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.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
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.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
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.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
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.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
Updated from 2003, these guidelines on infection prevention and control for cystic fibrosis provide recommendations for people with CF, their families, and health care providers to help reduce the spread of germs in the clinic and hospital setting, as well as in everyday life.
Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update
Saiman L, Siegel JD, LiPuma JJ, Brown RF, Bryson EA, Chambers MJ, Downer VS, Fliege J, Hazle LA, Jain M, Marshall BC, O'Malley C, Pattee SR, Potter-Bynoe G, Reid S, Robinson KA, Sabadosa KA, Schmidt HJ, Tullis E, Webber J, Weber DJ. Infect Control Hosp Epidemiol. 2014 Aug;35 Suppl 1:S1-S67. Epub 2014 Jul 1.PMID: 25025126
The 2013 guidelines for infection prevention and control (IPC) for cystic fibrosis provide an update to the 2003 guidelines. The guidelines were created by an interdisciplinary team using a systematic process to review and grade evidence for each recommendation. Key changes in the 2013 guidelines include the recommendations for:
This update also recommends against universal mask use by health care workers.
Recent studies on airborne spread of CF pathogens and guidelines for nontuberculous mycobacteria (NTM) in CF patients support the 2013 guidelines. Key unanswered questions regarding IPC for CF patients include how to schedule patients in clinic and what Airborne Precautions to take for cases of suspected NTM infection.
In 2014, an update to the 2003 Guideline for Infection Prevention and Control for CF was published. The rationale for the guideline update was to incorporate:
In 2011, an interdisciplinary committee of health care professionals, parents of children with CF, and an adult with CF developed the scope of the guidelines using the population, intervention, comparison, and outcomes (PICO) format. A systematic review of the literature was performed to address the PICO questions. The committee used the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe the strength of evidence for recommendations adopted from other sources such as the Centers for Disease Control and Prevention and World Health Organization. However, limited evidence was found by the systematic review for other recommendations and the GRADE system could not be used. These IPC recommendations for CF were made based on expert consensus (anonymous voting with at least 80 percent agreement by the committee members).
Selected recommendations from the 2013 guideline are presented. An * denotes those recommendations that were modified or new when compared to the 2003 guideline. Relevant studies published since the 2013 guideline are also provided and support the recommendations.
Education: Provide education to all health care personnel and people with CF and their families regarding IPC practices. Educational materials should be age-appropriate and language-appropriate.
Audits of IPC practices: CF care teams should collaborate with their IPC teams to develop protocols, checklists, and audits to standardize implementation of practices for cleaning and disinfecting multiuse items and surfaces in the health care environment.*
100% Committee Consensus
Hand hygiene: All health care professionals and people with CF and family members and friends should perform hand hygiene with alcohol-based hand rub or antimicrobial soap and water when hands could be potentially contaminated with pathogens.
Contact precautions: All health care personnel should implement Contact Precautions (i.e., wear a gown and gloves) when caring for all people with CF, regardless of respiratory tract culture results.*
Six-foot rule: All people with CF, regardless of their respiratory tract culture results, should be separated by at least six feet (two meters) from other people with CF in all settings, to reduce the risk of droplet transmission of CF pathogens. This does not apply to members of the same household.*
Isolation precautions for nontuberculous mycobacteria: The CF Foundation concludes that there is insufficient evidence at the time of publication of this document for or against placing people with CF who are infected with NTM on Airborne Precautions.*
2013 CF IPC guideline
Mask use: All people with CF should wear a surgical (procedure, isolation) mask when in health care settings to reduce the risk of transmission or acquisition of CF pathogens.*
Anticipating psychosocial impact of IPC guidelines: Centers should identify specific concerns for the potential psychosocial impact of the IPC guidelines for people with CF and identify strategies including an available counselor, to minimize the negative impact.*
Microbiology surveillance: All CF centers should review their quarterly surveillance reports to assess the incidence and prevalence of respiratory tract pathogens at their centers.*
Molecular typing: Molecular typing should be performed using an appropriate genotyping method (e.g., pulsed-field gel electrophoresis, random-amplified polymorphic, DNA polymerase chain reaction (PCR), repetitive sequence-based PCR, or multi-locus sequence typing).*
Minimize time in common areas of CF clinic: CF clinics should schedule and manage people with CF to minimize time in common waiting areas by using staggered clinic schedules, placing people with CF immediately into an exam room, using a pager system to alert people with CF that an exam room is available, and keeping people with CF in their exam room while the care team rotates into the room.
Pulmonary function tests (PFTs): PFTs should be performed in one of the following ways:
Hospital room: People with CF should be placed in a single-patient room.*
Activities outside hospital room: Centers should evaluate people with CF case-by-case and in accordance with institutional IPC policies regarding the pursuit of activities outside the hospital room.*
Handheld disposable nebulizers: Handheld disposable nebulizers should be managed as follows:
Events and activities: Only one person with CF should attend any camp, educational retreat, or CF Foundation-sponsored indoor event. Family members without CF and individuals with CF who live together in the same household may attend these activities.*
Care of home nebulizers: Nebulizers used in the home should not be disinfected with acetic acid (vinegar), bleach solutions, or benzalkonium chloride (e.g., “Control III”).*
* Denotes those recommendations that were modified or new when compared to the 2003 guideline
Several areas had insufficient evidence to make a recommendation and additional research is needed.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Alex H. Gifford, M.D., FCCP (Dartmouth-Hitchcock Medical Center, Lebanon, N.H.), William Stoudemire, M.D. (University of North Carolina, Chapel Hill, N.C.), and Lisa Saiman, M.D., MPH (Columbia University Medical Center and New York-Presbyterian Medical Center, New York)
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