Learning Objectives
By the end of this section, you will be able to:
- Discuss the pathophysiology, risk factors, and clinical manifestations associated with polycythemia
- Describe the diagnostics and laboratory values in polycythemia
- Apply nursing concepts and plan associated nursing care for the patient with polycythemia
- Evaluate the efficacy of nursing care for the patient with polycythemia
- Describe the medical therapies for the care of polycythemia
A hematologic condition marked by an abnormal increase in the number of RBCs in the body is called polycythemia. This condition can be primary (genetic) or secondary (caused by other diseases or conditions, such as chronic lung disease, kidney disease, cardiopulmonary shunts) (Haider & Anwar, 2023). Polycythemia increases the blood’s viscosity (thickness), resulting in complications including blood clots, cardiovascular issues, and reduced circulation. When caring for patients with polycythemia, the primary objectives are to identify its underlying causes, take appropriate diagnostic steps, and devise tailored management strategies to minimize potential risks while increasing overall well-being for patients.
Pathophysiology of Polycythemia
The pathophysiology of polycythemia can be divided into two major subcategories: primary and secondary. Primary polycythemia, or polycythemia vera, is a myeloproliferative disorder, which means it originates from the bone marrow (Pillai et al., 2023). A genetic mutation causes an abnormal response to the hormone EPO, an important hormone that stimulates RBC production from the kidneys, causing erythropoiesis (RBC production) to increase independently of the body’s natural regulatory mechanisms (Pillai et al., 2023). Overproduction of RBCs increases blood viscosity, impairing blood flow and increasing the risk for thrombosis, stroke, or myocardial infarction.
When EPO stimulation occurs as an adaptive reaction to other conditions, secondary polycythemia occurs. Recall that EPO (erythropoietin) is normally released by the kidneys as a response to low oxygen levels in blood. The abnormal release triggers the bone marrow to increase RBC production. This can lead to impaired blood flow, with potential complications including stroke or myocardial infarction. Conditions that can trigger secondary polycythemia include chronic hypoxia from respiratory or cardiovascular illnesses, congenital heart disease, and certain tumors such as renal cell carcinoma. Smoking and living at high elevation can also trigger excess RBC production.
Primary and secondary polycythemia both can result in complications arising from increased blood viscosity, making it imperative that its root causes be addressed to reduce any risk for thrombotic events. Treatment strategies may include phlebotomy to lower RBC counts, medication to address symptoms, and management of any secondary conditions underlying polycythemia. An interdisciplinarian team approach is key to optimize management and avoid possible complications related to polycythemia.
Clinical Manifestations
Clinical presentation of polycythemia varies from patient to patient. Symptoms may be nonspecific and may include fatigue, headache, and dizziness (Haider & Anwar, 2023). If large vessel occlusion occurs due to clumping of excess blood cells, symptoms will result in the occluded vessel. For example, if viscous blood clumps and creates a clot in a coronary artery, the patient may report chest pain, shortness of breath, fatigue, or nausea. If a clot develops in a brain artery, the patient may experience weakness (generalized or one sided), visual changes, or difficulty with speech.
Diagnostics and Laboratory Values
Diagnosing polycythemia requires performing various laboratory and diagnostic procedures that assess RBC counts as well as determine their source. Primary polycythemia often results in lower or normal EPO levels, while secondary polycythemia usually causes an increase. The following tests are used to help diagnose either type of polycythemia:
- A CBC count identifies Hb and Hct levels and compares them with the normal ranges. It also measures RBCs.
- Bone marrow aspiration and biopsy may be performed to evaluate the composition of blood cells as well as rule out other conditions that may exist in the bone marrow. Primary polycythemia affects bone marrow with increased cell volume due to increased RBC precursors.
- For accurate detection of primary polycythemia, genetic tests must look specifically for JAK2 mutations. An extensive diagnostic approach is key to distinguish primary from secondary polycythemia and provide appropriate management strategies.
- A simple pulse oximeter can measure the presence of hypoxia, which can stimulate RBC production and lead to secondary polycythemia.
- Chest x-rays and computed tomography (CT) scans can help identify lung diseases or tumors, which may be a cause of secondary polycythemia. Ultrasound or magnetic resonance imaging visual tests can also identify organ enlargement, such as of the spleen or liver, resulting from polycythemia.
- An arterial blood gas analysis can give information about blood pH and oxygenation. These data can help identify secondary causes of polycythemia, which may be causing the body to produce more RBCs.
- The cause of a thrombotic event can be identified by testing for clotting factors and blood components.
An extensive diagnostic approach is key to distinguishing primary from secondary polycythemia and providing appropriate management strategies. Patients suspected of polycythemia should undergo extensive evaluation by health-care providers.
Nursing Care of the Patient with Polycythemia
Nurses play an invaluable role in recognizing and interpreting signs in patients experiencing polycythemia. Through diagnostic studies, the nurse can recognize the overproduction of RBCs. Nursing actions include monitoring vital signs and clinical manifestations, completing a physical exam, and administering ordered treatments.
