Disease disclosure in individuals with cystic fibrosis: Association with psychosocial and health outcomes

INTRODUCTION

Research with children and adolescents with CF suggests that they may attempt to conceal their CF symptoms and diagnosis in order to appear “normal” to their peers (1). As patients with CF grow older, they may experience life changes such as shifts in peer groups, beginning college, or getting married (2). While parents of younger children and adolescents often take the lead in disease disclosure situations, older adolescents and adults may be disclosing independently for the first time to new friends or in novel workplaces. During each of these transitions, patients with CF must decide if, when, and how to disclose their disease status. Disclosure methods may be implicit, such as using oxygen in front of others, or explicit, where a patient directly discusses their diagnosis with someone. Further, some situations may call for direct disease disclosure, such as missing work due to illness. Because of the aforementioned life changes, such as peer relationships and workplace issues, disclosure is a particularly important construct to evaluate among older adolescents and adults.

Previous research with adults with CF indicates that people with higher rates of disclosure report greater illness severity (3,4) and better social support (4). Disclosure has been named as both a barrier and facilitator of adherence (5). Adults with CF report disclosing most frequently to close others, and they have described negative repercussions and fear of negative consequences when disclosing to romantic partners and in the workplace (4). Disclosure research in other chronic illnesses has supported findings in CF research, such that higher disclosure is associated with greater social support (6), fewer symptoms of depression (7), and better adherence (8). Disclosure (in the format of expressive writing) has been linked to better physical health in people with (9) and without (10) a chronic illness. No quantitative research has been conducted examining comfort in disclosure-related activities (e.g., discussing CF or performing CF treatments in front of others) following the initial act of disclosing. This may be an important area to explore, if disclosure is conceptualized as an ongoing process rather than a static act (3).

Most previous research on disclosure in CF has been qualitative or descriptive. For instance, research has described types of disclosure (3), perception of CF-related nutritional behaviors (11), and goals of web-based disclosure (12). This is the first study in CF to examine disease disclosure using a validated assessment tool. Additionally, this study makes a novel contribution to the literature by assessing comfort in disclosure-related activities. The primary goal of this paper was to quantify disease disclosure in individuals with CF and evaluate its association with psychosocial and health outcomes. Specifically, we examined the prevalence of disclosure to different social groups (e.g., romantic partners, close and casual friends, bosses, and coworkers) and correlates of disclosure (e.g., social support, emotional functioning, depressive symptoms, medication adherence self-efficacy, medication adherence, lung function, and body mass index [BMI]). Based on research in other chronic illnesses (6–10), we expected higher levels of disclosure to be associated with greater levels of social support, greater emotional and social functioning, fewer depressive symptoms, higher levels of self-efficacy to adhere to medications, greater medication adherence, and lower lung function and BMI.

METHODS

Procedures

Data for this study were collected at the baseline assessment of a randomized-controlled trial (RCT) comparing two adherence interventions. Participants completed baseline surveys online prior to a clinic visit. Inclusion criteria were: confirmed diagnosis of CF, age 16 years or older, scheduled upcoming CF clinic visit, clinical stability (e.g., not on intravenous medications for the previous 14 days), and prescription of inhaled mucolytic, inhaled antibiotic therapy, chronic macrolide therapy, and/or hypertonic saline therapy for the last year. Exclusion criteria were: Burkholderia cepacia complex within the past two years, history of a lung transplant, or participation in the pilot study for the adherence intervention study. Detailed information about study recruitment and procedures is provided elsewhere (13). This study was reviewed and approved by the study site’s Institutional Review Board.

Questionnaires

Demographics.

Participant demographic information was obtained from questionnaires completed by the participant at their baseline evaluation. Participants self-reported their age, gender, ethnicity, educational level, and insurance status.

Disclosure.

