Sedentary time in people with obstructive airway diseases.

Sedentary time (ST) and light-intensity physical activity (LIPA) are movement behaviours associated with important health outcomes, but are not widely explored in respiratory diseases. We aimed to describe their volume and/or accumulation patterns in moderate-severe COPD, bronchiectasis and severe asthma using the accurate postural-based accelerometer activPAL, contrasting these values with a non-respiratory population. We also sought to test the cross-sectional associations of these behaviours with disease characteristics by diagnostic group, and as a combined label-free disease group. RESULTS: Adults with COPD (n = 64), bronchiectasis (n = 61), severe asthma (n = 27), and controls (n = 61) underwent cross-sectional measurements of volume and/or accumulation patterns of ST and LIPA. The prevalence and characteristics, and associations with exercise capacity, health-status, airflow-limitation, dyspnoea, systemic inflammation and exacerbations were analysed. ST volumes in COPD were higher than that of bronchiectasis and severe asthma. Values in bronchiectasis and severe asthma were similar to each other and controls (≈8.9 h/day). Their accumulation patterns were also significantly better than in COPD, but similar if not worse compared to controls. LIPA volumes in bronchiectasis and severe asthma were also higher than those of COPD (p < 0.05) and controls. In bronchiectasis and COPD, lower levels/better patterns of ST accumulation, as well as higher LIPA volume were associated with better clinical characteristics. These associations may be mediated by airflow limitation. CONCLUSIONS: The discordance between engagement in ST volume versus ST patterns highlights the importance of accounting for both these different yet complementary metrics. ST and LIPA are low-intensity activities associated with important clinical characteristics in people with chronic respiratory diseases. TRIAL REGISTRATION: Not applicable.

Clinical impact of depression and anxiety in patients with non-cystic fibrosis bronchiectasis.

INTRODUCTION: Bronchiectasis is a chronic suppurative disease characterized by abnormal bronchial dilatation. The nature of bronchiectasis may have negative impact on psychological status, however it is poorly studied in relation to clinical indices, particularly the severity of disease. Primary aim of this study is to detect depression and anxiety in patients with non-cystic fibrosis bronchiectasis and to evaluate its relationship with disease severity indexes. MATERIALS AND METHODS: Ninety (male/female= 37/53; median age 45 years) stable non-cystic fibrosis bronchiectatic adult patients were enrolled into this study. Dyspnea scores, number of exacerbations and hospital admissions within the last year, body-mass index, pulmonary function tests, sputum cultures, bronchiectasis disease severity indexes (BSI and FACED) were assessed. Anxiety and depression were evaluated by using the Turkish version of the hospital anxiety and depression scale questionaire. RESULT: Anxiety was diagnosed in 30% of patients and depression was diagnosed in 41% of the participants. Female participants had significantly higher rates of depression (55% vs. 22%; p= 0.002). Exacerbation rates within the last year were higher among the subjects with anxiety, moreover, patients with depression had shorter duration of disease. BSI and FACED severity indexes increased with longer duration of disease (5.6 ± 5.0 yrs in mild group vs. 10.1 ± 9.2 yrs in moderate-to-severe group, p= 0.035 and 5.7 ± 5.4 yrs in mild group vs. 12.1 ± 9.7 yrs in moderate-to-severe group, p= 0.001, respectively), however, anxiety and depression were not related with BSI and FACED severity indexes. CONCLUSIONS: Patients with non-cystic fibrosis bronchiectasis have an increased risk for depression and anxiety. Duration of disease and higher exacerbation rate are related with psychological status and indexes increased with longer duration of disease. Early detection and taking the necessary measures to improve the psychological state is necessary for the overall management of these patients.

Quality of Life Questionnaire-Bronchiectasis: a study of the psychometric properties of the Brazilian Portuguese version.

