Correction to: Assessment of stigma in patients with cystic fibrosis.

An amendment to this paper has been published and can be accessed via the original article.

Development and psychometric validation of a cystic fibrosis knowledge scale.

BACKGROUND AND OBJECTIVE: Well-developed and validated measures of cystic fibrosis (CF) knowledge are scarce. The purpose of the present study is to develop and validate a CF knowledge scale that is brief, easy to use, self-administered and demonstrates clinical utility. METHODS: A comprehensive literature search generated a pool of scale items; an expert panel of CF team members reviewed and provided recommendations for item inclusion. A focus group of CF patients and family members (n = 12) then reviewed the items for face validity and reading clarity. To evaluate the validity and reliability of the newly developed CF knowledge scale, it was administered to several different samples including CF patients (n = 45), respirology patients (n = 100), health-care providers (n = 74) and university student samples (psychology students, n = 71; medical students, n = 36). RESULTS: Internal consistency of the scale was high, with an alpha coefficient for the overall sample of .95 (n = 326). The scale also demonstrated excellent construct validity. CONCLUSIONS: This study is an important first step in a line of research that aims to develop and empirically validate a psycho-educational adherence intervention for improving quality of life and treatment outcomes among adult CF patients. The CF knowledge scale has potential applications as a clinical teaching tool with patients and health-care providers and could be used as an outcome measure in CF educational intervention studies aimed at optimizing CF treatment knowledge, adherence and quality of life among CF patients.

Assessment of stigma in patients with cystic fibrosis.

BACKGROUND: Research that explores stigma in Cystic Fibrosis (CF) is limited. Productive cough, repeated lung infections, and periods of serious illness requiring hospitalizations are among common symptoms of CF. These symptoms may cause a negative perception by others. We developed a CF-specific Stigma Scale and tested its psychometric properties. METHODS: We conducted a focus group with 11 participants including adult patients with CF (n = 5) and their informal caregivers (n = 6). The thematic content of the focus group was analyzed to find key themes. We developed a CF-specific Stigma Scale and assessed its psychometric properties in a 3-month prospective cohort study of adult CF outpatients (n = 45). RESULTS: Stigma emerged as consistent concern for people living and caring for those with CF, affecting both patients' lives and health through the focus group. Using the newly developed CF Stigma scale, the mean baseline score was 16.6 (SD = 4.5, Range = 10-25). The CF Stigma Scale demonstrated robust psychometric properties: 1) Internal consistency: α = 0.79; 2) Mean inter-item correlation: 0.30 with good test-retest reliability; 3) Convergent validity: Positive associations with depression, severity of CF symptoms and anxiety; negative associations with validated quality of life scores were observed. CONCLUSIONS: Stigma is measurable and significantly impacts the lives of CF patients. Further research should investigate the role of stigma in patients living with CF.

Breathlessness catastrophizing relates to poorer quality of life in adults with cystic fibrosis.

BACKGROUND: Cystic Fibrosis (CF) is the most common genetic disease affecting children and young adults in Canada. It is a multi-system disease, however lung disease is largely responsible for mortality. Treatment advances have resulted in increased life expectancy and a subsequent need to better understand psychosocial issues associated with quality of life in adults living with CF. Emerging research suggests that anxiety and depression are related to poorer health-related quality of life (HRQoL) in CF patients, but there is little research examining cognitive processes, such as breathlessness catastrophizing. The present study addresses this gap in the literature. METHODS: Participants in this study are based on a convenience sample of patients recruited during their regular CF clinic appointments at a tertiary care center. Forty-five adults (Mage = 30.73 years) completed measures of lung function, depression, anxiety, pain, breathlessness catastrophizing, and HRQoL at one time point. RESULTS: Results of a hierarchical multiple regression indicate that increased breathlessness catastrophizing was related to poorer HRQoL, after controlling for lung function, depression, anxiety, and pain (p < .05). Depression, pain, and breathlessness catastrophizing all emerged as significant unique predictors of HRQoL. CONCLUSIONS: Breathlessness catastrophizing is a potential target for clinical intervention and might impact HRQoL. Further research on breathlessness catastrophizing in CF is warranted including longitudinal studies to examine the mechanisms by which breathlessness catastrophizing relates to HRQoL and treatment outcomes in CF.

A Cross-Sectional Study of the Psychological Needs of Adults Living with Cystic Fibrosis.

