Since the “War on Cancer” was declared in 1971, advances in cancer treatment and prevention have accomplished considerable success in reducing mortality and morbidity in many tumor types, and more Americans than ever are surviving cancer. Today, an estimated 15.5 million cancer survivors are alive in the United States, representing nearly 5% of the population.1 These numbers are projected to grow to 20.3 million by 2026, and to 26.1 million by 2040, an increase of almost 11 million from 2016.1 Among US cancer survivors, approximately 67% live at least 5 years after receiving their diagnosis, and 10% live 25 years or longer.2
As the number of cancer survivors continues to grow, patient aftercare has taken on a more important role. A cancer survivor can be described as any person who has been diagnosed with cancer, from the time of diagnosis through the balance of life. Although the term “survivor” is often applied to individuals who have completed active treatment, survivorship can be conceptualized in 3 phases, including3:
- The time from diagnosis to the end of the initial treatment
- The transition from treatment to extended survival
- Long-term survival.
Cancer survivors face a number of treatment-related challenges as they proceed through the care continuum, including medical care follow-up, managing the long-term and late effects of therapy, and monitoring for recurrence. In addition, cancer survivors are at an increased risk for additional cancers.4
The spectrum of cancer care includes physical, economic, and psychosocial issues that may arise from the time of diagnosis through the end of life. The period directly after active cancer treatment is particularly difficult for some survivors.4 After active treatment, there may be less frequent contact with the treatment team, anxiety about the possibility of recurrence, fewer social supports, and difficulty adjusting to a “new normal,” all of which can contribute to psychological and emotional distress during this transition period. Survivors may also experience residual pain after surgery and other long-term and late effects of treatment.
Even survivors who do not experience acute psychological distress or physical pain may face challenges in optimizing their health after a cancer diagnosis.4 In addition, cancer survivors face practical and economic challenges, including limitations in work and daily activities, obtaining health insurance coverage and accessing healthcare, and increasing medical care costs.5 Although these challenges can apply to all types of cancer, the remainder of this supplement will focus on survivorship of patients with hematologic malignancies, and, in particular, multiple myeloma.
Survival Trends in Multiple Myeloma
Although it is a relatively uncommon cancer, the burden of multiple myeloma is considerable for patients, family members, and caregivers. In 2016, an estimated 30,330 new cases of multiple myeloma will be diagnosed, and approximately 12,650 deaths are expected to occur.6
Although the incidence of multiple myeloma has modestly increased over the past few decades, overall survival has risen dramatically. From 2006 to 2012, the 5-year survival rate increased to an estimated 48.5% compared with 26.3% in 1975.7 Furthermore, the results of a new analysis of data from the Surveillance, Epidemiology, and End Results program found that the estimated 10-year and 20-year survival rates for multiple myeloma also have increased appreciably in recent years (Table 1).8 As a result of these advances in survival, more patients than ever are living with multiple myeloma—an estimated 95,688 people in the United States alone in 2013.7
Advances in the Treatment of Multiple Myeloma
Although multiple myeloma remains incurable, the goal of therapy has shifted from palliation to one of curative intent. Multiple myeloma is characterized by remission and subsequent relapse, during which the disease will inevitably become refractory to treatment.9 However, treatment advances are lengthening the time between relapses. Because the period between relapses becomes progressively shorter with each successive line of therapy, treatments that extend the interval of progression-free survival (and, by surrogacy, overall survival) offer the best available options in the absence of a true cure.9
Much of the increase in survival rates has coincided with the advent of newer therapies. Beginning with the introduction of the first proteasome inhibitors and immunomodulatory agents more than a decade ago, the depth of response, overall survival, and progression-free survival have improved dramatically when the results of autologous stem-cell transplantation and regimens containing novel agents are compared with those of conventional chemotherapy.10 Improved first-line regimens combining immunomodulatory agents and proteasome inhibitors are now widely used. Most patients respond to these treatments, keeping the disease at bay longer.11
Therapeutic advances continue to the present day, with 4 new anti–multiple myeloma agents approved by the US Food and Drug Administration in 2015 alone, and many more drugs are in clinical development. As supporting clinical evidence continues to emerge, novel agents with complementary mechanisms of action are increasingly used in combination with one another in the relapsed or refractory setting, and multiple myeloma treatment paradigms continue to evolve.12
