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.
Highlighted is recently published work from the CF Foundation’s Care Center Network and our colleagues from outside of the United States.
This column highlights recently published work from the CF Foundation's Care Center Network and our colleagues from outside of the United States. Data from the Foundation's Patient Registry was also used in some of this work. We congratulate the authors of these manuscripts and sincerely thank the many contributors who made this work possible.
Knapp EA, et al.
Annals of the American Thoracic Society, published 2016; 13(7):1173-9.
This manuscript describes the history of the CF Foundation Patient Registry from its inception in the 1960s to the present as well as methods for collection, security and processing of data. To assess generalizability of the data, two methods were used to estimate the number of people with CF in the United States (approximately 33,800) and, thus, 81 percent to 84 percent of the population is captured in the registry. An additional 6 percent of people with CF cared for at a CF Foundation-accredited center do not participate in the registry. Audits of the registry data showed that 95 percent of outpatient clinic visits and 90 percent of hospitalizations are entered into the registry. Data in the registry matched that of the medical record 82.6 percent to 99.9 percent depending on the specific data element. Of patients entered in the registry in 2009, only 9.4 percent could not be accounted for by data entries in 2013 or recorded deaths. Read more here.
Of note, the journal editor commented: “The article this month by Knapp and colleagues features one of the most fully developed disease registries in all of medicine. The current publication offers a benchmark and roadmap for the development of other observational patient registries.”
All of you should be proud of your contributions to the success of the registry. Thanks for all that you do!
Stanojevic S, et al.
Thorax, published online, August 2016.
Data from the Toronto CF Database were used for this retrospective cohort study of patients receiving care at the Toronto CF Center from 2009 to 2014. The investigators systematically measured the effects of pulmonary exacerbations treated with oral antibiotics on short-term and long-term clinical outcomes. Overall, pulmonary symptoms were treated with oral antibiotics four times more commonly than with intravenous antibiotics. They found that a significant proportion of patients treated orally did not recover baseline lung function. They also showed a cumulative effect of orally treated exacerbations on rate of decline in FEV1 percent predicted. In summary, the investigators demonstrated that these “mild” exacerbations treated with oral antibiotics are important events that deserve further attention from researchers and clinicians. Read more here.
Flume PA, et al.
Journal of Cystic Fibrosis, published online May 24, 2016.
This randomized, double-blind, placebo-controlled trial conducted at 45 CF care centers in the United States (from Dec. 2012 to Jan. 2015) compared three cycles of 28 days of inhaled aztreonam or placebo three times daily alternating with 28-day open-label tobramycin inhalation solution. Enrollment was limited for various reasons (competing studies, adoption of continuous alternating therapy in clinical practice, etc.) resulting in randomization of only 90 participants, well short of the planned enrollment of 250 participants. Thus, despite clinically significant reductions in exacerbation rates (25.7 percent) and rates of respiratory-related hospitalizations (35.8 percent) in the group on alternating inhaled antibiotics, statistical significance was not reached. Unfortunately, this is a lost opportunity to rigorously assess the efficacy of continuous alternating therapy, a practice that has been widely adopted in clinical practice. Read more here.
Munck A, et al.
Journal of Cystic Fibrosis, published 2016; 15(4):531-9.
This prospective observational study performed in 10 countries focused on the incidence, associated factors and treatment modalities for distal intestinal obstruction syndrome (DIOS). The incidence of DIOS was similar in children and adults with CF. Medical treatment failed only in those with complete DIOS. There was a strong association between DIOS and history of meconium ileus, pancreatic insufficiency, CF-related liver disease, CF-related diabetes and chronic pseudomonal infection in adults. A delay between onset of symptoms and seeking medical care was associated with a longer time to resolution of the problem. This paper is an important addition to our knowledge about this relatively common complication of CF. Read more here.
Buu MC, et al.
Chest, published 2016; 149(2):380-9.
This retrospective analysis of CF Foundation Patient Registry data compared Hispanic to non-Hispanic California residents diagnosed with CF from 1991 to 2010. Hispanics had a lower survival rate 18 years after diagnosis, compared to non-Hispanics (75.9 percent vs. 91.5 percent; p <0.0001). Hispanics had an increased risk of death, adjusted for socioeconomic status and clinical risk factors, compared to non-Hispanics (hazard ratio 2.81; 95% confidence interval 1.70-4.63). There was no difference in access to CF center care, age at diagnosis or nutritional status; however, Hispanics had a lower FEV1 percent predicted at age 6 compared to non-Hispanics (77 percent predicted vs. 89 percent predicted; p <0.0001). This study shows that the relationship of ethnicity and mortality is complex and likely multifactorial. A better understanding of the root causes of the survival difference between Hispanics and non-Hispanics will hopefully point the way in closing the gap. Read more here.
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