Cancer is the leading cause of death in North Carolina. This issue of the NCMJ discusses cancer prevention, screening, treatment, and survivorship; disparities in incidence and mortality; and ethics of clinical trials. Highlighting the importance of comprehensive data for understanding cancer, original articles in this issue address how medical homes can reduce health care utilization among breast cancer patients and how distance to care affects receipt of radiation therapy.
Living With Advanced Cancer: Unmet Survivorship Needs
The month of October, with its emphasis on early detection and celebration of lives saved—with cheery pink everything—felt happy to me. All of this changed in May 2010. All of a sudden I was no longer a happy success story of early detection. The happy pink month of October became a sort of taunt rather than a celebratory time, and I had no idea where I fit in when people were lining up by years of survivorship. Am I a survivor still? Fortunately, thanks to the best medical care available and a whole-system approach to healing, I am still alive today, almost 4 years later, but incurable cancer with never-ending treatment affects my entire life. My partner and teenage children live with my cancer every day as much as I do and experience its profound effects on their lives. The ups and downs of struggles with receiving news about the cancer and its progression, my variability in being able to be fully present with my family, and the ongoing uncertainty about my prognosis affect the entire family.
PJ, a 54-year-old woman with metastatic breast cancer
There are approximately 14 million cancer survivors in the United States , but we do not know how many of them are living with cancer that cannot be cured. Although such individuals represent a significant number of cancer patients, there are no precise statistics to characterize this important but neglected group. This uncertainty is emblematic of the broader gap in survivorship research and service delivery for these patients, who are not fully covered by typical survivorship guidelines. To be sure, the challenges of living with an advanced, incurable illness are substantial and are uniquely different from those faced by patients whose cancer has not recurred. For many individuals with incurable illness, the specter of their own mortality becomes increasingly powerful and unavoidable [2, 3]. Greater awareness of the survivorship needs of this population will help to ensure that they receive appropriate care.
Defining Advanced Cancer
Part of the difficulty in identifying the unique survivorship needs of patients with advanced cancer is the challenge of defining this heterogeneous population. For this commentary, we consider advanced cancer survivors to be individuals living with either an incurable cancer (ie, a solid tumor with distant metastases) or chronic cancer (eg, chronic lymphocytic leukemia). Different cancer advocacy and funding organizations, as well as individuals affected by cancer, define cancer survivors in various ways. The National Coalition for Cancer Survivorship defines survivorship as beginning at the time of diagnosis and continuing through the balance of the person’s life; because family members, friends, and caregivers are impacted by the survivorship experience, they are also included in this definition . Such a broad conceptualization encompasses men and women who are cancer-free as well as those living with cancer, either continuously or intermittently. Unsurprisingly, many patients have not embraced the term cancer survivor—either because it is too early in their journey to know whether they will survive or because it has become painfully clear that they will not. In an international survey of 1,342 women with metastatic breast cancer, the results of which were published in 2010 , 61% of respondents considered themselves to be cancer survivors.
The unique needs of patients with life-limiting or chronic cancer are rarely emphasized in current approaches to cancer survivorship, yet the number of such patients is growing, as improvements in cancer treatment allow patients with advanced cancer to live longer. For example, the 5-year survival rate for metastatic breast cancer was 10% in the 1970s. Today, as many as 23% of patients with metastatic breast cancer will survive for at least 5 years , and it is estimated that more than 150,000 women and men are living with metastatic breast cancer in the United States . Similarly, targeted therapies for diseases such as chronic myeloid leukemia  and metastatic colorectal cancer  have led to significant improvements in survival. We contend that growing numbers of cancer patients fall into a gap between survivorship programs, which are focused on wellness and surveillance, and end-of-life specialty care.
Emerging survivorship research and programmatic priorities, such as treatment summaries and care plans, may hold less relevance for patients living with advanced cancer. For example, a major component of survivorship recommendations is the combination of screening for new malignancies and surveillance for recurrence [10, 11], but this is of no consequence for patients with widely metastatic disease. Once the prospect of cure is no longer realistic, clinical priorities appropriately shift to clarifying goals of care, extending life, managing physical symptoms, fostering psychological adaptation, and maximizing quality of life. The challenges of spiritual distress and medical decision making also come to the forefront in this group, as disease progression forces these individuals to face new dilemmas.
Available data reveal that patients living with advanced cancer experience a substantial burden of physical and psychological symptoms that can profoundly affect their quality of life. In a systematic review published in 2007 , symptom prevalence was assessed in more than 25,000 patients with incurable cancer, and these patients commonly cited symptoms of fatigue (74%), pain (71%), lack of energy (69%), weakness (60%), and appetite loss (53%). Similarly, a 2007 meta-analysis published in Annals of Oncology  estimated that 64% of patients living with advanced, metastatic, or terminal disease experience moderate to severe pain, compared with 33% of cancer patients who had completed curative treatment. These data demonstrate that many patients living with advanced disease experience undertreated somatic symptoms. Patients and their families may not realize that much of this suffering is preventable. Resources such as booklets from the National Cancer Institute—including Coping with Advanced Cancer  and When Someone You Love Has Advanced Cancer —are helpful recent contributions. Still, we are long overdue for improved advocacy efforts and detailed guidelines on symptom management in advanced cancer.
