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Published in final edited form as: Psychooncology. 2008 Aug;17(8):783–789. doi: 10.1002/pon.1352

Psychological Functioning in Persons Considering Genetic Counseling and Testing for Li-Fraumeni Syndrome

Susan K Peterson 1, Rebecca D Pentz 2, Salma K Marani 3, Patricia A Ward 4, Amie Blanco 5, Denise LaRue 6, Kristen Vogel 7, Tamara Solomon 8, Louise C Strong 9
PMCID: PMC9535398  NIHMSID: NIHMS1734567  PMID: 18688785

Abstract

Li-Fraumeni syndrome (LFS) confers an increased risk of multiple types of cancer in both children and adults. Clinical genetic testing for deleterious germline p53 gene mutations can identify most LFS-affected families. We evaluated factors associated with cancer-specific distress and perceived self-efficacy in coping with a positive genetic test result among persons at risk of having deleterious p53 mutations. One hundred thirty five persons from 15 LFS-affected families were invited to take part in a study that offered p53 genetic counseling and testing and to complete psychosocial measures. Participants (n=92) were more likely to be younger and female than nonparticipants (n=43). In multivariate analyses, greater cancer-specific distress was associated with having a lower quality of life, a higher perceived risk of having a p53 mutation, no personal history of cancer and a greater number of FDRs affected with cancer. Lower perceived self-efficacy in coping with a positive test result was associated with greater cancer worry, higher decisional conflict about p53 testing, and having no personal history of cancer. Individual perceptions about cancer risk and p53 genetic testing, as well as personal experience with FDRs’ cancer diagnoses and deaths, should be addressed during the counseling and testing process for LFS-affected families.

Keywords: p53, Li-Fraumeni, cancer, oncology, psychosocial

INTRODUCTION

Li-Fraumeni syndrome (LFS) is a rare, autosomal dominant condition associated with deleterious germline mutations in the p53 gene [1]. LFS-affected individuals have up to a 90% lifetime risk of developing multiple primary cancers of various types. The most commonly occurring malignancy in LFS is pre-menopausal breast cancer, and other cancers associated with this syndrome include soft-tissue sarcomas, osteosarcomas, acute leukemia, adrenocortical tumors, brain tumors and a variety of childhood and adult onset tumors [1]. Clinical genetic testing for germline p53 mutations can identify more than 50% of LFS-affected families [2–4]. The cancer risks faced by children and young adults with LFS, particularly young women, are considerable. Nearly 20% of individuals with a germline p53 mutation develop cancer before age 20 [5], and survivors of these childhood cancers may be at increased risk for developing additional tumors [6]. Among female p53 mutation carriers, the risk of developing cancer by the age of 45 (primarily breast cancer) is estimated at 84%, with nearly a 100% lifetime risk [5, 7]. Male carriers have an estimated 41% risk of developing cancer by 45 years of age and a 70% to 90% lifetime risk. [5, 7]. There is no consensus regarding risk management guidelines for most LFS-associated malignancies, with the possible exception of early breast cancer screening for women [8–10]. Thus, p53 mutation carriers may receive little reassurance in terms of available cancer prevention or risk management options.

The importance of attending to individuals’ psychological functioning before and during genetic counseling for inherited cancer risk is widely recognized [11–13]. Psychological factors have been related to deciding to undergo genetic testing for hereditary breast, ovarian and colorectal cancers [14]. Findings from studies that have evaluated psychological outcomes after genetic testing for inherited cancer syndromes suggest that pre-test psychological status, including coping status and distress, may influence post-test functioning [12, 15, 16]. However, prospective studies of psychological functioning in individuals who are considering genetic counseling and testing for LFS are lacking.

People in LFS-affected families may face unique psychosocial challenges given the predisposition for developing several types of cancer from childhood through adulthood. Members of LFS-affected families may repeatedly experience cancer diagnoses, and possibly deaths, in parents, siblings, children, or other relatives, resulting in considerable psychological impact. Studies of close relatives of patients with breast cancer have described the effects of the patient’s cancer diagnosis and death on the relatives’ distress and psychological adjustment [17–19]. Similarly, experiencing a parent’s cancer during childhood was identified as a risk factor for psychological distress among persons who underwent genetic counseling for hereditary breast, ovarian or colorectal cancer [20]. Diagnosis of a pediatric cancer is an acutely distressing occurrence for parents and siblings of the patient, and can present a crisis for many families [21]. Having a family history of multiple cancers may pose a significant psychological burden for LFS-affected families, as it exposes members to the potential for repeated experiences of grief and threats to their personal well-being [22].

