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Original Investigation |

Sinonasal Quality of Life in Children With Cystic Fibrosis

Dylan K. Chan, MD, PhD1; Sharon McNamara, MN2; Jason S. Park, MD, PhD1; Jame Vajda, BS2; Ronald L. Gibson, MD, PhD2; Sanjay R. Parikh, MD3
[+] Author Affiliations
1Division of Pediatric Otolaryngology, Department of Otolaryngology–Head and Neck Surgery, University of California, San Francisco
2Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, Seattle, Washington
3Division of Pediatric Otolaryngology, Seattle Children’s Hospital, Seattle, Washington
JAMA Otolaryngol Head Neck Surg. 2016;142(8):743-749. doi:10.1001/jamaoto.2016.0979.
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Importance  Sinusitis 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.

Objective  To evaluate the incidence and quality-of-life impact of chronic rhinosinusitis (CRS) in an unbiased cohort of children with CF.

Design, Setting, and Participants  Survey 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.

Main Outcomes and Measures  Surveys 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.

Results  Of 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.

Conclusions and Relevance  In 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.

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Figure 1.
Chronic Rhinosinusitis and Sinonasal-5 Survey

Survey administered to consecutive patients presenting for quarterly routine checkup in a cystic fibrosis clinic.

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Figure 2.
Sinonasal-5 (SN-5) Findings in 41 Patients

A, Lower SN-5 scores and higher visual analog scale (VAS) scores each indicate better quality of life. B, Lines indicate best-fit linear regression.

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Figure 3.
Sinonasal-5 (SN-5) and Chronic Rhinosinusitis (CRS) in 41 Patients

A, Mean and individual domain scores for the SN-5 in children with or without CRS according to American Rhinologic Society guidelines. B, Overall visual analog scale (VAS) score for children with and without symptoms consistent with CRS. Error bars represent 95% CIs.

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Figure 4.
Historical Comparison

Mean Sinonasal-5 (SN-5) domain score from 5 historical studies8,1215 and children with cystic fibrosis (CF) with and without symptoms consistent with chronic rhinosinusitis (CRS) according to American Rhinologic Society criteria. Error bars represent 95% CIs.

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