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Supportive Care
Valerie Burger, RN, MA, MS, OCN®—Associate Editor
Providing Care for Previvors: Implications for Oncology Nurses Suzanne M. Mahon, RN, DNSc, AOCN®, APNG
activities might include more aggressive screening than those recommended for people of average risk (American Cancer Society, 2013). Consequently, previvors often are confronted with complicated and emotionally charged decisions regarding management strategies (Tercyak, Mays, DeMarco, Sharff, & Friedman, 2012).
stood that her risk reduction for breast cancer would be more than 97% with PMs (Bevers et al., 2010; Zagouri et al., 2013), and she completed that at age 31 years, followed by immediate breast reconstruction with tissue expanders and implants. She has been undergoing pelvic examinations every six months with a gynecologic oncologist, CA 125 test, and pelvic ultrasound. She presents to the gynecologic oncologist to consider prophylactic salpingo-oophorectomy (PSO). She is informed that the risk for ovarian cancer would be reduced by as much as 96% if completed before age 45 years (Finch, Evans, & Narod, 2012). Mrs. T also is informed that debate exists about the need for removal of the uterus at the time of the PSO because a small interstitial portion of the tube is left on the uterus in a PSO; however, few systematic reviews exist, and the risk of ovarian cancer appears similar to that of women who have a full hysterectomy (Gadducci, Biglia, Cosio, Sismondi, & Genazzani, 2010).
Case Study
Management
Mrs. T is a 38-year-old married mother of an 11-year-old daughter and a 13-yearold son. She works part-time as a business manager in an auto parts company. At age 31, she tested positive for a known BRCA2 mutation, associated with a lifetime risk of developing breast cancer of about 90%, and a 45% risk of developing ovarian cancer (Lindor, McMaster, Lindor, & Greene, 2008). Mrs. T was informed by the genetics professional about her lifetime risks for developing cancer and her options for surveillance, chemoprevention, and prophylactic surgery, both before testing and in follow-up sessions. She under-
Many previvors will initially manage risk with increased surveillance until the stress and worry about cancer risk motivates the individual to pursue prophylactic surgery (Hoskins, Roy, & Greene, 2012). The integration of the diagnosis of hereditary risk takes time, and subsequent decisions about prophylactic surgery often are influenced by previvor experiences with family members affected by cancer, experiences with prophylactic surgery, and whether they perceive their healthcare provider as genuinely concerned about their psychosocial needs and concerns (Hamilton, Williams, Bowers, & Calzone, 2009; Howard, Balneaves, Bottorff, & Rodney, 2011).
Previvors are individuals who are survivors of a genetic predisposition for developing cancer. They often are confronted with difficult decisions about management of risks that might include aggressive screening and prophylactic surgery. Psychosocial challenges exist for the affected individual, their partners, and offspring. Oncology nurses need to be aware of the complex and special needs of this ever-growing population. Suzanne M. Mahon, RN, DNSc, AOCN®, APNG, is a professor in the Department of Internal Medicine and in the School of Nursing at Saint Louis University in Missouri. The author takes full responsibility for the content of the article. The author did not receive honoraria for this work. No financial relationships relevant to the content of this article have been disclosed by the author or editorial staff. Mahon can be reached at [email protected], with copy to editor at [email protected]. Key words: distress; ethical issues; genetics/genomics; patient/public education; prevention and detection; survivorship; surgery Digital Object Identifier: 10.1188/14.CJON.21-24
A
bout 10% of individuals diagnosed with cancer have a genetic predisposition, many of which can be detected with genetic predisposition testing (Weitzel, Blazer, MacDonald, Culver, & Offit, 2011). Once a mutation is identified in a family, other first-degree relatives have a 50% chance of also having the mutation. The term “previvor” became a buzzword in 2007, and describes an individual who is a survivor with a genetic predisposition for developing cancer (Cruz, 2007). The public revelation by Angelina Jolie that she is BRCA positive and has undergone prophylactic mastectomies (PMs) has heightened awareness of the needs of previvors (Kluger & Park, 2013). Previvors represent an ever-growing population of patients with specific psychosocial and healthcare needs, and they need comprehensive and impartial information to help them decide how to manage this substantial risk. Ideally, primary prevention strategies are used, which include prophylactic surgery and, sometimes, chemoprevention. Secondary prevention
