Sharon McNamara

Changes in cystic fibrosis sputum microbiology in the United States between 1995 and 2008

The study objective was to identify changes in cystic fibrosis (CF) sputum microbiology over 13 years.

Risk factors for age at initial Pseudomonas acquisition in the cystic fibrosis epic observational cohort

BACKGROUNDnRisk factors for initial Pseudomonas aeruginosa (Pa) acquisition, particularly environmental exposures, are poorly understood. We aimed to identify such risk factors in order to inform prevention strategies and identify high-risk populations.nnnMETHODSnThe study cohort included all participants in the U.S. EPIC Observational Study who had no prior Pa-positive respiratory cultures (N=889). Cox proportional hazard models were used to test the effects of factors on age at first Pa-positive respiratory culture.nnnRESULTSnCystic fibrosis (CF) genotype functional class had an important effect on age at initial Pa acquisition (hazard ratio (HR) comparing minimal to residual CFTR function 2.87 (95% CI 1.88, 4.39)). None of the modifiable risk factors evaluated, including cigarette smoke, hot tub use, breastfeeding, or daycare, was associated with age at Pa acquisition. Similarly, newborn screening was not associated with age at Pa acquisition (HR 0.85, 95% CI 0.66, 1.09). Key associations were validated in a CF Foundation National Patient Registry replication cohort.nnnCONCLUSIONSnGiven the ubiquitous presence of Pa in the environment, it may be that many imposed lifestyle changes will have less impact on age at initial Pa acquisition than genetic determinants.

Randomized Trial of Biofilm Testing to Select Antibiotics for Cystic Fibrosis Airway Infection

In cystic fibrosis (CF), conventional antibiotic susceptibility results correlate poorly with clinical outcomes. We hypothesized that biofilm testing would more accurately reflect the susceptibilities of bacteria infecting CF airways.

Escherichia coli dysbiosis correlates with gastrointestinal dysfunction in children with cystic fibrosis

Cystic fibrosis gastrointestinal disease includes nutrient malabsorption and intestinal inflammation. We show that the abundances of Escherichia coli in fecal microbiota were significantly higher in young children with cystic fibrosis than in controls and correlated with fecal measures of nutrient malabsorption and inflammation, suggesting that E. coli could contribute to cystic fibrosis gastrointestinal dysfunction.

Respiratory viruses in children with cystic fibrosis: viral detection and clinical findings

Please cite this paper as: Burns et al. (2011) Respiratory viruses in children with cystic fibrosis: viral detection and clinical findings. Influenza and Other Respiratory Viruses 6(3), 218–223.

Feasibility of using pedometers to measure daily step counts in cystic fibrosis and an assessment of its responsiveness to changes in health state.

BACKGROUNDnEvaluation of physical activity is integral to the assessment of daily physical function and a potential objective outcome measure for clinical trials. We evaluated the feasibility of using pedometers to measure physical activity in adolescents and adults with cystic fibrosis (CF) and assessed the responsiveness of its measurement to changes in health state.nnnMETHODSnParticipants were recruited through two CF clinics in Seattle, WA. Subjects were instructed to use their pedometer for at least one ill and two well periods (each lasting 7 days). Step rate was calculated as steps per hour of use. Daily symptoms were also recorded using the CF Respiratory Symptom Diary (CFRSD). Generalized estimating equation linear regression was used to compare mean step rate between health states and by self-reported symptom category.nnnRESULTSnWe enrolled 30 CF patients with a mean (±SD) age of 22 (±7) years and a mean forced expiratory volume in 1s (FEV(1)) of 57% (±25%) predicted. The mean period step rate increased from 397 (95% CI 324-497) steps/hour when ill to 534 (95% CI 413-654) steps/hour when well (p=0.015). Pedometer-recorded step rate also correlated with self-reported physical activity items on the CFRSD.nnnCONCLUSIONnStep rate measured with a pedometer correlates significantly with changes in health status and self-reported activity, and could be used as an outcome measure in CF.

Sinonasal Quality of Life in Children With Cystic Fibrosis

IMPORTANCEnSinusitis is the most common otolaryngologic complaint in children with cystic fibrosis (CF). However, basic knowledge about the effect of sinusitis on these children is lacking.nnnOBJECTIVEnTo evaluate the incidence and quality-of-life impact of chronic rhinosinusitis (CRS) in an unbiased cohort of children with CF.nnnDESIGN, SETTING, AND PARTICIPANTSnSurvey study of consecutive pediatric patients with CF presenting for routine quarterly evaluation at a tertiary CF clinic at an academic pediatric hospital. Surveys were completed during the period from December 2012 to January 2013.nnnMAIN OUTCOMES AND MEASURESnSurveys designed to assess major criteria for diagnosis of CRS and a validated pediatric sinonasal quality-of-life instrument, the Sinonasal-5 (SN-5). Statistical analysis was performed to evaluate association between demographic features and survey responses.nnnRESULTSnOf the 102 consecutive eligible patients, 47 children (46%) aged 2 to 20 years (mean [SD] age, 12.9 [5.6] years; 24 [51%] female) completed the surveys. Depending on the exact diagnostic criteria used, 5 (11%) to 18 (38%) of children with CF had CRS. Mean domain (2.16; 95% CI, 2.02-2.30) and overall visual-analog scale (8.26; 95% CI, 8.01-8.51) scores on the SN-5 were consistent with minimal effect on quality of life and comparable to historical posttreatment scores. Mean scores on nasal obstruction (3.07; 95% CI, 2.80-3.34) and sinusitis (2.68;; 95% CI, 2.42-2.94) were the most affected domains, whereas allergy (1.83; 95% CI, 1.65-2.01), emotional disturbance (1.76; 95% CI, 1.56-1.96), and activity restriction (1.43; 95% CI, 1.31-1.57) were minimally affected. Children with a diagnosis of CRS had higher mean SN-5 scores (2.60; 95% CI, 2.31-2.89 vs 2.05; 95% CI, 1.90-2.20; difference of 0.55; 95% CI, 0.29-0.80). Twenty-five patients (53%) had undergone some treatment for sinusitis. There was no association between SN-5 score and CRS treatment history.nnnCONCLUSIONS AND RELEVANCEnIn this study, the incidence of symptomatic CRS was high, but quality-of-life impact was relatively low among children with CF. Use of standardized assessment scales, including consensus diagnostic criteria and validated quality-of-life surveys, may be helpful to guide referral and management decisions.

