight management, and stress reduction techniques.
  • Recognizing and Reporting Symptoms: Instruct clients on recognizing and promptly reporting any new or worsening symptoms, such as chest pain, shortness of breath, neurological changes, or bleeding.

Evaluation of Nursing Care

Evaluation is an ongoing process in nursing care. Nurses continuously assess the effectiveness of interventions by monitoring the client’s response to treatment and progress toward achieving expected outcomes. This involves:

• Regularly reviewing expected outcomes: Determine if outcomes have been met, partially met, or not met within established timeframes.
• Monitoring for symptom management: Assess the client’s reports of fatigue, pruritus, headaches, and other symptoms, and evaluate the effectiveness of interventions in alleviating these symptoms.
• Evaluating for prevention of complications: Monitor for signs and symptoms of thrombosis, bleeding, infection, and other potential complications.
• Reviewing laboratory and diagnostic results: Track CBC results, coagulation studies, and other relevant laboratory values to assess the effectiveness of medical and nursing interventions.
• Seeking client feedback: Incorporate client feedback to understand their experiences, identify areas for improvement, and ensure care is patient-centered.

If outcomes are not being met or are only partially met, the nursing care plan should be revised in collaboration with the client and the healthcare team. This iterative process of assessment, intervention, and evaluation ensures that nursing care remains responsive to the client’s evolving needs.

RN Recap: Polycythemia Vera

Polycythemia vera is a complex hematological disorder requiring comprehensive nursing care. By understanding the pathophysiology, assessment findings, nursing diagnoses, and evidence-based interventions, nurses can significantly contribute to improving the health and well-being of individuals living with PV. Effective nursing care focuses on preventing life-threatening complications like thrombosis, managing debilitating symptoms, and empowering clients through education and support to actively participate in their care.

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[8] “Erythromelalgia.jpg” by Herbert L. Fred, MD and Hendrik A. van Dijk is licensed under CC BY 2.0

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[16] Open RN Project. (2024, June 23). Health Alterations – Chapter 3 Hematological – Polycythemia [Video]. YouTube. CC BY-NC 4.0 https://youtu.be/n-1241VmL00?feature=shared