Distress experienced by LFS families and their perceived ability to cope with their increased cancer risk may affect their decisions regarding p53 genetic testing and follow-up care. Patenaude et al. [23] reported that 39% of people in LFS-affected families initially agreed to undergo germline p53 genetic testing, a rate significantly lower than that for BRCA1 testing by other families in the same study. Reasons for refusing testing in that study included worry about upsetting themselves or their relatives with positive test results, limited awareness of familial cancer risk and potential benefits of testing, and difficulty traveling to the testing center [23]. Genetic testing for LFS may pose a decisional dilemma for at-risk individuals, as the desire to learn about their p53 mutation status may conflict with distress about the possibility of being a carrier, and about the potential consequences of this knowledge for themselves and close family members [22]. The testing decision may be complicated further by the lack of effective options for the prevention or early detection of many LFS-related cancers.

To gain a better understanding of the psychological status of individuals considering p53 genetic counseling and testing, we evaluated factors associated with cancer-specific distress and perceived self-efficacy in coping with a positive gene test result among persons who were at risk of having a deleterious p53 gene mutation. The aims of the present study were: 1) to compare the demographic and medical characteristics, and family cancer experiences, of participants and nonparticipants in a study offering genetic counseling and testing for LFS; and, 2) to determine the psychological and other factors related to cancer-specific distress and perceived self-efficacy in coping with a positive p53 gene test result in persons considering genetic counseling and testing for LFS.

MATERIALS AND METHODS

Study Population

Our final sample included 92 persons from 15 kindreds who had previously participated in research regarding the genetics of LFS at The University of Texas M. D. Anderson Cancer Center (MDACC) [5, 24, 25][Strong et al., unpublished data][26]. Deleterious p53 germline mutations had been identified in each kindred through the previous research. However, no persons in our sample had undergone genetic counseling or clinical p53 genetic testing before the present study. Individuals were eligible for the study if they were at least 18 years of age, spoke English, and were at a 25% or 50% risk of having a p53 gene mutation. Persons with a personal history of any cancer, except non-melanoma skin cancer were classified as “affected”, whereas all others were classified as “unaffected”.

Study Procedures

This paper describes the first phase of a longitudinal study that offered genetic counseling and clinical p53 germline mutation testing to families who had previously taken part in research on the genetics of LFS [12]. The study was approved by the M.D. Anderson Institutional Review Board. Eligible persons received a letter inviting them to take part in a study that offered genetic counseling about their personal and familial cancer risk, with the option of free p53 genetic testing. Research staff subsequently followed up with prospective participants by telephone to determine their interest in the study. After obtaining informed consent, we asked participants to complete baseline psychosocial questionnaires (described below). In a later phase of the study, those who underwent counseling and testing were asked to complete similar questionnaires after disclosure of their test results. All participants had the option of receiving genetic counseling and testing without completing the psychosocial questionnaires.

One hundred thirty-five eligible persons were invited to participate, and 92 (68%) agreed to complete the baseline psychosocial questionnaire. The remaining 43 who declined, did not return follow-up telephone calls, or could not be reached were classified as nonparticipants.

Measures

All study questionnaires were administered by telephone. A description of the measures in the baseline questionnaire, as well as other variables included in this study, is provided below.

Dependent variables

Cancer-specific distress.

This variable was measured using the 15-item revised Impact of Event Scale (IES), which determines the extent to which a person is experiencing signs or symptoms of intrusive thoughts, or periods of avoidance, blocking, or denial of distress [27]. In this study, we used the IES to assess distress specific to having a family history of cancer. Overall scores range from 0 to 75, with higher scores indicating greater cancer-specific distress.

Perceived self-efficacy in coping with test results.

We developed an item to measure participants’ perceived self-efficacy in coping with the detection of an LFS-associated p53 gene mutation. Participants were asked to indicate, on a scale from 0% to 100%, how confident they were that they could cope with the possibility of being a p53 gene mutation carrier, with higher scores indicating greater self-efficacy.

Independent variables

Quality of life.

Participants completed the Quality of Life Index (QLI) [28, 29], a multidimensional rating scale that encompasses four life domains, measured by family, health/functioning, psychological/spiritual, and socioeconomic subscales. Scores range from 0 to 30, and are calculated for the overall QLI as well as for each subscale.

