J. Eder

Lung clearance index: Normal values, repeatability, and reproducibility in healthy children and adolescents†

There is increasing interest in using the Multiple Breath Washout technique and the lung clearance index (LCI) for detecting early pulmonary changes, for example, in cystic fibrosis lung disease. However, there are still limited data regarding equipment specific reference ranges, repeatability and reproducibility. The aim of this prospective study was to assess within‐test repeatability, short term reproducibility and long term reproducibility, and to establish normal values for the LCI in healthy children and adolescents using the sidestream ultrasonic flow sensor (EasyOne Pro, MBW Module, ndd Medical Technologies, Switzerland). Fourty‐four volunteers (5.3–20.3 years) were recruited for the 1st test. Twenty‐two out of 44 were measured on a 2nd test occasion after an interval of 1 hr (2nd test). Thirty‐four out of 44 agreed to come back for a follow up test 6–15 months later (3rd test). Mean LCI (SD) was 6.2 (0.4), 6.3 (0.4), and 6.0 (0.4) at the 1st, 2nd, and 3rd test. The upper limit of normal was 7.0 for all subjects. Within‐test repeatability was 5.1%. Short‐term reproducibility (1st test vs. 2nd test) was 4.2% with a mean difference of −0.13 (95% CI −0.350; 0.087). Long‐term reproducibility (1st test vs. 3rd test) was 5.1%, with a mean difference of 0.017 (95% CI −0.016; 0.348). With this low variability of the LCI for both, within and between tests, our study demonstrates reliability and robustness of equipment, protocol and analysis and the reliability of the MBW technique in general. The present data will help to interpret the effect of therapeutic interventions and interpretation of longitudinal data in patients with pulmonary diseases. Pediatr Pulmonol. 2009; 44:1180–1185.

Feasibility and Variability of Measuring the Lung Clearance Index in a Multi-Center Setting

The Lung Clearance Index (LCI) is superior to spirometry in detecting early lung disease in cystic fibrosis (CF) and correlates with structural lung changes seen on CT scans. The LCI has the potential to become a novel outcome parameter for clinical and research purposes. However longitudinal studies are required to further prove its prognostic value. Multi‐center design is likely to facilitate realization of such studies.

Tracking Lung Clearance Index and chest CT in mild cystic fibrosis lung disease over a period of three years

INTRODUCTION Lung disease remains the main cause of morbidity and mortality in patients with Cystic Fibrosis (CF). To detect lung disease before clinical symptoms become apparent, sensitive tools are essential. Spirometry is used for monitoring, but the FEV1 remains frequently normal throughout childhood. The Lung Clearance Index (LCI) calculated from Multiple Breath Washout (MBW) was introduced at the CF centre Innsbruck in 2007 for assessing ventilation inhomogeneity in patients with mild lung disease. We hypothesized that LCIs in 2007 are of prognostic value for the presence or absence of structural lung changes in later years. METHODS Between 2007 and 2010 MBW, spirometry and ultra-low-dose HR-CT were prospectively tracked in 36 patients (6-53 years) with a mean FEV1 ≥ 80% predicted in 2007. RESULTS At study start the majority of patients had abnormal CT scores and LCI results. While CT and spirometry remained largely stable throughout the study, LCI results slightly improved but still correlated with CT scores in 2010. LCI results in 2007 correlated with CT scores in 2010 while FEV1 did not. In 86% the LCI value in 2007 was indicative for the presence or absence of structural lung changes in 2010. CONCLUSION The LCI is a sensitive tool for detecting and tracking pulmonary changes. Extended structural changes are unlikely if the LCI is normal. The LCI has the potential to be used for monitoring the progression of early CF lung disease and assessing the effect of treatment in both clinical care and research settings.

Long-term improvement of lung clearance index in patients with mild cystic fibrosis lung disease: Does hypertonic saline play a role?

To assess whether long-term inhalation with hypertonic saline is able to halt the progression of mild CF lung disease, we analysed longitudinal data of lung clearance index (LCI) and spirometry. A total of 34 patients with mild lung disease (FEV1 ≥ 70% of predicted) had at least one LCI result before and ≥2 LCI measurements after start of hypertonic saline (HS) therapy. After a mean follow-up of 39.7 (SD 7.4) months after starting HS, LCI improved significantly from 7.89 (SD 1.35) at baseline to 6.96 (SD 1.03), and 19/34 patients had a normal LCI value at the last measurement. No decrease in mean FEV1 was observed. Thus, ventilation inhomogeneity can improve in patients with mild lung disease.

Psychological resilience and intolerance of uncertainty in coping with cystic fibrosis.