OBJECTIVE: To evaluate the psychometric properties of the Brazilian Portuguese version of the Quality of Life Questionnaire-Bronchiectasis. DESIGN: Cross-sectional study. SETTING: Outpatient clinic. SUBJECTS: Clinically stable individuals with a diagnosis of bronchiectasis. MEASURES: The evaluations performed were spirometry, incremental shuttle walk test, Saint George's Respiratory Questionnaire, and the modified Medical Research Council dyspnea scale. The Quality of Life Questionnaire-Bronchiectasis was administered twice (seven to 14 days apart). Psychometric analyses were performed as follows: reliability, construct validity, criterion validity, and interpretability. RESULTS: In total, 108 individuals (48 ± 14 years, 61 women) participated in the study. Internal consistency was considered adequate (Cronbach's alpha ⩾ 0.70) for the majority of scales (from 0.58 to 0.93). Test-retest coefficients were moderate to excellent (intraclass correlation coefficients from 0.70 to 0.93). In the construct validity, 35 of 37 items correlated more strongly with their assigned scale than a competing scale. The convergent validity showed significant correlations between scales of the Quality of Life Questionnaire-Bronchiectasis with modified Medical Research Council dyspnea scale, and incremental shuttle walk test (r from 0.20 to 0.59). A low to moderate correlations was revealed between all scales of the Quality of Life Questionnaire-Bronchiectasis and the Saint George's Respiratory Questionnaire domains (r from 0.26 to 0.70). The standard error of measurement was acceptable. Ceiling effects were found for the Social Functioning and Treatment Burden scales. CONCLUSIONS: The Quality of Life Questionnaire-Bronchiectasis is a reliable, valid instrument with adequate internal consistency for the evaluation of the impact of bronchiectasis on the health-related quality of life of Brazilian adults.

Anxiety and depression in bronchiectasis: Response to pulmonary rehabilitation and minimal clinically important difference of the Hospital Anxiety and Depression Scale.

The aims of the study were to evaluate the responsiveness of Hospital Anxiety and Depression Scale-Anxiety (HADS-A) subscale and HADS-Depression (HADS-D) subscale to pulmonary rehabilitation (PR) in patients with bronchiectasis compared to a matched group of patients with chronic obstructive pulmonary disease (COPD) and provide estimates of the minimal clinically important difference (MCID) of HADS-A and HADS-D in bronchiectasis. Patients with bronchiectasis and at least mild anxiety or depression (HADS-A ≥ 8 or/and HADS-D ≥ 8), as well as a propensity score-matched control group of patients with COPD, underwent an 8-week outpatient PR programme (two supervised sessions per week). Within- and between-group changes were calculated in response to PR. Anchor- and distribution-based methods were used to estimate the MCID. HADS-A and HADS-D improved in response to PR in both patients with bronchiectasis and those with COPD (median (25th, 75th centile)/mean (95% confidence interval) change: HADS-A change: bronchiectasis -2 (-5, 0), COPD -2 (-4, 0); p = 0.43 and HADS-D change: bronchiectasis -2 (-2 to -1), COPD -2 (-3 to -2); p = 0.16). Using 26 estimates, the MCID for HADS-A and HADS-D was -2 points. HADS-A and HADS-D are responsive to PR in patients with bronchiectasis and symptoms of mood disorder, with an MCID estimate of -2 points.

Bronchiectasis Information and Education: a randomised, controlled feasibility trial.

BACKGROUND: There has been comparatively little patient information about bronchiectasis, a chronic lung disease with rising prevalence. Patients want more information, which could improve their understanding and self-management. A novel information resource meeting identified needs has been co-developed in prior work. We sought to establish the feasibility of conducting a multi-centre randomised controlled trial to determine effect of the information resource on understanding, self-management and health outcomes. METHODS/DESIGN: We conducted an unblinded, single-centre, randomised controlled feasibility trial with two parallel groups (1:1 ratio), comparing a novel patient information resource with usual care in adults with bronchiectasis. Integrated qualitative methods allowed further evaluation of the intervention and trial process. The setting was two teaching hospitals in North East England. Participants randomised to the intervention group received the information resource (website and booklet) and instructions on its use. Feasibility outcome measures included willingness to enter the trial, in addition to recruitment and retention rates. Secondary outcome measures (resource use and satisfaction, quality of life, unscheduled healthcare presentations, exacerbation frequency, bronchiectasis knowledge and lung function) were recorded at baseline, 2 weeks and 12 weeks. RESULTS: Sixty-two participants were randomised (control group = 30; intervention group = 32). Thirty-eight (61%) were female, and the participants' median age was 65 years (range 15-81). Median forced expiratory volume in 1 s percent predicted was 68% (range 10-120). Sixty-two of 124 (50%; 95% CI, 41-59%) of potentially eligible participants approached were recruited. Sixty (97%) of 62 participants completed the study (control group, 29 of 30 [97%]; 95% CI, 83-99%; 1 unrelated death; intervention group, 31 [97%] of 32; 95% CI, 84-99%; 1 withdrawal). In the intervention group, 27 (84%) of 32 reported using the information provided, and 25 (93%) of 27 of users found it useful, particularly the video content. Qualitative data analysis revealed acceptability of the trial and intervention. Web analytics recorded over 20,000 page views during the 16-month study period. CONCLUSION: The successful recruitment process, high retention rate and study form completion rates indicate that it appears feasible to conduct a full trial based on this study design. Worldwide demand for online access to the information resource was high. TRIAL REGISTRATION: ISRCTN Registry, ISRCTN84229105. Registered on 25 July 2014.

