Rationale: Most children with cystic fibrosis (CF) experience a slow decline in spirometry, although some children continue to be at risk for more significant lung disease progression. Chest computed tomography (CT) scans have been shown to be more sensitive to changes in lung disease than spirometry and may provide a means for predicting future lung disease progression.

Objectives: We hypothesized that Brody chest CT scan scores obtained in 2000 in a prospectively monitored cohort of children with CF would be associated with the most recent measures of lung disease severity.

Methods: Brody chest CT scan scores were calculated for 81 children enrolled in the Wisconsin CF Neonatal Screening Project. Multivariable linear regression was used to determine associations between Brody scores and the most recent (age 21 yr or June 30, 2010, whichever was later) measures of CF lung disease.

Measurements and Main Results: The mean observation time after the chest CT scan was 7.5 years. Brody chest CT scan scores were significantly associated with the most recent measures of spirometry (P < 0.001) and Wisconsin and Brasfield chest radiograph scores (P < 0.001). The strength of this association was much stronger than spirometry obtained near the time of the chest CT scan (P < 0.01) but not chest radiograph scores.

Conclusions: Chest CT scan scores are associated with future lung disease severity, and quantitative chest imaging (chest CT scan and chest radiograph scores) is more strongly associated with future lung disease severity than measures of spirometry. These findings may help clinicians identify patients at risk of future lung disease progression.

Background

With the rapid implementation of cystic fibrosis (CF) newborn screening (NBS), quality improvement (QI) has become essential to identify and prevent errors. Using Process Failure Modes and Effects Analysis (PFMEA), we adapted this method to determine if it could be applied to discover and rank high priority QI opportunities.

Methods

Site visits to three programmes were conducted, and PFMEA exercises were accomplished in Colorado, Massachusetts and Wisconsin with 23 experienced professionals. During each of these comprehensive sessions, participants identified and ranked potential failures based on severity, occurrence and detection to calculate risk priority number (RPN) values.

Results

A total of 96 failure modes were generated and ranked in a list of the 20 riskiest problems that show no significant discordances by site, although there were differences by profession of the rater, particularly nurses.

Conclusions

Our results illustrate that the PFMEA method applies well to CF NBS and that steps requiring communication and information transfer are perceived to be the highest risks. The number of identified failure makes and their potential impact demonstrate considerable overall risk and a need for ongoing QI.

Background

Chronic pulmonary infection with Pseudomonas aeruginosa (PA) is responsible for significant morbidity and mortality in cystic fibrosis (CF). Because of the limited studies evaluating early exposure and the progression of genetic variability of PA, our goal was to assess PA in young children with CF followed in two clinic types.

Methods

A total of 39 infants with CF diagnosed through newborn screening were randomly assigned to either a segregated (PA-free) or mixed (PA-positive) clinic at two different CF centers, one of which replaced an older, mixed clinic where nosocomial acquisition was suspected. Oropharyngeal (OP) swab cultures were examined with subsequent genotyping to characterize the strains of PA isolated.

Results

We found that 13/21 segregated clinic patients and 14/18 mixed clinic patients showed positive PA, with median acquisition ages of 3.3 and 2.2 years, respectively (P=0.57). The median time to PA acquisition, however, was significantly longer in the new clinic with proper hygiene precautions compared to an old site (5.0 vs 1.7 years, P<0.001). The majority of subjects isolated a single genotype of PA or AP-PCR types during the study period with 8 subjects clearing the isolate after only one positive culture. The development of chronic colonization yielded the predominance of a single major genotype or AP-PCR type.

Conclusions

Segregation of infants and young children with CF in PA negative or PA positive clinics did not alter the time to first PA isolation in this randomized assessment of facilities with hygienic precautions. During the early infection period where PA is first isolated in young children with CF, patients cleared different PA strains until a predominant strain established permanent colonization.

Background

A comparison of the longitudinal progression of lung disease in cystic fibrosis patients identified through newborn screening (NBS) in cohorts located in two different countries has never been performed and was the primary objective of this study.

Methods

The study included 56 patients in Brittany diagnosed through NBS between 1989 and 1994 and 69 similar patients in Wisconsin between 1985 and 1994. The onset and progression of lung disease was radiographically quantified using the Wisconsin Chest X-ray (WCXR) scoring system. A single pediatric pulmonologist blinded to all identifiers scored the films.

Results

Generalized estimating equation analyses adjusted for age, genotype, sex, pancreatic insufficiency, and meconium ileus showed worse WCXR scores in Brittany patients compared to Wisconsin patients (average score difference=4.48; p<0.001). Percent predicted FEV1 was also worse among Brittany patients (p<0.001).

Conclusions

The finding of milder radiographically-quantified lung disease using the WCXR scoring system, as well as better FEV1 values, may be explained by variations in nutrition, environmental exposures, or healthcare delivery.

Introduction

Telephone quitlines are an effective way to provide evidence-based tobacco dependence treatment services at the population level. Information about what services quitlines offer and how those services are used may improve their reach to the smoking population.

Methods

The North American Quitline Consortium surveyed state quitlines in 2005 and 2006 to get information about quitline services, funding, and use. We report changes between 2005 and 2006.

Results

By 2006, all 50 states, the District of Columbia, and Puerto Rico had quitlines, and annual mean reach was approximately 1% of US adult smokers (aged 18 years or older). Significant increases were seen in mean quitline reach, mean per capita funding for quitline services, and provision of free cessation medications; otherwise, few changes were seen in quitline services.

Conclusions

Quitlines have the potential to serve a large percentage of smokers. Between 2005 and 2006, gains in the number, reach, and per capita funding for quitline services in the United States were seen. Although this represents progress, further research and investment to optimize quitline service delivery and reach are required for quitlines to fulfill their potential of improving the health of the American population.