Twelve Diseases that Changed Our World

Irwin W. Sherman

• Publisher: ASM Press
• Year: 2007
• Print ISBN: 9781555814663
• eBook ISBN: 9781683673538
• Edition: 1
• Topic: Sciences biologiques
• Subtopic: Maladies infectieuses

Covers the history of twelve important diseases and addresses public health responses and societal upheavals.

• Chronicles the ways disease outbreaks shaped traditions and institutions of Western civilization.
• Explains the effects, causes, and outcomes from past epidemics.
• Describes a dozen diseases to show how disease control either was achieved or failed.
• Makes clear the interrelationship between diseases and history.
• Presents material in a compelling, clear, and jargon-free prose for a wide audience.
• Provides a picture of the best practices for dealing with disease outbreaks.

Twelve Diseases That Changed Our World

Irwin W. Sherman

ASM Press

Contents

• Preface
• 1 The Legacy of Disease: Porphyria and Hemophilia
• 2 The Irish Potato Blight
• 3 Cholera
• 4 Smallpox: the Speckled Monster
• 5 Bubonic Plague
• 6 Syphilis: the Great Pox
• 7 Tuberculosis: the People's Plague
• 8 Malaria
• 9 Yellow Fever: the Saffron Scourge
• 10 The Great Influenza
• 11 AIDS: the 21st Century Plague
• Epilogue
• Notes
• Index

Preface

The literature on the impact of disease on history is large. It chronicles how illness has affected Western civilizations: in the 14th century plague broke the Malthusian stalemate and provided the impetus to restructure European societies; during the past two centuries genetic diseases altered the fates of the British, Spanish, and Russian royal families and contributed to the rise of Lenin, Franco, and Hitler; in the last 100 years we have witnessed how increased opportunities for disease transmission have decimated populations, created panic, and fostered discrimination. We continue to be painfully aware of the power a disease can wield in effecting social and political changes on a grand scale and how it can reveal and exacerbate social tensions. In the past, disease played a role in colonial expansion in the Americas and Africa and, through demographic pressure and starvation, forced a mass migration of the Irish people; tomorrow in different places and in different ways, another disease may do the same.

Historical perspectives of disease can be valuable for a better understanding of how we, and our forebears, survived the onslaught of “plagues” and how we might avoid some of their consequences: confrontations between immigrants and nativists, discrimination against those with different lifestyles, and the social and political disruptions due to incapacitation and death. Of equal value, and much needed, is an examination of the attempts to control disease and how it was possible to improve the public health. In short, this book is about the lessons we have or should have learned from our past encounters with unanticipated outbreaks of disease and how such understanding can be put to use when future outbreaks occur.

The recent SARS and AIDS pandemics clearly show that our lives, as well as the political and economic fortunes of the developed world and emerging nations, can be influenced by the appearance of a contagious disease. In 2004, alarm bells went off as avian influenza spread across the globe, killing millions of domestic fowl and 113 people. The public asked what measures would be needed to stop its spread so that another 1918 to 1920 flu pandemic, which killed tens of millions of people, would not occur. In 2006 cholera swept through West Africa, striking 20,000 people, and in the United States mumps—no longer thought to be a threat because of childhood vaccination—broke out in Iowa and quickly spread to neighboring states, affecting 1,000 people.

These unanticipated epidemics provoke questions. What is needed to curtail the transmission of a disease? What will it take to contain a disease so that protective measures can be instituted? These questions, perplexing and complex, need answers. To simply catalog past diseases and tell of their historic consequences would not be of lasting value to the general public. Rather, it was my feeling that the answers to how we might deal with “coming plagues” could be better obtained by an examination of how past encounters with disease allowed for better control and improved health.

