Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
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Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
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People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
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The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
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Hemoptysis and pneumothorax are complications reported in people with cystic fibrosis. This document reflects the Cystic Fibrosis Foundation's Pulmonary Therapies Committee recommendations for the treatment of hemoptysis and pneumothorax.
Flume PA, Mogayzel PJ, Robinson KA, et al. Cystic Fibrosis Pulmonary Guidelines: Pulmonary Complications: Hemoptysis and Pneumothorax. Am J Respir Crit Care Med. 2010 Aug 1;182(3):298-306. doi: 10.1164/rccm.201002-0157CI.PMID: 20675678.
The Cystic Fibrosis Foundation Pulmonary Therapies Committee recognized that insufficient data existed to develop evidence-based recommendations. The Delphi process was used to formalize an expert panel's consensus and develop explicit care recommendations. These guidelines were designed to answer common questions encountered in the management of people with cystic fibrosis with hemoptysis and pneumothorax.
The committee noted that the guidelines were designed for general application in most individuals, but should be adapted to meet the specific needs of individual patients. Updated publications relevant to the management of hemoptysis and pneumothorax in CF are reviewed below, with citations listed in the further reading section.
A multidisciplinary committee was assembled to perform a literature search and develop specific questions regarding the management of pneumothorax and hemoptysis in people with CF. A separate expert panel composed of CF clinicians, interventional radiologists, and lung transplantation surgeons was selected by committee members to complete the survey questions, rating the appropriateness of each statement using a Likert scale. Delphi methodology was used to quantify group consensus. Numeric responses to the questions were summarized and applied to a priori definitions to determine levels of consensus. The expert panel completed the survey twice, allowing refinement of recommendations.
The patient with scant hemoptysis should contact their health care provider if it is the first-ever episode, or if it is persistent.
The patient with at least mild hemoptysis should contact their health care provider.
The patient with scant hemoptysis may not require admission to the hospital.
The patient with massive hemoptysis should always be admitted to the hospital.
The patient with scant hemoptysis but without signs of a pulmonary exacerbation may not need antibiotics.
The patient with at least mild hemoptysis should be treated with antibiotics.
For patients with scant hemoptysis, there was moderate consensus that NSAIDS should be stopped.
The patient with at least mild hemoptysis should stop NSAIDS.
The patient with massive hemoptysis who is clinically unstable should be treated with BAE.
The patient with massive hemoptysis should not undergo bronchoscopy before BAE.
There was not sufficient panel consensus regarding performance of a computed tomography (CT) of the chest before BAE to make a recommendation. See Updates and Future Directions section below.
There was no panel consensus to make a management consensus for strategy.
BiPAP should not be withheld from patients with scant hemoptysis.
For patients with mild to moderate hemoptysis, there was no consensus to make a recommendation regarding withholding BiPAP.
BiPAP should be withheld from patients with massive hemoptysis.
Lung resection should be performed for patients with CF with massive hemoptysis only as a final therapeutic option.
The patient with scant hemoptysis should not stop airway clearance therapies.
There was increased concern from the expert panel regarding continuance of airway clearance in patients with mild to moderate hemoptysis, but there was not sufficient consensus to make a recommendation.
The patient with massive hemoptysis should stop all airway clearance therapies.
The patient with scant hemoptysis should not stop aerosol therapies.
For patients with mild to moderate hemoptysis (all therapies included) and for patients with massive hemoptysis (aerosolized therapies other than hypertonic saline), the expert panel felt the benefits of therapies outweighed the risks and therapy should be withheld only if it seemed to exaggerate or provoke bleeding.
The patient with massive hemoptysis should stop aerosolized hypertonic saline.
The patient with a small pneumothorax but who is otherwise clinically stable may be closely observed in the outpatient setting.
The patient with a large pneumothorax should always be admitted to the hospital.
The patient with a small pneumothorax should have a chest tube placed if there is clinical instability.
The patient with a large pneumothorax should have a chest tube placed.
The patient with a first pneumothorax should not undergo pleurodesis to prevent recurrence.
The patient with a recurrent, large pneumothorax should undergo pleurodesis to prevent recurrence.
For the patient with CF with a pneumothorax who is undergoing pleurodesis, the preferred method is surgical pleurodesis (defined as pleurodesis performed with a thoracoscope or through a limited or full thoracotomy).
The expert committee could not gain a consensus or deliver a recommendation for management on this question. Some panelists suggested a pneumothorax was a manifestation of a pulmonary exacerbation, others required additional evidence of a pulmonary exacerbation before treating the patient with antibiotics.
BiPAP should be withheld from patients with pneumothorax as long as the pneumothorax is present.
The patient with a pneumothorax should not fly on a plane for two weeks after the pneumothorax has resolved.
The patient with pneumothorax should not lift weights (>5lbs) for two weeks after the pneumothorax has resolved.
The patient with pneumothorax should not perform spirometry for two weeks after the pneumothorax (both small and large) has resolved.
The panel was not able to come to a recommendation regarding exercise after resolution of a pneumothorax irrespective of the size of the pneumothorax.
The expert committee was unable to reach consensus to make a recommendation. However, the occurrence of a pneumothorax is more frequent in patients with severe pulmonary impairment, and pneumothoraces are discussed in the literature for referral of patients with CF to lung transplant centers.
Some airway clearance therapies, specifically positive expiratory pressure and intrapulmonary percussive ventilation, should not be used in patients with pneumothorax.
There was no consensus reached by the expert panel on withholding other methods of airway clearance, although a higher rating was given to withholding airway clearance therapies for the patient with a large pneumothorax.
The patient with pneumothorax should not stop aerosol therapies.
As the majority of information regarding these medical management options remains at the case report level of evidence or retrospective review, no recommendations can be given and additional study is needed.
Review of published literature since 2010 shows very little new information regarding the management of pneumothorax in people with CF.
There was lack of consensus regarding the following questions in the original guideline:
The authors could not identify any new information to guide recommendations for these situations, and they remain unanswered questions.
There was consensus in the original guidelines that surgical pleurodesis is the preferred method in patients with CF. A Cochrane review from 2012 reviewed published literature to look at this question. The authors could not identify any clinical trials looking at the efficacy or safety of chemical or surgical pleurodesis in patients with CF. As a result, no recommendations to the preferred method of management of pleurodesis could be made.8
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Jason Fullmer, M.D., (Dell Children's Medical Center) and Lynne Quittell, M.D., (Columbia University)
The guidelines were published in August 2010, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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