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.
Aspergillus species is a fungus that often lives in the airways of children and adults with cystic fibrosis. When people develop an allergic reaction to Aspergillus, it is called allergic bronchopulmonary aspergillosis.
Aspergillus is very common in our environment. Most people breathe in Aspergillus spores every day without getting sick. However, people with cystic fibrosis or other diseases that weaken their immune systems, are at a higher risk of developing health problems because of Aspergillus. Some types of allergic bronchopulmonary aspergillosis (ABPA) are mild and others are very serious.
ABPA affects about 2 to 19 percent of the people with CF. It causes inflammation in the lungs and can cause further scarring and fibrosis if untreated.
ABPA is more common in people older than 6 years. People with CF with the following characteristics are more likely to be diagnosed with ABPA compared to those who are not:
Although the symptoms of ABPA are similar to CF symptoms, if you have ABPA, your health often gets worse without a clear reason or you may not respond to antibiotics that are used to treat typical CF exacerbations. Wheezing is more common in people with CF who have ABPA, but because the symptoms of ABPA and CF overlap, symptoms alone cannot be used to make the diagnosis.
Diagnosing ABPA in people with CF is difficult. It may often take longer because many of the criteria used to diagnose ABPA are the same as the usual symptoms of CF. Below is a list of the criteria:
It is important to mention that many people with CF have a sputum culture with Aspergillus. This does not mean you have ABPA. Your body's (immune system) response to Aspergillus and your symptoms will determine if you have ABPA.
Gina Hong, MD, MPH, at the University of Pennsylvania Medical Center, answers questions about diagnosing and treating ABPA and whether there is a connection between Aspergillus and hemoptysis.
Treatment for ABPA involves prevention and treatment of acute episodes, or flare-ups. Treatment of ABPA has two parts. Inflammation in the lung is treated with corticosteroids. Fungal colonization is treated with antifungal medicines.
Although there are no studies for the use of anti-fungals in people with CF with ABPA flare-ups, reducing the fungal burden in the respiratory tract may reduce long-term risk of disease progression. Further research is needed.
Oral corticosteroid pills or liquid, such as prednisolone (brand names include Pediapred® and Prelone®), prednisone tablets, and methylprednisolone (brand names include Medrol®) tablets, have anti-inflammatory effects. The course of medicine is much longer than the short courses used for asthma. An individual with ABPA may use steroids for many weeks and be slowly weaned off of them while symptoms and lung function studies are checked. Once all the symptoms have gone away, the steroids are often stopped. Although inhaled steroids are not used to treat ABPA flare-ups, they are often used between flare-ups to help control symptoms.
Side effects of oral and liquid steroids are related to the amount and length of time a person takes them. Side effects of taking corticosteroids may include:
Antifungal medicines like itraconazole (brand names include Sporanox®) reduce the amount of fungus in the lungs. This may lower the chance of the disease getting worse over time. Itraconazole is used one to two times a day for about three to six months, or until all symptoms have gone away. It is better absorbed when taken on an empty stomach. If you are on an acid-reducing medicine (brand names Prilosec, Nexium®, Protonix®, Pepcid®, Zantac®), you should take itraconazole with eight ounces of an acidic beverage, such as cola or orange juice to make sure you are able to absorb all of the medicine.
Side effects of taking itraconazole may include:
Because steroids can have significant side effects and ABPA can be difficult to treat, CF providers may offer a therapy that targets one particular part of the immune system (so-called “biologic” therapies). One such therapy, omalizumab (Xolair®), has been used to target IgE, which is often very elevated in patients with ABPA. This therapy has not been formally studied in people with CF with ABPA.
Watch the webcast below featuring Jeff Wagener, M.D., to learn more about:
Reference to any specific product, process or service does not necessarily constitute or imply its endorsement, recommendation or favoring by the Cystic Fibrosis Foundation. The appearance of external hyperlinks does not constitute endorsement by the Cystic Fibrosis Foundation of the linked websites, or information, products or services contained therein.
Information contained on this site does not cover all possible uses, actions, precautions, side effects or interactions. This site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
FDA-approved drug information is available at www.dailymed.nlm.nih.gov/dailymed.
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