Leprosy Book
Leprosy Book
Leprosy Book
Anthrax Malaria
Botulism Meningitis
Campylobacteriosis Mononucleosis
Cholera Pelvic Inflammatory
Disease
Ebola
Plague
Encephalitis
Polio
Escherichia coli
Infections Salmonella
Gonorrhea SARS
Hepatitis Smallpox
Herpes Streptococcus
(Group A)
HIV/AIDS
Staphylococcus
Human Papillomavirus
aureus Infections
and Warts
Syphilis
Influenza
Toxic Shock
Leprosy
Syndrome
Lyme Disease
Tuberculosis
Mad Cow Disease
Typhoid Fever
(Bovine Spongiform
Encephalopathy) West Nile Virus
LEPROSY
Alfica Sehgal
FOUNDING EDITOR
The Late I. Edward Alcamo
Distinguished Teaching Professor of Microbiology,
SUNY Farmingdale
FOREWORD BY
David Heymann
World Health Organization
CHELSEA HOUSE PUBLISHERS
VP, NEW PRODUCT DEVELOPMENT Sally Cheney
DIRECTOR OF PRODUCTION Kim Shinners
CREATIVE MANAGER Takeshi Takahashi
MANUFACTURING MANAGER Diann Grasse
http://www.chelseahouse.com
First Printing
1 3 5 7 9 8 6 4 2
Sehgal, Alfica.
Leprosy/Alfica Sehgal.
p. cm.—(Deadly diseases and epidemics)
Includes bibliographical references and index.
ISBN 0-7910-8502-3
1. Leprosy. I. Title. II. Series.
RC154.S38 2005
614.5'46—dc22
2005010391
All links and web addresses were checked and verified to be correct at the time
of publication. Because of the dynamic nature of the web, some addresses and
links may have changed since publication and may no longer be valid.
Table of Contents
Foreword
David Heymann, World Health Organization 6
1. Historical Overview 8
5. Host-pathogen Interactions 45
Glossary 78
Notes 81
Bibliography 82
Further Reading 83
Websites 84
Index 85
Foreword
In the 1960s, many of the infectious diseases that had terrorized
generations were tamed. After a century of advances, the leading
killers of Americans both young and old were being prevented with
new vaccines or cured with new medicines. The risk of death from
pneumonia, tuberculosis (TB), meningitis, influenza, whooping
cough, and diphtheria declined dramatically. New vaccines lifted the
fear that summer would bring polio, and a global campaign was
on the verge of eradicating smallpox worldwide. New pesticides
like DDT cleared mosquitoes from homes and fields, thus reducing
the incidence of malaria, which was present in the southern United
States and which remains a leading killer of children worldwide.
New technologies produced safe drinking water and removed the
risk of cholera and other water-borne diseases. Science seemed
unstoppable. Disease seemed destined to all but disappear.
But the euphoria of the 1960s has evaporated.
The microbes fought back. Those causing diseases like TB
and malaria evolved resistance to cheap and effective drugs. The
mosquito developed the ability to defuse pesticides. New diseases
emerged, including AIDS, Legionnaires, and Lyme disease. And
diseases which had not been seen in decades re-emerged, as the
hantavirus did in the Navajo Nation in 1993. Technology itself
actually created new health risks. The global transportation
network, for example, meant that diseases like West Nile virus
could spread beyond isolated regions and quickly become global
threats. Even modern public health protections sometimes failed,
as they did in 1993 in Milwaukee, Wisconsin, resulting in 400,000
cases of the digestive system illness cryptosporidiosis. And,
more recently, the threat from smallpox, a disease believed to be
completely eradicated, has returned along with other potential
bioterrorism weapons such as anthrax.
The lesson is that the fight against infectious diseases will
never end.
In our constant struggle against disease, we as individuals
have a weapon that does not require vaccines or drugs, and that
is the warehouse of knowledge. We learn from the history of sci-
6
ence that “modern” beliefs can be wrong. In this series of
books, for example, you will learn that diseases like syphilis
were once thought to be caused by eating potatoes. The inven-
tion of the microscope set science on the right path. There are
more positive lessons from history. For example, smallpox was
eliminated by vaccinating everyone who had come in contact
with an infected person. This “ring” approach to smallpox
control is still the preferred method for confronting an
outbreak, should the disease be intentionally reintroduced.
