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.
Nontuberculous mycobacteria are a group of bacteria that live in soil, swamps, and water sources.
There are more than 100 types of nontuberculous mycobacteria (NTM), and more are being found every year. NTM are very hardy and can survive many disinfectants and severe environmental conditions. Mycobacterium abscessus (M. abscessus) and Mycobacterium avium (M. avium Complex or MAC) are the most common types of NTM found in people with cystic fibrosis.
The group of bacteria that make up the different types of NTM are “cousins” to the bacteria that cause tuberculosis (TB). These were often called “atypical mycobacteria” in the past. Despite this relationship, NTM are distinct and cause a different lung disease than TB.
NTM have been found in the sputum of a growing number of people with CF since the 1990s. NTM can cause infections in many organs of the body, but for people with CF, the infection is nearly always in the lungs and can cause NTM lung disease. In 2017 in the US, approximately 13 percent of people with CF had a positive culture for a NTM species. From 2010 to 2016, 20 percent of people with CF grew NTM at least once.
NTM can enter the lungs directly through exposure to the environment. Because the organisms live in so many different environments, it is difficult to avoid all possible environmental sources of infection. Consider taking steps to limit breathing in water, soil, or dust. It is unclear if NTM can be passed from one person to another, so consider taking precautions to minimize the risk of cross-infection.
In North America, nearly 75 percent of NTM are MAC and 25 percent are M. abscesses. In general, MAC is seen in older people who have more mild disease and M. abscessus is associated with more advanced disease and a more rapid decline in lung function.
Watch the webcast below featuring Jerry Nick, M.D., to learn more about:
NTM lung disease can be challenging to diagnose. NTM is diagnosed by analyzing special cultures of your sputum for the presence of NTM. NTM infections can be detected only if these special cultures are ordered by your doctor. Clinical guidelines recommend yearly screening for NTM.
Sputum samples can be taken from what you cough up, from sputum-induction (with saline), or from bronchoscopy (when a fiber-optic tube is passed into your lungs to get samples). To help make sure the test is accurate, the sample must be taken from the lungs and not from a throat swab.
An important thing to remember is that even if NTM is found in your sputum culture, it does not mean that you have NTM lung disease. Typically, after a positive culture for NTM, your doctor will confirm it with one or more additional cultures. If cultures for NTM are repeatedly positive, your doctor may get a high-resolution chest computed tomography (CT) scan to look for clues of NTM disease and find out its extent. Unfortunately, even with a CT scan, it can be difficult to distinguish NTM lung disease from the normal findings seen in CF lung disease in people who do not have NTM lung disease.
Even if NTM is found in your sputum culture, it does not mean that you have NTM lung disease.
NTM can take up to eight weeks to grow, so you may have to wait some time until you receive your results. This slow growth makes NTM much harder to diagnose and treat.
Like typical CF infections from Pseudomonas aeruginosa and Staphylococcus aureus, NTM can chronically infect (“colonize”) the airways. Most people with a single positive NTM culture will not require treatment. The decision to treat NTM depends on other positive cultures, symptoms that suggest lung disease, such as weight loss, decreased energy, chronic fevers, night sweats, and trends in your lung function.
Because infections with typical CF bacteria can have similar features and are so common, your CF care team may treat these bacteria aggressively to see if your symptoms and lung function improve. If they do not, it may suggest NTM lung disease. Although NTM is naturally resistant to many antibiotics, treatments are available and research is underway to find more.
Once you and your CF care team decide that you should be treated for NTM lung disease, you will likely be started on at least three antibiotics at the same time. You may need to take these antibiotics for 12 months or longer. Because you would be taking so many medications for such a long period of time, it is important to review any possible interactions with your other medications with all your medical providers and pharmacist.
The goal is to decrease the amount of infection in your lungs. By doing this, your care team will try to improve your symptoms and decrease the amount of damage to your lungs. It may also mean your sputum will no longer grow NTM, although this does not happen in all individuals.
Treatment is typically divided into two phases: an intensive first phase followed by a continuation phase. During the first phase, you will usually take oral antibiotics, as well as an intravenous (IV) antibiotic. During the continuation phase, you may take one or more oral antibiotics, and possibly an inhaled antibiotic.
The exact combination of antibiotics that your doctor prescribes will depend on the type of NTM that is in your lungs. Tests will determine which antibiotics are the best at treating your particular infection. More tests are done to watch for side effects or allergies that you might develop during months of treatment.
You will be asked to provide blood samples routinely to monitor levels of the drugs in your body. You may also need to have your hearing and vision tested regularly. This is to check for signs of toxicity caused by some antibiotics.
Your doctor may frequently change your medications during NTM treatments if you experience side effects or allergies that begin to interfere with your quality of life or are potentially harmful. More information about medications used to treat NTM lung disease and their side effects can be found at ntminfo.org.
In addition to the NTM medications, it is very important to continue your standard CF medications and airway clearance. Often, people with CF and NTM need to do more airway clearance than usual.
Since 2010, the Foundation has carefully tracked NTM in its Patient Registry at the species level and is working with researchers to identify risk factors that make people with CF more likely to get NTM. Learn more about research on NTM. Talk to your care team about new research and possibilities of participating in clinical trials related to NTM and CF.
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