Distinguished CF Physician/Scientist, Dr. Paul di Sant'Agnese, Dies at Age 91

Dr. Paul di Sant'Agnese in his lab.

It is with profound sadness that we inform the cystic fibrosis (CF) community of the death of Dr. Paul di Sant’Agnese, one of  the founding fathers of both the Cystic Fibrosis Foundation and of CF care in the United States. Dr. di Sant’Agnese, recently passed away in Washington, D.C. at the age of 91, after experiencing failing health over the past several years.

Dr. di Sant’Agnese will be remembered by the CF community as being a physician, researcher, mentor and role model.  He became one of the first clinicians who devoted his life to understanding and conquering CF, while he was at Columbia Hospital in New York City.  And, he was the first to recognize--during the heat wave of 1949--that babies with CF were at special risk for heat prostration.  This astute observation led to his discovery that the sweat is abnormal in CF. This work not only resulted in the development of the CF sweat test--which, today, 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.

After participating in the establishment of the CF Foundation in 1955, Dr. di Sant’Agnese moved to the National Institutes of Health (NIH) where he became chief of the NIH Pediatric Metabolism Branch, and professor of pediatrics at Georgetown University.  During his career at the NIH, he published numerous articles on CF and continued to publish articles related to the disease well into the 1990s.

In addition to Dr. di Sant’Agnese’s wonderful personal contributions to the field of CF, his legacy will continue through the individuals he trained during his years at the NIH.  Many of the current leaders in CF research and care had their first exposure to the challenges and opportunities in CF while working with Dr. di Sant’Agnese at the Pediatric Metabolism Branch of the NIH.  No single individual in CF has had such a profound impact on so many people.  Dr. di Sant’Agnese energized each of these individuals by his inquisitive mind and his encyclopedic recall of medical literature.  At the same time, he continued to care for patients at the NIH, many of whom remember him as being a compassionate caregiver.  His approach to his fellows--that all were part of a large family devoted to a common goal--was the forerunner of the cooperative activities of CF clinicians that have been so essential for the progress in this disease.

In recognition of his outstanding contributions, the CF Foundation named two awards to honor the work of this outstanding physician/scientist. The Paul di Sant’Agnese Distinguished Scientific Achievement Award is a prestigious award given to scientists and clinicians who have made seminal contributions to CF research arena, including the three co-discoverers of the CF gene in 1989.  This award honors the tradition of excellence that Dr. di Sant’Agnese pursued throughout his distinguished medical career.  Further, the CF Foundation recently initiated The Paul di Sant’Agnese Society to pay tribute to those generous individuals who include the CF Foundation in their estate and planned giving.

Dr. di Sant’Agnese is survived by his wife of 62 years, Betty, who resides in Bethesda, Md.  Dr. di Sant’Agnese’s legacy will continue through all the people whose lives he touched in so many ways.

Posted 07/19/05

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