Social support.

We administered the 12-item short form of the Social Support Questionnaire (SSQ) [30]. The SSQ is a 12-item measure that assesses two dimensions of social support: the number of persons available for support, and satisfaction with that source of support (scores range from 6 to 36). Higher scores indicate both greater availability of and satisfaction with social support.

Perceived risk.

For unaffected persons, perceived risk of developing cancer was assessed by a single item: “In your opinion, compared with other persons your age, would you say your chances of getting cancer are: 1=much lower, 2=a little lower, 3= about the same, 4=a little higher, 5=much higher.” Affected participants were asked to rate their perceived risk of developing another cancer. All participants were asked to rate their perceived risk of having a deleterious p53 gene mutation on a continuous scale ranging from 0% to 100%. These items were adapted from a core set of measures recommended by the Cancer Genetics Studies Consortium [31].

Cancer worry.

We used the three-item Cancer Worry Scale to assess the current frequency of worry about developing cancer, and the effect of that worry on mood and the ability to perform daily activities [32]. Scores range from 1 to 4, with higher scores indicating a higher degree of cancer worry.

Decisional conflict.

The Decisional Conflict Scale [33] assessed uncertainty or perceived difficulty in making a decision about genetic testing. This validated scale uses a 5-point Likert response format to measure uncertainty in choosing from among various decision alternatives, and factors that contribute to uncertainty such as feeling informed, feeling clear in one’s values, and perceived support in decision-making. The perceived quality of decision-making also is measured. Responses are added and averaged to obtain scores ranging from 1 (low decisional conflict) to 5 (high decisional conflict).

Family experience with LFS-related cancer diagnosis and death.

To assess subjects’ family experiences with LFS-related cancer diagnosis and death, we used data obtained from the family pedigrees to quantify the numbers of LFS-related cancer diagnoses and deaths in their FDRs. For participants, we queried the pedigrees based upon the date the baseline questionnaire was completed. For nonparticipants, we queried pedigrees based upon the date that individuals were invited to participate in the study. We stratified the data gathered from the pedigrees based on participants’ and nonparticipants’ ages and included the following variables in our analyses: 1) number of FDRs with an LFS-related cancer diagnosis; 2) number of LFS cancer-related deaths in FDRs when participants or nonparticipants were younger than 21 years of age; and, 3) number of LFS cancer-related deaths in FDRs when participants or nonparticipants were 21 years of age or older.

Sociodemographic and medical characteristics.

We assessed age, gender, race/ethnicity, education, marital status, and number of children. Data regarding personal medical histories were obtained from family pedigrees.

Statistical Analysis

We compared participants and nonparticipants with regard to their demographic characteristics and the variables describing their family experience with LFS-related cancer diagnoses and deaths. For participants, we conducted univariate and multivariate regression analyses to examine variables associated with our main outcomes, cancer-specific distress and perceived self-efficacy in coping with a positive test result. Predictor variables with p < 0.05 on univariate analysis were selected for inclusion in the multivariate analysis. Cancer-affected versus unaffected status was included as a control variable in multivariate modeling. To account for any possible correlation in the dependent variables among individuals from the same kindred, we used a random regression model (PROC MIXED) to model the relationships in SAS statistical software. We also evaluated kindred effects for our outcome variables using intraclass correlation coefficients, and found low to modest correlations in our multivariate analysis of cancer-specific distress and perceived self-efficacy outcomes (0.01 and 0.28, respectively). Statistical significance was set at p < 0.05, and regression coefficient estimates, F-statistics and p-values were used to summarize the results of the analysis.

RESULTS

Characteristics of participants and non-participants.

Table 1 summarizes the differences in the demographic and family cancer history characteristics of participants and nonparticipants. The two groups did not differ on any demographic characteristics except age and sex, as participants were more likely to be female and were younger than nonparticipants. Taken together, the 135 subjects had an average of two FDRs who had been diagnosed with an LFS-associated cancer. Seventy-four percent (n=68) of all participants had experienced at least one FDR’s death from an LFS-associated cancer, with 26% (n=24) first experiencing such an event before age 21. More participants (74%) than nonparticipants (50%) had experienced an FDR’s death from an LFS-associated cancer at any age (p=0.01).

Table 1.

