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.
Quarterly visits to a CF Foundation-accredited care center are the foundation of your treatment plan. By partnering with your care team, you can also adjust your treatment plan as new therapies become available, or your needs change over time.
When you're seen at a CF Foundation-accredited care center, your care team will recommend treatment based on CF
clinical care guidelines. These guidelines are based on the latest medical evidence and best practices that make up specialized CF care so that you can receive the safest and most effective treatments.
CF Foundation clinical care guidelines recommend that people ages 6 and older visit their care center at least four times, receive four sputum cultures and perform at least two pulmonary function tests (PFTs) per year. There are other annual guidelines
for CF care, some of which are listed below.
At regular care center visits, your care team will monitor your health and work with you to develop and revise your treatment plan. Tests taken during your visit can help your team detect small changes in your health before you begin to feel badly and
prevent small problems from becoming big ones.
The team may want to schedule shorter follow-up visits to closely monitor any emerging conditions or help you prepare for upcoming travel or transitions in your life, such as going away to college or starting a new job.
“Like many others with CF, I've recently had to adapt to telehealth appointments.” -- Lydia Sand, an adult with CF, from the CF Community Blog
Care teams are made up of a multidisciplinary group of health
care professionals. These professionals are trained to work together to provide specialized care. A typical visit to your CF care center may include:
Your nurse will measure your temperature, blood pressure, height and weight. Measuring your height and weight is particularly important for people with CF because it helps your care team calculate your body mass index (BMI) and determine your body composition.
and BMI percentile help your care team compare growth, nutrition and digestive
function in people with CF to people without CF. If your BMI has gone up or down, it will help your care team understand how well your treatment plan is working.
Your nurse may also collect samples of your blood to check for things such as:
Your blood samples will be sent to a laboratory to be tested and later analyzed by your CF care team. These results will help your team determine if your treatment plan should change or if you should come in for a follow-up visit. A member of your care
team might share the results with you by phone or during a later visit. If your results are normal, you may not hear from your care team at all. Regardless, if you have any questions about when your test results will come in, what your test results
are or what they mean, you can always contact your care team.
Your respiratory therapist will perform your pulmonary function test (PFT) using a machine that you blow into called a spirometer. An important result of your PFT is the FEV1 (forced expiratory volume). This measures how much air you can forcefully
breathe out of your lungs in one second. This is a way to measure your lung function. FEV1 is a good measure of how much mucus is blocking your large airways.
Another important measure during PFTs, especially for children, is called the FEF 25/75% (forced expiratory flow). The FEF 25/75% is a good measure of how clear or blocked your smaller airways are. The FEF 25/75% measures the rate of airflow when you
have exhaled between 25 to 75 percent of the air in your lungs.
Additional measures include how much air your lungs move in and out, how fast they move it, and how well they exchange gas
(move oxygen into the blood and carbon dioxide out of the blood).
The results of your PFT during one care center visit can capture how well your lungs work at that specific point in time. But comparing that specific result to results from previous visits can help your care team see changes in your lungs over longer
periods of time. Your respiratory therapist will review your PFT results with you to help you understand what they mean.
You will likely be asked or induced to cough during your PFTs. You will be asked to spit the mucus you cough up, also known as your sputum, into a container. Your respiratory therapist will send this sputum sample to the lab for testing. The results of
the test, or throat culture, will show what
kinds of germs, such as types of bacteria, are growing in your lungs. The detection of specific strains of bacteria will determine
which antibiotics to use. The results are typically available in a week to 10 days.
During your visit, your dietitian will review your diet and BMI and discuss any challenges you are facing, such as constipation or acid reflux. Your dietitian is trained to teach you how to adjust your diet so
that you can get the calories and nutrients you need to meet your nutrition
goals, which will help your lung function. He or she may also discuss alternative ways to get additional calories such as adjusting vitamins or digestive enzymes, adding dietary supplements or tube feeding.
Maintaining emotional wellbeing is an important part of managing a chronic disease like CF. This is why your care team may talk to you about how you're feeling and offer to screen you for conditions such as depression and anxiety. These conditions, if left untreated can interfere with your ability to effectively manage your disease and ultimately, the way you want to live your life.
In addition to addressing your medical needs, you also may find help paying for your care or treatments. Your care team may include or refer you to professionals, such as a social worker or reimbursement specialist, who can work with your insurance provider
or a patient
assistance program to help you access affordable care or treatment options.
You also should let your care team know about changes that might affect your ability to get or pay for the health care or medications you need. Job or employment status changes can affect your health insurance
coverage, which can affect where you go to receive your care or which pharmacy you can use to order your medications. If you receive Medicaid, a change in your income could
also affect your eligibility.
Cystic Fibrosis Foundation Compass is a personalized service that can help you -- and your care
team -- with insurance, financial, legal and other issues. Contact Compass at:844-COMPASS (844-266-7277)Monday – Friday, 9 a.m. – 7 p.m. ET
Your care team will review your treatment plan with you during your care center visit. This is your opportunity to discuss what is and what is not working.
Although your test results are important, it is important for you to be honest and discuss how you think your treatment plan is going, any difficulties you're having fitting in your daily treatments and any changes you'd like to make.
Your care team may be experts in providing specialized CF care, but you are the expert on living with your CF. This includes:
You can tell your care team how you feel compared to what your normal is. Your test results may not indicate an issue, such as the beginning of an infection or potential exacerbation (a worsening of symptoms), but changes you sense in your body may be
important signals. If you notice that you are coughing more or feel less energetic than usual, be sure to talk to your care team about it.
These changes can include short-term activities like a camping
trip or international travel or more significant events in your life like, starting a new relationship, planning for a family, moving away for college, starting a new job
or leaving the work force entirely.
By discussing changes in how you feel, physically or emotionally, and changes in your schedule or lifestyle, your care team may recommend adjusting your treatment plan. Ways that your treatment plan could change include trying different medications or
adjusting the dose or the way you take an existing medication; trying another type of airway
clearance technique; and changing your diet or your fitness
routine. These changes may be temporary or last until your health or lifestyle needs change again.
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