K. N. Lai
Hong Kong, China
Liu Bei, the founding emperor of the state of Shu-Han, 7th century. The revered leader may have had Marfan syndrome.
These items, part of the Gerald Chow Memorial Lecture delivered to the Hong Kong College of Physicians, illustrate the many connections between medicine and the humanities, as well as exemplifying how illness shapes the course of human events and how even mild congenital anomalies may have catastrophic outcomes.
Franklin Delano Roosevelt (FDR), serving his third term of the presidency of the United States of America, was recorded in July 1941 to have had his systolic blood pressure fluctuate between 160 to 180 mmHg. By 1943 his health began to decline. He lost weight, his face thinned, and he had shortness of breath. At first his personal physician, Vice Admiral Ross T. McIntire, diagnosed “flu and bronchitis”; but a second consultant, Dr. Howard G. Bruenn, diagnosed hypertension, left ventricular failure, and bronchitis. He recommended digitalis, a low salt diet, and bed rest. FDR’s blood pressure rose from 168/108 mmHg in March 1944 to 260/150 mmHg in January 1945. At the Yalta conference on the night of February 3, 1945, his blood pressure was 260/150 mmHg. Sleepless, puffing and sitting up all night, he complained of headache and was unable to concentrate. He had a 9 a.m. meeting the next morning with Stalin and Churchill. At this meeting decisions were made that eventually resulted in the dominance of eastern Europe by the Soviet Union. Roosevelt, despite his failing health, seemed to think that he and Stalin would personally iron out any difficulties after the war ended, but he died two months later, leaving historians to speculate on how differently things may have turned out in Europe if antihypertensive drugs would have been available at the time.
Napoleon Bonaparte was exiled in 1815 to the island of Saint Helena, 1870 km from the west coast of Africa. Within three years his health deteriorated and he died at the age of fifty-two in 1822. As he was in the British custody, it was believed throughout Europe that he had been murdered. Napoleon’s physician, François Carlo Antommarchi, performed the autopsy and found the cause of death to be stomach cancer, but did not sign the official report. The stomach ulcer was 4 cm in diameter.
Between 1955 and 1961 Dr. Sten Forshufvud reviewed the diaries of Napoleon’s valet and proposed that Napoleon had died from arsenic poisoning. This conclusion was supported by 1960 a study from Glasgow in which the toxicologist Patrick Kintz stated the type of arsenic found in Napoleon’s hair shafts was the most toxic mineral type. But in 2008 researchers analyzed samples of Napoleon’s hair collected at different ages and also from his family and other contemporaries. All the samples had extremely high arsenic levels, showing that Napoleon’s body had been heavily contaminated with arsenic since childhood rather than being murdered with it. People were constantly exposed to arsenic in glues and dyes, such as Scheele’s Green, a pigment invented in the 18th century.
In 2005 Swiss scientists were able to obtain twelve pairs of Napoleon’s trousers, four from before his exile and eight in exile. Collating postmortem information of weights and waist measurements of patients dying from stomach cancer, they estimated Napoleon had lost eleven to fifteen kilograms in the last six months of his life, concluding that Napoleon died of stomach cancer. Furthermore, Napoleon’s father and his sister Pauline also died of stomach cancer.
Vaccination against smallpox. Emperor Kangxi, the second emperor of the Qing Dynasty, had smallpox at the age of five and became immune to it, succeeding to the throne because all his brothers had died of the disease, as did his father and two of the later Qing emperors. In the palace of the Qing Dynasty there was a temple for the goddess T’ou-Shen Niang Niang, who supposedly spread flowers from heaven leading to smallpox infection. She actually had four ministers who spread measles, chickenpox, scarlet fever, and pockmarks. Keen to prevent the spread of smallpox, Kangxi encouraged a smallpox vaccination program called nasal insufflation. Mild smallpox cases were selected as donors, scabs from their pustules were ground into powder or mixed with a grain of musk and bound in cotton, the infected material was packed into a silver blowpipe, and then puffed up the patient’s nostril. Variolated cases were treated as infectious as those who had acquired the disease naturally. The Chinese practiced this for at least 200 years before Jenner introduced smallpox vaccination in England in 1796.