Disclosure was assessed using the Cystic Fibrosis Disclosure Questionnaire (CFDS). Information from qualitative interviews and existing disclosure literature was used to develop the CFDS (5,14). The CFDS asks participants to report whether they have disclosed to all, some, or no people in a relationship category (e.g., Romantic Partner, Close Friends, Casual Friends, Boss, and Coworkers). Participants who are not in a romantic relationship or unemployed are directed to skip categories that are not relevant. If they have disclosed, then they rate their comfort in discussing their CF with and their comfort in performing CF treatments in front of those people on a Likert scale from 1=Not at all comfortable to 10=Completely comfortable. Our sample demonstrated good internal consistency (α=.71−.83).

Social Support.

Social support was assessed using the Medical Outcomes Study Social Support Survey (MOS-SS), a 19-item measure of functional social support validated for use with independently living chronically ill adults (15). Responses are rated on a Likert scale of 1=None of the time to 4=All of the time, and summed for an Overall Support Index. Our sample had good internal consistency (α=.85).

Emotional and Social Functioning.

We used the 5-item Emotional Functioning and 6-item Social Functioning subscales of the 50-item Cystic Fibrosis Questionnaire-Revised (CFQ

R), a self-report scale of health-related quality of life validated for use with older adolescents and adults with CF (16). Responses are rated on either a Likert scale assessing frequency/difficulty of an item or a binary true/false response. Higher scores signify better emotional and social functioning. Our sample demonstrated excellent internal consistency for the CFQ-R (α=.91), but poor internal consistency for the Emotional (α=.13) and Social (α=.48) Functioning subscales. This may be due to the low number of questions for each subscale (17).

Depression.

The Center for Epidemiological Studies-Depression (CES-D) Scale is a 20-item self-report measure designed to assess depressive symptomatology in older adolescents and adults over the previous week (18). Responses are rated on a four-point Likert scale ranging from 0=Rarely or none of the time [less than 1 day] to 3=Most or all of the time [5–7 days], with total scores ranging from 0–60. Higher scores indicate more depressive symptoms. In our sample there was excellent internal consistency (α=.91).

Self-efficacy.

The Medication Self-Efficacy Questionnaire - General Subscale is a 14-item self-report measure validated for use with older adolescents and adults to assess medication adherence self-efficacy when faced with challenges such as being in a hurry or during the weekend (19). Each question is preceded by the prompt “How sure are you that you can do your CF treatments the way the doctor said when…” Responses are rated on a ten-point Likert scale ranging from 1=Not at all sure to 10=Completely sure, with total scores ranging from 14–140. Results indicated excellent reliability (α = 0.96).

Medication Adherence.

Pharmacy refill data were used to calculate a composite medication possession ratio (CMPR; 20). Participants provided the name and contact information for all pharmacies they used to obtain CF medications during the previous 12 months. Each pharmacy was faxed a letter requesting refill records, along with a copy of the patient’s signed consent form. For each prescribed pulmonary medication, a ratio was calculated using the following procedure: the sum of all days of medication supply dispensed during the previous year, divided by the number of days each medication was prescribed in the same interval. Because medications dispensed during hospitalizations are not captured in pharmacy records, the number of days hospitalized during the interval was subtracted from the denominator. Values were capped at 100% and ratios for each medication were averaged across all prescribed medications to determine a composite score (20).

Health Outcomes.

Lung function was measured by FEV₁% predicted values, and body mass index (BMI) was used as a nutritional health marker. These health outcomes were extracted from the CFF patient registry using the value closest to the date of the baseline survey that was not during a pulmonary exacerbation.

Statistical analyses

We provided descriptive data on the prevalence of disclosure in our sample and examined associations between disclosure, psychosocial variables, adherence, and health outcomes. Spearman’s rho correlations were calculated to determine the association between comfort in discussing CF and doing therapies in front of the social group and psychosocial, adherence, and health outcome variables. T-tests were used to determine group differences on health outcomes based on whether the person had or had not disclosed his/her CF diagnosis to members of the social group. Post-hoc Bonferroni corrections were conducted to specify differences in comfort in discussion with and comfort performing treatments in front of discrete groups of people. SPSS version 22 was used for all statistical analyses (IBM Statistical Product and Service Solutions 22.0; IBM Corp., Armonk, NY). P-values <0.05 were considered statistically significant.

