Sagan's Disease

Ed Uthman, MD

Diplomate, American Board of Pathology

24 Dec 1996

This week I noted with sadness the death of astrophysicist, science writer, Cosmos writer/narrator, novelist, and Cornell professor, Carl Sagan, age 62. News reports stated he died from "pneumonia" resulting from "myelodysplastic or preleukemic syndrome." Presumably he had a myelodysplastic syndrome, causing neutropenia, causing pneumonia.

I was a great admirer of Sagan. In my opinion, he was the second greatest science popularizer of my lifetime (after Isaac Asimov). Like Asimov, Sagan never surrendered to the distractions of irrationality and was a great defender of the faith regarding the scientific method (cf., Linus Pauling, with his vitamin C nonsense; Robert Heinlein with his decades-long middle-age crisis; Arthur C. Clarke with his hollow mysticism).

[OK, so Sagan got into that stupid fight with Apple Computer over the "butthead astronomer" remark, but he was probably sick by then, so I'll cut him some slack. And a lot of us were disappointed when he split up with the lady who drew those cool Voyager pictures of the naked man and woman, but that's none of our business, right?]

I have long thought that the group of conditions dubbed "myelodysplastic syndromes" needs an eponymous designation more than any other common disease. "Myelodysplastic syndrome" not only lacks euphony and mellifluousness, but it just doesn't provide any satisfaction in penning it on a diagnosis line. Also, I hate the term "syndrome." It sounds like something from a cheap screenplay ["So, what is it, Bones?" "He's dead, Jim. Kerulian syndrome... every atom of manganese in his body has been destroyed by the virus."]

I figure that no one has any special privilege to create eponyms, so I will forever refer to MDS parenthetically as "Sagan's disease." Maybe it will catch on. Heck, the way I see it, Sagan is more deserving of immortality that Lou Gehrig (and that one should be changed to "Hawking's disease"). And what did that Hageman guy do anyway? Didn't he work on the railroad or something? Big deal.

By the way, Carl Sagan went to Cornell after being denied tenure at Harvard. Whom do you have to sleep with to become a tenured Harvard professor anyway?

Medical Addendum

29 May 2000

Since writing the above article, I have read both of the excellent biographies of Carl Sagan published in 1999 (the authors were Keay Davidson and William Poundstone, respectively). While neither author is a physician, both are seasoned science writers and offer some insight into Sagan's medical condition. These should be mentioned here, as they amplify and correct some of the items I covered above.

The first symptom that led to the MDS diagnosis was the observation by his wife, Annie Druyan, of a large bruise on Carl's arm. Presumably this was purpura resulting from thrombocytopenia, although neither biography explicitly states this. Apparently the diagnosis of MDS was made very quickly, without the prolonged "let's wait and see" process that tends to characterize many of these cases. This suggests to me that he had a particularly severe lesion from the get-go, and that the clinical and pathologic findings were 4+. In fact, biographer Poundstone remarks that the doctor was suspicious that the blood sample had been misidentified, in that Sagan's activity level (he was traveling at the time the results became known) was too robust for what the lab indicated.

Between the first symptoms in 1994 and his death in late 1996, Sagan received a total of three bone marrow transplants from his sister, Cari. The first two failed, and the third was preceded by total body irradiation. Although the last graft was said to have "taken," his last remaining months were characterized by a shocking decline, such that he was unrecognizable even to longtime friends. Poundstone writes (p.381):

Sagan made plans to see [astronomer and SETI pioneer] Frank Drake while he was in San Francisco [to address the National Science Teachers' Association]. While Drake was waiting for Sagan to arrive at the restaurant, he idly watched a waiter help a stooped, elderly man shuffle slowly through the restaurant. With a shock, Drake realized the "old man" was Carl.

After giving two talks to the NSTA, Carl became very ill and had to be taken back to Seattle (where he had received all his marrow grafts) for his last hospital admission. There he was diagnosed with pneumonia. No microbial etiology was established, and it was concluded that the pneumonia was due to the total body irradiation given months before (radiation is a well-established cause of diffuse alveolar damage). His official cause of death (shorty after midnight, 20 December 1996) was given as pneumonia as a complication of MDS.

Although Sagan was an astronomer by training, he was extremely well versed in biology. Many of his writings (e.g., The Dragons of Eden and Memories of Forgotten Ancestors) deal with purely biological/ethological topics. He is considered by many to be the Father of Exobiology (the study of life on other worlds, sometimes referred to archly as "the only area of scientific study that has no subject"). From this rich background and from his own harrowing health experiences (decades of suffering with achalasia, including nearly dying from a surgical procedure designed to correct it, and surviving a "botched" appendectomy) one would think he would have had a lot of interest in medicine. There is little in his writing to suggest he did, though. I wonder if his own long race from malady and death made him fear looking too closely into the Grim Reaper's recipe book.

[See also my personal essay, "How Carl Sagan Changed My Life"]

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