In addition to the introduction of new agents and combinations, efforts to develop strategies that prevent or delay disease progression have resulted in the concepts of consolidation and maintenance therapy. Consolidation can be defined as short-term therapy or a second stem-cell transplant that is administered after induction therapy or autologous stem-cell transplant to enhance the rate and depth of a previously obtained response.13 Maintenance therapy is low-dose treatment administered over a long period, with the goal of extending response duration and minimal toxicity after induction therapy, stem-cell transplant, and/or consolidation therapy.13,14 Low-dose lenalidomide is a common maintenance regimen for patients with multiple myeloma; bortezomib is also used for some patients as a single agent or in combination with lenalidomide.14 Clinical trials have shown that lenalidomide maintenance prolonged the time to progression or progression-free survival by 17 to 19 months compared with placebo.15-17 As a result of these therapeutic advances, researchers and clinicians are more optimistic than ever about the prognosis of patients with multiple myeloma.11
In addition to therapeutic advances, patients are receiving treatment earlier in the course of the disease. Historically, the definition of “active multiple myeloma” required treatment to be initiated at the first sign of end-organ damage (per the hypercalcemia, renal insufficiency, anemia, or bone lesions [CRAB] criteria).18 However, in recent years, there has been increased recognition of the need to prevent end-organ damage rather than merely treat it. As a result, the International Myeloma Working Group recently expanded its definition of active multiple myeloma by adding cases that do not meet the classic CRAB criteria.19 Although these definition changes may increase the prevalence of active multiple myeloma, they will also introduce a larger group of patients to treatment before morbidity from the diagnosis affects them.
Unmet Needs in Patients with Multiple Myeloma
As patients continue to live longer with multiple myeloma, they may face a number of challenges. The assessment of access to healthcare, treatment-related toxicities, disease-specific comorbidities, and quality of life have been identified as issues that need to be addressed throughout the continuum of cancer care.20
Overall, patients with hematologic cancers, including those with multiple myeloma, suffer from poorer health status than the general population. In particular, fatigue and pain substantially impact quality of life.21 Maintaining overall health status and quality of life has important practical implications for these individuals. Many patients with multiple myeloma must continue working to support their families and themselves. Therefore, the ability to be healthy enough to work is of vital importance.22 However, despite the need to maintain employment, many individuals with cancer or a history of cancer are either unemployed or underemployed. Because health insurance is tied to employment for many in the United States, the lack of employment may lead to the inability to pay for treatment and necessary follow-up. Subsequently, unemployment may lead to credit problems and bankruptcy, exacerbating the other stressors experienced by patients and their families.23
A number of survey-based analyses have endeavored to quantify the burden of multiple myeloma treatment on patients and their families. One report from the Cancer Experience Registry found that 32% of respondents reported depleting their savings as a result of the cost of their treatment; 22% borrowed against or used money from retirement; and 35% reported cutting their grocery expenses. Furthermore, approximately 33% of respondents reported that they were often or always upset about money and the cost of care, and 47% expressed concern about health insurance or had money worries. Despite these findings, only 56% of respondents reported that a member of their healthcare team talked to them about resources related to getting financial help or financial counseling, and only 28% reported that their healthcare team ever discussed the impact of multiple myeloma on personal finances.24
In another survey, 59 of 100 patients reported that treatment costs were higher than expected, with 36 of 100 reporting that they applied for financial assistance. The use of savings to pay for multiple myeloma treatment was common (43 of 94 patients), and 21 of 98 patients reported borrowing money to pay for medications. On multivariate analysis, younger age, nonmarried status, longer duration since diagnosis, and lower household income were significantly associated with higher financial burden for patients.25
Part of the financial stress felt by patients has resulted from the rising out-of-pocket cost burden incurred by patients and their families. Over the past decade, employers have relied increasingly on cost-sharing to manage the use of medical services and the resulting costs. The number of employers offering high-deductible health plans has grown almost 300% since 2009. In an effort to contain premiums, the average health insurer in-network deductibles and out-of-network deductibles increased by approximately $500 and $1000, respectively.26,27
In addition to the financial stress associated with the treatment of multiple myeloma, adding to the expense is the fact that few institutions in the country have multiple myeloma treatment programs; therefore, patients and their families often find that treatment requires travel and lodging expenses. The participants in one study reported financial effects in the areas of employment, disability coverage, health/medical and life insurance, and retirement. Many patients reported substantial out-of-pocket expenses.22
