SPECTRUM OF PULMONARY EXACERBATION IN CHILDREN AND ADOLESCENT WITH CYSTIC FIBROSIS AND PHYSICIANS’ PRACTICES: EXPERIENCE FROM TERTIARY CARE CENTER FROM LOWER-MIDDLE INCOME COUNTRY

Authors

  • Danish Abdul Aziz Aga Khan University Hospital Karachi, Pakistan
  • Shahira Shahid Aga Khan University hospital
  • Anusha Alam Aga Khan University Hospital, Karachi-Pakistan
  • Namrah Aziz Aga Khan University Hospital, Karachi-Pakistan
  • Haissan Iftikhar Aga Khan University Hospital, Karachi-Pakistan
  • Fatima Mir Aga Khan University Hospital, Karachi-Pakistan

Abstract

Background: Pulmonary exacerbations (PEx) are major contributor of significant morbidity and mortality in CF patients. Managing PEx needs standardization and without standard local practice guidelines there will be significant variation in practice in managing these children. The aim of this study is to analyse the clinical management of PEx in our setup and to document variation in practices among physicians. Methods: Children and adolescents ≤18 years with CF pulmonary exacerbations admitted at high dependency unit (HDU) or wards were included in the study. Frequency of different intravenous antibiotic combinations were documented along with use of different inhaled antibiotics and inhalation therapy. Practices of different physician were further studied with regards to use of systemic steroids, oral azithromycin and inhaled antibiotics. One way ANOVA was used to assess differences between physicians’ practices. Results: Fifty-seven patients were selected according to the inclusion criteria for 114 different exacerbations. Mean pulmonary exacerbation (PEx) for a patient (events/person-year) over five years were 3.16±1.41 per year and average length of stay was 5.7±4.4 days. Combination of intravenous ceftazidime and amikacin was the most frequently used regimen (28.07%).  Five different physicians dealing with majority of the exacerbations (n=74) were studied further. Variability among consultants was significant in using systemic steroids (21.42–92.30%), use of maintenance oral azithromycin (0–80%) and inhaled antibiotics (0-86.6%). Conclusion: Significant variation exists in practices of physicians dealing with CF PEx. Variability observed in our study will definitely provide openings for local CF experts to come up with standardized inpatient exacerbation guidelines.

Author Biography

Danish Abdul Aziz, Aga Khan University Hospital Karachi, Pakistan

Assistant Professor DEPARTMENT OF PAEDIATRICS

References

Sanders DB, Bittner RC, Rosenfeld M, Hoffman LR, Redding GJ, Goss CH. Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation. Am J Respir Crit Care Med 2010;182(5):627–32.

Waters V, Stanojevic S, Atenafu EG, Lu A, Yau Y, Tullis E, et al. Effect of pulmonary exacerbations on long-term lung function decline in cystic fibrosis. Eur Respir J 2012;40(1):61–6.

Sanders DB, Bittner RC, Rosenfeld M, Redding GJ, Goss CH. Pulmonary exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis. Pediatr Pulmonol 2011;46(4):393–400.

Morgan WJ, Wagener JS, Yegin A, Pasta DJ, Millar SJ, Konstan MW, et al. Probability of treatment following acute decline in lung function in children with cystic fibrosis is related to baseline pulmonary function. J Pediatr 2013;163(4):1152–7.

Rosenfeld M, Emerson J, Williams-Warren J, Pepe M, Smith A, Montgomery B, et al. Defining a pulmonary exacerbation in cystic fibrosis. J Pediatr 2001;139(3):359–65.

Heltshe SL, Goss CH, Thompson V, Sagel SD, Sanders DB, Marshall BC, et al. Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis. Thorax 2016;71(3):223–9. Ref no 6&22 are same

Mayer-Hamblett N, Rosenfeld M, Emerson J, Goss CH, Aitken ML. Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. Am J Respir Crit Care Med 2002;166(12):1550–5.

Castellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F, et al. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros 2018;17(2):153–78.

Villanueva G, Marceniuk G, Murphy MS, Walshaw M, Cosulich R. Diagnosis and management of cystic fibrosis: summary of NICE guidance. BMJ 2017;359:j4574.

