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CF Care Center Corner

The history of cystic fibrosis (CF) is a reflection of the advances in medicine. Our knowledge has gone from a description of a clinical problem to an understanding of the genetic, cellular and molecular basis of the disease. Dr. Dorothy Andersen, a pathologist, first described the disease while working at Columbia Presbyterian Medical Center over 75 years ago. A decade later another physician at Columbia University Medical Center, Dr. Paul di Sant’Agnese, was the first to recognize CF babies were at special risk for heat prostration during the heat wave of 1949. This led to the realization that salt loss was the cause and the development of the sweat test. The sweat test not only remains the cornerstone of the diagnosis of the disease but also opened up areas of research that ultimately helped to identify the basic defect in CF. Dr. Carolyn Denning worked with Dr. Andersen at Columbia and served as director of the Cystic Fibrosis and Pediatric Pulmonary Disease Center at Babies Hospital. She was the first cystic fibrosis center director in the United States to use a multidisciplinary team approach to manage the disease. The rich history of cystic fibrosis at Columbia played an important role in the establishment of one of the first Pediatric Pulmonary divisions in the country under the leadership of Professor Robert Mellins. The physicians at the CF Center at Columbia have had the good fortune of having been mentored or collaborated with Dr. Mellins, including the current CF Center Director, Dr. Hossein Sadeghi.

The multidisciplinary team as envisioned by Dr. Denning is the backbone of our CF clinical care. We also realize that families are an important part of the team. Our recently developed CF family advisory group provides additional supporting venue for our community.  This concept will enable families to share their ideas, difficulties and solutions to their struggles.  Our center has initiated extra quality improvement projects such as discharge medication sheet, transition from pediatric to adult care, CF related diabetes screening and audiology screening to enhance our clinical practice. Our primary goal in treatment is to maximize nutritional gains and minimize deterioration in lung function throughout a child’s life until a cure is found.  The future CF treatments will be individually focused on the definitive therapy based on the patient’s CF mutation and the clinical status of the patient at that time. Thanks to extensive research and medical advancement, the era of controlling symptoms has shifted to finding a cure for every class of CF mutation.

The advances in CF have had a major impact on survival. To accommodate the management and needs of adults with CF, we established the adult CF Center over 10 years ago under the direction of Dr. Emily Dimango. The Center is located at Columbia in the Gunnar Essiason CF Center. Together with the Pediatric CF center we are part of the Therapeutic Development Network and are currently carrying out clinical trials in adults and children.

Additionally, the ColumbiaDoctor’s Cystic Fibrosis Center at Stamford Hospital is part of our clinical services in Connecticut.  This team has its own dedicated full time nurse, part time social worker, respiratory therapist, dietitian and a naturopath.

Members of other divisions in the Medical Center work closely with the CF Center in clinical care and research and contribute to the national efforts of the Cystic Fibrosis Foundation. The Pediatric Infectious Disease team led by Drs. Lisa Saiman, Paul Planet and Alice Prince are nationally recognized leaders in CF-related research and clinical care. Dr. Saiman co-chaired the committee that recently published the infection control guidelines for CF. The Naomi Berrie Center is a world-renowned center for diabetes care. Dr. Sarah Lusman-Shrager of the Pediatric Gastroenterology division was selected to participate in the Cystic Fibrosis Foundation’s Developing Innovative Gastroenterology Training program. Columbia University Medical Center has one of the largest lung transplant programs in the United States. The center hosts the annual Kossoff Lecture which for the past 36 years has brought speakers who have been on the cutting edge of CF care and research.

The CF center was recently selected from among eleven centers, and is currently one of the six teams to participate in the ‘OneCF Learning and Leadership Collaborative’ initiative from now until the March of 2016.   The aim of this project is to improve capability of CF centers and to improve CF outcomes.

In summary, Columbia University Medical Center has the expertise to offer state of the art clinical practice as well as access to the latest CF clinical trials and research.

Respectfully,

Hossein Sadeghi, M.D.