Translation and linguistic validation of the Bronchiectasis Health Questionnaire (BHQ) into Danish.

INTRODUCTION: Patients with bronchiectasis suffer from breathlessness, cough and sputum production, which impairs quality of life. The Bronchiectasis Health Questionnaire (BHQ), a short and disease-specific questionnaire, has previously been developed and validated in English. The aim of this study was to translate and validate the BHQ in Danish, using established cross-cultural validation methods. METHODS: To create a Danish version of the questionnaire, this study used linguistic validation, a pilot study in 17 patients and forward-backward translation. Thereafter, 111 patients with bronchiectasis were asked to complete the BHQ (BHQ1) as well as the St George Respiratory Questionnaire (SGRQ), and one in three patients were asked to repeat the BHQ after two weeks had passed (BHQ2). RESULTS: There was a significant convergent validity between the BHQ1 and the SGRQ (ρ = -0,826, p = 0.0001), a satisfactory correlation coefficient between the BHQ1 and the BHQ2 (0.739) and a lower limit of agreement of -15.96 and 20.56 in the Bland Altmann plot. CONCLUSIONS: The BHQ is translated and validated in Danish and retains good validity properties. This questionnaire is ready for use in daily clinical practice among Danish-speaking patients. FUNDING: none. TRIAL REGISTRATION: not relevant.

COPD Assessment Test in Bronchiectasis: Minimum Clinically Important Difference and Psychometric Validation: A Prospective Study.

BACKGROUND: Health-related quality of life (QoL) is one of the most important end points in bronchiectasis (BE). However, the majority of health-related QoL questionnaires are time-consuming or not validated in BE. The COPD Assessment Test (CAT) is an easy-to-use questionnaire. The objective of this study was to perform a complete validation of the CAT in BE. METHODS: This was an observational, multicenter, prospective study in patients with BE. Psychometric properties of the CAT were measured: internal consistency (Cronbach α), repeatability (test-retest; intraclass correlation coefficient), discriminant validity (correlation with severity scores), convergent validity (correlation with some validated QoL questionnaire and other clinical variables of interest), longitudinal validity (measuring before and after each exacerbation during follow-up to determine the sensitivity to change and responsiveness), predictive validity to future exacerbations, and finally minimum clinically important difference. RESULTS: Ninety-six patients were included and followed up for 1 year. Their mean age was 62.2 (15.6) years (79.2% women). The CAT showed excellent internal consistency (α, 0.95) and repeatability (intraclass correlation coefficient, 0.95). The validity of the CAT was excellent in all the measures (almost all with a Pearson coefficient > 0.40) except for the correlations with severity scores (Pearson coefficient between 0.22 and 0.26). Sensitivity to change before and after exacerbations was set at between 5.4 and 5.8 points. A CAT value ≥ 10 points showed prognostic value for patients with more than one exacerbation, and finally the minimum clinically important difference was set at 3 points. CONCLUSIONS: The CAT presented excellent psychometric properties and is a questionnaire that is easy to use and interpret in patients with BE.

Validation of the COPD Assessment Test (CAT) as an Outcome Measure in Bronchiectasis.