Another shortcoming is that traditional methods of assessing symptom burden and functional status may underestimate the degree of disability experienced by patients with metastatic disease. In a recent study of cardiopulmonary function in breast cancer survivors, Jones and colleagues  found that quantitative cardiopulmonary exercise testing revealed marked functional impairment as measured by peak oxygen consumption, although these survivors had normal cardiac function as measured by left ventricular ejection fraction. The impairment was significantly greater in the cohort of survivors with metastatic disease than in the cohort of women with nonmetastatic disease . Furthermore, untreated symptoms rarely occur in isolation; most patients who experience significant physical symptom burden also face challenges due to the lifestyle and psychosocial changes that accompany their illness.
The psychological adaptation to living with incurable disease can present challenges that are as great or greater than the physical consequences of advanced cancer and its treatment. Within the context of cancer survivorship, patients with advanced disease often feel marginalized or isolated [17, 18]. Approximately 20% of all patients with advanced cancer have symptoms consistent with a depressive disorder, and nearly 14% meet diagnostic criteria for an anxiety disorder . Sources of emotional distress in this group typically differ from those of patients with earlier-stage disease. Psychological concerns—such as distress over anticipated death, fear of being a burden to loved ones, and social isolation—take on heightened meaning for these patients.
Coordination of Care
Given the magnitude and persistent nature of treatment and symptom burden in patients with advanced cancer, a coordinated team approach is needed to achieve compassionate and cost-effective treatment. Coordination of care (eg, between the patient’s oncologist and his or her primary care provider) is one of the most important and problematic aspects of cancer survivorship . Several studies have suggested that survivors often fail to receive recommended post-treatment care [20-22]. Improved coordination of care holds the promise of decreasing survivors’ exposure to medically unnecessary or duplicate testing . The possibility of participating in a clinical trial is another unique aspect of care coordination for patients with advanced cancer (and those for whom standard care approaches have failed). Here the challenge is to balance current treatment options and outcomes with optimal timing of and eligibility for investigational treatment opportunities. VT, a patient with advanced ovarian cancer, illustrates this challenge when she says, “I realize now that what I should have done was look for a clinical trial before my last course of gemcitabine. It turns out that I’ve had too many courses of chemo and developed too much toxicity to qualify for the clinical trial I really wanted.”
Emerging models of palliative care can inform how we approach advanced cancer survivorship. Many patients with advanced cancer live for months or years with excellent performance status. For these patients, a survivorship orientation based on the notion that patients will live for decades is no more appropriate than a recommendation to “get one’s affairs in order” and pursue “comfort care.” Patients with advanced cancer deserve full membership in the global survivorship movement.
Survivorship programs must embrace modern palliative care and incorporate its central tenets: aggressive symptom management, clarification of goals of care, and improved communication between and among patients, caregivers, and providers. In 2010 a randomized controlled trial of early palliative care for patients with metastatic non–small-cell lung cancer  demonstrated clinically meaningful improvements in quality of life, mood, and survival compared with patients who received standard care. Other studies [25, 26] have also demonstrated that early introduction of palliative care improves quality of life. As a result, the American Society of Clinical Oncology recommended in 2012 that palliative care be integrated into standard oncologic care from the time a person is diagnosed with metastatic or advanced cancer .
Integrated palliative care has been shown to yield substantial improvements in patient outcomes, but we do not yet know how to best tailor palliative and supportive care for patient subgroups or how to identify strategies to enhance patients’ trust and willingness to engage in this treatment. A single approach to palliative medicine will not address all patient needs. As cancer care has become targeted, so too can supportive care. There is also a lack of customized palliative care for specific populations, such as parents with dependent children, adolescents and young adults, and older adults.
A dedicated effort is needed to raise awareness of the psychosocial consequences of living with advanced cancer and to identify strategies to improve quality of life and medical care for this unique group. Policies, research initiatives, and programs that deliver optimal care to these individuals are overdue. Validated clinical interventions for cancer survivors who have completed curative therapy may also hold promise for patients living with advanced disease. For example, a promising area of future research is the value of physical exercise in promoting longevity and quality of life among advanced cancer patients [28, 29]. Further evaluation of psychosocial support interventions also merits attention. Although early research  indicated that participation in supportive-expressive group therapy might prolong survival for women with metastatic breast cancer, more recent evidence [31-33] suggests that it does not influence survival but does improve mood and pain perception—particularly among those who have higher distress at baseline. Finally, we wholeheartedly endorse the growing national movement of patient- and family-centered cancer care, which has increasingly recruited patients and caregivers into the operations of cancer centers. The voices of cancer-free patients are invaluable to clinicians and administrators in designing better ways to provide oncology care, as are the insightful perspectives offered by those with incurable cancer.
To sum up, patients with life-limiting or chronic cancer constitute a unique and neglected group of survivors whose needs have been largely unmet. However, we can help these patients live their lives as comfortably and meaningfully as possible through new models of survivorship care and the adoption of modern palliative care principles. Survivorship programs for patients living with advanced cancer should attend to the specific needs of young adult populations and should more actively address the psychological impact of cancer on patients and their families. Clinical program and research strategies to preserve performance status and minimize somatic symptoms are needed for these neglected patients.
Financial support. This manuscript was supported in part by the University Cancer Research Fund and by the Livestrong Foundation.
Potential conflicts of interest. E.M.P. and D.L.R. have no relevant conflicts of interest.
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Eliza M. Park, MD assistant professor, Department of Psychiatry, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
Donald L. Rosenstein, MD director, Comprehensive Cancer Support Program, University of North Carolina at Chapel Hill; professor, Departments of Psychiatry and Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
Address correspondence to Dr. Eliza M. Park, 170 Manning Dr, CB #7305, Chapel Hill, NC 27599 (firstname.lastname@example.org).