The Amazons of Greek mythology were fierce female warriors from Asia Minor or Ukraine, who under Queen Penthesilea and her sister Hippolyta participated in the Trojan War. In classical Greek, Amazon was given a popular etymology as from a-mazos, “without breast,” based on the tradition that Amazons had their right breast cut off or burnt out, so as to use a bow more freely and throw spears more easily.
Marfan syndrome. Liu Bei, a famous king of Shu Han (now Sichuan) at the time of the Three Kingdoms (220-280 A.D.) had many Marfanoid features. He could touch his knees while standing; had joint hypermobility; and lived to almost seventy years. He looked like an orangutan and probably suffered from Marfan syndrome. But he also had winged ears, was very tall, had a long arm span, and could see his ears. This condition is now recognized as joint hypermobility syndrome, its cardinal features being blue sclerae, atypical ears (also called “winged ears”), abnormal nose, prominent chin, marfanoid habitus, and hyperflexion of more than 90° of the joints of the hand.
Supernumerary nipples are a not uncommon inborn anatomical anomaly. It was recorded that the father of King Wu, who established the Chow Dynasty about 12thcentury B.C., was born with four nipples and commanded respect from his people. By contrast, in western society they were called “Devil’s Marks and Witches’ Marks,” and women having them were labelled as witches and tortured by the Inquisition. These “Devil’s Marks” were stabbed by a celibate and “chaste” priest with nasty looking knives called “witchprickers.” This was commonly practiced until the 17th century. As described by Reverend John Bell (1705), minister of Gladsmuir in Scotland: “The witch marks is sometimes like a blew spot, or a little flat or red spots, like flea biting; sometimes also the flesh is sunk in, and hallow . . . I myself have seen it in the body of a confessing witch, like a little powder mark, of a blue colour, somewhat hard . . .” Thus only 300 years ago, the western church still viewed this inborn anomaly as an evil expression for which one would be tortured or burnt at the stake.
Duplex pupils are malformations of the pupil with adhesion of the sclera leading pupil from O to ∞ shape. They are similar to the compound eye of insects and do not affect the visual image. In the western world they were considered to be a token of “evil eyes” (McDaniel WB. Perspective in Biological Medicine 1971; 15: 72-79). But the ancient Chinese believed that persons with two pupils were holy persons, born with blessings from heaven and exceptional talents, even immortality. At least six important ancient Chinese rulers had duplex pupils, namely Cangjie, who invented the Chinese characters in the neolithic ages; Shu— an ancient neolithic king; Xiang Yu, who overthrew the Qin Dynasty at 221 B.C. and believed even at the time of his death that he had divine powers despite his defeat by Liu Bang; two other kings (Luo Lu Kuang and Fish Club) in the fourth and fifth century A.D; and Li Yu, a famous poetic king in the kingdom of Tang around 950 A.D., who also believed he would not lose his kingdom but was captured by the emperor of the Sung Dynasty and died in captivity.
Achondroplasia is shown in a statue from the Cairo museum in a man, his wife, and his two children. The man was a wealthy achondroplasic dwarf married to a normal looking woman. The children looked normal. Achondroplasia occurs sporadically in about 75% of cases or is inherited as an autosomal dominant genetic disorder (25%), resulting in abnormal cartilage formation due to a change in the DNA for fibroblast growth factor receptor 3 (FGFR3). The British, who liked to raise dogs of different physical appearances, successfully bred in the 19th century different species of dogs with short legs such as dachshunds, basset hounds, and bulldogs. In 2009 a gene that created the modern short-legged dog was discovered, acquired by mutation at least 300 years ago, when modern dog breeding began. The extra gene causes overproduction of a protein that disrupts growth during fetal development, providing a link between achondroplastic humans and short-leg dogs.
|The Toilet of Bathsheba, 1643, Rembrandt, Louvre, Paris.