BACKGROUND: Objective assessment of symptoms in bronchiectasis is important for research and in clinical practice. The COPD Assessment Test (CAT) is a short, simple assessment tool widely used in COPD. The items included in the CAT are not specific to COPD and also reflect the dominant symptoms of bronchiectasis. We therefore performed a study to validate the CAT as an outcome measure in bronchiectasis. METHODS: The CAT was administered to two cohorts of bronchiectasis patients along with other quality of life questionnaires. Patients underwent comprehensive clinical assessment. One cohort had repeated questionnaires collected before-and-after treatment of acute exacerbations. We analyzed convergent validity, repeatability, and responsiveness of the score and calculated the minimum clinically important difference (MCID) using a combination of distribution and anchor-based methods. RESULTS: In both cohorts there were positive correlations between the CAT and the St. George's Respiratory Questionnaire (r = 0.90, P < .0001 and r = 0.87, P < .0001). There was an inverse relationship between CAT and Quality of Life - Bronchiectasis Respiratory Symptoms Scale (r = -0.75, P < .0001) and Leicester Cough Questionnaire score (r = -0.77, P < .0001). Patients with more severe disease, based on the bronchiectasis severity index, had significantly higher CAT scores. CAT also correlated with FEV1 % predicted and 6-min walk distance (6MWD). CAT increased significantly at exacerbation and fell at recovery. The intraclass correlation coefficient for two measurements four-weeks apart while clinically stable was 0.88 (95% CI, 0.73-0.95, P < .0001). An MCID of 4 was most consistent. CONCLUSIONS: CAT is a valid, responsive symptom assessment tool in bronchiectasis. The MCID is estimated as 4 points.

Coping and psychopathology in children with malignancy and bronchiectasis.

AIM: We aimed to evaluate the coping styles and social support perceived by the children with two different chronic diseases (cancer and bronchiectasis), their mothers' coping styles and compare them with a control group without any chronic physical or psychiatric disorder. METHODS: Our sample consisted of 114 children and adolescents, with an age range from 9 to 15 years. The data were collected by using schedule for affective disorders and schizophrenia for school-age children-present and lifetime version, kid-coping orientation to problems experienced (Kid-COPE), social support appraisals scale (SSAS), and COPE. RESULTS: All three groups were similar with respect to age and sex distribution. Around 50% to 60% of the children in both patient groups had a psychiatric diagnosis. Remarkably, 30% of the children had an internalizing disorder. The most commonly used coping style by the mothers was religious coping in all groups. Kid-COPE scores did not significantly differ between groups. The scores on Family and Friend subscales of SSAS in the bronchiectasis group were significantly lower when compared with those of participants in hematology-oncology and control groups. CONCLUSION: Chronic medical illnesses may have a similar psychological impact on children regardless of disease-specific clinical presentations and outcomes. Future studies need to focus on identifying protective and risk factors that potentially mediate psychosocial well-being.

Non-CF Bronchiectasis as a Possible Indicator of a Primary Immunodeficiency: Diagnosis, Clinical Course, and Quality of Life in a Pediatric Cohort.

BACKGROUND: Non cystic fibrosis bronchiectasis (NCBE) is an increasingly recognized chronic, progressive respiratory disorder with significant morbidity also in children and adolescents. METHODS: We longitudinally assessed a cohort of 35 pediatric patients with NCBE and investigated underlying diagnosis, symptoms, clinical course, treatment, and quality of life. RESULTS: NCBE were diagnosed at a mean age of 9.5 (±5.3) years. In half of the children NCBE were found prior to identification of the causative diagnosis. Primary immunodeficiency (PID) was identified as the underlying diagnosis in 24/35 (68%) cases, of which two-thirds showed antibody deficiency. In the 11 non-PID cases ciliopathies were most common (n=7). Clinical aspects such as manifestation age, cough or dyspnea symptoms, and exacerbation frequency did not differ significantly between PID and non-PID patients. Likewise, quality of life (QoL) was equally reduced in both groups. Lung function test parameters were stable under appropriate therapy in all children. The majority in both groups was insufficiently vaccinated against influenza and pneumococci. CONCLUSION: Our data indicates that NCBE need to be especially appreciated as a presenting sign of PID in pediatric patients. Thus, occurrence of NCBE should warrant rigorous diagnostics to identify the underlying condition. In our cohort NCBE themselves rather than the causative diagnoses seem to dictate the clinical course of disease and reduce QoL in children. More intensive efforts have to be undertaken to vaccinate patients according to recommendations.

Aetiology and disease severity are among the determinants of quality of life in bronchiectasis.