Demographic and Other Characteristics of Study Participants and Non-participants, N=135

Characteristic Participants (N = 92) Non-participants (N = 43)
Mean age in years (SD, range)a 49.7 (15.7, 18–81) 57.3 (17.2, 21–91)
Mean number of FDRsb diagnosed with LFS-associated cancer (SD, range) 2.1 (1.6, 0–7) 1.9 (1.9, 0–8)
% (N) % (N)
Gendera
  Female 61 (56) 37 (16)
Race/ethnicity
  White 79 (73) 81 (35)
Personal history of cancer (affected) 22 (20) 30 (13)
FDR died from LFS-related cancer when subject ≥ 21 years 55 (51) 41 (17)
FDR died from LFS-related cancer when subject < 21 years 26 (24) 21 (9)
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a

p<0.05

b

FDR: first degree relative

Factors associated with cancer-specific distress

The mean score for the IES was 11.3 (SD=14.6) for unaffected participants and 7.2 (SD=8.9) for those who were affected; however, the difference in mean scores was not statistically significant. On univariate analysis, higher levels of cancer-specific distress were associated with higher levels of cancer worry, lower social support, lower quality of life, greater perceived risk of developing cancer and having a p53 gene mutation, and a greater number of FDRs diagnosed with cancer. In our final multivariate model, variables that were associated with greater cancer-specific distress included unaffected cancer status, higher levels of cancer worry, lower quality of life, greater perceived risk of having a p53 gene mutation, and a greater number of FDRs diagnosed with cancer. The model accounted for 54% of the variance in cancer-specific distress.

Factors associated with perceived self-efficacy in coping with a positive p53 genetic test result

The mean score on our self-efficacy measure for unaffected participants was 79.6%, and the mean score for affected participants was 89.7%; however, this difference was not statistically significant. The following variables were significantly associated with lower levels of perceived self-efficacy in coping with a positive p53 test result in univariate analysis: higher levels of cancer worry, greater cancer-specific distress, and, higher levels of decisional conflict. In multivariate analysis, variables that were associated with lower perceived self-efficacy in the final regression model included unaffected cancer status, higher levels of cancer worry, and higher decisional conflict (Table 3). The model accounted for 37% of the variance in perceived self-efficacy.

Table 3.

Results of Random Regression Analysis Evaluating Correlates of Perceived Self-Efficacy in Coping with a Positive Test Result, N = 92

Variable Estimate F-value p
Unaffected cancer status 11.4 4.5 0.0365
Cancer worry −17.9 16.1 0.0001
Decisional conflict −10.3 12.0 0.0009
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DISCUSSION

Our findings offer insight regarding the psychological status of persons who may consider genetic counseling and testing for LFS, and characteristics of those who may or may not pursue an opportunity to receive p53 counseling and testing services. Most people invited to participate in this study agreed to do so, with higher participation rates observed among women and younger persons. Younger persons may perceive genetic counseling as an opportunity to learn about cancer risk, and the option of testing as relevant to decisions regarding marriage or childbearing. Younger persons also may be more likely to have children under age 18 and may thus have a desire to learn more about their children’s risk, or to learn about possible cancer prevention and risk reduction options so that they can avoid an untimely death from an LFS-associated cancer. Women with LFS have an increased risk for breast cancer, particularly at a young age, and may be more likely to explore the options of genetic counseling and testing because of the desire to identify potential risk reduction strategies, such as screening or risk-reducing surgery.

It has been suggested that the experience of being part of an LFS family may dampen interest in seeking genetic counseling and services due to the potentially threatening nature of learning one’s mutation status and the possible consequences for oneself and one’s family [22]. In contrast, we found that strength of family cancer history, as indicated by the number of cancer-affected FDRs, did not differ between participants and nonparticipants. Participants experienced more LFS-related cancer deaths among their FDRs, which suggests that they may have desired information about the underlying reason for their relatives’ mortality. It also is possible that participants’ experiences with their relatives’ diagnoses and deaths may have resulted in increased perceptions of their own cancer risks, which have been positively correlated with participating in genetic counseling and testing for other hereditary cancer syndromes [34, 35].

It is reassuring that for the families included in this study, experiencing relatives’ cancer diagnoses and deaths may not necessarily deter most individuals from seeking information about their inherited cancer risk. Nonetheless, it is important to note that a substantial proportion of all individuals approached for this study had experienced cancer diagnoses and deaths in parents, siblings or children - even at young ages. Family-level variables, such as the individual and collective experiences of cancer diagnosis and death, are important to attend to, as they may influence important outcomes such as adjustment to cancer risk notification and family communication about hereditary cancer risk [36].