RESULTS

Participant Characteristics

Participants were 128 individuals with CF with the majority being Caucasian (93%) and male (53%), with a mean age of 29 years (SD = 11.1). Full sample characteristics are displayed in Table 1. Outcome variable descriptives are displayed in Table 2. Nearly one-half of the sample had mild lung disease (48.4% with FEV₁≥70%), and met BMI recommendations (43% of males and 42% of females had BMI ≥ 23 or 22, respectively). Participants reported high levels of social support, good emotional and social functioning, and few symptoms of depression. Medication self-efficacy was reported to high and adherence was 44% on average.

Table 1.

Sample Characteristics

	n (%)
Male	68 (53%)
Age, mean year (SD)	29.2 (11.1)
Ethnicity
Caucasian	119 (93%)
African American	4 (3%)
Latino	1 (1%)
Other	4 (3%)
Marital status
Single	64 (50%)
Married	49 (38%)
With a partner	11 (9%)
Divorced	4 (3%)
Insurance status
Private	109 (85%)
Public	19 (15%)
Disease severity
Mild	62 (48%)
Moderate	51 (40%)
Severe	15 (12%)

Table 2.

Outcome Variable Descriptives

	M (SD)
MOS-SSa	60.4 (15.5)
CFQ-Rb Emotional Functioning subscale	73.5 (19.5)
CFQ-Rb Social Functioning subscale	70.2 (19.9)
CES-Dc	10.8 (9.3)
Medication Self-Efficacy Questionnaire-General subscale	6.9 (2.1)
CMPRd	44.4 (26.7)
FEV1 % predicted	63.7 (23.3)
Body mass index	22.7 (3.8)

Note.

^aMOS-SS = Medical Outcome Study Social Support Survey;

^bCFQ-R = Cystic Fibrosis Questionnaire-Revised;

^cCES-D = Center for Epidemiological Studies – Depression;

^dCMPR = Composite Medication Possession Ratio

Prevalence of Disclosure in Different Relationship Types

Of the 90 participants reporting a romantic partner, almost all (97%) reported disclosing their diagnosis to him/her. Participants reported high levels of comfort discussing their CF with their partner (M=9.2, SD=1.9) and doing their treatments in front of their partner (M=9.4, SD=1.7). The majority of participants (70%) reported disclosing their CF to all of their close friends, while 24% reported disclosing to some of their close friends. With regard to disclosure to casual friends, 17% reported disclosing to ‘all’ of their casual friends, and 62% reported disclosing to ‘some’ of their casual friends. Participants described moderately high levels of comfort in discussing their CF with close friends (M=8.3, SD=2.4) but less comfort doing their treatments in front of close friends (M=6.9, SD=3.1). Comfort levels were, on average, lower with discussing CF with casual friends (M=6.5, SD=2.9) and doing treatments in front of casual friends (M=4.9, SD=3.2).

Of the 68 participants working full or part-time, 71% disclosed their CF diagnosis to their boss. Employed participants reported mean comfort levels of 7.4 (SD=3.0) discussing their CF with their boss and a mean comfort level of 5.3 (SD=3.7) doing their treatments in front of their boss. Eighteen percent disclosed to all of their co-workers, and 35% disclosed to some of their coworkers. Comfort levels in discussing their CF (M=6.9, SD=3.0) and performing treatments (M=4.8, SD=3.6) in front of coworkers were similar to comfort levels in discussing and performing treatments with casual friends. See Figures 1 and 2.

Figure 1.

Rates of disclosure to others

Figure 2.

Participants’ average comfort in discussing CF and doing treatments in front of others. Mean scores for each group are shown above the bars.

Social Support

Social support was not related to whether or not a participant disclosed his or her diagnosis. Greater social support was associated with more comfort discussing CF with a romantic partner (Rho=.31, p=.003), close friends (Rho=.25, p=.006), casual friends (Rho=.20, p=.041), and bosses (Rho=.24, p=.045). Greater social support was also related to more comfort performing CF treatments in front of a romantic partner (Rho=.34, p=.001), close friends (Rho=.27, p=.002), and bosses (Rho=.26, p=.029).

