CRS Impact on Patients with Cystic Fibrosis Not Well Known

What is the impact of chronic rhinosinusitis (CRS) on pediatric patients with cystic fibrosis (CF)?

Bottom line
Despite the high frequency of CRS in the pediatric CF patient population, its impact on quality of life is not well understood. Currently there is no validated, disease-specific, quality of life instrument, available to assess the impact of CRS on the pediatric CF patient population.

Background: Individuals with CF have an incidence of CRS approaching 100%, which is often associated with nasal polyposis (6%–48%); however, data from the U.S. Cystic Fibrosis Foundation’s (CFF) CF Patient Registry indicates that only 2%–3% of pediatric patients per year have sinus disease requiring surgery. Other single-center studies have documented that approximately 10%–20% of CF patients require surgical management of their sinus disease. Despite the high prevalence of CRS in the CF population, little is known about the optimal treatment, indications for surgery, and outcomes of treatment for CF CRS, leading to wide variation in practice patterns.

Study design: PubMed and EMBASE literature review.

Synopsis: PubMed and EMBASE electronic databases were searched using Boolean searches that incorporated mesh headings and plain language for quality of life, symptom evaluation, pediatric patients, and sinusitis/rhinosinusitis. Studies were included if the study primarily evaluated a pediatric CF-CRS population and the primary outcome measure was quality of life evaluation. The search generated a total of 34 unique articles, and seven articles met the criteria for inclusion. The SN-5 is a questionnaire that has been validated in the non-CF patient population ages two through 12. By design, it is to be completed by the patients’ proxy/primary caregiver. The investigators say this review highlighted the fact that there is a paucity of literature evaluating the effects on quality of life for pediatric patients with CF-associated CRS. Currently, no disease-specific quality of life measure exists for this patient population. Although the SN-5 has shown promise, the fact that it is only validated in such a limited patient population leaves no validated measurement tool for patients between the ages of 12 and 18. It is likely that the true impact of CRS on pediatric CF patients will not be known until a validated measure exists that encompasses all patients younger than 18.

Citation: Virgin FW. Clinical chronic rhinosinusitis outcomes in pediatric patients with cystic fibrosis. Laryngoscope Investig Otolaryng. Published May 31, 2017. DOI: 10.1002/lio2.78.