Thread by @DavidSteensma

HematologyTweetstory #25: Dr. Walter Clement Noel, the first patient clearly described with what is now known as sickle cell disease (SCD). I published this fascinating & tragic story in 2010 @MayoProceedings following @USPS release of a stamp about SCD./1
mayoclinicproceedings.org/article/S0025-…

Credit is due to Todd L. Savitt, PhD, a medical historian @EastCarolina, who did the legwork flying to Chicago & Grenada in 1987 & identified Dr. Noel from sketchy details in a 1910 case report. He published his findings in a paper in @JAMA_current in 1989./2

In a 2010 paper in #JNMA @NationalMedAssn, Dr. Savitt described just how he tracked Noel's history down. It required both persistence and serendipity. Savitt has published extensively about race and medicine - the intersection of which is clearly part of this story, too. /3

Walter Clement Noel (1884-1916) was born on a large estate in the hilly bush country at the north end of Grenada (photo: @StateDept), a now-independent Caribbean island nation that was then a British colony. Noel was of African descent; his family included wealthy landholders./4

Despite chronic health problems - discussed more below - and a bout of yaws at age 10, Noel went to #Barbados and attended #HarrisonCollege in Bridgetown (left: Barbados National Trust photo), a secondary school founded in 1733 that is still in existence. He was a good student./5

In September 1904, Mr. Noel sailed from Barbados to New York on SS Cearense (postcard from 1906 when it ran aground off New Jersey). During the week-long journey, he developed a leg ulcer (common SCD complication) which in that pre-antibiotic era was treated with topical iodine.

Noel had suffered leg ulcers as a child, thought “due to the bruises & scratches that were frequently produced as he ran about barefoot through the streets and brush”. There were also “times when he was bilious & the whites of the eyes tinged with yellow" - hemolysis, surely./7

Noel then traveled by train to Chicago, where he had been accepted as a dental student at the “Chicago College of Dental Surgery” (merged with @LoyolaChicago in 1926) on the city's West Side. He took a room on West Congress Street in the heart of the Chicago medical district./8

It was unusual in 1904 for a student of African descent such as Noel to study in the US outside of what are now called “Historically Black Colleges and Universities (HBCUs)”, eg Meharry. But Noel was 1) rich, and 2) foreign, which opened doors./9 mmc.edu/education/sod/…

In late November 1904, Noel developed respiratory problems – today we’d call that acute chest syndrome. He finally sought medical attention at Presbyterian Hospital in Chicago (now part of Rush) & was evaluated by intern Ernest Irons (1877-1959), depicted in old age below./10

Dr Irons performed a peripheral blood smear - still a relatively recent addition to the clinical testing battery in 1906 - and noted that Mr Noel's blood smear contained “many pear-shaped and elongated forms—some small.” Below are the actual original lab report & cell counts./11

Irons discussed the case at length with James Herrick (depicted), his supervising physician. A thorough search for potential causes of the oddly shaped cells (original images from Noel below) was unrevealing; malaria or a parasitic infection was suspected but never confirmed./12

Noel eventually recovered and returned to school. Throughout the next 2½ years, he was sick a few more times: hospitalized briefly for bronchitis, then confined for 2 months at Frances Willard Hospital (depicted) for “a bilious & muscular attack” (we'd now call it pain crisis)/13

Irons also made house calls to Noel's rooms for more pain & bronchitis episodes. He kept dutiful case notes for years. Clearly Irons & Noel got on well; Noel is described as “bright and intelligent” in the records. Irons gave all his notes to Herrick at the end of residency./14

Herrick presented Noel's case at a national meeting in 1910, and then published a detailed report - without Irons as an author! - later that same year. Then he turned his attention to other matters; he is given credit for being the first to describe myocardial infarction in 1912.

We don’t have to feel too bad for Dr. Irons, though, even though Herrick really screwed him on credit for the discovery. He later achieved distinction as a rheumatologist, and he was Dean of @RushMedical, also president of @ACPinternists and @AmerMedicalAssn in the 1940s./16

A few months after the Noel case was published, a second, similar case was described in rural Virginia by Benjamin Washburn in the “Virginia Medical Semi-Monthly”. The patient was a cook/housemaid named Ellen Anthony. (Savitt found that case, too.) Next report was in 1915./17

By the early 1920s, enough experience had accumulated that Vernon Mason named this "new" illness sickle cell anemia - later became SCD since many other features besides anemia. In 1949 Linus Pauling (1901-1994) called it “the first molecular disease”. Note the cells on stamp./18

Despite his illnesses, Noel graduated in 1907 from dental school with his entering class (!) & returned to Grenada. He set up a private dentistry practice in the capital city of St George's. His mother owned the building where the practice was located; Dr Noel lived upstairs./19

Few details are available about Dr Noel's life between the time of his return to Grenada & his final illness. He never married. In 1915, he drafted a will, suggesting he was becoming worried about his health. In April 1916, he overexerted himself attending a horse race./20

He traveled all day across Grenada, then bathed. He soon developed a “chill,” followed by a serious respiratory infection. His condition steadily worsened, and he died in May 1916 in his home, at age 32. His death certificate lists the cause as, “asthenia from pneumonia.”/21

Dr. Walter Clement Noel is buried in a churchyard that overlooks the Caribbean Sea in the parish of Sauteurs. He rests next to his sister Jane, who also died in early adulthood of lung disease (maybe also had SCD?), and his father, who died at age 36 years of renal causes./22

Of course Dr. Noel was not the first *person* with SCD, just the first described clearly with blood smear images. A genetic analysis published in 2018 suggested the respinsible beta globin mutation arose for the first time ~7300 years ago in N Africa./23 cell.com/ajhg/fulltext/…

And there are some suggestive early case reports. Africanus Horton (James Beale) of present-day Sierra Leone described in 1872 that some of his West African patients had unexplained recurrent bone pain. Probably some of these individuals had SCD./24
scinfo.org/2016/06/14/ear…

I'd like to believe that, given the advent of gene therapy for SCD, this condition which has caused so much suffering over the centuries is going to become extinct. Here's hoping! A shout-out to @ASH_hematology for investing much effort & money in sickle cell research./End