INTRODUCTION: Quality of life (QoL) is known to be impaired in patients with bronchiectasis, which is generally attributed to exacerbations and chronic pulmonary symptoms. The aim of this study was to determine if aetiology and disease severity are associated with QoL in bronchiectasis. METHODS: We conducted a retrospective analysis of clinical stable patients with bronchiectasis. Diagnostic workup into the aetiology of bronchiectasis was conducted according to the current guidelines. QoL was measured by QoL-B questionnaire (QoL-B), data on sputum microbiology, pulmonary function tests and the disease severity were obtained. RESULTS: The aetiology of bronchiectasis was investigated in the total of 200 patients. The most commonly identified aetiologies were post-infective (39.5%) and idiopathic (12.5%). Patients with chronic obstructive pulmonary disease (COPD)-related bronchiectasis showed a significant lower QoL (P < .05) as compared to the other aetiologies. In the total population, an increasing disease severity as measured by FACED, E-FACED and the bronchiectasis severity index was correlated with a lower QoL. CONCLUSIONS: Our results showed that QoL in bronchiectasis is related both to aetiology, with worse QoL in COPD-related bronchiectasis, and to disease severity, which suggests more attention in advance for these specific patient groups with bronchiectasis.

Extrapulmonary associations of health status in severe asthma and bronchiectasis: Comorbidities and functional outcomes.

BACKGROUND: Severe asthma and bronchiectasis are heterogeneous diseases that contribute to disability beyond the pulmonary system. The magnitude of the impact that these extrapulmonary features has on health-related quality of life (HRQoL) is unknown. METHODS: We analysed the cross-sectional relationships between HRQoL (St. George's Respiratory Questionnaire; SGRQ) and extrapulmonary characteristics, including physical activity (steps/day), anxiety and depression, isometric leg strength, systemic inflammation, and several comorbidities in adults with severe asthma (n = 70) and bronchiectasis (n = 61). RESULTS: Participants with severe asthma and bronchiectasis had similar SGRQ total scores (mean scores 43.7 and 37.8 for severe asthma and bronchiectasis; p > 0.05), and similar pulmonary and extrapulmonary characteristics. The associations between extrapulmonary variables and HRQoL did not differ according to diagnosis (all interactions p > 0.05). Greater anxiety and depressive symptoms, fewer steps/day and greater systemic inflammation were statistically associated with poorer HRQoL in both diseases (p < 0.05). Lower isometric leg strength in severe asthma, and greater Charlson Comorbidity Index in bronchiectasis were also associated with poorer HRQoL (p < 0.05). In the multivariable regression model performed in the combined disease groups, anxiety and depression, steps/day, systemic inflammation and isometric leg strength remained independently associated with HRQoL. Associations between extrapulmonary characteristics and SGRQ domains were stronger for the activity and impact domains, than symptoms. CONCLUSION: In severe asthma and bronchiectasis, extrapulmonary features including physical activity and leg strength have a significant impact on HRQoL, especially within the activity and impact domains. These features should be considered as part of the assessment of these conditions, and they may represent additional treatment targets to improve HRQoL.

Azithromycin or erythromycin? Macrolides for non-cystic fibrosis bronchiectasis in adults: A systematic review and adjusted indirect treatment comparison.

Non-cystic fibrosis (non-CF) bronchiectasis is a condition characterized by an airway inflammatory response to bacterial pathogens. Frequent exacerbations have a major influence on the quality of life. Macrolide antibiotics have not only antibacterial but also immune-regulation effects. It is proved that macrolides have a benefit in preventing exacerbations. However, it is still uncertain whether azithromycin or erythromycin is more effective and safe. The purpose of this study was to answer the following question: Which kind of macrolide antibiotic is more effective and safe in preventing non-CF bronchiectasis exacerbation? We conducted a systematic review to identify randomized clinical trials published up to May 2017 that reported on macrolides for non-CF bronchiectasis and an adjusted indirect treatment comparison (AITC) between macrolides to evaluate their efficacy and safety. The direct comparison meta-analysis found that macrolides decreased the rate of exacerbation of non-CF bronchiectasis (risk ratio (RR) = 0.45; 95% confidence interval (CI) 0.36-0.55) with heterogeneity ( I2 = 63.7%, p = 0.064). The AITC showed that azithromycin had a significantly lower bronchiectasis exacerbation rate than erythromycin (RR = 0.35; 95% CI: 0.403-0.947). Azithromycin increased the risk of diarrhea and abnormal pain. This meta-analysis suggested that long-term treatment with macrolides significantly reduced the incidence of non-CF bronchiectasis exacerbation. Moreover, azithromycin is more efficient than roxithromycin and erythromycin in preventing exacerbation.

Paediatric and adult bronchiectasis: Monitoring, cross-infection, role of multidisciplinary teams and self-management plans.