Clinical Journal of Oncology Nursing • Volume 18, Number 1 • Supportive Care
21
In the case of a positive BRCA mutation, these decisions can be particularly stressful and complicated for young women whose peers typically are confronting fewer and much less emotionally charged decisions (Hoskins et al., 2012). The plethora of negative opinions and reactions that these women face can be overwhelming and is in direct contrast to the support that many individuals encounter when diagnosed with cancer. The diagnosis is accompanied by varying degrees of stigmatization and opinion that regard prophylactic surgery as drastic. Stigmatization is accompanied by feeling isolated, being labeled, and feeling different from those who do not carry a mutation, even if the exterior appearance of the woman remains unchanged after prophylactic and reconstructive surgery (den Heijer et al., 2012). Previvors who choose PMs make concessions. Despite advances, reconstruction is not perfect and requires a series of procedures that can take months to complete. Changes in body image are very real. Even if the woman does not appear different in clothes, she and her partner
know her physical body has changed (Gopie et al., 2012). Not all women are satisfied with the cosmetic outcome. Most women report substantially less worry about developing breast cancer after surgery is completed, but it comes after the sacrifices and changes that accompany prophylactic surgery (Litton et al., 2009).
Support Family members, particularly partners, can be a source of psychosocial support as previvors make test-related decisions (Tercyak et al., 2012). The importance of social support from family members should not be underestimated (DeMarco et al., 2010; Sherman, Kasparian, & Mireskandari, 2010). The impact of the diagnosis of a mutation can lead to significant psychosocial distress and stress for the partners as well. Distress is decreased in partners who are with the woman during both the pretest and disclosure meetings with the genetics professional (den Heijer et al., 2011). Better long-term adjustment may
TABLE 1. Web-Based Resources for Previvors and Families Agency
Resources
Bright Pink www.brightpink.org
Provides resources and support for women with a hereditary risk for breast and ovarian cancer • Educational booklets in English and Spanish • Activities and educational programs for women at increased risk • One-to-one peer support
FORCE (Facing Our Risks of Cancer Empowered) www.facingourrisk.org
Provides resources and support for previvors and women with hereditary breast and ovarian cancer syndromes • Online support • Local support groups • Mastectomy photo gallery • Education and publications • Spouse or partner support • Advocacy
GeneTests www.ncbi.nlm.nih.gov/ sitesGeneTests/?db=GeneTests
Provides resources for health professionals on genetic syndromes • Expert-authored peer-reviewed disease publications • Resource list • Laboratory directory
National Society of Genetic Counselors www.nsgc.org
Provides resources for patients and for healthcare professionals related to genetic syndromes • Searchable list of genetics professionals • Professional statements on issues related to BRCA testing
Sharsheret www.sharsheret.org
Provides resources and support for women of Jewish ancestry who have a genetic predisposition for developing cancer • Phone support • Local support groups • Education and publications
22
be associated with open communication with family members and partners (den Heijer et al., 2011). Encouraging couples to talk about their feelings may promote better adjustment and intimacy and, for this reason, couples are encouraged to attend counseling and physician appointments together. One possible source of psychosocial support and education for previvors might include timely supportive information from Internet-based resources (Tercyak et al., 2012). Some previvors and their family members may prefer Internet-based resources to face-to-face psychosocial services and counseling because they offer anonymity when exploring sensitive topics, as well as providing direct access to factual information (see Table 1).
