© Oncology Nursing Society. Unauthorized reproduction,
in part or in whole, is strictly prohibited. For permission to photocopy, post
online, reprint, adapt, or otherwise reuse any or all content from this
article, e-mail pubpermissions@ons.org.
To purchase high-quality reprints, email reprints@ons.org.
December 2013, Supplement to Volume 17, Number
6
Introduction
The Changing Landscape of Multiple Myeloma: Implications for
Oncology Nurses
Sandra Kurtin,
RN, MS, AOCN®, ANP-C, and Beth Faiman, MSN, APN-BC, AOCN®
Scientific advancements relative to diagnostic
evaluation, risk-adapted treatment selection, and supportive care strategies
for multiple myeloma (MM) have been developed in the past decade, which
provides hope for patients living with MM. However, the disease remains
incurable for the majority of patients, and continued clinical trials are
necessary to refine existing therapeutic strategies and develop new approaches
to treatment. Hematopoietic stem cell transplantation (HSCT), in particular
autologous HSCT, remains an important component in the overall treatment
paradigm for MM. This requires a well-organized team approach with ongoing
communications and collaboration with community providers and other
specialists. The majority of care for patients with MM is provided in the
outpatient setting, relying on the active participation of both the patient and
caregiver(s) for successful clinical outcomes. This supplement is prepared by
members of the International Myeloma Foundation Nurse Leadership Board, which
is dedicated to improving the care of patients with MM and their caregivers.
The introduction serves to provide an overview of MM today and to summarize the
articles included in this supplement.
Multiple myeloma (MM) is a plasma cell neoplasm
characterized by excess paraprotein secretion with
secondary organ effects including renal, bone, bone marrow, neurologic, and
immune dysfunction. About 22,350 new cases of MM are projected in 2013 (12,440
men, 9,910 women), with 10,710 deaths (6,070 men, 4,640 women) (Wallin & Larson, 2011). Risk factors for MM include
advanced age, male gender, obesity, and African American descent (American
Cancer Society [ACS], 2013; Perotta et al., 2013).
The incidence of MM in 2013 in African American men was estimated at 14.4 per
100,000, more than double the 6.6 per 100,000 for Caucasian men (ACS, 2013).
Similarly, African American women are more likely to develop MM compared to
Caucasian women (9.8 per 100,000 versus 4.1 per 100,000). MM is listed as the
10th most common type of cancer for both African American men and women, the
10th leading cause of cancer death in men, and the seventh leading cause of
cancer death in women (National Cancer Institute [NCI], 2010). The cause of the
increased incidence in the African American population has not been explained
and emphasizes the need for continued investigation into genetic predisposition
to this disease.
Previous studies evaluating occupational exposure in
MM have been limited by small sample size and variable measures for exposure to
selected chemical compounds. Perotta et al. (2013)
conducted a pooled analysis of five international case-controlled studies,
including 1,959 patients with MM and 6,192 control participants, evaluating the
association of occupational chemical exposure and the incidence of MM. Among a
wide range of work categories, gardeners, plant nursery workers, and crop
farmers were the most likely to be exposed to pesticides and showed a 50%
increased risk of developing MM in this analysis (odds ratio [OR] = 1.5, 95%
confidence interval [CI] [0.9, 2.3]). Metal processors (OR = 1.55, 95% CI
[0.98, 2.35]) and women working in the housekeeping or cleaning professions (OR
= 1.32, 95% CI [1, 1.76]) also showed increased risk attributed to exposure to
a range of potentially harmful substances such as arsenic, cadmium, lead, and
various cleaning solutions. The data emphasize the need to continue efforts in
identification of risk factors for MM and pursuit of opportunities to develop
prevention strategies.
Disease and Treatment
The disease continuum of MM encompasses distinct
clinical diagnoses, each defined by clinical and diagnostic criteria (see Figure 1). Monoclonal gammopathy
of undetermined significance is an asymptomatic premalignant condition that
precedes myeloma and does not require immediate treatment (Rajkumar,
2010). A 1% per year risk exists of progressing to MM; however, the overall
risk of progression to MM or a related plasma cell disorder is higher in
patients with higher paraprotein levels, an abnormal
kappa/lambda serum-free light chain ratio, and non–immunoglobulin-G (IgG) subtypes (Agarwal & Ghobrial, 2012; Rajkumar, 2010; Rajkumar et al., 2005). Smoldering myeloma (SM) is a more
advanced premalignant and asymptomatic precursor to MM with distinct clinical
findings and a greater risk of progression to MM (Rajkumar,
2010). Clinical trials are ongoing to evaluate the role of disease-modifying
treatment in the setting of SM.