Flume PA, Mogayzel PJ, Robins KA, Goss CH, Rosenblatt Rl, Kuhn RJ, et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med 2009;180(9):802–8.

Sanders DB, Solomon GM, Beckett VV, West NE, Daines CL, Heltshe SL, et al. Standardized treatment of pulmonary exacerbations (STOP) study: observations at the initiation of intravenous antibiotics for cystic fibrosis pulmonary exacerbations. J Cyst Fibros 2017;16(5):592–9.

Steinraths M, Vallance HD, Davidson AG. Delays in diagnosing cystic fibrosis: Can we find ways to diagnose it earlier? Can Fam Physician 2008;54(6):877–83.

Mir TA, Ashraf M, Ahmed K, Chowdhary J, Rehana B, Ahmed J. Clinical profile, diagnostic delay, and genetic make-up of cystic fibrosis in Kashmir, India. Lung India 2011;28(2):97–100.

Brumback LC, Baines A, Ratjen F, Davis SD, Daniel SL, Quittner AL, et al. Pulmonary exacerbations and parent‐reported outcomes in children< 6 years with cystic fibrosis. Pediatr Pulmonol 2015;50(3):236–43.

Blanchard AC, Horton E, Stanojevic S, Taylor L, Waters V, Ratjen F. Effectiveness of a stepwise Pseudomonas aeruginosa eradication protocol in children with cystic fibrosis. J Cyst Fibros 2017;16(3):395–400.

Stressmann FA, Rogers GB, Marsh P, Lilley AK, Daniels TW, Carroll MP, et al. Does bacterial density in cystic fibrosis sputum increase prior to pulmonary exacerbation? J Cyst Fibros 2011;10(5):357–65.

Smith S, Rowbotham NJ, Charbek E. Inhaled antibiotics for pulmonary exacerbations in cystic fibrosis. Cochrane Database Syst Rev 2018;10(10):CD008319.

Wagener JS, Rasouliyan L, VanDevanter DR, Pasta DJ, Regelmann WE, Morgan WJ, et al. Oral, inhaled, and intravenous antibiotic choice for treating pulmonary exacerbations in cystic fibrosis. Pediatr Pulmonol 2013;48(7):666–73.

Hester KL, Powell T, Downey DG, Elborn JS, Jarad NA. Glucocorticoids as an adjuvant treatment to intravenous antibiotics for cystic fibrosis pulmonary exacerbations: a UK Survey. J Cyst Fibros 2007;6(4):311–3.

Tepper RS, Eigen H, Stevens J, Angelicchio C, Kisling J, Ambrosius W, et al. Lower respiratory illness in infants and young children with cystic fibrosis: evaluation of treatment with intravenous hydrocortisone. Pediatr Pulmonol 1997;24(1):48–51.

Dovey M, Aitken ML, Emerson J, McNamara S, Waltz DA, Gibson RL. Oral corticosteroid therapy in cystic fibrosis patients hospitalized for pulmonary exacerbation: a pilot study. Chest 2007;132(4):1212–8.

Heltshe SL, Goss CH, Thompson V, Sagel SD, Sanders DB, Marshall BC, et al. Short-term and long-term response to pulmonary exacerbation treatment in cystic fibrosis. Thorax 2016;71(3):223–9. Ref no 6&22 are same

Dmello D, Nayak RP, Matuschak GM. Stratified assessment of the role of inhaled hypertonic saline in reducing cystic fibrosis pulmonary exacerbations: a retrospective analysis. BMJ Open 2011;1(1):e000019.

Dentice RL, Elkins MR, Middleton PG, Bishop JR, Wark PA, Dorahy DJ, et al. A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis. Thorax 2016;71(2):141–7.

Nichols DP, Odem-Davis K, Cogen JD, Goss CH, Ren CL, Skalland M, et al. Pulmonary outcomes associated with long-term azithromycin therapy in cystic fibrosis. Am J Respir Crit Care Med 2020;201(4):430–7.

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Published

2021-06-28