Myeloproliferative neoplasms (MPNs) are a group of rare progressive and chronic hematological cancers that most commonly impact adults older than 60 years (Arber et al., 2016; Titmarsh et al., 2014). Although labeled as incurable cancers, MPNs are managed as chronic conditions, primarily in the outpatient setting. There are three main types of MPNs: essential thrombocythemia, polycythemia, and myelofibrosis (Mesa, Jamieson, et al., 2017; National Comprehensive Cancer Network [NCCN], 2019a). The cancers arise in the bone marrow and are characterized by an overproduction of one or more blood cell components (white blood cells, red blood cells, and/or platelets).

Because an overproduction of blood components places the patient with an MPN at increased risk for thrombotic and cardiovascular events (Enblom et al., 2015; Kamiunten et al., 2018), prevention of thrombotic and cardiovascular events is the priority of medical treatment, followed by management of a myriad of symptoms specific to MPNs. Although individual symptoms experienced by patients with MPNs may vary widely, Table 1 identifies the 10 most commonly experienced symptoms (Emanuel et al., 2012). Disease burden remains high, causing interferences with work, quality of life (QOL), and social activities (Harrison et al., 2017). Professional case managers in the outpatient setting are well suited to support patients with chronic conditions such as MPNs, manage their symptomatology, and equip them with an array of important self-care practices. The purpose of this article is to (a) review management and symptomatic burden of patients with MPNs, and (b) describe the role of professional case managers in coordinating outpatient services with the health care team and educating patients for better self-care management.

TABLE 1 Most Common MPN Symptomsa

Brief Case Study

FC, age 64, speaks limited English (Spanish is her primary language). Today, she has come with her spouse to the community outpatient center where her primary health care needs have been managed for the past 3 years because her polycythemia vera symptoms have been progressively worsening over the past 6 months. The diagnosis of polycythemia vera was made 2 years ago when FC's serum hemoglobin and hematocrit levels were found to be excessively high (18 and 48 g/dl). She was referred to a hematologist who confirmed the diagnosis of polycythemia vera with a positive bone marrow biopsy and a positive Janus kinase (JAK2) mutation (present in 98% of patients with polycythemia vera) (NCCN, 2019a). In addition to polycythemia vera, FC's history is unremarkable except for obesity and frequent headaches. Although her polycythemia vera is managed by the hematologist, FC continues to visit the community outpatient care center for her primary health care needs; at this visit, her main concerns are increased fatigue, diminished appetite, inability to work, anxiety about her prognosis, and abnormal laboratory values (hemoglobin 16 g/dl and hematocrit 48 g/dl). Her vital signs and all other laboratory values are normal. FC's spouse expresses concern that FC is becoming depressed. A physical assessment will be completed after their interview with the professional case manager. Table 2 highlights specific interventions to address FC's concerns and the professional case manager's priorities.

TABLE 2 Case Study (FC): What Are the Case Manager's Concerns?

Implications for Professional Case Management Practice

Professional case managers are increasingly needed in outpatient settings to ensure population health because older adults with chronic conditions such as MPNs have complex medication regimens and experience high health care utilization (Bankar et al., 2020; Mehta et al., 2014). Patients with MPNs are generally older with increased comorbidities; are at higher risk for arterial and venous thrombosis; and require more health care, laboratory, and transfusion services (Bankar et al., 2020). Patients with MPNs also experience greater health care expenses related to acute care for thrombotic and cardiovascular complications, which may be prevented (Bankar et al., 2020). For example, although FC does not arrive to the community outpatient center with an obvious thrombosis or cardiovascular complaint, the professional case manager should consider each visit as an opportunity to review thrombotic and cardiovascular risk factors. Patient education about smoking avoidance, eating a cardiac healthy diet, and routine exercise is essential for wellness and prevention of complications (NCCN, 2019a). In light of COVID-19, the professional case manager can educate the patient, family, and team that patients with MPNs are at increased risk for infections due to both disease biology and medical treatment. Professional case managers can collaborate with hematologists to manage and coordinate the multifaceted care needed by these patients, thus reducing costs and, potentially, complications and hospitalizations (Bankar et al., 2020).

