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 for colorectal cancer screening for adults with CF.
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.
Based on available data from the Cystic Fibrosis Foundation Patient Registry, the median age of onset of colorectal cancer (CRC) in patients with cystic fibrosis is roughly 20-30 years younger than in the general population.
Although colonoscopic data among people with CF remain limited, studies from the United States, Australia, and Canada have consistently shown increased prevalence of pre-cancerous adenomatous colon polyps (precursor growths that have potential to transform into cancer over time), including advanced neoplasia defined by size and/or histopathologic criteria. Furthermore, animal and human studies suggest that altered expression of the cystic fibrosis transmembrane conductance regulator (CFTR) gene increases colon cancer risk and may accelerate its progression.
In the general population, mortality from CRC is reduced by screening and early intervention. As most CRC arises from a well-defined adenoma-to-cancer sequence, it is possible to prevent cancer by identifying and removing pre-cancerous polyps during screening colonoscopies.
Given the increased prevalence of adenomatous colonic polyps at a younger age -- as well as the increasing evidence of CFTR mutations as a contributing genetic risk factor -- CF should be considered a genetic adenomatous polyposis and colon cancer syndrome. This distinction is critically important to the future care of aging people with CF.
Immunosuppression associated with organ transplantation (particularly following lung transplantation) has been associated with increased risk for CRC development in CF. Any screening recommendations would need to incorporate specific guidance for people with CF who have undergone solid organ transplantation.
Watch a video of Alexander Khoruts, M.D., to learn more about the CRC screening recommendations.
An expert task force composed of 18 members performed a comprehensive literature review, focusing on the following topics: cancer risk, organ transplantation, and procedure and preparation pertaining to CRC screening. The committee held multiple meetings to analyze the available data and synthesize recommendations.
An independent analysis of the benefits and harms of screening, as well as resource requirements, was performed by the Department of Public Health at Erasmus Medical Center, Netherlands. The recommendations also received public comments from the CF community of physicians and patients after being presented at the 2016 North American Cystic Fibrosis Conference.
The final recommendations were included only if they reached 80 percent consensus threshold among the task force members.
The CF Foundation recommends that all decisions on colorectal cancer screening and surveillance in individuals with cystic fibrosis be based on shared decisions between the provider and individual with CF about treatment, co-morbidities, safety, and quality of life.
The CF Foundation recommends that all colorectal cancer screening and surveillance for individuals with cystic fibrosis are jointly managed by CF health care professionals and an endoscopist.
The CF Foundation recommends colonoscopy as the screening examination for colorectal cancer in individuals with cystic fibrosis.
The CF Foundation concludes that the evidence is insufficient to recommend the use of CT colonography, stool-based tests, or flexible sigmoidoscopy in individuals with cystic fibrosis for the purpose of colorectal cancer screening.
The CF Foundation recommends that colorectal cancer screening begin at age 40 in individuals with cystic fibrosis with continued re-screening every five years.
The CF Foundation recommends that individuals with cystic fibrosis who are 30 years of age and older and have adequately recovered after receiving a solid organ transplant begin colorectal cancer screening within two years of transplant, except when they have had a negative colonoscopy within the past five years.
The CF Foundation recommends continued colorectal cancer re-screening every five years in individuals with cystic fibrosis who have received a solid organ transplant.
The CF Foundation recommends that individuals with cystic fibrosis who have undergone a solid organ transplant and had colonoscopy that had any adenomatous polyps have surveillance colonoscopy in three years, unless a shorter interval is indicated by individual findings, with subsequent intervals based on the most recent endoscopic examination.
The CF Foundation recommends that adults with cystic fibrosis undergoing a colonoscopy receive intensive regimens for bowel preparation to allow for optimal examination. The intensive regimen should include: 3-4 washes (minimum of 1-liter purgative per wash) with the last wash occurring within 4-6 hours prior to the exam.
Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.
James M. Abraham, M.D., (University of Minnesota) and Alexander Khoruts, M.D., (University of Minnesota)
The guidelines were published in February 2018, they were reviewed in April 2019 and it was determined that no update is needed at this time.
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