Hereditary Risk Statistically, mutation carriers have a 50% chance of passing the mutation to the offspring. Many previvors have not yet completed their families and have to deal with the stressors of realizing they can pass the risk to subsequent children. In a study of 284 previvors, 44% experienced extreme worry about passing a mutation to subsequent generations (Staton, Kurian, Cobb, Mills, & Ford, 2008). Young previvors also need to consider whether they have PMs before or after childbearing is complete, which is challenging and associated with concessions; previvors must choose between breastfeeding and decreased worry about cancer risk (Hoskins et al., 2012). For single women, fears can occur about what a future partner may think about the decision to have PMs, and how to explain such decisions (Hamilton & Hurley, 2010). Many mothers choose surgery because they fear a premature death and leaving their children without a mother. The decision to have a prophylactic PSO means acknowledging that childbearing is complete. A related issue previvors must address is when and how to discuss hereditary risk with their children (Tercyak et al., 2011). Parents may or may not convey information about risk of having a mutation accurately (Patenaude et al., 2013). Many parents are faced with the decision to tell children when a mother undergoes prophylactic surgery (Farkas Patenaude et al., 2012). The importance of honesty and
February 2014 • Volume 18, Number 1 • Clinical Journal of Oncology Nursing
Cancer prevention and early detection for other cancers • Colonoscopy starting at age 50 years and every 7–10 years • Annual skin examination • Tobacco elimination • Regular use of sunscreen, protective clothing, and hats; minimize ultraviolet light exposure. • Maintain body weight. • Increase fresh fruit and vegetable intake; decrease fat intake. • Increase intentional exercise to 150 minutes per week. Management of bone health risks • Counseling regarding the importance of daily weight-bearing exercise • Bone density testing every one to two years starting at the time of the PSO • Vitamin D 1,000 IU per day and calcium 1,500 mg per day Management of peritoneal cancer risks • Yearly pelvic examination to assess for peritoneal malignancies • Discussion with provider regarding risks and benefits of CA 125 testing for peritoneal malignancies • Counseling regarding subtle symptoms of primary peritoneal cancer Menopause management • Hormone therapy considered in eligible premenopausal patients with a balanced discussion of the potential risks and benefits • Education about nonhormonal means to manage vulvarvaginal dystrophy • Education about hot flash management and possible evidence-based methods of management PSO—prophylactic salpingo-oophorectomy
FIGURE 1. Long-Term Follow-Up in Previvors With BRCA Mutations Following Prophylactic Surgery Note. Based on information from American Cancer Society, 2013; Chapman et al., 2011; Finch et al., 2012; Gadducci et al., 2010; Kaplan et al., 2011; Matloff et al., 2009; Sturdee & Panay, 2010.
words to use, and the importance of longterm open communication. Decision aids to assist with this procedure are available (Peshkin, DeMarco, & Tercyak, 2010). In general, testing of adolescents is not recommended. Testing usually is completed at a point in which it would influence decisions regarding screening or prevention. Most adolescents do not need prophylaxis. Although many pediatricians and primary care providers will order genetic testing on adolescents if requested by parents, testing probably is best managed by a genetics professional who is experienced with genetic risk assessment pretest counseling (O’Neill et al., 2010). Genetic testing often is recommended by age 25 years, or 10 years before the first cancer diagnosis in the family because that is when surveillance would begin. Although many do test negative, for those who test positive, it can be frightening, particularly for younger women who do not have peers experiencing similar choices.
Conclusions Mrs. T opted to have a total hysterectomy, including PSO, because she had two relatives who were diagnosed with ovarian cancer around age 45 years. She will require ongoing management of the side effects related to her prophylactic surgical choices (see Figure 1). Mrs. T was informed of the risks of premature menopause, including bone loss, vaginal dryness, and hot flashes (Matloff, Barnett, & Bober, 2009). Although she experienced relief and liberation in knowing she had taken all possible steps to prevent cancer, she did experience significant hot flashes and some vaginal dryness. The lifetime risk for developing peritoneal cancer is still at least 3%–4%, and longterm surveillance is needed (Chapman et al., 2011).