Our world has experienced so many diseases that it would be pointless to deal with all of them. In fact, it would be a nearly impossible task, and, if achieved, it would be numbing to read. Instead, I have selected a dozen diseases that have shaped our history and illuminated the paths taken in finding measures to control them. Porphyria and hemophilia (chapter 1) influenced the political fortunes of England, Spain, Germany, Russia, and the United States; late blight (chapter 2) spawned a wave of immigration that changed the politics of the United States; cholera (chapter 3) stimulated sanitary measures, promoted nursing, and led to the discovery of oral rehydration therapy; smallpox (chapter 4) led to a vaccine that ultimately eradicated the disease; plague (chapter 5) promoted quarantine measures and attenuated vaccines were the result of outbreaks of tuberculosis (chapter 6); syphilis (chapter 7) provided the impetus for cure through chemotherapy; and malaria and yellow fever (chapters 8 and 9) provided the basis for vector control. However, despite these successes, two pandemics—influenza (chapter 10) and HIV/AIDS (chapter 11)—continue to elude control. In this book I try to answer why this is so.

The message of this book is simple: understanding past outbreaks of disease can better prepare us for those in our future. The twelve diseases chosen have influenced the way we look at sickness and show how they resulted in public health measures and other interventions to stem the spread of that disease and others. To eliminate the fear and confusion surrounding “coming plagues,” I describe the ways we have succeeded in bringing certain diseases under control and, in other cases, our failures. My purpose in writing this book for the general reader is to show that despite the challenges which an unanticipated illness may place before us, the future is not without hope or remedy.

1 The Legacy of Disease: Porphyria and Hemophilia

In 1962 the U.S. President John F. Kennedy said, “Life is unfair. Some people are sick and others are well.” He, of course, was referring to himself and the persistent rumors about his ill health. Forty years later, an examination of his medical records revealed that he had Addison's disease, a life-threatening lack of adrenal gland function, as well as osteoporosis and persistent digestive problems. He was given pain killers (demerol and methadone), stimulants, and antianxiety agents, as well as hormones (hydrocortisone and testosterone) to keep him alive, especially during times of stress. Although doubts linger whether President Kennedy's physical ailments influenced the manner by which the Cuban missile crisis was handled or whether they affected other political decisions, it is clear that for many world leaders, including Great Britain's King George III, several of Queen Vizctoria's children and grandchildren, Tsar Nicholas II of Russia, and Alfonso XIII and Generalissimo Franco in Spain, as well as, indirectly, the leaders of Nazi Germany, sickness was the seed for historical change.

Porphyria

Madness in the monarchy

Mary Queen of Scots (1542 to 1587) had a mysterious ailment. At the age of 24 she wrote, “Oftentimes I have great pains . . . ascending unto my head . . . it descends to my stomach so that it makes me lack an appetite . . . and there is sickness with great vomit . . . excuse my writing, caused by the weakness of my arm . . . wherewith we are tormented.” In 1570, when she had another attack, her symptoms were described by her physician: “terrible pains in the side made worse by every movement, even breathing. She vomited continuously, more than 60 times, and eventually brought up blood. She became delirious, and two days later she lost her sight and speech, had a series of fits, remained unconscious for some hours and was thought to be dead. Yet within 10 days she was up and about again. She had unquiet and melancholy fits, convulsions, shivering, difficulty in swallowing, altered voice, weakness of arms and legs so that she could neither write, walk or even stand unaided.” The onset of her symptoms was rapid and suggested to some in her court that she was being poisoned. Others judged her to be hysterical. It is most likely, however, that Mary Queen of Scots was neither hysterical nor the victim of poisoning. Instead, she and many of her descendants probably suffered from an inherited disorder—a curse of British royalty—that would alter the course of world history.