Exploring the psychological impact of such experiences should be an important component of genetic counseling for LFS families, as our findings indicate possible effects on distress and perceived self-efficacy in coping with genetic test results. Although we observed modest cancer-specific distress levels overall, psychological and personal factors associated with higher levels of distress in our population were similar to those identified in other studies [37, 38]. Lower quality of life has been associated with distress in previous research [11, 16], and warrants further investigation, as these results may reflect the broader psychosocial impact of one’s experience with LFS. Our results indicated that individuals who experience distress and worry regarding their cancer risk may perceive themselves to be less confident in their ability to cope with a positive p53 gene test result. In addition, the association of higher levels of decisional conflict with lower self-efficacy may reflect a struggle with difficult decisional considerations that may be rather unique among p53 mutation carriers, given their relatively limited options for effective prevention and early detection interventions. These findings have implications for future research as well as for offering psychological support for individuals from LFS families, as they may be relevant to decisions about receiving test results and surveillance or risk reduction options.

There are some limitations to the present study that should be noted. The cross-sectional design of the study does not permit us to determine whether specific causal relationships exist between our predictor and outcome variables. Also, we were limited to using demographic and family history data to evaluate differences between participants and nonparticipants. Knowledge of psychological factors that characterize nonparticipants may provide additional insight regarding the role of distress or other psychosocial variables in influencing individuals’ decisions to participate in this study. Finally, our sample was largely white, and was recruited from a selected population of families, many of whom had previously participated in research; thus, our findings may not be generalizable to all LFS-affected families.

Future research should explore the longer-term psychological impact of LFS-associated cancer risk, particularly as individuals progress through p53 genetic counseling and testing. Efforts also should be directed toward disseminating findings of psychosocial research on LFS-affected families to oncologists and primary health care providers, and mental health professionals, as there is likely to be limited knowledge about the clinical and psychological aspects of this rare cancer syndrome. Such efforts will help improve the awareness and understanding of the psychosocial needs of LFS-affected families, and may facilitate strategies to promote optimal psychological adjustment and coping with the substantial cancer risks associated with this syndrome.

Table 2.

Results of a Random Regression Analysis Evaluating Correlates of Cancer-Specific Distress, N = 92

Variable Estimate F-value p
Unaffected cancer status −9.9 11.9 0.009
Cancer worry 10.1 22.4 <0.0001
Number of FDRs diagnosed with LFS cancer 2.8 9.3 0.0035
Quality of life (QOL) −0.7 4.6 0.0349
Perceived risk of having a p53 mutation 0.1 5.7 0.0191
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ACKNOWLEDGEMENTS

This research was supported by the American Cancer Society MRSGT-04-204-01-CPHHPS (Peterson), the National Cancer Institute, National Institutes of Health CA34936 (Strong), the Kleberg Human Cancer Genetics Development Award (Pentz and Strong), and the National Cancer Institute through The University of Texas M. D. Anderson Cancer Center Support Grant (5 P30 CA016672). We thank Christina Diaz, Sapna Kapoor, MiWon Park, Jenny Patel, Gloria Robertson, Doris Sembera, and Beatty Watts for their assistance with data collection. Finally, we thank the participants for their valuable contributions to our study.

Contributor Information

Susan K. Peterson, Department of Behavioral Science, The University of Texas MD Anderson Cancer Center, Houston, TX

Rebecca D. Pentz, Department of Hematology and Oncology, Emory University School of Medicine, Atlanta, GA

Salma K. Marani, Youth and Family Cancer Prevention Program, The University of Texas MD Anderson Cancer Center, Houston, TX

Patricia A. Ward, Baylor Genetics, Baylor College of Medicine, Houston, TX

Amie Blanco, UCSF Cancer Genetics and Prevention Program, San Francisco, CA.

Denise LaRue, Population Health Transformation, Harris Health System, Houston, TX.

Kristen Vogel, Center for Medical Genetics, NorthShore University Health System, Evanston, IL.

Tamara Solomon, Baylor College of Medicine, Houston, TX.

Louise C. Strong, Department of Genetics, The University of Texas MD Anderson Cancer Center, Houston, TX

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