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.
Many people with cystic fibrosis experience hemoptysis. Although that can be serious and is often frightening, most often it's not as serious as you might think. Let me set the record straight.
David Orenstein, M.D., M.A.
July 28, 2017
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Hemoptysis, or coughing up blood, is one of the scariest complications of cystic fibrosis, although it doesn't have to be. “Hemoptysis” is also the name of a heavy metal band, and that is truly scary! Hemoptysis, the medical condition, is relatively common in adults with CF and rare in young children.
As many as 50 percent of adults with CF will occasionally see some streaks of blood in the mucus they spit out, and a much smaller percentage of patients (3-5 percent of those older than 15 years) will cough considerably more blood up. Although hemoptysis can be fatal, it rarely is, even among people who cough up a lot of blood, which is defined as more than one cup (8 ounces) in about 20 minutes.
Most often, hemoptysis is evidence of increased infection.
Like most infections, infections that cause hemoptysis are nearly always treatable. The increased infection in one small area of the lung irritates a small blood vessel, causing it to bleed. The difference between coughing more frequently and spitting out blood is that it can be very frightening to see the blood. To put this in context, even though it might look like your whole lung is bleeding, it's not. If you were to spread out someone's lungs (Don't do it! This is just a teaching point.), they would just about cover the surface of a tennis court. Well, if someone has hemoptysis, the area that's bleeding is likely to be the size of a little pebble on that tennis court.
Treating minor hemoptysis (streaks of blood in mucus) is just like treating any lung infection: increase or change antibiotics (oral, inhaled, or perhaps intravenous [IV]) and improve airway clearance. Some doctors suggest taking extra vitamin K, as that can help blood clot.
If you're bringing up a lot (more than one cup) of pure blood, you need to let your CF care team know, and you may need to be hospitalized. You will definitely need antibiotics, likely by IV. Depending on how much blood you have lost, you might even need a blood transfusion.
CF experts disagree on the role of airway clearance for someone who's bringing up large amounts of blood. Without question, gentle airway clearance is good (Clear those airways! Get rid of blood, which is a hospitable environment for germs! Open up areas of the lung for breathing!). But more vigorous methods, such as the vest, chest physical therapy, and positive expiratory pressure, may or may not be useful.
If brisk bleeding continues, some CF experts recommend pulmonary artery embolization, a specialized procedure where a small catheter is threaded through blood vessels to the lung, and a small plug is injected to block off the bleeding vessel. This often works, but the result may be temporary, and like so many medical procedures, this one carries some risk. Your CF care team will recommend treatment that is appropriate for you.
People with CF have asked if there are factors or medicines that make hemoptysis more likely. The simple answer is no, but each person is different. Individuals and their lungs don't always follow what the textbooks say. So, if you notice that whenever you do “x,” or take medicine “y,” you seem to have more hemoptysis, let your care team know, and they'll likely say, “Well, let's avoid x or y.”
Not all people with CF experience hemoptysis. For those who do, it's likely the result of a lung infection that has gotten worse. If it happens to you, let your CF care team know. They will almost always be able to treat this sometimes scary problem successfully.
David Orenstein, M.D., M.A.
Cystic Fibrosis Center Director Emeritus
David Orenstein, M.D., M.A., is the Antonio J. and Janet Palumbo Professor of Cystic Fibrosis, and CF Center Director Emeritus at Children’s Hospital of Pittsburgh. A CF doctor since 1975, he has published more than 200 articles and chapters. Of his three CF-related books, Cystic Fibrosis: A Guide for Patient and Family is in its 4th edition, and is used in many CF centers. When David is not working with patients or doing CF research, he spends time with his wife and son, swimming, reading, running, or knitting, though usually not at the same time.
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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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