Home Self-Collection of Nasal Swabs for Diagnosis of Acute Respiratory Virus Infections in Children With Cystic Fibrosis

Abstract Background Understanding the importance of respiratory viruses in children with cystic fibrosis (CF) has been limited because of challenges using clinic- or hospital-based diagnostic testing. We conducted a pilot study to assess feasibility of home self- (or parent-) collection of nasal swabs (NS). Methods Cystic fibrosis patients aged 6–18 years with new respiratory illness participated. In clinic, a deep nasal flocked swab was collected by research staff and compared with an anterior foam NS obtained after instillation of saline spray. At home, up to 2 self-collections of paired foam NS (with and without saline) were collected and mailed for real-time polymerase chain reaction (PCR) testing. Results Paired swabs were collected from 28 patients: 18 sets in clinic (deep nasal vs saline foam NS) and 43 sets at home (saline vs dry foam NS) with 9 (50%) and 35 (81%) virus detections, respectively. Home-collected NS were obtained closer to illness onset, with a mean difference in symptom days of −2.3 between home and clinic collections (95% confidence interval [CI] −3.5, −1.2; P < .001). Rhinovirus comprised 73% of virus detections; the difference in mean PCR cycle threshold values for rhinovirus between swabs collected at home versus clinic was −3.8 (95% CI −6.8, −0.9; P = .014), indicating significantly higher viral load for home-collected swabs. Conclusions Home-collected foam NS had a higher positivity rate compared with clinic-collected swabs, likely because collection was closer to illness onset. Home self-collection is feasible and well tolerated for timely respiratory virus diagnosis and provides a novel approach for clinical diagnostics and surveillance of respiratory virus infections among CF patients.

Initial development and pilot testing of observer-reported outcomes (ObsROs) for children with cystic fibrosis ages 0–11 years

PURPOSEnPatient-reported outcomes are important clinical trial endpoints. Young children may not be able to reliably report on how they feel or function, so observer-reported outcomes (ObsROs) may be more appropriate for them. The purpose of this study was to develop and pilot field test electronic parent-reported observational instruments for children with cystic fibrosis (CF) 0-6 and 7-11years of age.nnnMETHODSnWe performed concept elicitation interviews with parents of children with CF ≤11years of age to elicit the respiratory signs they could observe at baseline and during an acute respiratory illness. The resulting instruments were refined based on interviews with parents and clinicians. We conducted a pilot field test to evaluate test-retest reliability and the ability of items to distinguish well and sick periods.nnnRESULTSnThe instruments consist of 17 items assessing respiratory signs and observable CF-related impacts. Test-retest reliability was acceptable for both age groups but discrimination was low for ages 7-11, likely reflecting less direct observation of older children by their parents.nnnCONCLUSIONSnAn ObsRO for children with CF ages 0-6 appears promising, while self-report may be more appropriate for children >6years of age. Next steps for the 0-6year old instrument will be utilizing it as an exploratory endpoint in clinical trials to enable item reduction, scale development, and further reliability and validity testing. Ultimately, this ObsRO could be a promising endpoint for early intervention trials in young children with CF.

Age-related heterogeneity in dental caries and associated risk factors in individuals with cystic fibrosis ages 6–20 years: A pilot study

BACKGROUNDnThe literature conflicts regarding dental caries risk in cystic fibrosis (CF) relative to controls.nnnMETHODSnProspective, observational study of age-related heterogeneity in caries rates and potential risk factors in individuals with CF ages 6-20 at a single clinic in Washington state (N=85). Caries rates for enrolled CF participants and historical controls from NHANES were compared using cubic spline regression models. Generalized linear regression models identified correlates of age and caries in CF.nnnRESULTSnChildren ages 6-9 with CF had significantly lower caries than controls (Holms P<0.05). There was no difference for ages 10-20 by CF status (Holms P>0.05). Various biological/intraoral, medical, and behavioral factors were associated with caries and age in CF.nnnCONCLUSIONSnYounger children with CF may be protected from caries, but there is apparent loss of protection in early adolescence associated with multiple risk factors. Additional studies are needed to confirm these findings.