Treatment is indicated when a patient has active MM
with evidence of end-organ damage as defined by the CRAB criteria (Calcium
elevation, Renal dysfunction, Anemia, and Bone disease).
The overall goal for treatment of MM is a complete response, with an acceptable
level of toxicity and quality of life (Palumbo & Cavallo,
2012). Achieving a complete response has been identified as a key factor in
improved progression-free survival and overall survival; however, achieving a
complete response does not imply eradication of the malignant clone. Survival
of patients with MM has improved significantly through continued clinical
investigation, the evolution of molecular and genetic profiling,
novel therapies, risk-adapted treatment selection, and better supportive care
(see Figures 2 and 3).
Despite these advances, MM remains incurable for the majority of patients with
expected relapses, each with unique clinical characteristics, patient
attributes, and treatment options (Palumbo & Anderson, 2011; Siegel & Bilotti, 2009) (see Figure 4).
Autologous hematopoietic stem cell transplantation
(AHSCT) remains an important treatment option for MM. Transplantation
eligibility is based on well-established clinical criteria and should be
considered at the time of diagnosis. Exposure to melphalan
and other stem cell toxic agents must be avoided prior to stem cell collection
(National Comprehensive Cancer Network [NCCN], 2013). Allogeneic HSCT remains
investigational and is generally reserved for patients with higher-risk disease
who have failed AHSCT and currently available novel therapies. It should only
be considered within the context of a clinical trial (NCCN, 2013; NCI, 2010).
The results of ongoing and future allogeneic HSCT trials will further elucidate
the role of nonmyeloablative or reduced-intensity
conditioning regimens in this setting.
Implications for Clinical Practice
HSCT requires planning and coordination from the
time a patient is considered a candidate for transplantation through the
post-transplantation period. The logistics of preparation, treatment,
follow-up, expected treatment-emergent adverse events, coordination of care
within and between settings, financial implications, and the patient-caregiver
dynamics must all be considered. The International Myeloma Foundation Nurse
Leadership Board is committed to improving the lives of patients living with
MM; therefore, this supplement provides a clinical guide to the care of
patients with MM undergoing HSCT. The primary focus is on AHSCT. This series of
articles also provides tools for forming a partnership with patients and
caregivers to improve self-management capabilities and, ultimately, improve
quality of life and clinical outcomes.
Miceli et al. (2013)
provides a road map to AHSCT for the patient with MM. A detailed description of
the role of AHSCT in the treatment of MM; eligibility criteria; and pretransplantation, peritransplantation,
and post-transplantation considerations for patients, caregivers, and providers
in multiple settings is offered. As previously mentioned, the patient
undergoing AHSCT will receive a bulk of his or her care in the outpatient
setting, and much of this will occur in the patient’s community.
Clinical guidelines are included to provide the
community oncology professional with tools to assist in collaborative
management of patients with MM undergoing AHSCT. Given the heterogeneity of the
MM population, an individualized approach to therapy is necessary, and
variability in treatment approaches based on patient-specific factors is
common. The article by Mangan, Gleason, and Miceli (2013) addresses the frequently asked questions
pertaining to common decision points in the process of HSCT, such as: Who are
good candidates for AHSCT? What is the optimal timing of an AHSCT? What is the
role of allogenic-HCT in the treatment of MM? And
what is the role of maintenance therapy following AHSCT?
Faiman, Miceli,
Noonan and Lilleby (2013) provide an update on
scientific developments pertaining to the process of HSCT relative to MM.
Common preparative regimens, techniques for stem cell mobilization and
collection, and management of the patient in the peritransplantation
and immediate post-transplantation period are described.
