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.
When your life is already jam-packed with nebulizing medications, why would you add one more?
Wendy Bullington, Pharm.D.
March 17, 2016
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As the pharmacist at an adult cystic fibrosis center for the past 10 years, I often have heard the frustrations that patients have regarding the time it takes to do their inhaled therapies. I really can't imagine. I have asthma, requiring me to take one inhaler twice daily, and I struggle to remember to do that consistently (I know … health care providers are the worst). I have such profound respect for my patients -- for you.
What I have learned over the past 10 years is that the quick tips I pass from patient to patient seem to be the most helpful. They may include anything from the way a patient manages adverse effects to cost-saving measures. So please, don't be shy with your care team about what is working for you -- or what isn't!
One of the goals I have as part of the care team is to help patients understand why we may add an additional medication when they are already juggling their current therapies with their day-to-day lives. Many of my patients let hypertonic saline fall through the cracks. With the main side effect being coughing (which isn't necessarily a bad thing), and the additional time it takes to nebulize, many of my patients skip this therapy. Why is it so important, then?
Hypertonic saline has been shown to improve lung function, decrease exacerbations and improve quality of life. Adding hypertonic saline can be a great option for my patients who are already taking dornase alfa (Pulmozyme®) and an inhaled antibiotic, but need to step up therapy because of increased symptoms or decreased FEV1. It is also an option for patients who have trouble bringing up sputum. This is where the side effect of coughing can be an advantage (as long as it is not serious). But for some people, the benefit they see from hypertonic saline doesn't seem to be worth the pressure of working another medication into their routine or the side effects they experience. That's where your care team can help.
Many medications involve balancing their therapeutic effects with their side effects. That's why it's important to tell your care team when side effects get worse or decrease your quality of life. For example, if you find that you cough too much or have increased shortness of breath, you have a couple of options. You can use an albuterol inhaler first, or you can decrease your hypertonic saline from 7% to 3%. This lower concentration may ease the irritation.
One of the few times we recommend calling your doctor, and waiting to take hypertonic saline, is if you are coughing up blood. Hypertonic saline can irritate the airways and make the bleeding worse.
If cost is an issue, some specialty pharmacies provide hypertonic saline for free if you order dornase alfa and inhaled antibiotics from them. The Cystic Fibrosis Foundation's patient assistance program, Compass, can also be a great resource.
We don't mean for hypertonic saline to be a burden, but rather an addition to your current arsenal of therapies. If you have any questions about adverse effects or how to better fit hypertonic saline into your schedule, talk to you pharmacist or respiratory therapist about ideas.
Wendy Bullington, Pharm.D.
Registered Pharmacist, Medical University of South Carolina
Wendy is a registered pharmacist and an adjunct associate professor for the South Carolina College of Pharmacy. She has been a part of the CF team at the Medical University of South Carolina for more than nine years, providing both inpatient and outpatient care. Wendy was part of the Strategic Action planning team and the Medication Adherence Strategic Planning Committee at the Cystic Fibrosis Foundation. She is currently a champion for the Partnerships for Sustaining Daily Care committee. Wendy’s professional passions include improving the “therapy/life” balance for people with CF -- maximizing effectiveness while minimizing time on individuals’ day-to-day lives. She also has an interest in improving transitions of care from the hospital to home.
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This site contains general information about cystic fibrosis, as well as personal insight from the CF community. It 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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