TY - JOUR
T1 - A physicians survey assessing management of pulmonary airway involvement in sickle cell disease
AU - Agrawal, Sabhyata
AU - Burton, William B.
AU - Manwani, Deepa
AU - Rastogi, Deepa
AU - De, Aliva
N1 - Publisher Copyright:
© 2019 Wiley Periodicals, Inc.
PY - 2019/7
Y1 - 2019/7
N2 - Background: Airway involvement in patients with sickle cell disease (SCD) involves recurrent episodes of acute chest syndrome (ACS), co-existent asthma, lower airway obstruction (LAO), or airway hyper-responsiveness/ bronchodilator response (AHR/BDR). With increased recognition that sickle cell (SC) airway inflammation may be distinct from asthma, our aim was to study regional and individual practices among pediatric pulmonologists and elucidate the patient characteristics that determine the diagnosis of asthma or SC airway inflammation. Methods: A cross-sectional web-based survey including 6 case scenarios on diagnosis and management of pulmonary manifestations of pediatric SC airway disease was conducted. The case scenarios, combined different risk factors for airway inflammation: history of recurrent ACS, atopy, family history of asthma, LAO, or AHR/BDR, with possible responses including − diagnosis of asthma, SC airway inflammation, both or neither. Results: Of the 130 responses, 83 were complete. “Asthma” was diagnosed when LAO (OR, 7.96 [4.28, 14.79]; p < 0.001), family history of asthma (OR 18.88 [5.87, 60.7]; p < 0.001), and atopy (OR 3.19 [1.74, 5.8]; p < 0.001) were present. “SC airway inflammation” was diagnosed when ACS (OR 3.95 [2.08, 7.51]; p < 0.001), and restrictive pattern on PFT (OR 3.75 [2.3, 6.09]; p < 0.001) were present in the scenarios. Regardless of the diagnosis, there was a high likelihood of initiating or stepping up inhaled corticosteroid as compared to prescribing montelukast. Conclusion: There is variability in the diagnosis and management of SC airway inflammation among pediatric pulmonologists. This study highlights the need for consensus guidelines to improve management of SC airway inflammation.
AB - Background: Airway involvement in patients with sickle cell disease (SCD) involves recurrent episodes of acute chest syndrome (ACS), co-existent asthma, lower airway obstruction (LAO), or airway hyper-responsiveness/ bronchodilator response (AHR/BDR). With increased recognition that sickle cell (SC) airway inflammation may be distinct from asthma, our aim was to study regional and individual practices among pediatric pulmonologists and elucidate the patient characteristics that determine the diagnosis of asthma or SC airway inflammation. Methods: A cross-sectional web-based survey including 6 case scenarios on diagnosis and management of pulmonary manifestations of pediatric SC airway disease was conducted. The case scenarios, combined different risk factors for airway inflammation: history of recurrent ACS, atopy, family history of asthma, LAO, or AHR/BDR, with possible responses including − diagnosis of asthma, SC airway inflammation, both or neither. Results: Of the 130 responses, 83 were complete. “Asthma” was diagnosed when LAO (OR, 7.96 [4.28, 14.79]; p < 0.001), family history of asthma (OR 18.88 [5.87, 60.7]; p < 0.001), and atopy (OR 3.19 [1.74, 5.8]; p < 0.001) were present. “SC airway inflammation” was diagnosed when ACS (OR 3.95 [2.08, 7.51]; p < 0.001), and restrictive pattern on PFT (OR 3.75 [2.3, 6.09]; p < 0.001) were present in the scenarios. Regardless of the diagnosis, there was a high likelihood of initiating or stepping up inhaled corticosteroid as compared to prescribing montelukast. Conclusion: There is variability in the diagnosis and management of SC airway inflammation among pediatric pulmonologists. This study highlights the need for consensus guidelines to improve management of SC airway inflammation.
KW - airway
KW - asthma
KW - inflammation
KW - pulmonary
KW - sickle cell disease
KW - survey
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U2 - 10.1002/ppul.24289
DO - 10.1002/ppul.24289
M3 - Article
C2 - 31012283
AN - SCOPUS:85064712634
SN - 8755-6863
VL - 54
SP - 993
EP - 1001
JO - Pediatric pulmonology
JF - Pediatric pulmonology
IS - 7
ER -