Care Coordination of Medication and Supportive Education

MPNs are rare diseases, and individuals may have different treatment options. The professional case manager should review NCCN clinical practice guidelines (NCCN, 2019a) on the management of patients with MPNs and the evidence for treatment options, such as routine phlebotomies to maintain target hematocrit <45 mg/dl; antiplatelet medications (low-dose aspirin); myelosuppressive medications (hydroxyurea); or weekly interferon-[alpha] therapy injections, which are safe and effective but have adverse effects (Bewersdorf et al., 2021). Adverse effects vary, so patient education about what to expect is essential. For example, hydroxyurea, a chemotherapeutic medication, may cause mouth and skin ulcers along with stomach upset and digestion interference. If the patient is intolerant of hydroxyurea, interferon (IFN) may be implemented (Tefferi & Barbui, 2018). The most common adverse effects of IFN are flu-like symptoms such as body aches, fever, and malaise (Bewersdorf et al., 2021), which can interfere with the patient's QOL; however, the professional case manager can support the patient and help them to manage symptoms. For example, some patients may need acetaminophen (Tylenol) and diphenhydramine (Benadryl) an hour before their weekly self-administrated subcutaneous IFN injection. Dosages of IFN are adjusted according to the patient's hematological responses and tolerance (which often takes several months to achieve) (Bewersdorf et al., 2021; NCCN, 2019a; Verstovsek et al., 2017). Genomics can also play an important role in the treatment plan for patients like FC, because targeted therapy such as JAK2 inhibitors may be needed if there is an inadequate response to other medications such as hydroxyurea. The professional case manager can collaborate with FC's hematologist and provide supportive education about medication titration and subsequent monitoring of complete blood counts. Patient education and care coordination also includes helping the patient to understand the rationale for a phlebotomy and what the procedure involves, and assisting with scheduling. Scheduling routine phlebotomy procedures and laboratory appointments on the same day can assist the patient in navigating the outpatient system. It is important for the professional case manager to explain that recommendations for phlebotomy for the patient with polycythemia vera are 300-450 ml of blood removed until the target hematocrit is reached (Barbui et al., 2018).

In FC's case, because her primary language is Spanish, her professional case manager should approach education, advocacy, and collaborative goals discussions involving pharmaceutical interventions, treatment options, and self-care with cultural and linguistic sensitivity. The NCCN provides a patient guide (which is available in Spanish) written in accessible language with easy-to-understand terms that explains MPNs and contains visual descriptions of treatments, medications, and procedures (NCCN, 2019c). The professional case manager can print this guide for the patient, highlighting their individual treatment options and suggested interventions.

Validate, Collaborate, and Facilitate

Despite recent improvements in medications, symptom burden continues to interfere with QOL for patients with MPNs (Harrison et al., 2017). Assessment of symptomatic burden should be completed at each visit using the MPN Symptom Assessment Form (MPN-SAF) (Emanuel et al., 2012; Scherber et al., 2011), which includes the most common symptoms experienced by patients (identified in Table 1); however, it should be noted that the MPN-SAF does not take into account the patient's QOL or activities of daily living. The MPN-SAF identifies the most clinically meaningful symptoms, but the patient may also be experiencing additional symptoms, such as numbness and tingling in hands and feet, cough, abdominal pain, and problems with sleep. The professional case manager can track symptoms as a means of tracking disease progression and response to current treatment. In FC's case, for example, her professional case manager should ask additional questions about the effects of her fatigue, appetite, headaches, and anxiety on her QOL. Clinician health care outcome goals for the patient are often discordant with the patient's long-term health goals (Harrison et al., 2017; Mesa, Miller, et al., 2017). Reviewing the MPN-SAF with the patient can promote discussion of how symptoms impact the patient on a daily basis. If the professional case manager becomes aware that such symptoms as fatigue, appetite change, or anxiety are affecting the patient's QOL and emotional well-being, an opportunity opens for discussing shared goals to improve outcomes.

A diagnosis of a rare type of cancer creates added stress for the patient and their caregivers and negatively affects their activities of daily living (Walpole et al., 2021). In this case study, both FC and her spouse expressed concerns about anxiety and depression. The professional case manager can validate the impact of added stress, educate patients and their families, and provide information about caregiver support resources as well as helpful tips for living with a chronic condition. FC's professional case manager, for example, could create a holistic, patient-centered, shared treatment plan that addresses her symptom burden and outpatient procedures, allowing for possible adjustments to medications and/or referrals for psychological support. By addressing the concerns of FC and her caregiver and helping them to navigate the outpatient health care environment and use resources effectively, her professional case manager can mitigate her hospitalization risks and improve their QOL.

Outcomes and Clinical Implications

Living with an MPN means living with chronic fatigue, a decreased QOL, and the need to prioritize energy demands, all of which have an impact on the patient and their caregiver/s (Rossau et al., 2021). The professional case manager can glean the information necessary for individually targeted psychosocial support and patient education through open communication about the patient's perceptions of how their symptoms impact their work-life balance, family, social activities, and personal sense of well-being. A key role of the professional case manager is facilitating shared decision-making related to well-being goals and treatment outcomes. Professional case managers have the potential to improve clinical outcomes for patients with chronic conditions such as MPNs by providing encouragement and psychosocial support as the patient learns about self-care management. It can be highly rewarding for the professional case manager to coordinate outpatient care that aligns medical care management with the individual priorities of patients with chronic conditions.

Conclusion

The professional case manager should use a holistic and collaborative approach to patient care in the outpatient setting for patients with chronic conditions such as MPNs. Care of chronic conditions can be fragmented, less cost-effective, and impersonal. The professional case manager can ameliorate difficulties and frustrations by (a) incorporating the patient's goals into treatment plans and decision-making, (b) involving caregivers in the process, and (c) coordinating medication administration and outpatient services to best meet the needs of the patient and their caregiver.

References