A physicians survey assessing management of pulmonary airway involvement in sickle cell disease

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
JournalPediatric Pulmonology
DOIs
StatePublished - Jan 1 2019

Fingerprint

Airway Management
Sickle Cell Anemia
Asthma
Inflammation
Physicians
Acute Chest Syndrome
Lung
Respiratory Hypersensitivity
Airway Obstruction
montelukast
Bronchodilator Agents
Pediatrics
Surveys and Questionnaires
Consensus
Adrenal Cortex Hormones
Guidelines

Keywords

  • airway
  • asthma
  • inflammation
  • pulmonary
  • sickle cell disease
  • survey

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

Cite this

@article{18abb7a08b3c437ea41a43a348c61b94,
title = "A physicians survey assessing management of pulmonary airway involvement in sickle cell disease",
abstract = "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.",
keywords = "airway, asthma, inflammation, pulmonary, sickle cell disease, survey",
author = "Sabhyata Agrawal and Burton, {William B.} and Manwani, {Deepa G.} and Deepa Rastogi and Aliva De",
year = "2019",
month = "1",
day = "1",
doi = "10.1002/ppul.24289",
language = "English (US)",
journal = "Pediatric Pulmonology",
issn = "8755-6863",
publisher = "Wiley-Liss Inc.",

}

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 G.

AU - Rastogi, Deepa

AU - De, Aliva

PY - 2019/1/1

Y1 - 2019/1/1

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

UR - http://www.scopus.com/inward/record.url?scp=85064712634&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85064712634&partnerID=8YFLogxK

U2 - 10.1002/ppul.24289

DO - 10.1002/ppul.24289

M3 - Article

JO - Pediatric Pulmonology

JF - Pediatric Pulmonology

SN - 8755-6863

ER -