References emphasizing that a mother is taking all steps to prevent future problems should not be underestimated. Parents may need assistance finding a balance between protecting and educating their children. For this reason, consultation with a genetics professional prior to disclosure is recommended to help parents consider the child’s maturity, potential approaches,
American Cancer Society. (2013). Cancer facts and figures 2013. Atlanta, GA: Author. Bevers, T.B., Armstrong, D.K., Arun, B., Carlson, R.W., Cowan, K.H., Daly, M.B., . . . Ward, J.H. (2010). Breast cancer risk reduction. Journal of the National Comprehensive Cancer Network, 8, 1112–1146. Chapman, J.S., Powell, C.B., McLennan, J.,
Clinical Journal of Oncology Nursing • Volume 18, Number 1 • Supportive Care
Crawford, B., Mak, J., Stewart, N., & Chen, L.M. (2011). Surveillance of survivors: Follow-up after risk-reducing salpingooophorectomy in BRCA 1/2 mutation carriers. Gynecologic Oncology, 122, 339–343. doi:10.1016/j.ygyno.2011.04.004 Cruz, G. (2007). The ten best buzzwords. Time, 170(26), 70. DeMarco, T.A., Nusbaum, R.H., Peshkin, B.N., Patenaude, A.F., Schneider, K.A., Garber, J.E., . . . Tercyak, K.P. (2010). Prevalence and correlates of mothers and fathers attending pretest cancer genetic counseling together. Patient Education and Counseling, 78, 29–33. den Heijer, M., Seynaeve, C., Vanheusden, K., Duivenvoorden, H.J., Bartels, C.C., Menke-Pluymers, M.B., & Tibben, A. (2011). Psychological distress in women at risk for hereditary breast cancer: The role of family communication and perceived social support. Psycho-Oncology, 20, 1317–1323. doi:10.1002/pon.1850 den Heijer, M., Vos, J., Seynaeve, C., Vanheusden, K., Duivenvoorden, H.J., TilanusLinthorst, M., . . . Tibben, A. (2012). The impact of social and personal resources on psychological distress in women at risk for hereditary breast cancer. PsychoOncology, 21, 153–160. doi:10.1002/ pon.1879 Farkas Patenaude, A., DeMarco, T.A., Peshkin, B.N., Valdimarsdottir, H., Garber, J.E., Schneider, K., . . . Tercyak, K.P. (2012). Talking to children about maternal BRCA1/2 genetic test results: A qualitative study of parental perceptions and advice. Journal of Genetic Counseling, 22, 303–314. doi:10.1007/s10897-012-9549-z Finch, A., Evans, G., & Narod, S.A. (2012). BRCA carriers, prophylactic salpingooophorectomy, and menopause: Clinical management considerations and recommendations. Women’s Health, 8, 543–555. doi:10.2217/whe.12.41 Gadducci, A., Biglia, N., Cosio, S., Sismondi, P., & Genazzani, A.R. (2010). Gynaecologic challenging issues in the management of BRCA mutation carriers: Oral contraceptives, prophylactic salpingo-oophorectomy, and hormone replacement therapy. Gynecological Endocrinology, 26, 568–577. doi:10.310 9/09513590.2010.487609 Gopie, J., Mureau, M.A., Seynaeve, C., Ter Kuile, M.M., Menke-Pluymers, M.B., Timman, R., & Tibben, A. (2012). Body image issues after bilateral prophylactic mastectomy with breast reconstruction in healthy women at risk for hereditary breast cancer. Familial Cancer, 12, 479–487. doi:10.1007/s10689-012-9588-5 23
Hamilton, R., & Hurley, K.E. (2010). Conditions and consequences of a BRCA mutation in young, single women of childbearing age. Oncology Nursing Forum, 37, 627–634. doi:10.1188/10.onf.627-634 Hamilton, R., Williams, J.K., Bowers, B.J., & Calzone, K. (2009). Life trajectories, genetic testing, and risk reduction decisions in 18–39 year old women at risk for hereditary breast and ovarian cancer. Journal of Genetic Counseling, 18, 147–159. doi:10.1007/s10897-008-9200-1 Hoskins, L.M., Roy, K.M., & Greene, M.H. (2012). Toward a new understanding of risk perception among young female BRCA1/2 “previvors.” Families, Systems, & Health, 30 (1), 32–46. doi:10.1037/ a0027276 Howard, A.F., Balneaves, L.G., Bottorff, J.L., & Rodney, P. (2011). Preserving the self: The process of decision making about hereditary breast cancer and ovarian cancer risk reduction. Qualitative Health Research, 21, 502–519. doi:10.1177/ 1049732310387798 Kaplan, M., Mahon, S., Cope, D., Keating, E., Hill, S., & Jacobson, M. (2011). Putting evidence into practice: Evidence-based interventions or hot flashes resulting from cancer therapies. Clinical Journal of Oncology Nursing, 15, 149–157. doi:10.1188/11.CJON.149-157 Kluger, J., & Park, A. (2013). The Angelina effect. Time, 181(20), 28–33. Lindor, N.M., McMaster, M.L., Lindor, C.J., & Greene, M.H. (2008). Concise handbook of familial cancer susceptibility syndromes (2nd ed.). Journal of the National Cancer Institute. Monographs, 2008(38), 3–93. doi:10.1093/jncimono graphs/lgn001 Litton, J.K., Westin, S.N., Ready, K., Sun, C.C., Peterson, S.K., Meric-Bernstam, F., . . . Arun, B.K. (2009). Perception of
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screening and risk reduction surgeries in patients tested for a BRCA deleterious mutation. Cancer, 115, 1598–1604. doi:10.1002/cncr.24199 Matloff, E.T., Barnett, R.E., & Bober, S.L. (2009). Unraveling the next chapter: Sexual development, body image, and sexual functioning in female BRCA carriers. Cancer Journal, 15, 15–18. O’Neill, S.C., Peshkin, B.N., Luta, G., Abraham, A., Walker, L.R., & Tercyak, K.P. (2010). Primary care providers’ willingness to recommend BRCA1/2 testing to adolescents. Familial Cancer, 9, 43–50. doi:10.1016/j.ygyno.2011.04.004 Patenaude, A.F., Tung, N., Ryan, P.D., Ellisen, L.W., Hewitt, L., Schneider, K.A., . . . Garber, J.E. (2013). Young adult daughters of BRCA1/2 positive mothers: What do they know about hereditary cancer and how much do they worry? Psycho-Oncology, 22, 2024–2031. doi:10.1002/pon.3257 Peshkin, B.N., DeMarco, T.A., & Tercyak, K.P. (2010). On the development of a decision support intervention for mothers undergoing BRCA1/2 cancer genetic testing regarding communicating test results to their children. Familial Cancer, 9, 89–97. Sherman, K.A., Kasparian, N.A., & Mireskandari, S. (2010). Psychological adjustment among male partners in response to women’s breast/ovarian cancer risk: A theoretical review of the literature. PsychoOncology, 19, 1–11. doi:10.1002/pon.1582
Staton, A.D., Kurian, A.W., Cobb, K., Mills, M.A., & Ford, J.M. (2008). Cancer risk reduction and reproductive concerns in female BRCA1/2 mutation carriers. Familial Cancer, 7, 179–186. doi:10.1007/ s10689-007-9171-7 Sturdee, D.W., & Panay, N. (2010). Recommendations for the management of postmenopausal vaginal atrophy. Climacteric, 13, 509–522. Tercyak, K.P., Hensley Alford, S., Emmons, K.M., Lipkus, I.M., Wilfond, B.S., & McBride, C.M. (2011). Parents’ attitudes toward pediatric genetic testing for common disease risk. Pediatrics, 127, e1288– e1295. doi:10.1542/peds.2010-0938 Tercyak, K.P., Mays, D., DeMarco, T.A., Sharff, M.E., & Friedman, S. (2012). Results of an online community needs assessment for psychoeducational interventions among partners of hereditary breast cancer previvors and survivors. Journal of Medical Internet Research, 14, 24–26. doi:10.2196/jmir.1847 Weitzel, J.N., Blazer, K.R., MacDonald, D.J., Culver, J.O., & Offit, K. (2011). Genetics, genomics, and cancer risk assessment. CA: A Cancer Journal for Clinicians, 61, 327–359. doi:10.3322/caac.20128 Zagouri, F., Chrysikos, D.T., Sergentanis, T.N., Giannakopoulou, G., Zografos, C.G., Papadimitriou, C.A., & Zografos, G.C. (2013). Prophylactic mastectomy: An appraisal. American Surgeon, 79, 205–212.
