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.
Lung Transplantation Surgery and Recovery
Surgery and recovery involves more than replacing your lungs. The process also includes making the physical and emotional adjustment to life with your new lungs.
When you arrive at the hospital for your transplant, you will have blood tests, a test to examine your heart called an electrocardiogram (EKG), and a chest X-ray.
Members of the surgical team will examine you and ask you questions. The
anesthesiologist will start an intravenous (IV) line in your arm or your hand. Additional IV lines will be placed in your neck, wrist, collarbone, or groin areas to monitor your heart and blood pressure and to take blood samples.
Piper Beatty Welsh was surrounded by family prior to her second double-lung transplant. She describes the transplantation process as, “life-saving, life-changing, and 100 percent life-affirming.”
You will be under general anesthesia for the surgery, so you will be unaware and won't feel any pain.
While you are under anesthesia, you will not be able to breathe on your own so a tube will be inserted through your mouth into your lungs. The tube will be attached to a machine called a ventilator, which will breathe for you during the surgery.
To prevent infection, the surgical team will thoroughly clean the areas of your skin they are preparing to cut and make an incision. Doctors will remove your unhealthy lungs (one at a time) and replace them with the donor lungs. The airways and blood vessels of the new lungs will be attached to your airways and blood vessels, one lung at a time.
Doctors will then close the incision with stitches or staples. A sterile bandage will be placed over the incision. One or more drainage tubes will be placed in your chest to let excess air, fluid, and blood escape from the surgical site and to allow the lungs to expand completely.
Finally, an additional IV tube may be placed in your back to deliver pain medication to you.
After the surgery, you will either be taken to a recovery room and then to the Intensive Care Unit (ICU) or directly to the ICU. Once you are in the ICU, you will be attached to machines that will measure your vital signs. The nurses and doctors will monitor you closely to make sure you are recovering safely.
You should expect some pain after surgery. To make you more comfortable, you will receive pain medicine through an IV. Your transplant team will want you to be comfortable enough to cough, sleep, and participate in exercise.
You will not be able to breathe on your own immediately. Until you are strong enough to do so, you will have a tube in your throat so that a ventilator can help you breathe. It may be removed after a few hours or not until a number of days after surgery.
Once the doctors feel you are strong enough, the ventilator will be removed. You may then be able to start drinking liquids. If you are able to tolerate liquids, you will slowly transition to eating solid foods.
Nurses, physical therapists, and respiratory therapists will help you with your physical therapy and breathing exercises to prepare you for moving out of the ICU. When your doctors think you are ready, you will move from the ICU to a room in a nursing or transplant unit. You will gradually be able to increase your activity by getting out of bed, sitting up in a chair, and taking walks around the unit.
Your IV tubes will remain in place throughout your recovery. You will receive immunosuppressive drugs through these tubes as soon as your surgery is complete. Your doctors will closely monitor the levels of these drugs in your blood to be sure that you're receiving exactly the right amount, as having either too much or too little could be harmful to you. As you recover, you may be given other IV medicines to support your blood pressure, heart, kidneys, and liver.
You will stay in the hospital until your doctors are confident that it is safe for you to leave. This is not always a smooth process. Having a lung transplant is traumatic for your body, and you will be particularly vulnerable to complications during this time.
Before you are discharged, the transplant team will work with you to ensure that you are prepared to manage your recovery at home.
Two adults with cystic fibrosis describe what their lives are like after transplant and address some of the challenges they faced during the recovery process.
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