Mary Stuart, Queen of Scotland since birth, was engaged at the age of 3 to Prince Francis, heir to the throne of France; at age 15, when she married him, he was already King Francis II, so she became Queen of France as well as of Scotland. Such glory did not last very long. Francis II died unexpectedly a year after the marriage, and Mary returned to Scotland, where she later married Henry Stuart, Lord Darnley, a relation of the English royal family who was described by historians as a drunkard and an imbecile. Mary did not trust Darnley with affairs of state, and she had several male secretaries who advised her. One of her favorites was David Rizzio, who provoked such jealousy in Mary's husband that he arranged for Rizzio to be murdered. Darnley himself was murdered a year later, and it was widely believed that James Hepburn, 4th Earl of Bothwell, had conspired with the Queen to kill her husband. Shortly thereafter, Mary married Bothwell. This, together with Mary's episodes of blindness, depression, and inability to speak or stand, so disturbed the Scottish nobility that they forced her to abdicate the throne. Mary sought refuge and protection in England, where her cousin Elizabeth I was queen. Mary was an ungrateful and tormented guest in England and became involved in plots to kill Elizabeth. The plots were discovered, and in 1587 Mary was beheaded.

When Elizabeth I died in 1603, Mary's son, James VI of Scotland, succeeded her as James I of England. The King had a disease similar to that of his mother. According to his physician, Sir Theodore de Mayerne, “He was afflicted with pain . . . under his ribs . . . he glows with heat, and his appetite falls off; he sleeps badly; he readily vomits, at times so violently that his face is covered with red spots for two or three days . . . very often he suffered from painful colic . . . with vomiting and diarrhea, preceded by melancholy and nocturnal rigors . . . he had such pain and weakness in the foot that it was left with an odd twist when walking . . . In 1616 . . . for 4 months he had to stay in bed or in a chair . . . In 1619 . . . he sweats easily . . . often suffers bruises . . . he is of exquisite sensitiveness and most impatient of pain . . . He often passed urine red like Alicante wine.” Although diagnosis of medical conditions in persons living so long ago is uncertain, it is very likely that the mysterious disease suffered by Mary Queen of Scots, her son King James, and many of their descendants was porphyria, derived from the Greek word “porphuros,” meaning “purple,” with its telltale sign of red-purple urine.

Gene failure

Porphyria, a hereditary error of metabolism, is linked to the body's production of the pigment hemoglobin, which gives color to our red blood cells and grabs oxygen molecules as blood courses through the lungs. Hemoglobin consists of a protein, globin, coupled to a nonprotein molecule, heme. Heme, an iron complex within a ring structure called porphyrin, is synthesized in the red cells and liver. The reverse of this process, that is, the breakdown of heme to salvage the iron, results in the formation of bile pigments which are stored in the gallbladder and function as a detergent to emulsify fats for easier digestive action; bile pigments also color the feces brown. If there is a block anywhere in the eight-step pathway of heme formation, heme is not produced and the porphyrin intermediates accumulate in a variety of tissues in the body.

The pathway of heme manufacture can be thought of as if it were a river flowing downstream with a series of eight waterwheels along the way; each waterwheel is a cellular factory for making heme intermediates, i.e., porphyrins. To allow for control of water flow, a series of sluice gates are positioned ahead of each waterwheel. For a waterwheel to turn, each sluice gate must be opened by a gatekeeper. When a gatekeeper cannot open a gate, the flow of water is interrupted; water accumulates behind the waterwheel and spills over. Similarly, in the pathway for the synthesis of heme, the gatekeepers are the eight biological catalysts, called enzymes, that allow a controlled flow of intermediates in the pathway. If a gatekeeper “falls asleep at the wheel,” i.e., a particular enzyme does not function properly (or is absent), the normal pathway to heme is blocked and porphyrins accumulate in front of the block. These increased amounts of porphyrins do their dirty work, causing abdominal pain and neuropsychiatric symptoms (such as those seen in Mary and James), although the precise molecular basis for this is not known. The porphyrins in the skin, when exposed to UV light, become “excited” and in this state react with molecular oxygen to form activated oxygen, which can lead to cell death, with redness and blistering of the skin and scarring. The porphyrin intermediates also spill over into the urine during an attack. In an individual with porphyria, fresh urine is colorless, but on exposure to air and light for several hours it turns the color of port wine.

How did James I get porphyria from his mother, Queen Mary? The disease was transmitted through inheritance, not by contagion. Porphyria is ...

Table of contents

• Twelve Diseases That Changed Our World