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February 2014 • Volume 18, Number 1 • Clinical Journal of Oncology Nursing
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Supportive Care
Valerie Burger, RN, MA, MS, OCN®—Associate Editor
Providing Care for Previvors: Implications for Oncology Nurses Suzanne M. Mahon, RN, DNSc, AOCN®, APNG
activities might include more aggressive screening than those recommended for people of average risk (American Cancer Society, 2013). Consequently, previvors often are confronted with complicated and emotionally charged decisions regarding management strategies (Tercyak, Mays, DeMarco, Sharff, & Friedman, 2012).
stood that her risk reduction for breast cancer would be more than 97% with PMs (Bevers et al., 2010; Zagouri et al., 2013), and she completed that at age 31 years, followed by immediate breast reconstruction with tissue expanders and implants. She has been undergoing pelvic examinations every six months with a gynecologic oncologist, CA 125 test, and pelvic ultrasound. She presents to the gynecologic oncologist to consider prophylactic salpingo-oophorectomy (PSO). She is informed that the risk for ovarian cancer would be reduced by as much as 96% if completed before age 45 years (Finch, Evans, & Narod, 2012). Mrs. T also is informed that debate exists about the need for removal of the uterus at the time of the PSO because a small interstitial portion of the tube is left on the uterus in a PSO; however, few systematic reviews exist, and the risk of ovarian cancer appears similar to that of women who have a full hysterectomy (Gadducci, Biglia, Cosio, Sismondi, & Genazzani, 2010).
Case Study
Management
Mrs. T is a 38-year-old married mother of an 11-year-old daughter and a 13-yearold son. She works part-time as a business manager in an auto parts company. At age 31, she tested positive for a known BRCA2 mutation, associated with a lifetime risk of developing breast cancer of about 90%, and a 45% risk of developing ovarian cancer (Lindor, McMaster, Lindor, & Greene, 2008). Mrs. T was informed by the genetics professional about her lifetime risks for developing cancer and her options for surveillance, chemoprevention, and prophylactic surgery, both before testing and in follow-up sessions. She under-
Many previvors will initially manage risk with increased surveillance until the stress and worry about cancer risk motivates the individual to pursue prophylactic surgery (Hoskins, Roy, & Greene, 2012). The integration of the diagnosis of hereditary risk takes time, and subsequent decisions about prophylactic surgery often are influenced by previvor experiences with family members affected by cancer, experiences with prophylactic surgery, and whether they perceive their healthcare provider as genuinely concerned about their psychosocial needs and concerns (Hamilton, Williams, Bowers, & Calzone, 2009; Howard, Balneaves, Bottorff, & Rodney, 2011).
Previvors are individuals who are survivors of a genetic predisposition for developing cancer. They often are confronted with difficult decisions about management of risks that might include aggressive screening and prophylactic surgery. Psychosocial challenges exist for the affected individual, their partners, and offspring. Oncology nurses need to be aware of the complex and special needs of this ever-growing population. Suzanne M. Mahon, RN, DNSc, AOCN®, APNG, is a professor in the Department of Internal Medicine and in the School of Nursing at Saint Louis University in Missouri. The author takes full responsibility for the content of the article. The author did not receive honoraria for this work. No financial relationships relevant to the content of this article have been disclosed by the author or editorial staff. Mahon can be reached at [email protected], with copy to editor at [email protected]. Key words: distress; ethical issues; genetics/genomics; patient/public education; prevention and detection; survivorship; surgery Digital Object Identifier: 10.1188/14.CJON.21-24
A
bout 10% of individuals diagnosed with cancer have a genetic predisposition, many of which can be detected with genetic predisposition testing (Weitzel, Blazer, MacDonald, Culver, & Offit, 2011). Once a mutation is identified in a family, other first-degree relatives have a 50% chance of also having the mutation. The term “previvor” became a buzzword in 2007, and describes an individual who is a survivor with a genetic predisposition for developing cancer (Cruz, 2007). The public revelation by Angelina Jolie that she is BRCA positive and has undergone prophylactic mastectomies (PMs) has heightened awareness of the needs of previvors (Kluger & Park, 2013). Previvors represent an ever-growing population of patients with specific psychosocial and healthcare needs, and they need comprehensive and impartial information to help them decide how to manage this substantial risk. Ideally, primary prevention strategies are used, which include prophylactic surgery and, sometimes, chemoprevention. Secondary prevention