Emotional and Social Functioning

Emotional and social functioning were not related to whether or not a participant disclosed his or her diagnosis. Higher social functioning was significantly related to greater comfort in discussing CF with close friends (Rho =.19, p=.031) and bosses (Rho =.28, p=.021). Higher social functioning was also associated with comfort performing treatments in front of close friends (Rho =.24, p=.008) and casual friends (Rho =.19, p=.044). Higher emotional functioning was significantly related to greater comfort discussing CF with bosses (Rho =.27, p=.024).

Depressive Symptoms

Depressive symptoms were not related to whether or not a participant disclosed his or her diagnosis or their comfort performing treatments in front of others. Fewer depressive symptoms was associated with greater comfort discussing CF with a romantic partner (Rho =−.23, p=.031).

Medication Adherence Self-Efficacy

Medication adherence self-efficacy was not related to whether or not a participant had disclosed his or her diagnosis to others or reported comfort discussing CF with others. Higher medication adherence self-efficacy was associated with greater comfort doing CF treatments in front of a romantic partner (Rho =.28, p=.008) and close friends (Rho =.24, p=.009).

Medication Adherence

Adherence was not related to a participant’s comfort discussing his or her CF or performing treatments in front of others. Participants who had disclosed to close friends (M=45.5, SD=26.9) demonstrated better objectively measured adherence (e.g., pharmacy refill data) than participants who had not disclosed to close friends (M=24.7, SD=15.8; t(125)=−3.021, p=.021).

Health Outcomes

Lung function was not associated with a participant’s comfort discussing his or her CF with others or performing treatments in front of others. Participants who reported disclosing their CF diagnosis to their boss (M=66.4, SD=22.5) had lower lung function than participants who had not disclosed to their boss (M=78.9, SD=23.8; t(91)=2.402, p=.018). Participants who had disclosed to coworkers (M=67.3, SD=22.7) demonstrated worse lung function than participants who had not disclosed to coworkers (M=78.7, SD=24.9; t(94)=2.174, p=.032). BMI was not related to any disclosure variables.

DISCUSSION

This study was the first to quantitatively explore disease disclosure in individuals with CF using a validated assessment, empirically examine the impact of disclosure on psychosocial and health outcomes, and describe comfort discussing CF or performing CF treatments in front of others in individuals with CF. Findings from this study indicate that individuals with CF have a high level of comfort discussing their CF diagnosis with and performing treatments in front of close friends and romantic partners. Aside from close friends and romantic partners, participants most frequently disclosed to bosses and coworkers. These results are consistent with findings of previous research, with participants disclosing more frequently to close others than to supervisors or coworkers (4). Social support, employment, and medication adherence self-efficacy were identified as the three main psychosocial correlates of comfort discussing CF or performing CF treatments in front of others. Additionally, medication adherence was associated with disclosure to close friends.

Social Support

There was a strong association between comfort discussing CF or performing CF treatments in front of others and social support and social functioning, particularly with close friends. Adolescence and young adulthood development is typically characterized by a loosening of emotional ties with families and growing number and importance of close friendships (22–24). Because half our sample was comprised of single young adults, friends likely serve as an important source of CF-specific and general social support for these participants. Thus it is no surprise that disclosure comfort in our sample was associated with social support and functioning. Indeed, previous CF research with adolescents suggests that social support from friends, specifically, impacts adherence and quality of life (25). Future research should explore whether disclosure is a function of seeking social support or if greater perceived social support aids in disclosure.

