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.
Reflecting the continued momentum we're making in the fight against cystic fibrosis, the latest Patient Registry data show steady gains in survival for people with CF. With this good news comes a lot of excitement, along with some questions.
Bruce C. Marshall, M.D.
November 16, 2017
Coming to Expect More From My Life
Finally Finding Someone Who Gets It
At this year's North American Cystic Fibrosis Conference (NACFC), Preston W. Campbell, III, M.D., president and CEO of the Cystic Fibrosis Foundation, shared
exciting news from the latest Patient Registry report. He announced that the median predicted age of survival for people
with CF had increased to 47 years. This was good news for our community, but it also raised questions, such as “What does this number mean and what caused the jump?”
Like many statistics, median predicted age of survival, or life expectancy at birth, is used to track progress in the health of people with CF -- as a population -- who are followed by the Foundation's Patient Registry. This and much more information
is shared with the CF community through the Patient Registry Annual Data Report.
CF clinicians and researchers also use the Registry data to design clinical trials, study CF treatments,
and inform quality improvement initiatives in specialized CF care.
What Does This Number Mean?
Based on the 2016 Registry data, the life expectancy for a baby born with CF in 2016 is 47 years of age. This means that half of all babies born in 2016 are expected to live to be 47 years old or older.
As a statistical prediction, this figure does not account for potential benefits of CFTR modulators or ongoing improvements in care. Most important, this number does not predict how long any individual will live because it does not take into consideration
individual characteristics, like mutations, disease severity, or response to treatments. If you have questions about your personal health, discuss them with your care team who can offer specific guidance and support.
What Caused the Jump?
CF is a rare disease and small changes in the population can noticeably impact survival calculations. Life expectancy at birth is a calculation that can fluctuate from year to year.
Although we don't know for sure why there was a relatively large increase in the predicted survival for those born in 2016, we did see some key changes that may explain this jump. The Registry data showed that there were fewer deaths reported in 2016
than in 2015. Also, there was a noticeable increase in the number of lung transplants and this may have contributed to the decrease in number of deaths.
Is This a Trend That Will Continue?
Changes observed over a single year (from 2015 to 2016) are not enough to know if this increase in predicted survival is the beginning of a trend for children born with CF in the future. However, when we analyze the data collected across a wider time
span, like over 5 years, the overall trend shows steady gains in survival for people with CF. The most recent 5-year time span shows that the life expectancy for people born between 2012 to 2016 is 43 years of age. We expect this trend to continue,
reflecting how advances in treatment and care continue to help all people with CF live longer, fuller lives.
Until All Can Say “I Used to Have CF.”
As a community of families, volunteers, researchers, care team clinicians, and people with CF, we should be proud of the progress that our collective efforts have brought to bear over so many years.
Yet, as we celebrate this moment, we also know that too many are still dying before they can finish school, begin careers or start families. Many living with the disease struggle with additional complications like CF-related diabetes, anxiety, and depression, and many still face frequent hospitalizations. The daily rigors of CF treatment, which can take 2 hours per day or more, weighs heavily on many people with CF and their families.
In this journey to end CF, the Registry data are encouraging, reliably showing us that we're moving in the right direction and with great momentum. Together, we must continue to push forward so that we can slow the progression of this disease while working
toward the search for a cure.
Bruce C. Marshall, M.D.
Senior Vice President of Clinical Affairs, Cystic Fibrosis Foundation
Bruce Marshall is senior vice president for Clinical Affairs at the Cystic Fibrosis Foundation. He joined the organization in 2002 and directs the clinically related activities of the Foundation, including the care center network, quality improvement, clinical practice guidelines, the Patient Registry, and educational resources. Dr. Marshall was a tenured faculty member at the University of Utah School of Medicine, where he served as the founding director of the Adult CF Program from 1989 to 2002. Dr. Marshall earned a bachelor of arts degree at Johns Hopkins University and his medical degree at the University of Maryland School of Medicine. He earned a master’s degree in medical management from Carnegie Mellon University.
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This site contains general information about cystic fibrosis, as well as personal insight from the CF community. Opinions and experiences shared by members of our community, including but not limited to people with CF and their families, belong solely to the blog post author and do not represent those of the Cystic Fibrosis Foundation, unless explicitly stated. In addition, the site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
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