Cystic fibrosis is a lifelong condition that begins at birth and slowly progresses throughout a person’s life. It is among the most difficult chronic illnesses to treat, manage, and survive. Despite this, the field of cystic fibrosis has made leaps and strides in the last fifty years, more than doubling the lifespan of individuals diagnosed with the disease, and vastly improving the quality of life for those who must live with the disease for the whole of their lives.
At first, the field of cystic fibrosis research and treatment was directed at those who suffered from it the most: infants and children. Pediatric programs excel at treating individuals with cystic fibrosis, and much of the focus of treatment lies on the critical years of a child’s development. As the child grows, pediatric programs utilize the treatment options and therapies that have been made available to children, nurturing them into capable adults with manageable symptoms and action plans for living as healthy a life as possible.
Yet many find the transition from pediatric programs to adult treatment a difficult hurdle to cross. For those who live with cystic fibrosis, even a momentary lapse in treatment can give way to severe consequences. Infections that go untreated for more than a few days may prove fatal, or they may permanently damage a patient’s already vulnerable respiratory system. Without constant care and attention, individuals with cystic fibrosis stand at great risk of a severely adverse health outcome.
Dr. Elliott Dasenbrook MD, MHS serves as Director of the Cleveland Clinic Adult Cystic Fibrosis Program, and has spent his life treating difficult to manage diseases like cystic fibrosis. He specializes in optimizing health outcomes for individuals with cystic fibrosis and focuses on putting the patient at the head of their own care. Here is some of his best advice for those facing or making the transition into full adult care.
Make a smooth transition into an adult program
A good pediatric program will offer you a transition program to get you situated into an adult care program. This involves your pediatric doctors meeting with your new physicians and relaying all relevant patient history and recurring problems to the new caregivers. This takes the burden off of you and ensures that your new doctors know everything about what has been going on with you, and how to best provide your care.
Should you find yourself approaching the end of your pediatric care program and without a plan to transition, that’s okay. You will simply need to be a little more proactive about seeking out a good adult care program yourself, and then connecting your prospective physicians with your current physicians. The transfer of patient history and information is vital to ensuring that you continue with all existing therapies and medications, without new doctors struggling to figure out what works well for you and what doesn’t.
Care for your lungs first and foremost
Cystic fibrosis affects the lungs more than any other organ in the body, so it is imperative that you focus on your lung health as you enter adulthood. The more that you can prevent infection and maintain lung capacity, the better your overall quality of life, and the longer your lifespan will be. Part of ensuring lung health is choosing a care program or care provider who has extensive knowledge of cystic fibrosis and managing difficult to treat infection.
Dr. Dasenbrook recommends seeking out a board-certified pulmonologist; that is, someone whose primary scope of practice is within the respiratory system. Throughout your life, the one thing that you stand at constant risk of is lung infection or respiratory complications. Respiratory events will have significant long-term impact on your overall health, and most people with cystic fibrosis will experience significant decline if even a simple lung infection takes place.
Be active in your own health
Many pediatric programs excel in that they offer care to a child in a way that that child is comfortable with and can understand. Adult programs are not always so, and without proper proactivity, it might be easy to slip into a program that takes you in and turns you out without informing you well or getting to know what it is that you really need as a whole person. Since those with cystic fibrosis face a lifetime of clinical care, a good relationship with your physician is imperative.
You should always feel like and be at the center of your own care. Dr. Dasenbrook warns against physicians that act like “a dictator, telling you what to do,” without caring for your sensitivities and medical preferences. He says that “the leader of your cystic fibrosis team should be you!” In this sense, your medical care should feel like a partnership between you and all of your supporting caregivers. You should have the ability to make informed decisions about your care, and you have the right to feel respected and understood as you navigate life with cystic fibrosis.
Organize your team
You will want to have a structured team in place to help you manage your cystic fibrosis. Since time is often of the essence when it comes to responding to new developments and complications, you will want consistent, familiar faces to guide you and work with you as you seek out the best treatment options and therapies. Dr. Dasenbrook outlines four major players that you should have as part of your health care team.
First, you should have a pulmonologist who can optimize your lung health and offer you therapies and treatments against lung infection. Second, you should enlist the help of a dietician to help you control the nutrients that your body receives, preferably one familiar with cystic fibrosis. Third, you may want to enlist the help of a social worker who can help guide you through the emotional and everyday stresses of cystic fibrosis, including medical insurance and livelihood. Finally, a consistent nurse practitioner should be on hand to help communicate between all relevant parties, and to be the coordinating member of your team.
Plan on seeking out a lung transplant
No matter how hard you try, it is inevitable that your lung functioning will decline. Most patients with cystic fibrosis find that the set of lungs that they were born with are not capable enough to last them well into adulthood. Even for those who do not experience significant infection or lung damage, the wear and tear of such a difficult disease and the strain of development on the lungs can be enough to warrant a lung transplant. For those with cystic fibrosis, the best-case scenario is to receive a lung transplant at some point into adulthood.
For this reason, Dr. Dasenbrook recommends that you be aware of lung transplant options early on. It is better to enter a program with the hope of receiving a transplant in ten to fifteen years then it is to scramble after a major complication to receive a lung transplant reactively. Dr. Dasenbrook also notes that “[lung transplant centers] that do a large volume of lung transplants tend to have better outcomes.”
Managing cystic fibrosis as an adult does change somewhat from the pediatric programs that have given many children with cystic fibrosis the opportunity to grow into adulthood. However, managing the disease throughout should not be an overwhelming or complicated ordeal throughout a person’s lifetime, especially since those who have the disease have it for life. Optimizing your own health outcome and seeking out the best supporting medical staff is the best way to ensure that you live with your disease under control and absent from your everyday life.