Employment

Of the employed participants in this sample, most had disclosed their diagnosis to their boss; however, as previous literature has found (3,4), participants with more severe lung disease were more likely to disclose. These participants may have felt “pushed’ to disclose to bosses due to the visibility of their symptoms or hospitalization resulting in missed work (4,26). Additionally, a minority of this sample reported disclosing their diagnosis to coworkers. CF care team social workers or psychologists may want to address disclosure strategies for the workplace with patients. This conversation is especially important to have with patients who must perform treatments at work, as participants reported low levels of comfort doing treatments in front of bosses and coworkers who were aware of their diagnosis. These findings, along with social support findings discussed above, may indicate that adults with CF are not seeking or obtaining social support in the workplace. Future research should examine relations between disclosure, social support, and employment.

Medication Adherence Self-Efficacy

Medication adherence self-efficacy emerged as a significant positive correlate of performing treatments in front of close friends and romantic partners. Adherence was not significantly linked to disclosure in the workplace or to casual friends, which indicates that disclosure comfort in close relationships is most important in adherence. Previous CF research has linked higher self-efficacy with better lung function and quality of life (27) and higher medication adherence (13), suggesting that improving comfort in performing treatments in front of close others may be a way to support self-efficacy and thereby treatment adherence and quality of life. Because the data are cross-sectional, the converse may also be true, that people with high treatment self-efficacy possess the confidence to overcome barriers associated with disclosure.

Clinical Implications

Revealing one’s illness is the first step in disclosure, but talking about the illness and doing treatment in front of others is less comfortable for people with CF, even amongst close friends. However, disclosure is linked to more positive psychosocial outcomes. Medication adherence self-efficacy, social support, and social functioning are important areas to consider in the context of disclosure. CF care team members should inquire about a patient’s social network, workplace environment, and disclosure behaviors, specifically related to whom and how comfortable they are with disclosing. It may be particularly informative to know not only who knows about the diagnosis, but also whether the person is comfortable performing treatments in front of them. Subsequently, clinicians can determine whether the patient could benefit from further discussing disclosure. Evidence-based techniques such as problem-solving or role-playing strategies can be used to help patients practice disclosing to others (28), and how to handle unsupportive reactions. Multiple interventions already exist to address adherence in CF, using cognitive, cognitive-behavioral, and problem-solving techniques (29). Providers can also refer patients to a psychologist for outpatient treatment.

Another area for physicians to target is a patient’s medication schedule to determine any potential impact of disclosure status. For example, if a patient needs to perform treatments at work but is attempting to keep their CF diagnosis private, physicians should discuss this situation with the patient and collaboratively generate solutions. Disclosure should be considered as a potential adherence barrier and could be integrated into existing efficacious interventions.

Limitations

This study was not without limitations. These data were cross-sectional, which limits our ability to extrapolate directionality of results. Additionally, as the MOS-SS is meant to be administered to independent, community dwelling adults, it may not have fully captured social support for younger participants in this study still living with their parents. Finally, as in many studies using self-report data, respondent bias should be considered a limitation. Participants may attempt to increase social desirability when answering questions or they may forget or inaccurately recall information about past events (30). These biases may be relevant for this sample as the self-report data demonstrated limited associations with objective measures.

Conclusion

Our results are consistent with and extend prior findings regarding disclosure patterns in individuals with CF, with participants reporting more frequent disclosure to close others. Comfort in disclosure to friends appears particularly important to social support, social functioning, and medication adherence self-efficacy. Future research should explore the effect of age on disclosure in different situations, given that peers change (number and quality of the relationships) over the lifespan. Future research should also explore causal pathways between disclosure and adherence with other factors as mediators or moderators, such as regimen complexity. Findings from our study are the first to quantify disclosure in CF patients, and demonstrate that disclosure is an important construct associated with medication adherence self-efficacy, social support, and social functioning in individuals with CF.

Highlights.

• This study is the first to examine disclosure in adults with CF using a validated tool.

• Participants were more likely to disclose to romantic partners and close friends than to casual friends, bosses, or co-workers.

• Participants reported more comfort discussing CF with and doing treatments in front of romantic partners and close friends than other groups.

• Disclosure was associated with higher social support, social functioning, and medication adherence self-efficacy.

• Lower lung-function was associated with disclosure to bosses and co-workers.