Polycythemia vera (PV) is a rare progressive myelo-proliferative cancer with significant symptom burden. Patients with PV often experience symptoms that adversely affect quality of life, work productivity, and functional status. Oncology nurses are well suited to assess for symptom burden and to provide educational interventions that support patients and their families.
AT A GLANCE
- PV is characterized by elevated blood counts and symptom burden, which often influences quality of life.
- Oncology nurses can assess patients with PV for symptom burden while monitoring for thrombotic and vascular events.
- Oncology nurses can support patients with PV and their families by providing ongoing education.
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Polycythemia vera (PV) is a rare hematologic cancer characterized by elevated blood counts. The driver mutation for most patients is the Janus kinase 2 (JAK2) gene, which causes the bone marrow to overproduce blood products, such as red blood cells (Arber et al., 2016). Some patients may have elevated white blood cells and platelets. PV is one of three myeloproliferative neoplasms (MPNs), including essential thrombocythemia and myelofibrosis, which share a tendency for clotting and bleeding that increases patients’ health risks (Arber et al., 2016). Patients with essential thrombocythemia have an overproduction of platelets, and patients with myelofibrosis suffer from anemia, bone marrow fibrosis, splenomegaly, and elevated white blood cells (Tefferi, 2018; Tefferi & Barbui, 2019; Tefferi et al., 2017). It is essential that oncology nurses have sufficient knowledge of MPNs to assess risk factors and disease progression and to provide appropriate educational support and interventions as they assist patients toward wellness.
Diagnosis and Prognosis
Hematologists diagnose PV using the World Health Organization (WHO) classification system. A patient must meet all three major criteria or the first two major criteria and the minor criterion (Arber et al., 2016). The first major criterion is (a) hemoglobin greater than 16.5 g/dl in men or greater than 16 g/dl in women, (b) hematocrit greater than 49% in men or greater than 48% in women, or (c) increased red blood cell mass. The second major criterion is having bone marrow results that include tri-lineage proliferation with pleomorphic mature megakaryocytes. The third major criterion is the presence of a JAK2 mutation. The minor criterion is a subnormal serum erythropoietin level (Arber et al., 2016; Tefferi & Barbui, 2019).
It is not unusual for abnormal laboratory values, vascular complications, and thromboembolic events to occur before diagnosis of PV (Enblom et al., 2015). Because PV may progress slowly, nurses need to report abnormally elevated hemoglobin, hematocrit, and platelet levels, even if the patient appears asymptomatic, because complications may be avoided with early diagnosis and treatment.
Although PV is a progressive disease, it presents differently across populations. Most adults are diagnosed after age 60 years, so prognosis may be influenced by comorbidity and age-related health changes. Prognosis is also influenced by thrombotic and vascular events (Kamiunten et al., 2018). Survival estimates are 15 years, and less if the patient progresses to myelofibrosis (Szuber et al., 2019).
Treatment priorities for PV are to prevent or reduce risks for thromboembolic events; therefore, nurses must prioritize the management of risk factors. The National Comprehensive Cancer Network’s (NCCN’s) clinical guidelines for patients with MPNs provide consistent and standardized care for patients with PV. All patients are monitored for bleeding and thrombosis, while cardiovascular risk factors, such as high blood pressure, diabetes, and lifestyle, are assessed and monitored. Patients are encouraged to quit smoking, control their diabetes and hypertension, eat a healthy diet, and exercise regularly (NCCN, 2020b). Treatments recommended for low-risk patients include using low-dose daily aspirin and routine phlebotomy to maintain hematocrit less than 45%. If the patient’s disease progresses or symptom burden worsens, cytoreductive treatment (hydroxyurea or interferons) may be added. Signs of disease progression are thrombosis, increased need for phlebotomy, thrombocytosis, leukocytosis, increased night sweats, itching, and extreme fatigue. Treatment for high-risk patients includes low-dose daily aspirin, phlebotomy to maintain hematocrit less than 45%, and cytoreductive medications (NCCN, 2020b). Ruxolitinib, a JAK2 inhibitor, is prescribed for high-risk patients who do not respond to traditional treatment. Studies have found that ruxolitinib use led to improvement in symptom burden, particularly problems with concentration, fatigue, night sweats, and itching, which improved patients’ quality of life (Griesshammer et al., 2018; Mesa, Verstovsek, et al., 2016) (see Table 1).
Oncology Nurse Considerations
Although the primary treatment goals for PV are related to controlling blood counts and decreasing risks for thrombotic events, oncology nurses also evaluate symptom burden while assessing for risk factors and providing medication and/or treatment. PV symptom burden can be high, even when patients are considered to be low risk, and can interfere with quality of life, activities of daily living, work productivity, functional abilities, and employment status (Mesa et al., 2018; Mesa, Verstovsek, et al., 2018; Yu et al., 2018, 2019).
