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 people living with cystic fibrosis and their families face complicated issues related to getting the care they need. Cystic Fibrosis Foundation Compass makes sure that no one has to do it alone.
CF Foundation Compass is a service that helps people with CF and their families with navigating insurance options, connecting to legal information and experts, finding available financial resources, and tackling other life issues.
CF care team members are paramount in providing highly specialized care to people living with CF. CF Foundation Compass can help by serving as a strategic ally for care teams, so team members can focus on their patients’ care.
CF Foundation Compass can help you navigate insurance, financial, legal, and other issues you are facing. Use this online form to start your conversation with a Compass case manager today.
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.
These clinical care guidelines from the Cystic Fibrosis Foundation provide recommendations on how best to care for adults with CF.
Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic Fibrosis Adult Care: Consensus Conference Report. Chest. 2004 Jan; 125(1 Suppl):1S-39S.
Improved care has led to a steady increase in the number of adults with cystic fibrosis. The Cystic Fibrosis Foundation Patient Registry demonstrates that, in 2014, the number of adults with CF first exceeded the number of children with CF. With continued therapeutic advances, CF survival continues to improve, and the proportion of adults with CF increases.
Recognizing the unique needs of adults with CF, the CF Foundation convened a consensus conference in 1999 to address care standards for adults with CF. Their findings and recommendations were reported in a consensus conference report. Since that report, there have been many clinical advances and other CF clinical care guideline reports.
CF Foundation-sponsored benchmarking studies of high-performing adult and pediatric CF programs found that patient outcomes were more closely related to care systems, attitudes, practices, patient/family involvement, and improvement projects than to any specific care elements (Boyle MP et al. 2014).
In the Adult Care Consensus Conference Report, we summarize the following topics. CF Foundation guidelines that supersede this report are cited.
In June 1999, the CF Foundation convened a consensus conference to address care of adults with CF. The consensus conference report summarizes the findings of that meeting and incorporates additional data gathered from 1999-2002. All these recommendations were based on consensus opinions. Other CF Foundation and European Cystic Fibrosis Society (ECFS) guidelines are cited in the pertinent sections.
Multidisciplinary approach modeled on the pediatric system and provided by a team with training and experience in adult CF care.
Pulmonary Disease: Evaluation: Sputum Culture
Pulmonary Disease: Evaluation: Imaging
Pulmonary Disease: Management: Pulmonary Exacerbations
Pulmonary Disease: Management: Chronic Suppressive Antibiotic Therapy
Pulmonary Disease: Management: Airway Clearance and Exercise
Pulmonary Disease: Management: Mucolytic Agents
Pulmonary Disease: Management: Oxygen
Pulmonary Disease: Management of Complications
Criteria for Successful Transition
The CF center should support patients with career development through initiation of a dialogue about career planning and assisting the patients and their school counselors with regard to the implications of CF on careers and education.
Protection From Discrimination
Accommodations for Disability
Informing an Employer of a CF Diagnosis
Bone and Joint Disease
Advanced care planning should begin early in the course of a patient's illnesses.
Structured discussion and documentation should occur to ensure that a patient's wishes will be respected.
Patients should clarify their personal goals and the care they would like to receive in various situations as well as identify the person or persons whom they would like to make health care decisions on their behalf.
Palliative care may be beneficial for multidisciplinary management of symptoms that may cause suffering; efforts to maximize quality of life as defined by the patient and family; care provider education; treatment of psychological, social, and spiritual distress; and loss, grief, and bereavement support.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
Boyle MP, Sabadosa KA, Quinton HB, Marshall BC, Schechter MS. Key Findings of the US Cystic Fibrosis Foundation's clinical practice benchmarking project. BMJ Quality Safety. 2014 23:i15-i22. doi: 10.1136/bmjqs-2013-002369.
Farrell PM, White TB, Ren CL, et al. Diagnosis of Cystic Fibrosis: Consensus Guidelines from the Cystic Fibrosis Foundation. J of Pediatr. 2017 181:S4-S15.e1. doi: 10.1016/j.jpeds.2016.09.064.
Floto RA, Olivier KN, Saiman L, et al. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus recommendations for the management of nontuberculous mycobacteria in individuals with cystic fibrosis. Thorax. 2016 Jan; 71(1):88-90. doi: 10.1136/thoraxjnl-2015-207983.
Flume PA, Mogayzel PJ, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Resp Crit Care Med. 2009 Nov 1;180(9):802-8. doi: 10.1164/rccm.200812-1845PP. Epub 2009 Sep 3.
Mogayzel PJ, Naureckas ET, Robinson KA, et al. Cystic Fibrosis Pulmonary Guidelines: Chronic Medications for Maintenance of Lung Health. Am J Resp Crit Care Med. 2013 Apr 1; 187(7):680-9.
Flume PA, Robinson KA, O'Sullivan BP, et al. Cystic Fibrosis Pulmonary Guidelines: Airway Clearance Therapies. Respir Care. 2009 Apr; 54(4):522-37.
Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized trial. JAMA. 2003 290:1749-1756.
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.
Borowitz DS, Grant RJ, Durie PR. Use of pancreatic enzyme supplements for patients with cystic fibrosis in the context of fibrosis colonopathy. J Pediatr. 1995 Nov; 127:681-4.
Stallings VA, Stark LJ, Robinson KA. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008 May; 108(5):832-9. doi: 10.1016/j.jada.2008.02.020.
Schwarzenberg SJ, Hempstead SE, McDonald CM, et al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibros. 2016 Nov; 15(6):724-735. doi: 10.1016/j.jcf.2016.08.004. Epub 2016 Sep 3.
Moran A, Brunzell C, Cohen RC, et al. Clinical Care Guidelines for Cystic Fibrosis-Related Diabetes. Diabetes Care. 2010 Dec; 33(12):2697-708. doi: 10.2337/dc10-1768.
Sokol R, Durie PR. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. Recommendations for management of liver and biliary tract disease in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1999 28:S1-S13.
Hadjiliadis D, Khoruts A, Zauber AG, et al. Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations. Gastroenterology. 2018 Feb; 154(3):736-745.e14. doi: 10.1053/j.gastro.2017.12.012. Epub 2017 Dec 29.
Aris RM, Merkel PA, Bachrach LK, et al. Guide to Bone Health and Disease in Cystic Fibrosis. J Clin Endocrinol Metab. 2005 Mar; 90(3):1888-96. Epub 2004 Dec 21.
Yankaskas JR, Mallory GB. Lung Transplantation in Cystic Fibrosis: Consensus Conference Statement. Chest. 1998 Jan; 113(1):217-26.
Holly Keyt, M.D., (University of Texas at San Antonio) and James R. Yankaskas, M.D., (University of North Carolina at Chapel Hill)
The guidelines were published in January 2004, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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