Recognizing and Analyzing Cues
By completing vital signs and a thorough head-to-toe assessment, nurses can recognize cues from each body system suggesting the existence of polycythemia (Table 16.3). A subjective assessment may include the patient stating feelings of fatigue, early satiety (feeling full), abdominal pain, inactivity, problems with concentration, night sweats, pruritus, bone pain, weight loss, and fever. Elevations in Hb, Hct, and EPO levels are particularly important when identifying polycythemia. Additional cues may be obtained from a thorough patient history, which should include questions about the patient’s history of smoking and altitude exposure.
| System | Symptoms |
|---|---|
| General | Fatigue |
| Integumentary | Erythromelalgia (redness and a burning sensation), often of hands and feet Pruritus (itching) due to histamine release |
| Neurologic | Headache Visual disturbances, sometimes resulting in dizziness or disorientation Cognitive changes due to poor blood flow to the brain |
| Hematologic | Easy bruising and bleeding Increased occurrence of blood clots |
| Cardiovascular | Hypertension Peripheral edema caused by increased blood flow congestion |
| Respiratory | Dyspnea (shortness of breath) |
| Gastrointestinal | Hepatomegaly or splenomegaly due to increased blood volume |
| Renal | Increased urine flow due to increased blood volume |
| Musculoskeletal | Joint pain |
Prioritizing Hypotheses, Generating Solutions, and Taking Action
Acute management of polycythemia centers around treating the cause, if possible; preventing thrombotic events due to the overproduction of RBCs; and symptom improvement. Dependent on the degree of symptoms, nursing interventions are tailored to the patient’s presentation (Brennan-Cook, 2020).
Link to Learning
The Oncology Nursing Society endorses the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS) tool. This allows patients to track symptoms to report to the nurse and health-care team, who can then evaluate the severity of symptoms and create an effective plan of care.
Ultimately, nurses assist patients with symptom control and reduction of thrombotic risk (Lu & Chang, 2023). Education is important. For example, the patient might be taught to slowly increase their activity level in alignment with their fatigue. If night sweats are a sign, nurses can educate patients to identify triggers (e.g., alcohol, spicy food, caffeine) and to adjust their intake accordingly. For early satiety, nurses can help patients plan a diet that is heart healthy and high in protein, whole grains, fruits, and vegetables. For severe pruritus, nonpharmacological remedies such as cool showers and skin moisturizers are recommended. Patients with brain fog or difficulty concentrating may require pharmacological therapy, including additional education on the purposes of these medications. Given the propensity for infection, identification of fevers that deviate from target temperature parameters and a comprehensive workup may be indicated (Brennan-Cook, 2020).
Evaluation of Nursing Care for the Patient with Polycythemia
Evaluation of nursing care for polycythemia centers around symptom management and tailoring interventions and care plans specifically to address each patient’s unique needs. It is important to perform regular assessments to evaluate how interventions affect symptom improvement.
Evaluating Outcomes
A continuous evaluation process is crucial to improving care delivery, avoiding complications, and supporting overall health and well-being for each patient. Stabilization of an acute exacerbation and/or avoiding a thrombotic event are ideal outcomes, but there are specific outcomes nurses may identify (Pillai et al., 2023), including:
- A reduction of self-reported physical symptoms and objective signs help the nurse assess for changes or improvements, which can dictate next steps.
- The stabilization or normalization of RBCs, Hb, and Hct, and comparing the current results with previous results.
- The patient can articulate importance of compliance with pharmacological therapy, and verbalizing signs and symptoms of a thrombotic event.
- The patient can report reduced anxiety and depression and identify healthy coping mechanisms. Evaluating the patient’s social support system can also help identify what resources will be needed.
- The patient maintains target parameters for oxygen saturation, heart rate, and blood pressure.
Medical Therapies and Related Care
The goal of medical management of polycythemia is to lower RBC counts, prevent complications such as thrombosis, and improve overall symptoms. The following are major therapies for patients with polycythemia:
- A therapeutic phlebotomy is the removal of excess RBCs by reducing overall blood volume. If the patient has no genetic concerns or other comorbidities, the removed blood may be donated to a blood bank or service. The frequency and quantity of each reduction is determined by the Hb and Hct levels (Pillai et al., 2023).
- Management of cardiovascular risk factors includes management of hypertension and anticoagulation. Maintaining blood pressure at an ideal level is critical for minimizing cardiovascular events, and cholesterol management helps mitigate cardiovascular risks (Pillai et al., 2023).
- Medications such as ruxolitinib, an immune modulator, specifically target the abnormal JAK2-signaling pathway associated with polycythemia vera. Hydroxyurea is a myelosuppressive medication to decrease RBC production; interferon-α may also be used. Low-dose aspirin therapy helps decrease the risk of thrombosis by inhibiting platelet aggregation (Pillai et al., 2023).
- Regular monitoring and assessment via blood tests, including CBC counts, are used to track Hct levels and adjust treatment accordingly. Imaging studies may also be performed periodically to measure liver and spleen sizes as well as assess for potential complications (Pillai et al., 2023).
- Smoking cessation is another important strategy for patients with polycythemia because nicotine can exacerbate polycythemia’s complications significantly. Smoking cessation should be pursued immediately to decrease these effects (Pillai et al., 2023).
Treatment decisions should take into consideration factors such as a patient’s age, overall health status, and risk factors, as well as their response to previous interventions. Individualized treatment plans for patients with polycythemia must be created in conjunction with hematologists or health-care providers specializing in blood disorders. Their ongoing monitoring and adjustments are integral to managing this condition effectively and avoiding potential complications.