Bronchiectasis is a chronic lung disease associated with structurally abnormal bronchi, clinically manifested by a persistent wet/productive cough, airway infections and recurrent exacerbations. Early identification and treatment of acute exacerbations is an integral part of monitoring and annual review, in both adults and children, to minimize further damage due to infection and inflammation. Common modalities used to monitor disease progression include clinical signs and symptoms, frequency of exacerbations and/or number of hospital admissions, lung function (forced expiratory volume in 1 s (FEV1 )% predicted), imaging (radiological severity of disease) and sputum microbiology (chronic infection with Pseudomonas aeruginosa). There is good evidence that these monitoring tools can be used to accurately assess severity of disease and predict prognosis in terms of mortality and future hospitalization. Other tools that are currently used in research settings such as health-related quality of life (QoL) questionnaires, magnetic resonance imaging and lung clearance index can be burdensome and require additional expertise or resource, which limits their use in clinical practice. Studies have demonstrated that cross-infection, especially with P. aeruginosa between patients with bronchiectasis is possible but infrequent. This should not limit participation of patients in group activities such as pulmonary rehabilitation, and simple infection control measures should be carried out to limit the risk of cross-transmission. A multidisciplinary approach to care which includes respiratory physicians, chest physiotherapists, nurse specialists and other allied health professionals are vital in providing holistic care. Patient education and personalized self-management plans are also important despite limited evidence it improves QoL or frequency of exacerbations.

Health Perception and Behaviors in Adults With Bronchiectasis.

BACKGROUND: Bronchiectasis is a chronic debilitating suppurative disease that significantly impacts quality of life. Clinical outcomes like exacerbations, are usually physician centered; however, the patients' experience, health-related behaviors, and expectations have frequently been neglected. In addition, patients' health perceptions may be influenced by their culture. OBJECTIVE: To determine the health perception and behavior in adults with bronchiectasis. METHODS: We performed semi-directive interviews, which were audiotaped, with 60 adults with bronchiectasis between April 2016 and December 2016. Our interview focused on issues related to symptom perception, access to health-care resources and patient-physician communication, medication adherence, outcomes and expectations, quality of life, and social relationships. RESULTS: The subjects with bronchiectasis developed varying patterns of symptom perception (ranging from highly distressing to barely disturbing) and had conflicting opinions on whether and when they should seek health-care services (ranging from active consultations to being totally passive or resistant to seek health care). We observed certain discrepancies between symptom perception and health-related behaviors. Overall, medication adherence was suboptimal, but the subjects were willing to participate in clinical trials and receive complementary alternative medications despite concerns regarding adverse effects of prolonged treatment. There were concerns about the adverse effects of bronchiectasis on fertility and infectiousness to others, although most subjects disregarded these issues. CONCLUSIONS: The diverse symptom perception and health-related behaviors highlighted the need for evaluation and intervention in bronchiectasis. These findings will provide rationales for refining future health care through comprehensive (particularly psychological) interventions worldwide.

Psychiatric morbidity and quality of life in children and adolescents with cystic fibrosis.

Senses-Dinç G, Özçelik U, Çak T, Dogru-Ersöz D, Çöp E, Yalçin E, Çengel-Kültür E, Pekcan S, Kiper N, Ünal F. Psychiatric morbidity and quality of life in children and adolescents with cystic fibrosis. Turk J Pediatr 2018; 60: 32-40. The aim of this study was to investigate psychiatric disorders, depression and anxiety levels, and quality of life in children and adolescents with cystic fibrosis (CF), and to compare them with those of children with non-cystic fibrosis (non-CF) bronchiectasis and healthy controls. A total of 103 children and adolescents aged 7-16 years (35 CF, 28 non-CF bronchiectasis, 40 healthy) were evaluated using The Schedule for Affective Disorders and Schizophrenia for School Aged Children (K-SADS), The Child Depression Inventory (CDI), The State-Trait Anxiety Inventories for Children (STAI-C) and the Pediatric Quality of Life Inventory (PedsQL)-C. The three groups were not statistically different with respect to age, sex, and familial sociodemographic variables. 80% of the children and adolescents in the CF group were diagnosed with a psychiatric disorder, which was significantly more compared to those of the two other groups. The CF group had significantly greater rates of depressive and oppositional defiant disorder and the non-bronchiectasis group had a significantly greater rate of anxiety disorder than the control group. The depression and anxiety symptom levels were significantly greater and the quality of life levels significantly lower in both the CF and non-CF bronchiectasis groups than the healthy controls. In the CF group, the presence of any associated psychiatric disorder led to significantly lower total and psychosocial quality of life scores. In conclusion, CF is associated with poorer QOL in childhood. In order to improve quality of life in CF, the psychiatric conditions of children and adolescents should also be evaluated and their follow-up and treatment should involve a multidisciplinary team approach.