BACKGROUND Anxiety and depression are lower than to be expected in a considerable portion of cystic fibrosis (CF) patients. This outcome might be a result of substantial resilience and/or tolerance of uncertainty in coping with adversity. Research into resilience in cystic fibrosis is in its infancy. METHODS 57 adult CF patients participated in the study during their routine medical checkup. In addition to regular psychological assessment, the Intolerance of Uncertainty Scale (IUS) and the Resilience Scale (RS) were administered. The relative importance of IUS and RS in predicting quality of life in CF was explored. Bivariate correlations and predictive value of variables in multiple regressions on subscales of the Cystic Fibrosis Questionnaire-Revised (CFQ-R) were calculated. RESULTS Remarkably, resilience (personal competence and acceptance) was clearly elevated, whereas intolerance of uncertainty was comparable to healthy reference groups. In multiple regressions, personal competence emerged as strongest resilience variable in the prediction of quality of life. CONCLUSIONS CF patients in our study seem to be particularly resilient rather than cognitively avoidant. At this stage of research, fostering personal competence in CF patients is most promising in improving quality of life.

Low sodium status in cystic fibrosis—as assessed by calculating fractional Na+ excretion—is associated with decreased growth parameters

BACKGROUND In CF infants, normonatremic Na(+) depletion (NNaD), identified by fractional Na(+) excretion (FENa) values <0.5%, was recently linked to impaired growth. Our paper investigates the relationship between FENa and growth in CF children >2years. METHODS FENa values were calculated in 35 CF and 24 control children, and tested for correlations with z-scores for weight, height and BMI. RESULTS All CF children and controls had normal plasma Na(+) concentrations. A total of 25 of 35 (71.4%) CF patients had decreased FENa values <0.5% (group I). FENa results of 10 CF patients (group II) and 23/24 controls (group III) were normal. In Na(+)-depleted CF children, compared to normal controls, mean z-scores for weight (-0.18±0.87 vs +1.03±0.57, p<0.001), height (-0.06±0.89 vs +0.53±0.72, p=0.009) and BMI (-0.22±0.87 vs +1.00±1.06, p<0.001) were significantly reduced. Also, we found positive correlations between FENa values and z-scores for weight (r=0.521), height (r=0.292) and BMI (r=0.468), respectively. CONCLUSION NNaD may contribute to poor growth in CF.

WS09.4 The role of resilience in quality of life of adult patients with cystic fibrosis (CF)

Objectives Although several psychological studies on coping and quality of life suggested high levels of resilience in CF patients, empirical data on resilience in CF are barely existent. Thus, the purpose of the study was to investigate resilience in adult CF patients and its impact on quality of life. Methods 57 adult CF patients (18–59 years) participated in the study at their routine medical check-up. In addition to their regular psychological assessment including the Cystic Fibrosis Questionnaire – Revised (CFQ-R), the Resilience Scale (RS) was administered. The constructs were also correlated with physical parameters (age, gender and lung function). Results Patients reported high levels of resilience and overall good quality of life in all domains. Patients resilience was significantly higher compared to the large healthy reference group (t[2086] = 4.7510, p=0.0001) and was independent of gender (t[55] = 0.826, p=0.412), age (r = 0.076, p=0.573) and lung function (FEV1%, r=0.165, p=0.220). Significant associations between resilience and CFQ-R domains could be confirmed. Conclusion Our study is one of the first attempts to explicitely test resilience in CF patients and could demonstrate high levels of resilience in these patient group and positive correlations with quality of life. These results suggest that resilience could be seen as an important factor on quality of life and coping with CF. Therefore fostering resilience early in patients life should be encouraged.

The impact of psychosocial and socioeconomic factors on health status in children with cystic fibrosis

Aim: The study objective was the investigation of psychosocial variables affecting the disease trajectory in patient diagnosed with cystic fibrosis (CF). In addition, we analysed the impact of socioeconomic status on patients’ health status. Method: Between 2000 and 2009 all patients treated at the CF Centre at Innsbruck Medical University and aged below 18 (n = 103) were included in the study and assessed longitudinally. As measures of physical health status we recorded lung function values and body mass index (BMI). Psychosocial status was assessed using the Cystic Fibroses Problemchecklists (CFPC) and Hospital Anxiety and Depression Scale (HADS). Lung function and BMI were recorded quarterly. Psychosocial and sociodemographic variables were collected annually at routine examinations. Results: Our results suggest a strong association between mother’s marital status and lung function (mean FEV1: single parent 82.1% vs. parent with spouse 96.2%). In addition, we found an impact of mother’s educational status on lung function (mean FEV1: compulsory school 79.6% vs. university degree 98.6%). We did not find any significant association with BMI and psychosocial variables. Conlusion: A strong impact of education and marital status on the child’s health status was found, although in Austria treatment costs are fully covered by general health insurance and all patients receive the same treatment. We conclude that it is essential to turn attention to the parent’s marital and educational status. To be able to prevent a negative impact, further research is necessary to investigate how these factors affect lung function.