The availability of a caregiver is a prerequisite to
HSCT eligibility. Caregivers may include spouses or other family members,
friends, or volunteers. These individuals play a critical role in the effective
management of the patient prior to, during, and following an HSCT. Caregiver
stress and strain are common and may have a negative effect on the quality of
life of the patient and the caregiver. Kurtin, Lilleby, and Spong (2013) review
key components of the caregiver role for the patient with MM, common attributes
of caregiver stress or strain, and guidelines for assessment of caregiver
stress. Strategies for empowering the caregiver and resources and tools to
promote self-management are provided.
Conclusion
The scientific advances in the field of MM relative
to the pathobiology of the disease, identification of potential new targets for
therapy, mechanisms of resistance, and integration of new agents into the
existing treatment paradigm are ongoing. Integrating these changes into
clinical practice and anticipating continued developments is a challenge for the
oncology professional. HSCT remains an important component of the treatment
paradigm. Familiarity with eligibility criteria, pretransplantation
evaluation, the actual transplantation process, and supportive care for the
patient throughout the treatment continuum will improve the care of patients
with MM undergoing HSCT. Integrating tools and strategies for patient and
caregiver self-management as well as caregiver support will improve the active
participation and quality of life for both groups. Continued engagement and
collaboration with oncology professionals in support of the patient and
caregiver and in robust scientific discovery will be necessary to effectively
integrate these new techniques or strategies into the MM treatment and
supportive care paradigm.
The authors gratefully acknowledge Brian G.M. Durie, MD, Robert A. Kyle, MD, and Diane P. Moran, RN, MA, EdM, senior vice president of strategic planning at the
International Myeloma Foundation, for their critical review of the manuscript.
Implications for Practice
Ø
Understanding the current approach to the
treatment of multiple myeloma can help oncology nurses provide optimal
situations for their patients.
Ø
Knowing the disease characteristics for
multiple myeloma, smoldering myeloma, and monoclonal gammopathy of undetermined significance can aid in early
detection.
Ø
Examining the impact novel agents have had on
improving survival for patients with multiple myeloma can help oncology nurses,
patients, and caregivers understand possible treatment choices.
References
Agarwal,
A., & Ghobrial, I.M. (2012).
Monoclonal gammopathy of undetermined significance
and smoldering multiple myeloma: A review of the current understanding of
epidemiology, biology, risk stratification, and management of myeloma precursor
disease. Clinical Cancer Research, 19, 985–994. http://dx.doi.org/10.1158/1078-0432.CCR-12-2922
American Cancer Society. (2013).
Cancer facts and figures 2013. Retrieved from http://www.cancer.org/research/cancerfactsfigures/cancerfactsfigures/cancer-facts-figures-2013
Durie, B.G., Kyle, R.A., Belch, A., Bensinger,
W., Blade, J., Boccadoro, M., . . .
Van Ness, B. (2003). Myeloma management guidelines: A consensus report from
Scientific Advisors of the International Myeloma Foundation. Hematology
Journal, 4, 379–398. http://dx.doi.org/10.1038/sj.thj.6200312
Faiman,
B., Miceli, T., Noonan, K., & Lilleby,
K. (2013). Clinical update in bone marrow
transplantation in multiple myeloma. Clinical Journal of Oncology
Nursing, 17(Suppl., 2), 33–41. http://dx.doi.org/10.1188/13.CJON.S2.33-41
Kuehl,
W.M., & Bergsagel, P.L. (2002).
Multiple myeloma: Evolving genetic events and host interactions. Nature
Reviews: Cancer, 2, 175–187. http://dx.doi.org/10.1038/nrc746
Kumar,
S., Dispenzieri, A., Gertz,
M., Lacy, M., Lust, J., Hayman, S., . . . Rajkumar, S.V. (2012). Continued
improvement in survival in multiple myeloma and the impact of novel agents
[Abstract 3972]. Retrieved from http://myeloma.org/pdfs/ASH2012_Kumar_3865.pdf
Kurtin,
S., Lilleby, K., & Spong,
J. (2013). Caregivers of multiple myeloma
survivors. Clinical Journal of Oncology Nursing, 17(Suppl., 2),
25–32. http://dx.doi.org/10.1188/13.CJON.S2.25-32
Mangan,
P., Gleason, C., & Miceli, T. (2013).
