Evaluating the sinus and Nasal Quality of Life Survey in the pediatric cystic fibrosis patient population
Introduction
Cystic fibrosis (CF) is an autosomal recessive disorder with mutations in the CF transmembrane conductance regulator gene. This mutation occurs most commonly as a deletion of F508 [1]. Chronic rhinosinusitis (CRS) is significantly prevalent in the CF population with 74%–100% of CF patients experiencing symptoms of severe chronic rhinosinusitis [2]. Chaaban et all suggested that sinus disease in these patients is both reflective and predictive of pulmonary disease [2].
Health-related quality of life (QOL) instruments have previously been validated for adults with CRS [3], [4], [5]; however, there are no similar QOL instruments for pediatric CF patients with CRS. This lack of data makes it difficult for clinicians to determine how CRS affects QOL in pediatric CF patients, and further may complicate the selection of an appropriate and effective treatment plan.
Currently, the Sinonasal Outcome Test (SNOT-22) is commonly used to measure CRS symptoms in adults by evaluating various outcomes including physical problems, functional limitations, and emotional consequences [6], [7]. However, the SNOT-22 has not yet been evaluated and validated for use in pediatric populations. The Sinus and Nasal Quality of Life Survey (SN-5), specific to the pediatric population, asks caregivers to rate their children's CRS symptoms on a 7-point scale [8]. This survey evaluates 5 specific symptoms (sinus infection, nasal obstruction, allergy symptoms, emotional distress, and activity limitations). In addition to the symptomatic evaluation, caregivers evaluate the child's overall QOL on scale from 0 to 10. This survey has only been validated in children between the ages of 2–12, leaving those between the ages of 12–18 without a validated CRS QOL instrument.
The SN-5 was validated in a population of pediatric CRS patients but has not yet been formally evaluated in a CF population. There are recent papers looking at the use of SN-5 in CF patients [9], [10], [11]. When used to identify the CRS QOL burden for CF patients, Chan et al. showed that despite the high incidence of symptomatic CRS, the SN-5 results indicated that the disease impact was relatively low [9]. Wentzel et al. [10] similarly evaluated SN-5 for 50 patients with CF, and found that mean symptom frequency was negatively correlated with overall QOL score (r = −0.55, p = 0.00006). However, there does not exist a quality of life measurement that is specific to pediatric CF CRS. As such, there remains a need for a quality of life instrument that can be used to evaluate the overall impact of medical and surgical intervention in the short and long term. The goal of this study is to evaluate the SN-5 as a health-related QOL instrument for the pediatric CF patient population.
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Methods
This retrospective study analyzed previously completed SN-5 surveys. Survey data between 2012 and 2015 from pediatric cystic fibrosis patients were collected and complemented with clinical and sociodemographic data. Overall QOL scores and specific symptom scores were obtained from surveys completed at the first, baseline, and last follow up clinic visit in the three-year span. Patients, or patient caregivers, of all ages were asked to complete the surveys and were later analyzed by age groups
Results
A total of 266 patients completed surveys. Of these, 165 patients had both baseline and follow-up data. Mean age at baseline data collection was 8.70 ± 5.28 years. A majority of patients identified as Caucasian (97.0%) and non-Hispanic (98.2%). Additional demographic data are shown in Table 1. Mean baseline scores in the symptom domains ranged from 1.68 ± 1.34 for activity limitations to 3.08 ± 2.51 for sinus infections, where higher values indicate experiencing these symptoms more frequently (
Discussion
CRS affects a significant proportion of children with CF. Currently, a wide range of treatment practices exist, with a multicenter review identifying rates of sinus surgery for CF patients ranging from 1 to 24% across 29 pediatric hospitals [12]. This wide range resembles different surgical indications across the nation, suggesting the need for data to support a more standardized treatment. In order to create a more robust treatment algorithm, we must first identify the candidates who can
Conclusion
There remains a need for a validated CRS QOL tool for children with CF. Though the SN-5 has previously been described as a potential instrument, our data suggest that it may be more valuable in children ages 0–4. Given the variable utility of this survey based on patient age, this may not be the most appropriate metric to evaluate the impact of CRS in pediatric CF.
Funding
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Conflict of interest
The authors report no conflicts of interest.
Acknowledgements
We would like to thank Bavly Dawoud and Amita Bey for their contributions to this project. This research was supported in part by the Surgical Outcomes Center for Kids at Monroe Carell Jr. Children's Hospital at Vanderbilt and through the Section for Surgical Sciences at Vanderbilt University Medical Center. The research utilized REDCap which was funded by grant UL1 TR000445 from NCATS/NIH.
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