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.
Antibiotics fight infections caused by bacteria. They should not be used to treat infections caused by viruses (such as the flu) or types of fungi, which include yeasts and molds.
The buildup of thick, sticky mucus in the lungs makes people with cystic fibrosis more likely to develop bacterial infections that can last for short periods of time (known as acute infections or exacerbations) or for many years.
The good news is that many of these infections can be treated. To keep them in check, antibiotics are taken by people with cystic fibrosis as part of regular daily treatment. For lung exacerbations, people with CF may receive intravenous (IV) antibiotics -- that is, directly into the veins -- in addition to the inhaled or oral antibiotics.
See how inhaled antibiotics work to help control bacteria in your lungs.
Inhaled antibiotics should be taken last, after bronchodilators (if you take them), mucus thinners, and airway clearance techniques, so your lungs will be as clear of mucus as possible. This allows the antibiotics to reach deep into your lungs to treat the bacteria that cause infection.
The tables below list bacteria common among people with CF, the antibiotics that are commonly prescribed to treat them, and how they are taken.
Learn more about P. aeruginosa.
Learn more about MRSA.
Learn more about NTM.
Learn more about B. cepacia.
It is particularly important to take antibiotics exactly as they've been prescribed by your doctor, even if you no longer feel the symptoms of an infection. Not doing so can allow the remaining bacteria in your system to become resistant to the antibiotic, which will make it more difficult to treat.
Antibiotic-resistant infections are a real concern among people with CF and over time, can lead to fewer treatment options. Your CF care team will watch for signs of resistance and help you understand how to prevent or decrease the risk of developing infections from resistant bacteria.
Talk to your care team if you have questions about how to take the antibiotic, including the sequence for taking the drug in relation to your other treatments, what you should expect and do if you experience side effects, and what to do if you miss a dose or cannot complete the full course.
Alcohol interferes with the effectiveness of many antibiotics. It is a good rule to avoid consuming alcohol while taking antibiotics. Antibiotics also can decrease the effectiveness of oral contraceptives in women.
Antibiotics typically begin working 48 to 72 hours after you begin taking them. It may take a day or so before you start to feel better. As the antibiotics work to break up the mucus, coughing may increase, which is why some people feel worse before they feel better.
You may experience side effects when you take any medication, including antibiotics. When discussing any new medications or changes in dosages for medications you are already taking, be sure to ask your care team about:
Let your care team know if you feel any side effect that bothers you or makes it hard for you to continue taking this medication as prescribed. Your care team can work with you to help you manage side effects or to adjust your treatment plan.
You can learn more about antibiotics at DailyMed, which is a service from the National Library of Medicine that provides information about drugs, including dosages and possible side effects. You can learn more about specific antibiotics by searching for the medication from the main DailyMed page.
For IV antibiotics given in the hospital, clinic, or hospital, staff work with the appropriate individuals to provide the therapy. For IV antibiotics that will be administered at home, clinic, or hospital, staff work with home care agencies to arrange delivery and therapy. Oral antibiotics are typically available at retail pharmacies and mail-order pharmacies. Inhaled antibiotics are available through specialty pharmacies that are contracted with specific insurance plans. Medications from specialty pharmacies often need to be handled and stored specially and delivered quickly.
Insurance plan coverage for some antibiotics can vary. For antibiotics that are specifically indicated for CF treatment, most insurance providers should provide coverage. For antibiotics that are not specifically indicated for CF treatment, insurance coverage may be more difficult to arrange. For example, although the antibiotic colistimethate (Colistin®) is often prescribed as an inhaled treatment for P. aeruginosa, it is considered an injectable antibiotic. Because it is not specifically indicated to be used in that way, coverage may be declined by insurers. Check with your insurance provider to ensure that the antibiotic is covered and confirm what your out-of-pocket expenses may be.
You can also contact the Cystic Fibrosis Foundation Compass, a free, personalized service that can help you with insurance, financial, legal, and other issues. Dedicated Compass case managers can assist in coordinating benefits or providing information about benefits offered under your plans. Contact Compass at:
Monday - Friday, 9 a.m. - 7 p.m. ETcompass@cff.org
Inhaled antibiotics are used to fight or control bacteria that cause lung infections. Your CF care team will use the results of a sputum culture to see if you need to take an inhaled antibiotic. Inhaled antibiotics go right to where they are needed -- deep into the small airways in your lungs.
The following antibiotics are used to improve respiratory symptoms in people with CF who have Pseudomonas aeruginosa. The antibiotics especially made for people to inhale are:
Typically, patients do only one of these products every other month for 28 days. However, there are some people who alternate between taking aztreonam and tobramycin. For example, people take aztreonam for 28 days and then switch to tobramycin for another 28 days.
Aztreonam is approved for people ages 7 and older who have P. aeruginosa, but it has been studied in younger children. People taking aztreonam should take one single-dose vial three times per day with a recommended nebulizer.
Tobramycin inhalation solution and tobramycin inhalation powder are approved for people ages 6 and older who have P. aeruginosa, but the inhalation solution has been studied in younger children.
Aztreonam and tobramycin inhalation solution are administered with a nebulizer. There are several different types of nebulizer and compressor devices.
Your CF care team can show you how to use these devices correctly. With most types of nebulizers, the drug is put in a clean nebulizer cup and attached to a small air compressor. The compressor blows air through the nebulizer cup, which creates a mist that you inhale through a mouthpiece.
There are several different types of nebulizers and compressor devices, depending on which medication is being administered. Tobramycin inhalation powder uses a portable handheld device. Certain aerosol medications require you to use specific devices. Use only recommended nebulizers.
To take aztreonam inhalation solution:
Watch how to clean the Altera nebulizer.
Treatment should take about 15 minutes to complete. You should take the medication as close to 12 hours apart as possible. When you are finished, wash the nebulizer.
The inhalation powder form of tobramycin (TOBI® Podhaler™) is typically taken as four capsules, twice each day.
To take tobramycin inhalation powder:
Treatment should take between two and seven minutes to complete.
You should take the medication as close to 12 hours apart as possible and not less than six hours apart.
For complete instructions on how to take tobramycin inhalation powder, visit DailyMed, a service from the U.S. National Library of Medicine that provides FDA label information on marketed drugs.
Oral antibiotics are liquids, tablets, or capsules that you swallow. Oral antibiotics can be used to treat chronic bacterial infections or mild exacerbations.
Different oral antibiotics can have different effects and requirements. For example, certain antibiotics are taken once each day for several days. Others can be prescribed for longer periods of time. Some need to be taken with food, others on an empty stomach. Your CF care team members will prescribe the antibiotic they think will be most effective for your infection. They will provide instructions on how to take the antibiotic and tell you about possible side effects.
Intravenous (IV) antibiotics are liquid solutions with antibiotics that are delivered directly into your blood through a small tube called an IV catheter. They can be used to treat infections that are caused by bacteria that have not responded to oral antibiotics or infections that have progressed too far to be successfully treated with oral antibiotics.
IV antibiotics are often necessary to treat CF pulmonary exacerbations. People with pulmonary exacerbations who need IV antibiotics are often admitted to the hospital to start treatment so that the antibiotic dosage and potential side effects can be carefully monitored. Sometimes, if they are able to get the extra airway clearance and nutrition at home, they can complete the IV antibiotics at home.
IV antibiotics are typically administered by medical professionals when you are in the hospital. They can be delivered by placing an IV catheter in your arm. For home use, you may have a health care professional come to your home to teach you how to administer them.
The CF Foundation supports ongoing infection research, including research regarding inflammation.
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 dailymed.nlm.nih.gov/dailymed.
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