Breathe Easy Pediatrics Presents: Tidal Volume Episode 2 – Cystic Fibrosis Exacerbations

Tidal Volume is a new podcast from ATS Peds focusing on core concepts of pediatric pulmonology. The goal is to bring in depth focus on these core concepts for residents, fellows, early career pulmonologists or even senior faculty looking for a refresher. In episode 2, Ryan Thomas MD from Michigan State University discusses the Cystic Fibrosis exacerbations. There is a brief review of CF pulmonary physiology followed by a discussion of symptoms, signs, and causes of CF exacerbations. We then explore management and touch on some of the evidence for how we manage exacerbations.

Contact Information:
Tidal Volume Email:
 
Twitter Accounts:
Ryan Thomas, MD
ATS Peds:
@ATSPeds

Selected References:

Clinical Impact


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. 

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

Liou TG, Adler FR, Fitzsimmons SC, et al. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol 2001; 153: 345–352
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.

Causes

Asner S et al. Role of respiratory viruses in pulmonary exacerbations in children with cystic fibrosis. J Cyst Fibros. 2012;11(5):433. 
Zemanick ET, Wagner BD, Harris JK, et al. Pulmonary exacerbations in cystic fibrosis with negative bacterial cultures. Pediatr Pulmonol 2010; 45: 569–577

Regelmann WE, Elliott GR, Warwick WJ, et al. Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone. Am Rev Respir Dis 1990; 141: 914–921.

Antimicrobials

Szaff M, Høiby N. Antibiotic treatment of Staphylococcus aureus infection in cystic brosis. Acta Paediatr Scand 1982; 71: 821–826.
Pressler T, Szaff M, Høiby N. Antibiotic treatment of Haemophilus inuenzae and Haemophilus parainuenzae infections in patients with cystic fibrosis. Acta Paediatr Scand 1984; 73: 541–547.

Rayner RJ, Hiller EJ, Ispahani P, et al. Haemophilus infection in cystic brosis. Arch Dis Child 1990; 65: 255–258.
Briggs EC, Nguyen T, Wall MA, et al. Oral antimicrobial use in outpatient cystic fibrosis pulmonary exacerbation management: a single-center experience. Clin Respir J 2012; 6: 56–64.

Johnson C, Butler SM, Konstan MW, et al. Factors influencing outcomes in cystic fibrosis: a center-based analysis. Chest 2003; 123: 20–27.
Clinical Practice Guidelines for Cystic Fibrosis Committee. 1997. Clinical practice guidelines for cystic fibrosis. Bethesda,MD: the Foundation; 1997

Treatment Duration

Cystic Fibrosis Trust. Antibiotic treatment for cystic fibrosis. Report of the UK Cystic Fibrosis Trust Antibiotic Working Group. 3rd Edn. London, Cystic Fibrosis Trust, 2009.

Collaco JM, Green DM, Cutting GR, et al. Location and duration of treatment of cystic fibrosis respiratory exacerbations do not affect outcomes. Am J Respir Crit Care Med 2010; 182: 1137–1143.

Waters V, Stanojevic S, Klingel M, Chiang J, Sonneveld N, Kukkar R, Tullis E, Ratjen F. Prolongation of antibiotic treatment for cystic fibrosis pulmonary exacerbations. J Cyst Fibros. 2015 Nov;14(6):770-6. 

VanDevanter DR, Flume PA, Morris N, Konstan MW. Probability of IV antibiotic retreatment within thirty days is associated with duration and location of IV antibiotic treatment for pulmonary exacerbation in cystic fibrosis. J Cyst Fibros. 2016 Nov;15(6):783-790. 

SM Brown, IM Balfour-Lynn. Duration of intravenous antibiotic treatment for respiratory exacerbations in children with cystic fibrosis Arch Dis Child, 95 (7) (2010), p. p568
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