Patients with cystic fibrosis (CF) are considered at possibly higher risk if they contract the severe acute respiratory syndrome‐coronavirus‐2 (SARS‐CoV‐2) virus.1–3 Often, patients with CF develop more severe viral respiratory tract infections and have a greater risk of diminished lung function due to preexisting lung inflammation.4 We undertook a survey of pediatric CF patients to better understand how quarantine and anxiety regarding infection affected this patient population.
Materials and methods
This study was approved through the Institutional Review Board of Children’s Hospital Los Angeles (CHLA; CHLA‐19‐00462). Patients receiving care through CHLA completed surveys from December 30, 2019, to March 11, 2020, during routine CF visits as part of a prior study on CF and smell loss. Any child between the age of 6 to 22 years with CF presenting to the clinic for care was invited to participate in the initial quality‐of‐life survey. Los Angeles experienced school closures starting on March 16, 2020, and statewide stay‐at‐home orders starting March 19, 2020. Between April 15, 2020 and May 5, 2020, a phone call was made to guardians of patients who had completed the initial surveys asking for consent to send follow‐up surveys electronically. A reminder e‐mail was sent if no response was obtained and a final follow‐up phone call was made if necessary. Guardians who indicated they did not have access to e‐mail were asked to respond to the questionnaire verbally. The questionnaires and verbal surveys were available in both English and Spanish.
The survey consisted of a sinonasal disease assessment, CF quality‐of‐life assessment, and coronavirus disease 2019 (COVID‐19)‐specific questions. Adolescent patients ≥14 years personally completed the validated 22‐item Sino‐Nasal Outcome Test (SNOT‐22) and adult/adolescent Cystic Fibrosis Questionnaire‐Revised (CFQ‐R) whereas a guardian of children between 6 and 13 years completed the validated Sinonasal‐5 (SN‐5) and the parental CFQ‐R.5–7 Last, 10 questions regarding COVID‐19 were asked using a Likert forced‐choice scale.
Survey results were collected via Research Electronic Data Capture (REDCap; Vanderbilt University, Nashville, TN) and data analysis was completed in R (R Foundation for Statistical Computing, Vienna, Austria; https://www.r‐project.org/).8, 9 Results were compared using paired 2‐sided t tests, chi‐square tests, Cohen’s D, and multivariate linear regression.
Results
Seventy‐five patients were initially approached to be included in a study of CF and smell loss, and 72 patients between the ages of 6 and 22 years completed the initial sinonasal survey and CFQ‐R (3 patients declined to participate). For the survey of patients after stay‐at‐home orders, 4 patients were unable to be reached for consent, 68 patients were consented, and 50 completed the follow‐up survey. Four patients were excluded from sinonasal and CFQ‐R analysis because their initial surveys were performed during a “sick” or “hospital follow‐up visit” rather than a routine visit. Patients who completed the pre‐COVID‐19 surveys were 62% female; 57% identified as Hispanic, 26% as white, and 6% as black; 62% of patients had at least 1 F508 mutation; 13% had prior sinus surgery and 37% were on elexacaftor/tezacaftor/ivacaftor. There were no statistically significant differences in age, gender, or race of respondents who did or did not participate in the follow‐up survey.
Sinonasal symptoms
The mean SN‐5 and SNOT‐22 scores decreased after the stay‐at‐home order (Table 1).
SN‐5, SNOT‐22, and CFQR results pre‐COVID‐19 and post‐COVID‐19 stay‐at‐home order
| Pre‐COVID‐19 | Post‐COVID‐19 | p | ||
|---|---|---|---|---|
| Parameter | (mean ± SD) | (mean ± SD) | (paired t test) | Cohen’s D effect size |
| Sinonasal scores | ||||
| SN‐5 (Child) | 14.52 ± 7.19 | 11.13 ± 14.52 | 0.01 | 0.61 |
| SNOT‐22 (Adolescent) | 24.94 ± 17.08 | 15.33 ± 24.94 | <0.01 | 0.87 |
| CFQR Respiratory domain | ||||
| Child | 72.71 ± 21.96 | 88.29 ± 16.37 | <0.01 | 0.83 |
| Adolescent | 65.15 ± 24.79 | 85.96 ± 16.18 | <0.01 | 0.99 |
- CFQ‐R = Cystic Fibrosis Questionnaire Revised; SD = standard deviation; SN‐5 = Sinonasal 5; SNOT‐22 = 22‐item Sino‐Nasal Outcome Test.
CFQ‐R
Among both children and adolescents, there was a statistical improvement in the respiratory domain (Table 1). There were no other statistically significant changes in health quality‐of‐life in the other domains. There was no significant association between elexacaftor/tezacaftor/ivacaftor usage and change in Respiratory Domain score in a multivariate linear regression model.
COVID‐19
The distribution of Likert answers to the COVID‐19 survey questions are shown in Figure 1. A total of 50% of children or adolescents were primarily receiving their COVID‐19 information from family, friends, or teachers, and 32% from the news, whereas 6% were primarily receiving information from online sources and 4% from social media. There were no reports of COVID‐19‐like illnesses among participants or household members, which accounted for the additional questions.
Distribution of Likert answers to COVID‐19 Survey questions. COVID‐19 = coronavirus disease 2019.
Discussion
Both children and adolescents with CF had a statistically significant improvement in sinonasal symptom scores and in the Respiratory Symptom domain of the CFQ‐R after institution of COVID‐19 precautions, and the vast majority were following social distancing guidelines. The improvement in Respiratory Domain score was greater than the previously calculated minimal clinically important difference and the effect size was large.7
Improved hygiene habits, school closures, and social distancing may have helped to decrease the spread of other upper respiratory illnesses and thus improved patients’ reported sinonasal and respiratory symptoms. Routine multispecialty CF clinics were placed on hold; therefore, changes in health are unlikely to be due to changes in management.
Limitations of the study include the self‐reported nature of symptoms. No routine pulmonary function test data from the post–stay‐at‐home order period is currently available. Additionally, our patient population may not be representative of the larger national population of pediatric patients with CF. Further studies are necessary on how SARS‐CoV‐2 affects patients with CF and how quarantine regulations affect both socially and medically vulnerable pediatric populations.
Conclusion
Both children and adolescents with CF at an urban tertiary center have had a statistically significant improvement in sinonasal symptom scores and in the Respiratory Symptom domain of the Cystic Fibrosis Questionnaire‐Revised (CFQ‐R) after institution of state and county level COVID‐19 precautions.
References
