Lung boxing gloves hanging

The hidden villain

Pseudomonas aeruginosa is a major cause of bacterial lung infections in people with cystic fibrosis (CF). It can impair lung function and cause symptoms like coughing, wheezing, and changes in the amount of mucus.

Quick takeaways

  • Pseudomonas aeruginosa can hide in the lungs
  • Progression of Pseudomonas aeruginosa can be a problem
  • Feeling better does not mean it’s gone

Found in the lungs of nearly half of all people with CF, Pseudomonas aeruginosa can live deep in the lungs, which is why a negative culture doesn’t always mean you are infection-free. Pseudomonas aeruginosa could still be hiding.

“I've had Pseudomonas aeruginosa infections before. Does it ever go away?”

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The problem with Pseudomonas aeruginosa progression

Pseudomonas aeruginosa can progress over time. When it progresses, it becomes chronic. That means the infection does not always go away, and symptoms may linger and possibly get worse.

Pseudo-what?!

Hear: Pseudomonas aeruginosa
(soo-daMOE-nus A-ridge-a-NO-sa)

Untreated, it can progress to become a different strain of Pseudomonas aeruginosa, called mucoid, that can:

  • Be harder to treat and get rid of
  • Impair lung structure and function

Over time, Pseudomonas aeruginosa can cause permanent lung damage such as scar tissue and cysts in the lungs.

Are you keeping up
with your cultures?

Cystic Fibrosis Foundation (CFF) guidelines recommend 4 respiratory cultures annually.

You can feel better and still be infected

Just because you’re feeling better on your other CF treatments doesn’t mean you’re not infected with Pseudomonas aeruginosa. The only way to know for sure is to keep up with your quarterly cultures. If you’re unsure, talk to your doctor and care team.

References
  • Pseudomonas. Cystic Fibrosis Foundation website. Accessed June 1, 2021. https://www.cff.org/Life-With-CF/Daily-Life/Germs-and-Staying-Healthy/What-Are-Germs/Pseudomonas/
  • Tramper-Stranders GA, van der Ent CK, Wolfs TFW. Detection of Pseudomonas aeruginosa in patients with cystic fibrosis. J Cyst Fibros. 2005;4 Suppl 2:37-43.
  • Cystic fibrosis. US National Library of Medicine website. Updated August 18, 2020. Accessed June 1, 2021. https://medlineplus.gov/download/genetics/condition/cystic-fibrosis.pdf
  • Moore JE, Mastoridis P. Clinical implications of Pseudomonas aeruginosa location in the lungs of patients with cystic fibrosis. J Clin Pharm Ther. 2017;42(3):259-267. doi:10.1111/jcpt.12521
  • Hansen CR, Pressler T, Høiby N. Early aggressive eradication therapy for intermittent Pseudomonas aeruginosa airway colonization in cystic fibrosis patients: 15 years experience. J Cyst Fibros. 2008;7(6):523-530. doi:10.1016/j.jcf.2008.06.009
  • Stuart B, Lin JH, Mogayzel PJ Jr. Early eradication of Pseudomonas aeruginosa in patients with cystic fibrosis. Paediatr Respir Rev. 2010;11(3):177-184. doi:10.1016/j.prrv.2010.05.003
  • Cystic Fibrosis Foundation. 2019 Patient Registry Annual Data Report. Bethesda, MD: Cystic Fibrosis Foundation; 2019. Accessed June 1, 2021. https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2019-Patient-Registry-Annual-Data-Report.pdf
  • Cunningham JC, Taussig LM. Respiratory System. In: An Introduction to Cystic Fibrosis: For Patients and Their Families. 6th ed. Cystic Fibrosis Foundation; 2013:41-58.
  • Li Z, Kosorok MR, Farrell PM, et al. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. JAMA. 2005; 293(5):581-588.
  • Saiman L, Siegel JD, LiPuma JJ, et al. Infection prevention and control guideline for cystic fibrosis: 2013 update. Infect Control Hosp Epidemiol. 2014;35 Suppl 1:S1-S67. doi:10.1086/676882
  • Cystic fibrosis. Mayo Clinic website. Accessed June 1, 2021. https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/diagnosis-treatment/drc-20353706
  • Bacteria and Antibiotic Resistance In People with Cystic Fibrosis. Cystic Fibrosis Foundation website. Accessed June 1, 2021. https://www.cff.org/PDF-Archive/Bacteria-and-Antibiotic-Resistance-in-People-With-CF
  • Svedberg M, Gustafsson P, Tiddens H, Imberg H, Pivodic A, Lindblad A. Risk factors for progression of structural lung disease in school-age children with cystic fibrosis. J Cyst Fibros. 2020;19(6):910-916. doi:10.1016/j.jcf.2019.10.014
  • Calthorpe RJ, Smith SJ, Rowbotham NJ, et al. What effective ways of motivation, support and technologies help people with cystic fibrosis improve and sustain adherence to treatment? BMJ Open Respir Res. 2020;7(1):e000601. doi:10.1136/bmjresp-2020-000601
  • Bregnballe V, Schiøtz PO, Boisen KA, Pressler T, Thastum M. Barriers to adherence in adolescents and young adults with cystic fibrosis: a questionnaire study in young patients and their parents. Patient Prefer Adherence. 2011;5:507-515. doi:10.2147/PPA.S25308
  • Bishay LC, Sawicki GS. Strategies to optimize treatment adherence in adolescent patients with cystic fibrosis. Adolesc Health Med Ther. 2016;7:117-124. doi:10.2147/AHMT.S95637
  • Zobell JT, Moss J, Heuser S, Roe L, Young DC. Understanding the expanding role of pharmacy services in outpatient cystic fibrosis care. Pediatr Pulmonol. 2021;56(6):1378-1385. doi:10.1002/ppul.25283

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