Clinical Journal of Oncology Nursing • Volume 18, Number 1 • Supportive Care
21
In the case of a positive BRCA mutation, these decisions can be particularly stressful and complicated for young women whose peers typically are confronting fewer and much less emotionally charged decisions (Hoskins et al., 2012). The plethora of negative opinions and reactions that these women face can be overwhelming and is in direct contrast to the support that many individuals encounter when diagnosed with cancer. The diagnosis is accompanied by varying degrees of stigmatization and opinion that regard prophylactic surgery as drastic. Stigmatization is accompanied by feeling isolated, being labeled, and feeling different from those who do not carry a mutation, even if the exterior appearance of the woman remains unchanged after prophylactic and reconstructive surgery (den Heijer et al., 2012). Previvors who choose PMs make concessions. Despite advances, reconstruction is not perfect and requires a series of procedures that can take months to complete. Changes in body image are very real. Even if the woman does not appear different in clothes, she and her partner
know her physical body has changed (Gopie et al., 2012). Not all women are satisfied with the cosmetic outcome. Most women report substantially less worry about developing breast cancer after surgery is completed, but it comes after the sacrifices and changes that accompany prophylactic surgery (Litton et al., 2009).
Support Family members, particularly partners, can be a source of psychosocial support as previvors make test-related decisions (Tercyak et al., 2012). The importance of social support from family members should not be underestimated (DeMarco et al., 2010; Sherman, Kasparian, & Mireskandari, 2010). The impact of the diagnosis of a mutation can lead to significant psychosocial distress and stress for the partners as well. Distress is decreased in partners who are with the woman during both the pretest and disclosure meetings with the genetics professional (den Heijer et al., 2011). Better long-term adjustment may
TABLE 1. Web-Based Resources for Previvors and Families Agency
Resources
Bright Pink www.brightpink.org
Provides resources and support for women with a hereditary risk for breast and ovarian cancer • Educational booklets in English and Spanish • Activities and educational programs for women at increased risk • One-to-one peer support
FORCE (Facing Our Risks of Cancer Empowered) www.facingourrisk.org
Provides resources and support for previvors and women with hereditary breast and ovarian cancer syndromes • Online support • Local support groups • Mastectomy photo gallery • Education and publications • Spouse or partner support • Advocacy
GeneTests www.ncbi.nlm.nih.gov/ sitesGeneTests/?db=GeneTests
Provides resources for health professionals on genetic syndromes • Expert-authored peer-reviewed disease publications • Resource list • Laboratory directory
National Society of Genetic Counselors www.nsgc.org
Provides resources for patients and for healthcare professionals related to genetic syndromes • Searchable list of genetics professionals • Professional statements on issues related to BRCA testing
Sharsheret www.sharsheret.org
Provides resources and support for women of Jewish ancestry who have a genetic predisposition for developing cancer • Phone support • Local support groups • Education and publications
22
be associated with open communication with family members and partners (den Heijer et al., 2011). Encouraging couples to talk about their feelings may promote better adjustment and intimacy and, for this reason, couples are encouraged to attend counseling and physician appointments together. One possible source of psychosocial support and education for previvors might include timely supportive information from Internet-based resources (Tercyak et al., 2012). Some previvors and their family members may prefer Internet-based resources to face-to-face psychosocial services and counseling because they offer anonymity when exploring sensitive topics, as well as providing direct access to factual information (see Table 1).
