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 cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
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.
Deciding to get on the double-lung transplant waiting list was a very difficult decision, emotionally. I was lucky my care team recommended early referral. It gave me the time to process my emotions about transplant and make an educated decision.
February 28, 2019
Creative Fuel Writing Through Suffering
A CF Nurse Practitioner Talks IPC and Five Feet Apart
“It's official. As of yesterday afternoon, I am listed on the transplant list for new lungs,” I posted on my Facebook wall. An outpouring of love and support
grew by the hour in the form of comments following my post. Two days later I was still looking back at it -- and all of the comments -- still digesting that was MY post!?! My mind bounced all over the place during the course of each day from fear
to excitement to anxiety to hope, with the question “How did I get here?” in between. I want to share with you how I got here, how I decided to list for transplant,
and what I think helped me the most.
It started four years ago. I had worked full time as a nurse for 22 years, the last five of it working as a case manager from the comfort of my home. I couldn't have imagined a better job! Working from home cut out my commute time and allowed me more
time for my daily routine of nebulizer treatments, chest therapy, exercising,
and eating all day long, as well the added perk of working in my pajamas if I chose. I mean, what could be better?
And still, the decline came, despite a dream job and my best efforts. It was drastic and seemed to come out of nowhere, my forced expiratory volume (FEV1) fell from 45 percent to 26 percent seemingly overnight. I found myself so out of breath
just taking a shower, that I'd have to sit down and recover for a few minutes before having the breath to get dressed. So into the clinic I go, and my doctor says the "T" word asking me if it's something I want to pursue, or not. Transplant. WHAT?? For ME??
My mind was instantly whirling. I heard myself say "yes" and I remember hearing the words "early referral" and then jumping to the plan for intravenous (IV) antibiotics.
By the time I drove home, I had decided I needed to stop working. I wasn't sure, yet, how it would work financially, but I knew I'd have to figure it out. I reached out to my employer and started making CF my full-time job, in hopes that I could keep
that "T" word at arm's length for as long as possible.
That's exactly what I've done for the past four years now. My doctor told me I am a great example of when stopping work can help stabilize and benefit your CF health. I was hoping I'd go even longer before I'd have to seriously consider transplant. To
be honest, I never thought I'd have to seriously consider transplant. I have two rare mutations and was hoping one of the cystic fibrosis transmembrane conductance regulator (CFTR) modulators would be found to work for me, or I thought maybe I'd be in the group that would go through gene editing as my "cure." It's been hard to realize that
transplant really is in my future.
This past year I have had to work even harder than normal to maintain my low lung function, with multiple steroid bursts and rounds of IV antibiotics. I've now got a chest port and do monthly hydration at home. I need more rest, and -- this past month -- I have become oxygen-dependent.
Each day is like the movie, Groundhog Day. I do my treatments and chest PT and take my medications. I get plenty of rest, eat as much as I can, and exercise, with the normal household chores intertwined. I carefully plan my social outings and
include days of rest before and after.
I realize my health status is not great compared to other people my age without CF. There is no way I could keep up if I continued to work, or do all that I see others doing on social media. But, still, when someone asks how I am doing, my standard response
is always "I'm hanging in there."
I have come to find that people with CF are so strong and resilient. It's a blessing -- in many cases -- but in trying to decide when is the right time to list for transplant, it can also be a curse, or I am finding it is with me.
It's amazing and crazy how I have adjusted over the years to each decline in my lung function, so much so that I was shocked when my doctor brought up the topic of transplant at 26%.
And though I have only maintained a 35 percent lung function for four years, I am now used to it, so I still doubted if it was the right time to move forward with transplant evaluation, testing, and listing. I knew I had been having more bad days, but
it's hard to keep track of when or how often. Knowing that my CF doctor and the transplant team were suggesting it was time to list and awaiting my answer, my mom suggested I use a calendar and mark each day with how I felt leading up to my next clinic
So I grabbed my calendar and to keep it easy, I used a green and red marker. Each evening as I did my last breathing treatment, I'd evaluate my day, putting a green mark on the calendar if it had been a good day, and a red box if I had a bad day. Many
days I marked both red and green as I found most days seemed to be, meaning I had a rough morning but the day got better, or maybe I felt good in the morning but by evening I was wiped out or not feeling well. I was basing this solely on my physical
symptoms such as energy and pain levels, and shortness of breath. By the time my clinic visit rolled around, I had a full calendar month colored in.
To my surprise, there was a lot of red -- more red than I wanted to see and more red than I felt comfortable seeing, especially knowing I had been on prednisone, hydration, and oral antibiotics that month. And that is what helped pushed me over the edge
to say, "OK, I'm ready to list." Living with CF you learn to be strong and you learn to keep on going and I think with that mentality I could keep on going until I literally drop. So, this calendar helped me see my current health status in a different
way and be able to make a decision that was difficult mentally and emotionally, where my instinct would normally be to say, "I'm okay, I can keep going." This calendar gave me a perspective to measure with, where I could override my emotional response
and use my brain to look at the data and know it was time to move forward in this process.
As I sit here, now listed for transplant, I am thankful. I'm thankful that I have an amazing care center close to me that not only does transplants, but does a lot of them, and even takes some of the more difficult cases. I am thankful that I was accepted
at my care center, as being a good candidate to list.
One of the things I'm most thankful for is that my doctor referred me to the transplant team and process early. Although it scared me to hear the word, it gave me the luxury of time.
I had time to think about my decision; time to research the enormity of the commitment; time to reach out and talk with others who had been through this and find out what post-transplant life looks like; time to come to terms with where my health was;
time to fight and see if I could make it better; time for the transplant team to get to know me as a person; time to digest that this next step was right for me; time to get affairs in order; and time to mentally and physically prepare myself and
I'm not sure when "the call" will come, or how it will go, but I do know that I feel I'm doing the right thing at the right time with the right team and that it has been a long process to come to peace with all of that. I know it is scary, but I'd highly
recommend early transplant referral so that others will have the opportunity of time to think, research, digest, fight, discuss, and prepare as much as possible. Whatever their final decision may be, at least they had the luxury of time to figure
it out. And, as we are all well aware, time is one of the luxuries people with CF rarely get.
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Adult with CF
Jennifer was diagnosed with cystic fibrosis at birth. She worked as a nurse for 22 years before transitioning to disability in 2015. She's been involved with the CFF for many years, fundraising with Great Strides since 2004 and wraparound events, helping to co-chair the Adult Advisory Committee, serving on the Adult Advisory Council, and participating in CF Peer Connect and Community Voice. Jennifer's passions and hobbies include listening to music, cooking, exercising, writing, reading, being with her dogs, traveling, and spending time with friends and family. She has a 23-year-old daughter, is married, has two dogs, and lives in Pennsylvania.
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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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