In addition to financial pressures, patients with multiple myeloma may have medical needs that are not directly related to their cancer. Multiple myeloma typically is a disease that afflicts older adults. Older individuals are more likely to experience non–cancer-related comorbidities that may impact their overall health and quality of life.3
Survivorship Care Planning
To help meet the often daunting needs of patients with cancer, the Institute of Medicine (IOM) recommends that, to facilitate optimal survivorship care, a survivorship care plan should be provided to every patient on the completion of treatment.28 The survivorship care plan is comprised of a personalized record of care and a health maintenance plan as a means of improving patient-reported and health-related outcomes. Survivorship care plans can include summaries of the patient’s cancer type and treatment history, schedules for follow-up screening, potential posttreatment issues, signs of recurrence, guidelines for lifestyle modifications, and important community resources. This information can provide valuable guidance during the often challenging transition from active cancer treatment back to community-based care, which is a period that is frequently characterized as lacking in coordination.2
Although the IOM recommendation served as the impetus that initially spearheaded the development of cancer survivorship care plans, there has subsequently been limited consensus on the key features of these plans or how to operationalize plan delivery. It should also be noted that survivorship plans must be tailored for each cancer type because of the disease- and population-specific variables that characterize each condition. In 2011, the International Myeloma Foundation Nurse Leadership Board (NLB) addressed this issue by publishing consensus statements in an effort to guide the development of survivorship care plans for patients with multiple myeloma.20
The consensus statements provide a blueprint to assist treatment teams in developing comprehensive survivorship care plans that address several key health domains, including general health maintenance screening and support, bone health, mobility and safety, renal health, and sexuality.20
Health promotion and disease prevention have been identified as critical areas for maintaining the overall health status and quality of life of multiple myeloma survivors. Multiple myeloma and its treatment-related side effects can contribute to an overall decline in health status by affecting multiple organ systems. In addition, most multiple myeloma survivors are older adults and are therefore at risk for a broad range of non–cancer-related comorbidities.29
To better meet the survivorship needs of this population, the NLB developed a health maintenance schedule that provides guidance on the timing and frequency of screening evaluations. Specifically, the NLB recommends screening for cardiovascular disease, other malignancies, endocrine disorders, bone health, sensory changes, psychosocial issues, nutrition, and other conditions that may be important to cancer survivorship, as is shown in Table 2.29
The majority of patients with multiple myeloma will experience a pathologic fracture over the duration of their illness. Because of multiple myeloma–related bone pathology, 80% of patients experience pain and diminished quality of life.30 Healthcare providers and nurses have the ability to identify patients who are at risk for pathologic fractures and other skeletal events that occur as a result of the physiologic alterations in bone metabolism associated with multiple myeloma.20
The identification and management of bone disease in patients with multiple myeloma are integral parts of long-term survivorship plans. Such plans should include interventions such as diagnostic monitoring, dietary counseling, medication administration, surgical procedures, and exercise promotion to minimize risk. Timely supportive care and the effective management of bone disease can significantly improve quality of life, enhance mobility, and reduce skeletal-related complications in patients with multiple myeloma.31
Mobility and Safety
More than 25% of older adults fall each year, and falling only once doubles the risk for subsequent falls. Falls may result in hip fracture and hospitalization.32
Patients with multiple myeloma are at an increased risk for impaired mobility resulting from the pathology of the disease, the side effects of therapy, and the patient’s age and comorbidities. In particular, fatigue, weakness, bone loss, sensory impairment, alcohol misuse, mental health issues, and other health conditions, such as arthritis, diabetes, and cardiovascular disorders, can contribute to decreased mobility and a risk for falling in these individuals. The increased morbidity associated with falls in older adults has an additional negative effect on the long-term prognosis for patients diagnosed with multiple myeloma.33
To help mitigate risk, patients with multiple myeloma may benefit from regular monitoring, laboratory and radiographic tests, assessment for risk for falls and injury, and safe activity and exercise programs that promote functional mobility. A functional mobility and safety survivorship care plan can help patients be more accountable for improving their overall health behaviors, thereby reducing the risk for falls. Plans should include a baseline assessment to evaluate the current risk and functional status, the promotion of physical activity to improve or manage immobility, and nutrition or weight management programs that are tailored to the individual patient’s needs.33