Hereditary Risk Statistically, mutation carriers have a 50% chance of passing the mutation to the offspring. Many previvors have not yet completed their families and have to deal with the stressors of realizing they can pass the risk to subsequent children. In a study of 284 previvors, 44% experienced extreme worry about passing a mutation to subsequent generations (Staton, Kurian, Cobb, Mills, & Ford, 2008). Young previvors also need to consider whether they have PMs before or after childbearing is complete, which is challenging and associated with concessions; previvors must choose between breastfeeding and decreased worry about cancer risk (Hoskins et al., 2012). For single women, fears can occur about what a future partner may think about the decision to have PMs, and how to explain such decisions (Hamilton & Hurley, 2010). Many mothers choose surgery because they fear a premature death and leaving their children without a mother. The decision to have a prophylactic PSO means acknowledging that childbearing is complete. A related issue previvors must address is when and how to discuss hereditary risk with their children (Tercyak et al., 2011). Parents may or may not convey information about risk of having a mutation accurately (Patenaude et al., 2013). Many parents are faced with the decision to tell children when a mother undergoes prophylactic surgery (Farkas Patenaude et al., 2012). The importance of honesty and
February 2014 • Volume 18, Number 1 • Clinical Journal of Oncology Nursing
Cancer prevention and early detection for other cancers • Colonoscopy starting at age 50 years and every 7–10 years • Annual skin examination • Tobacco elimination • Regular use of sunscreen, protective clothing, and hats; minimize ultraviolet light exposure. • Maintain body weight. • Increase fresh fruit and vegetable intake; decrease fat intake. • Increase intentional exercise to 150 minutes per week. Management of bone health risks • Counseling regarding the importance of daily weight-bearing exercise • Bone density testing every one to two years starting at the time of the PSO • Vitamin D 1,000 IU per day and calcium 1,500 mg per day Management of peritoneal cancer risks • Yearly pelvic examination to assess for peritoneal malignancies • Discussion with provider regarding risks and benefits of CA 125 testing for peritoneal malignancies • Counseling regarding subtle symptoms of primary peritoneal cancer Menopause management • Hormone therapy considered in eligible premenopausal patients with a balanced discussion of the potential risks and benefits • Education about nonhormonal means to manage vulvarvaginal dystrophy • Education about hot flash management and possible evidence-based methods of management PSO—prophylactic salpingo-oophorectomy
FIGURE 1. Long-Term Follow-Up in Previvors With BRCA Mutations Following Prophylactic Surgery Note. Based on information from American Cancer Society, 2013; Chapman et al., 2011; Finch et al., 2012; Gadducci et al., 2010; Kaplan et al., 2011; Matloff et al., 2009; Sturdee & Panay, 2010.
words to use, and the importance of longterm open communication. Decision aids to assist with this procedure are available (Peshkin, DeMarco, & Tercyak, 2010). In general, testing of adolescents is not recommended. Testing usually is completed at a point in which it would influence decisions regarding screening or prevention. Most adolescents do not need prophylaxis. Although many pediatricians and primary care providers will order genetic testing on adolescents if requested by parents, testing probably is best managed by a genetics professional who is experienced with genetic risk assessment pretest counseling (O’Neill et al., 2010). Genetic testing often is recommended by age 25 years, or 10 years before the first cancer diagnosis in the family because that is when surveillance would begin. Although many do test negative, for those who test positive, it can be frightening, particularly for younger women who do not have peers experiencing similar choices.
Conclusions Mrs. T opted to have a total hysterectomy, including PSO, because she had two relatives who were diagnosed with ovarian cancer around age 45 years. She will require ongoing management of the side effects related to her prophylactic surgical choices (see Figure 1). Mrs. T was informed of the risks of premature menopause, including bone loss, vaginal dryness, and hot flashes (Matloff, Barnett, & Bober, 2009). Although she experienced relief and liberation in knowing she had taken all possible steps to prevent cancer, she did experience significant hot flashes and some vaginal dryness. The lifetime risk for developing peritoneal cancer is still at least 3%–4%, and longterm surveillance is needed (Chapman et al., 2011).
References emphasizing that a mother is taking all steps to prevent future problems should not be underestimated. Parents may need assistance finding a balance between protecting and educating their children. For this reason, consultation with a genetics professional prior to disclosure is recommended to help parents consider the child’s maturity, potential approaches,
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February 2014 • Volume 18, Number 1 • Clinical Journal of Oncology Nursing
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