Acute promyelocytic leukemia (APL) is a rare hematologic disorder with approximately 600 to 800 new patients diagnosed per year in the United States.1,2 In contrast to other forms of leukemia, [ Read More ]
Best Practices in Patient Navigation – Acute Promyelocytic Leukemia
Best Practices in Patient Navigation: Acute Promyelocytic Leukemia
Barbara Barnes Rogers, CRNP, MN, AOCN, ANP-BC
As new trends emerge in the management of patients with cancer, an increasingly complex care delivery system continues to evolve. Although this certainly represents progress, it is important to realize that the number of treatment options available to patients may be overwhelming to them. The primary role of the oncology nurse navigator (ONN) is to improve the preparedness of the patient with cancer by providing education and psychological support.1 In addition, ONNs can offer logistical support, obtain referrals, assist with financial and insurance matters, and facilitate communication between the patient and other members of the healthcare team. Interestingly, the demand for nurse navigation is often impacted by the complexity of the disease.1 Acute promyelocytic leukemia (APL) is a highly malignant form of acute myeloid leukemia.2 The average age of patients at the time of diagnosis is between the ages of 20 and 50 years,3 a time when adults are typically assuming many roles, including spouse, parent, and employee. The management of patients with APL can be complex and requires immediate diagnosis to prevent morbidity and mortality.2 Since patients often present with bleeding and thrombosis, the initial goal of treatment is to reduce the risk of severe bleeding and death. Disseminated intravascular coagulation is the most common life-threatening complication associated with APL.3 Because of the high risk for this condition, a diagnosis of APL does not need to be confirmed to begin treatment.4
The ONN plays a key role in providing support to patients who are suspected of having APL. They can help patients better understand the diagnostic workup they are undergoing and the rationale for starting treatment even if a definitive diagnosis has not yet been made. They may also help locate financial resources for patients who need to take time off from their jobs during treatment.2
In general, the management of patients with APL is comprised of 3 phases: (1) induction, (2) consolidation, and (3) maintenance.3 This requires many patients to be on some type of therapy for several years. Molecular response rates are quite high in patients who are treated with induction therapy consisting of all-trans retinoic acid (ATRA) plus anthracycline-based chemotherapy, but the length of therapy may lead to anxiety in this relatively young patient population. In addition, patients may experience unusual complications associated with the disease itself or its treatment, including coagulopathy, differentiation syndrome, and prolonged QT interval (from arsenic trioxide). It is critical to recognize these complications and implement effective interventions as quickly as possible.3
Management of coagulopathy may include transfusions, administration of fresh frozen plasma, cryoprecipitate, and fibrinogen. When patients experience differentiation syndrome, they must be educated on the need to hold treatment with ATRA and arsenic trioxide until severe symptoms, such as renal failure or respiratory distress, resolve. They must also understand that medication dosages will need to be adjusted when treatment is resumed.2
The ONN must be aware of how a diagnosis of APL may impact a patient, and allow him or her the necessary time and space to express fears, anxieties, and concerns.5 Patients may need to be referred to a social worker or a psychiatrist to help them cope with their diagnosis and to provide additional resources.
Patients who have been diagnosed with APL and have maintained a complete response for at least 3 years have a very low incidence of late recurrences. Survivorship care includes medical care and ongoing surveillance to manage chronic and emerging comorbidities. In a 2014 study by Shetty and colleagues, the major cause of death in patients with a history of APL included second malignancies in 41% of the patient deaths, and cardiac diseases in 29% of the deaths.6 The ONN needs to impress upon
patients the need for ongoing surveillance for second malignancies and for follow-up with their primary care provider to ensure optimal management of cardiac-related comorbidities. As part of long-term follow-up and survivorship care, patients should be screened for second malignancies on a regular basis via mammograms and colonoscopies. In addition, they should be screened for cardiac issues such as hypertension and hyperlipidemia. Any signs of cardiovascular disease should be evaluated through stress testing.
The treatment of APL is complex and substantially different from other forms of acute leukemia. Therefore, patients need clear explanations regarding the treatment approaches that will be used, as well as education about disease- and drug-related complications that may arise. Other key roles of the ONN are to assist with logistic navigation through the healthcare system, provide a link between patients and providers, facilitate access to support networks and psychosocial assistance, and enable comprehensive follow-up care.
2. Walker DK, Held-Warmkessel J. Acute promyelocytic leukemia: an overview
with implications for oncology nurses. Clin J Oncol Nurs. 2010;14:747-759.
3. Peterson G, Trautman K, Hoffner B, Zakrocki J. Acute myeloid leukemia and acute leukemias of ambiguous lineage. In: Olsen M, Zitella L, eds. Hematologic Malignancies in Adults. Pittsburgh, PA: Oncology Nursing Society; 2013:101-156.
4. Sanz MA, Grimwade D, Tallman MS, et al. Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet. Blood. 2009;113:1875-1891.
5. Hamilton SJ, Martin DJ. A framework for effective communication skills. Nursing Times. 2007;103:30-31.
6. Shetty AV, Ravandi F, Alapati N, et al. Survivorship in APL: outcomes of acute promyelocytic leukemia patients after maintaining complete remission for at least 3 years. Blood. 2014;124:Abstract 954.