Rembrandt’s model may have suffered from lactation mastitis.
The Night Watch. Rembrandt painted The Militia Company of Captain Frans Banning Cocq between 1640 and 1642. It was called Nachtwacht by the Dutch and The Night Watch by Sir Joshua Reynolds because by the 18th century it had become so dimmed and defaced that it looked quite like a night scene. After it was cleaned, it was discovered to represent broad day-party of musketeers stepping from a gloomy courtyard into the blinding sunlight.
Rembrandt also painted two versions of the Toilet of Bathsheba, one in the in the Louvre in Paris, the other at the Metropolitan Museum of Art in New York. Both show Queen Bathsheba having a bath. In the Louvre painting medical researchers have observed a lumpy deformity in her left breast and have offered various hypotheses, including breast cancer. The model for the painting was Rembrandt’s partner, 28-year-old Hendrickje Stoffels. As she had neither surgery nor chemotherapy, it appears that rather than cancer she had lactation mastitis, lasting for about nine years before she died.
Scarlet fever and music. For the great maestro, Ludwig van Beethoven, the cause of death was well known. He suffered from alcoholic cirrhosis, infectious hepatitis, sarcoidosis, and Whipple’s disease. He was deaf and he also had syphilis. He had lead poisoning because syphilis was treated with lead at that time. But for Mozart the cause of death has long been debated, his medical history including syphilis (treated with mercuric salts), rheumatic fever, renal impairment due to vasculitis, and trichinosis from eating undercooked pork chops. During the last six months of his life he had fever, skin rash, limb pain, dropsy, gross edema, and renal failure. A more recent re-examination of the archives in Vienna at the time of 1791 A.D found that 5000 persons (males and females) aged 40 or less had died of similar clinical features in Vienna in six months. There was an epidemic streptococcal infection in middle Europe in the winter of 1791; and Mozart most likely died of acute nephritic syndrome due to post-streptococcal glomerulonephritis.
Another famous composer is Johann Strauss I—maestro of the waltz and first of the three generations of Strauss—died in Vienna in 1849 from scarlet fever contracted from one of his illegitimate children. As he was dying, the attending physician gave three instructions: clean the room, put him in a side room, and ask for a priest. There was no penicillin, or he would have been able to give a concert in two weeks.
Continuing the story of scarlet fever, there was an English engineer, Robert Whitehead, who invented the first generation of self-propelled torpedoes. When the British Naval Office did not recognize their importance, he went to Europe to sell his invention. Around 1870 he established a factory in Fiume, in the Austro-Hungarian Empire, which had a large navy with seaports in the Adriatic. Whitehead left his fortune to his grand-daughter Agathe Whitehead. In 1911 she married Captain Georg Ludwig van Trapp, who as submarine commander in the First World War sank with torpedoes 12 cargo vessels, the French armoured cruiser Leon Gambetta (12,600 tons), and the Italian submarine Nereide (225 tons). Agathe died of scarlet fever around 1920, and Von Trapp had to find a teacher for their seven children. He hired Maria Kutchera, a postulant from Salzburg intending to become a nun. She became their stepmother and matriarch of the Trapp Family Singers. Her story served as inspiration for a 1956 German film, then a Broadway musical, and subsequently the 1965 blockbuster film The Sound of Music. Before antibiotics many people succumbed to infection in early life, and deadly illnesses such as scarlet fever often shaped the course of events.
DR. K. N. LAI is honorary professor of medicine at the University of Hong Kong and the Chinese University of Hong Kong, and past president of the Hong Kong College of Physicians. Extract from his The Gerald Choa Memorial Lecture 2012. Medical Education. Can we introduce humanity without a textbook or syllabus?
Highlighted in Frontispiece Summer 2014 – Volume 6, Issue 3