Autologous hematopoietic stem cell transplantation for multiple myeloma:
Frequently asked questions. Clinical Journal of Oncology Nursing, 17(Suppl.,
2), 43–47. http://dx.doi.org/10.1188/13.CJON.S2.43-47
Miceli, T., Lilleby, K., Noonan,
K., Kurtin, S., Faiman, B.,
& Mangan, P. (2013). Autologous
hematopoietic stem cell transplantation for patients with multiple myeloma.
An overview for nurses in community practice. Clinical
Journal of Oncology Nursing, 17(Suppl., 2), 13–24. http://dx.doi.org/10.1188/13.CJON.S2.13-24
National
Cancer Institute. (2010). SEER stat fact sheets: Myeloma. Retrieved from http://seer.cancer.gov/statfacts/html/mulmy.html
National Comprehensive Cancer Network.
(2013). NCCN Clinical Practice Guidelines in Oncology: Multiple myeloma
[v.1.2013]. Retrieved from http://www.nccn.org/professionals/physician_gls/f_guidelines.asp#myeloma
Palumbo, A., & Anderson, K. (2011). Multiple myeloma. New England Journal of Medicine, 364,
1046–1060. http://dx.doi.org/10.1056/NEJMra1011442
Palumbo, A., & Cavallo, F. (2012).
Have drug combinations supplanted stem cell transplantation in myeloma? Blood,
120, 4692–4698. http://dx.doi.org/10.1182/blood-2012-05-423202
Perotta, C., Kleefeld, S., Staines, A., Tewari, P., De Roos, A.J., Baris, D., . . . Cocco, P. (2013).
Multiple myeloma and occupation: A polled analysis by the International
Multiple Myeloma Consortium. Cancer Epidemiology, 37, 300–305. http://dx.doi.org/10.1016/j.canep.2013.01.008
Rajkumar, S.V. (2010). Multiple myeloma: 2011 update
on diagnosis, risk-stratification, and management. American Journal of
Hematology, 86, 57–65. http://dx.doi.org/10.1002/ajh.21913
Rajkumar, S.V., Kyle, R.A., Therneau,
T.M., Melton, L.J., Bradwell, A.R., Clark, R.J., . . . Katzmann, J.A. (2005).
Serum free light chain ratio is an independent risk factor for progression in
monoclonal gammopathy of undetermined significance. Blood,
106, 812–817. http://dx.doi.org/10.1182/blood-2005-03-1038
Siegel, D.S., & Bilotti, E. (2009). New directions in therapy for multiple myeloma. Community
Oncology, 6(Suppl., 3), 22–30.
Vacca,
A., & Ribatti, D. (2006). Bone marrow angiogenesis in multiple myeloma. Leukemia,
20, 193–199. http://dx.doi.org/10.1182/blood-2005
-03-1038
Wallin,
A., & Larson, S.C. (2011). Body mass index and risk of
multiple myeloma: A meta-analysis of prospective studies. European Journal
of Cancer, 47, 1606–1611. http://dx.doi.org/10.1016/j.ejca.2011.01.020
Sandra Kurtin, RN, MS, AOCN®, ANP-C, is a nurse
practitioner and clinical assistant professor of medicine in the
Hematology/Oncology Division of the University of Arizona Cancer Center in
Tucson, and Beth
Faiman, MSN, APN-BC, AOCN®, is a nurse practitioner in
the Taussig Cancer Center at the Cleveland Clinic in Ohio. The authors received editorial support from Alita Anderson, MD, with Eubio
Medical Communications in preparation of this article supported by Sanofi Oncology. The authors are fully responsible for
content and editorial decisions about this article. Kurtin
serves as a consultant for Celgene Corporation,
Novartis Pharmaceuticals, Millennium: The Takeda
Oncology Company, and Onyx Pharmaceuticals. Faiman
has no financial relationships to disclose. The content of this article has
been reviewed by independent peer reviewers to ensure that it is balanced,
objective, and free from commercial bias. No financial relationships relevant
to the content of this article have been disclosed by the independent peer
reviewers or editorial staff. Kurtin can be reached
at sandra.kurtin@uahealth.com,
with copy to editor at CJONEditor@ons.org.
(Submitted July 2013. Revision submitted September
2013. Accepted for publication September 12, 2013.)
http://dx.doi.org/10.1188/13.CJON.S2.7-11