The other day I finally fulfilled a longtime wish: Meeting the venerable Dr. Bayard D. Clarkson, the senior author who first described the illness that bears his name or is known as Systemic Capillary Leak Syndrome, in an article published in the prestigious American Journal of Medicine in August 1960.
At the time, Clarkson was one of four MDs affiliated with The New York Hospital-Cornell Medical Center, nowadays known as New York Presbyterian Hospital-Weill Cornell Medical College, located in the vicinity of 68th Street and York Avenue in the Upper East Side of Manhattan. It happens to be the hospital I have been admitted to many times, whenever I have had a serious episode of SCLS while staying in New York City, and where I have one of my best doctors.
The article was entitled "Cyclical Edema and Shock Due to Increased Capillary Permeability” and it is a clinical study describing in painstaking detail (24 pages!) the travails of a “34 year old white woman of Italian descent who had been in excellent health until the age of thirty-two.”
The patient “was entirely well until February 1956 when … she awoke to note that her lower right forearm was swollen and that her voice was hoarse. Shortly thereafter nausea and vomiting developed, and she noted generalized swelling of the face, neck and all extremities. The nausea and vomiting subsided after 36 hours … but the generalized swelling persisted for about four days. She was seen by her family physician who made a diagnosis of rheumatism and treated her with aspirin and a ten-day course of penicillin injections.”
Nevertheless, the patient soon went on to have many of these swelling cycles and came under the care of Dr. Clarkson and colleagues, who ran all kinds of then-available tests and gave her various medications but, unfortunately, she died of a heart attack during a particularly severe episode in January 1959. The underlying reason for the sudden changes in her capillary permeability was not discovered.
As Clarkson and colleagues wrote, “It is apparent that our understanding of this unusual patient is far from complete. It hardly seems conceivable that this case could be truly unique. We hope that some of the recorded observations on our patient may assist in diagnosis and provide clues towards an understanding of the basic disorder in subsequent patients.”
That was the first and last SCLS patient Dr. Clarkson ever encountered, and the only article he ever wrote on SCLS. As most hematologists do, he went on to focus on various blood-related cancers (like leukemia, lymphoma and myeloma) which are far more prevalent than a rare disease like SCLS, and he devoted himself to a life of research rather than clinical practice. He developed a stellar professional reputation, authoring more than 400 scientific articles in journals and books, and serving as a long-time leader of the American Association for Cancer Research.
Still, I had been wanting to look him up some day, to tell him that the struggle with SCLS goes on to this day, and that unfortunately the passage of half a century has not brought us much closer to unlocking the secrets of SCLS. I figured, however, that Dr. Clarkson had long since retired and had probably moved to Florida or someplace like that, so there was no point in contacting him.
To my surprise, I recently learned that Clarkson still shows up for work every day even though he is nearing 84 years of age, and that he spends his time in the research facility that belongs to the prestigious Memorial Sloan-Kettering Cancer Center – located literally half a block from the hospital where he was on staff when that female patient was referred to him back in 1956! So that is why I decided to look him up, after all.
It took me a while to work up the courage to go and see him the other day, and when I entered his stunning corner office on the 17th floor, I introduced myself by saying: “Dr. Clarkson, my name is Arturo Porzecanski [long, deliberate pause] and I have Clarkson’s Disease.”
He was stunned – downright speechless. After regaining his composure, he invited me to sit down, and we spoke for about an hour, during which he recalled his first and only SCLS patient by name and in extensive detail, just as if he had treated her, say, last month. And you could tell that he was still so sad at his inability to understand her medical condition and to help keep her alive – 50 years ago, mind you! It made me wonder: Could it be that this experience had affected him to the point where he had decided to devote himself to research rather than clinical practice?
For my part, I briefed him on what little we have learned about SCLS in the decades that have passed, and about the RareShare community we have created. For his part, he made some suggestions of scientists to contact and research avenues to pursue.
So here is to your life and good health, Dr. Clarkson! It was a veritable honor to finally meet you!
PS: If you'd like to watch a recent, 2-part interview with Dr. Clarkson where he speaks about his career, go to www.youtube.com and run a search for his full name.