Articles and Updates from Phoenix Children's
The Adult and Pediatric Cystic Fibrosis (CF) Center at Phoenix Children’s provides multidisciplinary, specialized care for more than 500 people with CF, from newly diagnosed newborns to elderly adults who come from all over the state of Arizona to receive care. Our team is made up of physicians, nurse practitioners, nurses, dieticians, social workers, a psychologist, care navigators, child life staff and a research nurse who are all dedicated to providing the best care to our CF patients and their families.
Phoenix Children’s is one of only 2 CF centers accredited by the Cystic Fibrosis Foundation (CFF) in the state of Arizona, which ensures that rigorous standards for the care of people with CF are met. We have combined clinics with otolaryngology, endocrinology and gastroenterology providing coordinated and more convenient care as CF is a disease that effects other organ systems in addition to the lungs. We are also experts in connecting our patients and families with the resources they need to access all the medications, therapies, equipment and supplements they need to thrive.
New CF medicines
People with CF require daily therapies and multiple medications to maintain their health. We’re excited by the development of highly effective CFTR modulator medications that are the first to treat the primary problem in CF, which is abnormal function of the CFTR protein, which leads to thick and sticky secretions in the lungs causing lung infections and lung damage over time. All the previous medications and therapies for CF addressed the downstream effects of abnormal CFTR function, but these new medicines help this protein to function and lead to significant improvement in lung function, fewer lung infections, improved nutrition, and improved quality of life. Trikafta (elexacaftor/tezacaftor/ivacaftor) is the most recently approved CFTR modulator we have been able to use for patients 12 and above since October of 2019. It is expected to be approved for ages 6 to 11 in June of 2021. We are so excited to be able to offer this highly effective therapy to our younger CF patients, and are awaiting additional studies to evaluate if it is safe and effective for younger children as well. Unfortunately, not all patients with CF are candidates for modulator therapy. This depends on the genetic mutation an individual with CF carries, and fortunately, Trikafta is an option for patients who have the most common genetic mutation known to cause CF. In the meantime, the CF Foundation remains dedicated to funding research efforts aimed at finding more effective therapies for all patients with CF, and ultimately a cure for CF.
People with CF are living much longer and healthier lives thanks to research efforts, quality improvement practices, improved care guidelines and new medications and therapies. Since 2017, the number of adults with CF in the United states exceeds that of the pediatric population with CF, which is amazing progress for this disease that was once considered primarily a pediatric disease. The majority of our pediatric CF patients are now entering adulthood with normal lung function and plans for the future, and we are privileged to help them navigate life with CF.
Additionally, Phoenix Children’s also has a Cystic Fibrosis Patient Family Advisory Council (PFAC) which is made up of CF families, patient and a diverse group of Phoenix Children’s care team members. Learn more or fill out an application to join the CF PFAC here.