Living with Cystic Fibrosis (CF) is a challenge, but advances in medical care for people with CF have opened new opportunities for health. The scientific breakthroughs in recent years are a tribute to the extraordinary work of our clinical and research colleagues, to the Cystic Fibrosis Foundation, and especially, to our patients and their families, true partners in all of our endeavors. As “older” physicians, we have observed the evolution of this disease from the early days of our medical careers, when most people with CF did not plan for graduations, weddings, births, and productive professional careers, to the current era when expectations are high. The changes in CF care have been remarkable. Understanding the molecular genetics of cystic fibrosis transmembrane regulator protein (CFTR), the “CF gene” product, has been central to many of our new insights. It has helped us to see phenotype-genotype correlations, placed into context some of the long-observed but poorly understood microbiologic perturbations in CF, and has played a part in newborn screening and our current approach to the diagnosis of CF. A multisystem approach to this complex condition has led us not only to focus on the respiratory complications of the disease but also to recognize the influence of growth and nutrition, gastroenterologic, and endocrine aspects of the disorder as well. Despite advances in treatment, transplantation ultimately remains the best option for some individuals. Now that adults make up half the CF population in the United States, issues related to transition of care need to be addressed. Furthermore, we must expand our view to take into account not only the physical but also the psychosocial challenges of living with chronic illness. In the words of Sir William Osler, “the good physician treats the disease; the great physician treats the person who has the disease.” By taking both a translational and a holistic approach, we are convinced that people with CF will have steadily improved medical and personal outcomes. As you read the articles included in this issue, we hope you will share our optimism for the bright future ahead for those with CF.
It has been wonderful to collaborate with our exceptional colleagues, the outstanding authors who contributed to this compendium. Their enthusiasm to bring a wealth of new basic and clinical information to you in a comprehensive and understandable format is inspiring. We have enjoyed and appreciated working with them to create this issue of Pediatric Clinics of North America focused on Cystic Fibrosis.
This issue is dedicated to our fathers, Rabbi Eugene B. Borowitz and Victor M. Marshall, MD, both scholars and teachers, who passed away within days of each other as we were in the final stages of editing this issue. They inspired us to seek, understand, and transmit knowledge in an enduring quest to benefit others. We hope that as you read this issue you will take away new insights, and through our part in that process of ongoing education, their legacy will live on.
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