Kidney disease is a common complication in patients with multiple myeloma. Kidney dysfunction most often occurs in patients with a high tumor burden and usually manifests as cast nephropathy, which leads to tubuloepithelial-cell atrophy and interstitial fibrosis.34,35
Pathophysiologic renal alterations often result in decreased creatinine clearance. As the pathology progresses, it can negatively affect the clearance of anti–multiple myeloma therapies and other medications. Chronic kidney dysfunction can impact overall health status, putting patients at higher risk for additional complications, such as bone loss, myelosuppression, infection, and anemia.34
Fortunately, preventive screening and timely therapeutic interventions can limit kidney disease progression in patients with multiple myeloma. Therefore, renal function should be assessed at initial diagnosis and throughout therapy. Oncology nurses and other healthcare providers can play a key role in the early identification of renal insufficiency and in educating patients on the importance of liberal oral hydration and caution regarding the use of nonsteroidal anti-inflammatory drugs.34
Survivorship care plans are designed to identify kidney dysfunction via routine screening techniques. For patients with renal insufficiency, interventions such as the judicious use of supportive bone health agents can be implemented to minimize behaviors and environmental factors that may negatively affect renal function, thereby limiting disease progression and helping to manage renal complications.34
Sexual dysfunction is common in patients living with multiple myeloma. It may occur as a result of treatment, nerve root compression, or other comorbidities, such as hypertension, diabetes, or coronary artery disease. Men and women may be affected by sexual dysfunction, altering their relationships with their partners.36
If dysfunction is identified after conducting an assessment of sexual function, a number of pharmacologic and nonpharmacologic interventions can be implemented in men and women. A sexuality survivorship care plan focuses on defining sexual dysfunction, discussing risk factors for sexual dysfunction, providing tools for sexual assessment, and describing interventions for patients and their partners who may be experiencing a disruption of the sexual response cycle.36
As our understanding of cancer biology continues to evolve, leading to therapeutic breakthroughs in treatment, people can expect to live longer with cancer. However, the disease itself, and the negative effects of treatment, can lead to diminished overall health status and quality of life over time. Similar to many other tumor types, there have been impressive survival gains in multiple myeloma in the past few decades.7,8
However, patients may encounter a host of treatment-related issues and other challenges during and after the period of active treatment. Financial pressures, reduced social supports, stress on intimate relationships, and impaired overall health status are all relatively common and may drastically affect patients’ health status and psychosocial well-being.20 Because multiple myeloma is not curable at this time, fear of relapse or recurrence is a very real concern for multiple myeloma survivors.
Health system barriers, such as fragmented and poorly coordinated healthcare; a lack of education and awareness of the long-term effects of cancer and its treatment; limited knowledge of how to maximize health outcomes; and suboptimal patient–provider communication can exacerbate the challenges faced by multiple myeloma survivors.2
To optimize health and quality of life for multiple myeloma survivors, the development of a tailored survivorship care plan can help individuals and their families cope with cancer survival and get the most out of their lives. The NLB has developed consensus recommendations that address the key domains of multiple myeloma survivorship and can serve as a valuable template for the creation of a care plan suited to each individual survivor’s needs.20
1. National Cancer Institute Division of Cancer Control and Population Sciences. Updated October 17, 2016. https://cancercontrol.cancer.gov/ocs/statistics/statistics.html. Accessed November 8, 2016.
2. Daudt HM, van Mossel C, Dennis DL, et al. Survivorship care plans: a work in progress. Curr Oncol. 2014;21:e466-e479.
3. American Cancer Society. Cancer treatment and survivorship facts and figures: 2014-2015. www.cancer.org/acs/groups/content/@research/documents/document/acspc-042801.pdf. Accessed November 8, 2016.
4. Pratt-Chapman M, Simon MA, Patterson AK, et al. Survivorship navigation outcome measures: a report from the ACS patient navigation working group on survivorship navigation. Cancer. 2011;117(15 suppl):3575-3584.
5. Ekwueme DU, Yabroff KR, Guy GP Jr, et al. Medical costs and productivity losses of cancer survivors—United States, 2008-2011. MMWR Morb Mortal Wkly Rep. 2014;63:505-510.
6. American Cancer Society. What are the key statistics about multiple myeloma? Updated January 19, 2016. www.cancer.org/cancer/multiplemyeloma/detailedguide/multiple-myeloma-key-statistics. Accessed November 9, 2016.
7. National Cancer Institute Surveillance, Epidemiology, and End Results Program. SEER stat fact sheets: myeloma. http://seer.cancer.gov/statfacts/html/mulmy.html. Accessed November 9, 2016.
