Pulmonology

Cystic Fibrosis

Questions discussed in this category

Is there a role for chronic suppressive oral or inhaled therapy for recurrent Burkholderia cepacia pneumonia causing frequent hospitalizations in a patient with severe bronchiectasis with or without underlying cystic fibrosis?

3 Answers available

When do you consider the use of steroids in the management of acute exacerbations in patients with cystic fibrosis?

1 Answer available

What is your approach to the management of symptomatic Cystic Fibrosis carriers?

1 Answer available

How do you manage GI side effects related to CFTR modulator therapy?

2 Answers available

How do you approach the use of chronic azithromycin in patients with CF who may require therapy with IV tobramycin in the future?

Given the suggestion of the antagonistic effect of azithromycin on IV tobramycin in prior studies.

1 Answer available

Do you continue CFTR modulator therapy in patients with extra-pulmonary manifestations of CF after lung transplant?

1 Answer available

Do you routinely use sweat tests if you are going to titrate a CF patient onto a lower dose of ETI for any reason (allergy, mental health, etc) to assess the severity of their response to the lower dose?

1 Answer available

Do you routinely continue airway clearance therapies in the setting of mild to moderate hemoptysis in a patient with CF?

2 Answers available

Do you often see clinical improvement that is significant enough to de-list patients with CF for transplant after the initiation of CFTR modulator therapy?

2 Answers available

Do you de-escalate airway clearance therapies in patients with CF who experience an improvement in mucus production and clearance after CFTR modulator therapy is started?

1 Answer available

Papers discussed in this category

Ann Am Thorac Soc,

Evaluating the Impact of Stopping Chronic Therapies after Modulator Drug Therapy in Cystic Fibrosis: The SIMPLIFY Clinical Trial Study Design.

American journal of respiratory and critical care medicine, 2010-08-01

Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax.

Am J Respir Crit Care Med,

Rapid Improvement after Starting Elexacaftor-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2017-01

Cancer risk among lung transplant recipients with cystic fibrosis.

N Engl J Med, 2019 Oct 31

Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.

J Cyst Fibros, 2020 Jul 18

Effect of highly effective modulator treatment on sinonasal symptoms in cystic fibrosis.

Thorax, 2021 Oct 27

Testing the effects of combining azithromycin with inhaled tobramycin for in cystic fibrosis: a randomised, controlled clinical trial.

Journal of clinical medicine, 2020 Dec 23

Real-World Safety of CFTR Modulators in the Treatment of Cystic Fibrosis: A Systematic Review.

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