US Patient-Centered Research Priorities and Roadmap for Bronchiectasis.

Noncystic fibrosis bronchiectasis (bronchiectasis) is an increasingly common chronic lung disease that is difficult to manage because of a lack of evidence on which to base treatment decision-making. We sought to develop a practical list of US-based patient-centered research priorities and an associated roadmap to guide bronchiectasis research. We designed and administered a web-based patient needs assessment survey to establish broad research priorities, convened three stakeholder webinars to confirm the top priorities, obtained written stakeholder feedback, and completed a final consensus survey of objectives. The stakeholder panel consisted of clinical research experts in bronchiectasis, a seven-member patient advisory panel, and representatives from the two key patient advocacy organizations: COPD Foundation and NTM Info and Research Inc. Based on survey results from 459 patients with bronchiectasis, the stakeholder panel identified 27 patient-centered research priorities for bronchiectasis in the areas of bronchiectasis treatment and prevention of exacerbations, improving treatment of exacerbations and infections, improving health-related quality of life, predictors of poor prognosis, understanding the impact of underlying conditions, and conducting patient-centered clinical trials. These priorities should further inform the development and evaluation of both new and previously unproven therapies, with particular attention to the inclusion of patient-reported outcomes. We anticipate a great deal of progress will be made in the field of bronchiectasis in the next decade.

Pulmonary Rehabilitation Only Versus With Nutritional Supplementation in Patients With Bronchiectasis: A RANDOMIZED CONTROLLED TRIAL.

PURPOSE: Pulmonary rehabilitation (PR) is recommended for bronchiectasis, but there is little evidence of its efficacy in these patients. The aim of this study was to assess the effect of PR in normally nourished patients with noncystic fibrosis bronchiectasis compared with the effect of PR plus an oral nutritional supplement (PRONS). METHODS: A single-center randomized controlled trial, parallel treatment design in which participants were randomly assigned to receive PR for 12 wk or PR plus a high-protein nutritional supplement enriched with beta-hydroxy-beta-methylbutyrate. Outcome assessments were performed at baseline, 12 and 24 wk including cardiopulmonary exercise testing, health-related quality of life (HRQOL), bronchorrhea, dyspnea, psychological symptoms, spirometry, and exacerbations. RESULTS: Thirty patients were randomized into 2 groups of 15 participants. In both groups, cardiopulmonary exercise testing, HRQOL, dyspnea, and spirometry parameters significantly increased from baseline at 3 and/or 6 mo. CONCLUSION: PR improved exercise capacity, HRQOL, and respiratory parameters. The use of PRONS did not have a significant effect on the results.

"The missing ingredient": the patient perspective of health related quality of life in bronchiectasis: a qualitative study.

BACKGROUND: Bronchiectasis is a heterogeneous disease which affects quality of life. Measuring symptoms and quality of life has proved challenging and research is limited by extrapolation of questionnaires and treatments from other diseases. The objective of this study was to identify the major contributors to quality of life in bronchiectasis and to evaluate existing health related quality of life questionnaires in bronchiectasis. METHODS: Eight adults with bronchiectasis participated in one to one semi-structured interviews. These were recorded and transcribed verbatim. Thematic analysis was used to identify core themes relevant to disease burden and impact. Participant views on current health related quality of life questionnaires were also surveyed. RESULTS: Bronchiectasis symptoms are highly individual. Core themes identified were symptom burden, symptom variation, personal measurement, quality of life and control of symptoms. Themes contributing to quality of life were: social embarrassment, sleep disturbance, anxiety and modification of daily and future activities. Evaluation of 4 existing questionnaires established their individual strengths and weaknesses. A synthesis of the participants' perspective identified desirable characteristics to guide future tool development. CONCLUSIONS: This qualitative study has identified core themes associated with symptoms and quality of life in bronchiectasis. Current treatments and quality of life tools do not fully address or capture the burden of disease in bronchiectasis from the patients' perspective.