8. Pulte D, Jansen L, Brenner H. Most up-to-date long term survival estimates for common hematologic malignancies using the boomerang method. Poster presented at the 58th Annual Meeting of the American Society of Hematology; December 3, 2016; San Diego, CA.
9. Pratt G, Williams C. In pursuit of long-term disease control in multiple myeloma. 2016. http://keyopinions.info/downloads/in-pursuit-of-long-term-disease-control-in-multiple-myeloma. Accessed November 10, 2016.
10. Rajkumar SV. Treatment of myeloma: cure vs control. Mayo Clin Proc. 2008;83:1142-1145.
11. Fenichel MP. Treating multiple myeloma: the cause for optimism. J Natl Cancer Inst. 2014;106(5).
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18. International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. Br J Haematol. 2003;121:749-757.
19. Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol. 2014;15:e538-e548.
20. Bilotti E, Faiman BM, Richards TA, et al. Survivorship care guidelines for patients living with multiple myeloma: consensus statements of the International Myeloma Foundation Nurse Leadership Board. Clin J Oncol Nurs. 2011;15(suppl):5-8.
21. Allart-Vorelli P, Porro B, Baguet F, et al. Haematological cancer and quality of life: a systematic literature review. Blood Cancer J. 2015;5:e305.
22. Goodwin JA, Coleman EA, Sullivan E, et al. Personal financial effects of multiple myeloma and its treatment. Cancer Nurs. 2013;36:301-308.
23. Hodges AC. Working with cancer: how the law can help survivors maintain employment. Washington Law Rev. 2015;90:1039-1112.
24. Buzaglo JS, Miller MF, Karten C, et al. Multiple myeloma patient experience with financial toxicity: findings from the Cancer Experience Registry [abstract]. Blood. 2015;126:874.
25. Huntington SF, Weiss BM, Vogl DT, et al. Financial toxicity in insured patients with multiple myeloma: a cross-sectional pilot study. Lancet Haematol. 2015;2:e408-e416.
26. Health Research Institute. Medical cost trend: behind the numbers 2016. June 2015. www.achp.org/wp-content/uploads/pwc-hri-medical-cost-trend-2016.pdf. Accessed November 15, 2016.
27. Commonwealth Fund. How high is America’s health care cost burden? Findings from the Commonwealth Fund Health Care Affordability Tracking Survey, July-August 2015. www.commonwealthfund.org/publications/issue-briefs/2015/nov/how-high-health-care-burden. Accessed November 15, 2016.
28. Hewitt M, Greenfield S, Stovall E, eds. From Cancer Patient to Cancer Survivor: Lost in Transition. Washington, DC: National Academies Press; 2006.
29. Bilotti E, Gleason CL, McNeill A. Routine health maintenance in patients living with multiple myeloma: survivorship care plan of the International Myeloma Foundation Nurse Leadership Board. Clin J Oncol Nurs. 2011;15(suppl):25-40.
30. Roodman GD. Skeletal imaging and management of bone disease. Hematology Am Soc Hematol Educ Program. 2008:313-319.
31. Miceli TS, Colson K, Faiman BM, et al. Maintaining bone health in patients with multiple myeloma: survivorship care plan of the International Myeloma Foundation Nurse Leadership Board. Clin J Oncol Nurs. 2011;15(suppl):9-23.
32. Centers for Disease Control and Prevention. Home and recreational safety: Important facts about falls. Updated September 20, 2016. www.cdc.gov/homeandrecreationalsafety/falls/adultfalls.html. Accessed November 15, 2016.
33. Rome SI, Jenkins BS, Lilleby KE. Mobility and safety in the multiple myeloma survivor: survivorship care plan of the International Myeloma Foundation Nurse Leadership Board. Clin J Oncol Nurs. 2011;15(suppl):41-52.
34. Faiman BM, Mangan P, Spong J, Tariman JD. Renal complications in multiple myeloma and related disorders: survivorship care plan of the International Myeloma Foundation Nurse Leadership Board. Clin J Oncol Nurs. 2011;15(suppl):66-76.
35. Winearls CG. Acute myeloma kidney. Kidney Int. 1995;48:1347-1361.
36. Richards TA, Bertolotti PA, Doss D, McCullagh EJ. Sexual dysfunction in multiple myeloma: survivorship care plan of the International Myeloma Foundation Nurse Leadership Board. Clin J Oncol Nurs. 2011;15(suppl):53-65.