More on the Early History of Sickle Cell Disease

In a previous blog I talked about the contribution of a remarkable man and early African Nationalist, Dr Africanus Horton, , to the discovery of sickle cell disease. Perhaps a less savoury individual was Dr Robert Lebby Jr (1831-1907), Quarantine Officer in Charleston, South Carolina. In 1846 Lebby reported the autopsy findings in a male slave, who was hung after trying to escape (Case of absence of the spleen: Southern Journal of Medicine and Pharmacology 1846; volume 1, page 481). The poor, unfortunate man had leg ulcers and, to Lebby’s surprise, an absent spleen. Lebby also reported on conversations with other slave owners, from the plantations of South Carolina, who told him of slaves in their possession with jaundice and leg ulcers. Many or all of these individuals may have had mild forms of sickle cell disease. Neither Africanus Horton nor Robert Lebby were in a position to examine the blood of their patients. Although red blood cells had been observed under the microscope since the mid 1600’s (1658; Jan Swammerdam) it was not until Paul Ehrlich introduced his blood stain in 1879 that it became routine practice in research and clinical medicine to make and record observations on stained blood films.

Dr Robert Lebby

Dr Robert Lebby

Robert Lebby came from an old southern family and both his father and one of his sons were doctors. He served as a surgeon in the Confederate Army during the American Civil War (1861-1865) and, in fact, was present on the fateful day, 12th April 1861, when the first shots were fired by a Confederate battery at the Unionist stronghold of Fort Sumter. The subsequent, dreadful conflict killed over half a million men. Later, in about 1863, he wrote up his recollections of those events, which were published much later in the South Carolina Historical and Genealogical Magazine in 1911. He was also a successful cotton planter on James Island and a leading member of the South Carolina Agricultural Society, presumably where, in conversation, he learnt of the other planters observations on their negro slaves. He was appointed Quarantine Officer for the port of Charleston in 1882, a post he held for 24 years, being responsible for ensuring that infectious disease was not introduced into the city by the arrival of infected or contaminated sailors or goods. In 1886 he, along with the rest of the medical community, rallied round to help the survivors of the Charleston earthquake (estimated 6.6 to 7.3 on the Richter scale). This was an unusual event for that part of the world, but caused significant damage with most buildings in the city damaged and between 60 and 110 lives lost.

Most of the building in the city were damaged

Charleston Earthquake 1886; most of the buildings in the city were damaged

Despite the early seminal observations of Africanus Horton and Robert Lebby, the first clear report of sickle cell disease in the western scientific literature is generally credited to Dr James Herrick, who published his observations of a patient in Chicago in 1910. Although he did not name the condition, Herrick is credited with the first description of the abnormal red cells in his patient’s blood. On the centenary of his discovery in 2010, there was a symposium, organized by the National Institutes of Health in Bethesda, Maryland, to celebrate this achievement and the progress made in understanding sickle cell disease over the subsequent century.

Dr James Herrick

Dr James Herrick

James Herrick (1861 to 1954) was born, lived and worked in Chicago, he came from a pioneer family and his maternal grandfather had emigrated from England, travelling by covered wagon, and settling a homestead in Illinois, in what later became the suburb of Oak Park on the western edges of the city of Chicago. He seems to have been a cultured, if slightly austere man, with a lifelong interest in the early English author Chaucer, all of whose works he had apparently read. He qualified in Medicine from Rush Medical College, Chicago and was attending Physician at the Presbyterian Hospital.

The Presbyterian Hospital, Chicago, 1914

The Presbyterian Hospital, Chicago, 1914

He published 135 scientific papers, lived to the age of 93 years and received many honors from the American medical establishment. His main interest was in cardiology and he was the first to describe and propose a mechanism for angina pectoris and myocardial infarction (heart attacks). He was an early proponent of the electrocardiograph (ECG) for the diagnosis of heart disease.

An example of an early ECG machine purchased by James Herrick for use in the Presbyterian Hospital, Chicago. The subject looks a little nervous.

An example of an early ECG machine purchased by James Herrick for use in the Presbyterian Hospital, Chicago. The subject looks a little nervous.

In 1904 Herrick’s attention was drawn to a negro patient, who was training in dentistry at The Chicago College of Dental Surgery, by his intern (house officer) Dr Ernest Irons (1877 to 1959). The patient had all the classic features of what we would now call the acute chest syndrome, and spent 4 weeks in hospital recovering. It was Dr Irons who examined the patient’s blood microscopically and noted nucleated red blood cells, polychromatophillia and, critically, “ thin, elongated, sickle-shaped and crescent-shaped” red cells. Various tests were carried out to try and determine the significance of these red cells without any success. Dr Irons saw the patient on many subsequent occasions over the next 3 years although it appears that his consultant, Dr Herrick, only met the patient twice. Contact was lost in 1907 when the patient returned to Grenada.

Six years after the original observations Herrick presented the findings in a paper to the 25th Meeting of the Association of American Physicians in 1910 and subsequently published them (Peculiarly elongated and sickle-shaped red blood corpuscles in a case of severe anaemia: Archives of Internal Medicine 1910; volume 5, page 517). The presentation and paper caused virtually no interest and Herrick never returned to the problem, mentioning the paper only briefly in his autobiography, published many years later. A similar sequence of events followed Herrick’s presentation of his findings on myocardial infarction to the same august body in 1912, again there was no interest, despite the fact that Herrick felt that these were observations of tremendous importance. He later recalled “I read it, and it fell as flat as a pancake ……. I was sunk in disappointment”. Six years later Herrick re-presented the myocardial infarction findings, this time to great acclaim, his publication on sickle cell disease however languished unappreciated until the early 1950’s.

Dr Irons was mentioned in the 1910 paper, but he was not a co-author, despite the fact that he had made the original observations on the patient’s blood, had drawn Herrick’s attention to the possible connection between the patient’s severe anaemia and the unusual microscopical appearances and had provided on-going care. These days such a contribution would have guaranteed proper acknowledgement as a joint author.

Dr Ernest Irons

Dr Ernest Irons

Irons was born in Council Bluffs, Iowa, another Midwesterner, and had a parallel career to his mentor, James Herrick, also becoming an attending physician at the Presbyterian Hospital and President of The American College of Physicians. He seems to have had a close relationship with their patient, looking after him on multiple occasions, visiting him at home and keeping meticulous records of his observations, undertaking several blood examinations and drawing the “sickle-shaped” red blood cells in the patient’s medical records. However, his main interest was in bacteriology and infectious disease, and like Herrick, Irons never returned to the problem of the “peculiarly shaped red cells”. In his later years, as a medical elder statesman, Irons wrote extensively on the obligations and role of the doctor in society, the dangers of socialism and the threat of nationalization and the welfare state to the freedom of medical practice. James Herrick pre-deceased Ernest Irons by several years and the latter paid fulsome tribute to his senior colleague after his death (Transactions of the Association of American Physicians 1954, volume 67, page 15).

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About rogerjamos

I am a consultant haematologist who has worked in Hackney, London, UK with patients who have sickle cell disease for many years. Knowledge is power; the hope is that this blog will empower patients by putting them in touch with contemporary research into sickle cell disease and facilitating informed discussion on the issues raised. Dr Roger Amos MA, MD, FRCPath
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