Symptom burden should be assessed using the MPN Symptom Assessment Form Total Symptom Score (MPN-SAF TSS), a valid and reliable instrument recommended by the NCCN (2020b). The MPN-SAF TSS asks the patient to self-report on the 10 most common symptoms experienced by patients with MPNs (e.g., fatigue, early satiety, abdominal discomfort, inactivity, problems with concentration, night sweats, pruritus, bone pain, fever, unintentional weight loss) (NCCN, 2020b). The scores are totaled to provide the nurse with information about severity of symptom burden. This helps to facilitate individualized patient assessment, education, and treatment. If the patient skips a symptom on the checklist, the nurse should review this omission with them, because it will affect the total symptom score.
Fatigue is the most common symptom experienced by patients with PV (Mesa et al., 2018; Mesa, Miller, et al., 2016). In the MPN Landmark survey, fatigue was shown to affect quality of life and was often unrelated to low- or high-risk status (Mesa, Miller, et al., 2016). It is important for the oncology nurse to explore individual fatigue symptoms and their causes because fatigue may be multifactorial and related to medications and comorbidities. Suggested strategies for managing fatigue include setting priorities, pacing, delegating, increasing activities during peak energy levels, and limiting naps to less than one hour (NCCN, 2020a).
Inactivity is a frequent symptom of PV that may result from fatigue and interfere with sleep, affect social functioning, and decrease quality of life (Mesa et al., 2018). Many patients report missing social functions, canceling activities, or limiting activities because of fatigue and, therefore, lowering their quality of life (Mesa, Miller, et al., 2016). Oncology nurses can explore causes and factors related to the patients’ activity levels (Tolstrup Larsen et al., 2018). Oncology nurses can encourage patients to increase their activity levels gradually, despite fatigue and inactivity, along with physical therapy referrals.
Night sweats are a challenging symptom for many patients (Mesa, Verstovsek, et al., 2016). Night sweats are a scaffolding symptom that interferes with sleep and influences fatigue and daytime activity levels. It is important for oncology nurses to assess for night sweats, discuss triggers (e.g., alcohol, caffeine, spicy foods, smoking, stress), and offer suggestions for alleviation. Nurses can suggest online yoga programs or smartphone-based meditation applications to assist patients with fatigue, stress, and sleep (Huberty et al., 2018; Huberty, Eckert, Larkey, et al., 2019).
Patients with PV often experience early satiety (Mesa et al., 2018). Feelings of fullness or abdominal pressure may result from an enlarged spleen, causing patients to eat less and to experience unintentional weight loss. A cardiac-healthy diet high in nutrients, such as protein, whole grains, fruits, and vegetables, is recommended (NCCN, 2020b). Patients can be encouraged to eat small meal portions at frequent intervals throughout the day to maintain weight. Referrals to oncology nutritionists are also recommended (NCCN, 2020c).
Itching is a severe symptom, also defined in PV as aquagenic pruritus, which includes intense burning, tickling, and stinging from water contact (Siegel et al., 2013) and usually occurs after a warm shower. A study of aquagenic pruritus in 102 patients with PV found that 45% of patients had severe itching, and 33% avoided any contact with water on a daily basis (Lelonek et al., 2018). No recent evidence-based recommendations exist for alleviating severe pruritus, whereas newer medications for PV (JAK2 inhibitors) may influence pruritus symptoms that can be reported to the hematologist for an evaluation or change in treatment. Traditional treatment options, such as antihistamines, phototherapy, and serotonin reuptake inhibitors, are not recommended. Basic skin care with cooler showers and skin moisturizer may provide some relief by soothing the skin.
Concentration problems are not uncommon. Patients with PV often complain of difficulty focusing or being attentive (Yu et al., 2019). Concentration problems, headaches, brain fog, and other vasomotor symptoms should be considered when providing education to the patient. Meditation is suggested for inactivity, fatigue, and sleep problems, but may also help patients who experience difficulty concentrating (Huberty, Eckert, Dueck, et al., 2019). Bone pain and fever occur less often, but may be indicative of disease progression; therefore, nurses should assess for these symptoms and encourage patients to report them. Fever may be related to infection rather than to PV. Sources of infection should be investigated.
PV is a rare but progressive myeloproliferative cancer with significant symptom burden. Patients with PV can experience a myriad of symptoms. Although often subtle, these symptoms may adversely affect quality of life, work productivity, and functional status. Oncology nurses are well suited to assess for symptom burden and provide support with educational interventions to patients and their families.
About the Author(s)
Jill Brennan-Cook, DNP, RN-BC, CNE, is an assistant clinical professor in the ABSN Program in the School of Nursing at Duke University in Durham, NC. The author takes full responsibility for this content and did not receive honoraria or disclose any relevant financial relationships. Brennan-Cook can be reached at email@example.com, with copy to CJONEditor@ons.org.
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