BACKGROUND: Depression and anxiety are prevalent in people with cystic fibrosis (CF), yet psychological services are rarely accessible in CF clinics. This cross-sectional single center study reports on a psychological needs assessment of people with CF. METHODS: We asked adults attending a CF clinic, without integrated psychological services, to complete a psychological needs assessment survey that included items on: a) past access to psychological services (via a CF referral service), b) concerns relevant to discuss with a psychologist, and c) their likelihood of accessing psychological services if available at the CF clinic, and standardized measures of depression (CES-D) and anxiety (GAD-7). RESULTS: We enrolled 49 participants and 45 (91.8%) completed the survey. Forty percent reported elevated symptoms of depression and 13% had elevated anxiety. A majority of individuals (72.2% and 83.3%, respectively) indicated they would be likely to use psychological services, if available at the clinic. Concerns considered most relevant to discuss with a psychologist were: 1) worries (51.1%), 2) mood (44.4%), 3) life stress (46.6%), 4) adjustment to CF (42.2%), 5) life transitions (42.2%) and 6) quality of life (42.2%). CONCLUSIONS: This study highlights the rationale for screening adults with CF for depression and anxiety, and to facilitate provision of psychological services and preventative mental health interventions as an integral component of multi-disciplinary CF care.

Acute effects of viral respiratory tract infections on sputum bacterial density during CF pulmonary exacerbations.

BACKGROUND: Airway proliferation of Pseudomonas aeruginosa bacteria is thought to trigger CF exacerbations and may be affected by the presence of viral infections. METHODS: A 2-year prospective study was conducted on 35 adults with CF. P. aeruginosa sputum density was analyzed during stable, exacerbation and post exacerbation assessments. Upon exacerbation, samples were sent for PCR detection of respiratory viruses and the sputum density of P. aeruginosa in patients with a viral infection versus those without was compared. RESULTS: Twenty-two patients experienced 30 exacerbations during the study period; 50% were associated with a viral infection. There was no change in sputum density of P. aeruginosa from the stable to exacerbation state when measured by quantitative culture or by PCR. Virus-associated exacerbations did not result in significant increases in P. aeruginosa sputum density compared to non-viral exacerbations. CONCLUSION: Sputum density of P. aeruginosa was not increased at the time of CF exacerbation and was not influenced by the presence of viral infection.

Implementation of a cystic fibrosis lung transplant referral patient decision aid in routine clinical practice: an observational study.

BACKGROUND: The decision to have lung transplantation as treatment for end-stage lung disease from cystic fibrosis (CF) has benefits and serious risks. Although patient decision aids are effective interventions for helping patients reach a quality decision, little is known about implementing them in clinical practice. Our study evaluated a sustainable approach for implementing a patient decision aid for adults with CF considering referral for lung transplantation. METHODS: A prospective pragmatic observational study was guided by the Knowledge-to-Action Framework. Healthcare professionals in all 23 Canadian CF clinics were eligible. We surveyed participants regarding perceived barriers and facilitators to patient decision aid use. Interventions tailored to address modifiable identified barriers included training, access to decision aids, and conference calls. The primary outcome was >80% use of the decision aid in year 2. RESULTS: Of 23 adult CF clinics, 18 participated (78.2%) and 13 had healthcare professionals attend training. Baseline barriers were healthcare professionals' inadequate knowledge for supporting patients making decisions (55%), clarifying patients' values for outcomes of options (58%), and helping patients handle conflicting views of others (71%). Other barriers were lack of time (52%) and needing to change how transplantation is discussed (42%). Baseline facilitators were healthcare professionals feeling comfortable discussing bad transplantation outcomes (74%), agreeing the decision aid would be easy to experiment with (71%) and use in the CF clinic (87%), and agreeing that using the decision aid would not require reorganization of the CF clinic (90%). After implementing the decision aid with interventions tailored to the barriers, decision aid use increased from 29% at baseline to 85% during year 1 and 92% in year 2 (p < 0.001). Compared to baseline, more healthcare professionals at the end of the study were confident in supporting decision-making (p = 0.03) but continued to feel inadequate ability with supporting patients to handle conflicting views (p = 0.01). CONCLUSION: Most Canadian CF clinics agreed to participate in the study. Interventions were used to target identified modifiable barriers to using the patient decision aid in routine CF clinical practice. CF clinics reported using it with almost all patients in the second year.