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.
As a medical professional who has cystic fibrosis, I have experienced both sides of pain management and chafed under the new rules for prescribing pain medications brought about by a nationwide opioid crisis. Despite having to add yet another doctor to my long list of providers, a trip to a pain management specialist wasn't as bad as I thought.
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I had managed pain before in my personal and professional life, yet there I was sitting on the couch in tears, while my daughter watched cartoons beside me. The ribs on my right side hurt if I moved or took a deep breath -- and, God help me, if I coughed.
I had considered going to the ER. Had I broken a rib? It was two weeks until my next pulmonology appointment, and I had been out of prescription pain medication for at least that long. On top of that, I had been sick, which meant that I had been coughing
more. My rotation of Tylenol® and Advil® with some Valium® (as a muscle relaxer) every few hours got me to the point where I was comfortable enough to sit on the couch … in tears ... as long as nobody touched me.
As a nurse practitioner in a hospital, I frequently encounter and treat pain of all kinds. I used to enjoy pain management. I was able to deliver relief to my patients, using both narcotic and non-narcotic methods.
That was a different time. In the midst of a very serious opioid epidemic, the rules have become stricter with regard to prescribing pain medications. Addiction and drug misuse are very real problems. Now, patients with chronic pain may be required to
sign pain contracts and may be subject to periodic drug screening. There also are limits on dosage and quantity prescribed. We live in a time of mandatory specialist consults for certain patients who require more than the designated threshold of narcotics.
Clinicians must balance all of this without forgetting a patient's quality of life.
Sometime around 2014, I began regularly having pain to the ribs on my right side. I remember one day walking down the hallway holding my side after performing CPR on a patient.
It was around that time that I began taking prescription tramadol (Ultram®). For a few years, I was taking it up to four times per day, rotating with Tylenol and Advil, continuing my almost daily workout routine, going to school, working, and
being a father.
One day, I requested a refill and, instead of my usual quantity, I was given only enough for seven days. My pulmonologist's office messaged me and said because of new rules, this was the best they could do. I would have to request another refill when
the time came.
The time came around 10 days later. After a few refill requests, I received a message from the doctor's office. They wanted me to see a pain management specialist.
As a prescriber, I understood. However, the rules do make exceptions for palliative care, chronic pain, cancer pain, etc. For all intents and purposes, I could fall into
two of those categories -- chronic pain and palliative care.
As a patient, I was not thrilled about seeing a pain management specialist. If my use of pain medication had changed, or I needed something beyond Ultram, I would have been more accepting of this sudden referral; however, there was no discussion beforehand.
This meant adding ANOTHER doctor to my long list of providers -- and one who could change the way ANYONE is allowed to prescribe me any controlled substance.
The referral without any context from a doctor that I had a long relationship with just didn't seem right. It became clear at my pulmonology appointment that my suspicions were correct. My doctor had been out of town, and the providers that were covering
for her either were not aware of -- or were not comfortable prescribing -- my usual quantity of pain medication. They had not even given me enough medication to get me to my pain management specialist appointment, which was a month away.
In the meantime, my lung function decreased. My weight dropped between 5 and 10 pounds. I had greatly decreased the intensity and durations of my workouts, although I still tried to do a little bit. My quality of life had substantially declined. I was
in such severe pain that I could not sleep. I could barely make it through the workday, collapsing when I got home. I started snipping at my daughter because her snuggles had become too painful.
My pulmonologist promptly addressed all of this. She prescribed enough Ultram to get me to my pain management appointment. However, she also told me that it was not a bad idea to get a pain management specialist on board, as this is what she had been
doing with many of her CF patients with chronic pain.
Ultimately, the specialist was very laid back. He recommended an injection to the painful area between my ribs to decrease inflammation and desensitize a nerve that runs under each rib. Otherwise, if my pulmonologist was willing to prescribe Ultram -- which she was -- he said he would be hands off.
I appreciated that I was still in control of managing my pain, only now I had more options.
Most cystic fibrosis patients will have pain at some point, whether it is musculoskeletal pain, abdominal pain, or even arthritic pain. Some portion of these patients, like me, will go on to have chronic pain. I had periodic pleuritic pain caused by inflammation of the membrane that surrounds the lungs. My worst -- and most frequent -- pain was musculoskeletal. Some days it was there just enough to notice. Other days it felt
like I had been kicked in the ribs ... hard.
My point is that pain management is tricky business. You should have a thorough discussion about the risks, benefits, and treatment options with your doctor.
Being pain-free is unrealistic in many cases, and it is important to understand this. Opiates are not always the best option. Perhaps your best alternative is a TENS (Transcutaneous Electrical Nerve Stimulation) unit, that can be prescribed by a pain
management specialist, or a lidocaine patch (to numb the area). Or, like me, you could have a pain management specialist administer injections to either decrease inflammation or temporarily desensitize nerves.
Maybe the better choice is treatment for the anxiety and depression that are closely associated with severe pain, either by your primary care provider or by a psychiatrist.
There is also a lot of data correlating exposure to nature, meditation, and mindfulness with improvement in pain. Each patient's pain management plan should be individualized and should incorporate multiple treatments whenever appropriate.
And of course, high-dose medications, such as opiates, need to be used judiciously where there is chronic pain. If people with CF need these medications simply to function, how is pain going to be managed after a major surgery, such as a lung transplant, where muscles and bones are pulled, cut, and stretched? As patients build a tolerance to pain medications and require higher and higher doses, their pain eventually will be difficult or impossible
to adequately and safely manage. Furthermore, there is a very real risk of addiction and drug misuse. Over the years I have heard of a fair number of CF patients who have overdosed, whether intentionally or accidentally.
Pain medication will continue to be a sensitive subject. As law enforcement cracks down on prescribers for using opiates to treat pain, some clinicians have even lost licenses and have been sued, putting everybody on edge. Even me. I know that I have
discharged people from the hospital without meds, where I previously wouldn't have thought twice about giving them several days' worth of narcotic pain medication.
But pain can destroy a patient's quality of life. Balancing this with everyday function and the risks associated with pain medication is a very fine line. Therapies should be tailored to each person according to his or her goals. Having a realistic expectation
of pain management, a candid discussion with your providers, and an open mind to trying multimodal or out-of-the-box ideas are all crucial aspects of getting the most relief from your pain, while minimizing associated risks.
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Adult with CF
Joey, 31, was diagnosed with CF shortly after birth. He has participated in numerous clinical trials and research studies. When he is not working as a hospitalist nurse practitioner, he is most likely spending time with his daughter and the rest of his family, taking classes, or creating art.
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This site contains general information about cystic fibrosis, as well as personal insight from the CF community. Opinions and experiences shared by members of our community, including but not limited to people with CF and their families, belong solely to the blog post author and do not represent those of the Cystic Fibrosis Foundation, unless explicitly stated. In addition, the site 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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