At the same time, we are constantly adding new drugs, new
vaccines, and new information to the warehouse. Recently, the
entire human genome was decoded. So too was the genome
of the parasite that causes malaria. Perhaps by looking at
the microbe and the victim through the lens of genetics
we will be able to discover new ways to fight malaria, which
remains the leading killer of children in many countries.
Because of advances in our understanding of such diseases
as AIDS, entire new classes of anti-retroviral drugs have
been developed. But resistance to all these drugs has already
been detected, so we know that AIDS drug development
must continue.
Education, experimentation, and the discoveries that
grow out of them are the best tools to protect health. Opening
this book may put you on the path of discovery. I hope so,
because new vaccines, new antibiotics, new technologies, and,
most importantly, new scientists are needed now more than
ever if we are to remain on the winning side of this struggle
against microbes.
David Heymann
Executive Director
Communicable Diseases Section
World Health Organization
Geneva, Switzerland
7
1
Historical Overview
“My life could have been so different and maybe a lot shorter” said Hari,
a young man from a rural village in India. He was found to have con-
tracted kushtha when he was only nine years old. As was often the case
with this disease, he was about to be abandoned by his family, until a very
open-minded doctor found out about him. Luckily, the boy did not have
a severely infective form of the disease and his family was not at risk. Still,
everyone in his village was too frightened to touch him. They called him
cursed and wanted nothing to do with him. They only knew that some-
thing terrible was making people in their village very ill. Fortunately, the
doctor succeeded in allaying the villagers’ fears, and, after many sessions
and conversations with almost half of the villagers, the boy was able to
remain with his family. He was properly treated, and soon, the only
reminder of the disease we know as leprosy was a tiny scar on his
back. Listening to this young man of 20, today, it is obvious that, in
addition to its physical symptoms, the disease also brought the devastat-
ing pain of social stigma. The fear of being left alone also had a positive
effect, however. It helped him to become a social worker, which is a
matter of pride for him today. He has been teaching biology in a school
for several years, and his own life experience has certainly helped him
reach out to his students.
Kushtha, the name by which leprosy, or Hansen’s disease, is known in
India, is also the name used to document the disease in very early scrip-
tures of ancient Indian civilization. No matter what it is called, leprosy is
caused by a bacterium called Mycobacterium leprae, a microscopic
germ, which is closely related to the tuberculosis bacillus. Like tuber-
culosis, it is transmitted through breathing—through droplets emitted by
8
a person with an active case of the disease. Unlike tuberculosis,
however, it is not highly contagious. Most people (90–95%) have
a genetic immunity to leprosy, and leprosy experts generally say
that prolonged and intimate contact with a contagious individ-
ual is required for a susceptible person to acquire the disease.
The disease principally affects nerves and skin, with
other organs of the body being affected only in late stages.
Germs may be spread through coughing and sneezing. Many
patients develop deformities due to nerve damage, as the
disease runs its course. Contrary to popular misconceptions,
leprosy does not cause limbs to fall off or flesh to rot. The
bacillus does, however, damage the peripheral nerves, causing
insensitivity or lack of feeling. Nerve damage is the major
cause of disability associated with leprosy. The painlessness
that results from nerve damage allows affected individuals
to ignore cuts, burns, and other injuries sustained during
everyday activities. These injuries may become infected or
even gangrenous, and amputation is required in some cases.
As a result of these deformities, patients are often isolated,
affecting their social and economic well-being. According to a
statistics in 1991 an estimated 6 million people worldwide are
infected with leprosy.
Until the discovery of AIDS (acquired immune deficiency
syndrome), leprosy was the most feared infectious disease in
the world. Even today, however, leprosy’s effects still drastically
change the lives of millions of people—living mainly in Asia,
South America, and Africa. Brazil is the second-most affected
nation, after India.
In the last half of the 14th century, leprosy swept
though Europe, killing one-third of the population, but it
came and went quickly, like a great earthquake, followed by
a series of aftershocks. Today, the disease is most prevalent
in underprivileged areas, where crowded and unsanitary
conditions contribute to its spread. Leprosy has even been
known to develop in more developed nations, from time to
9
10 LEPROSY
did not drive Uzziah into the wilderness, like lesser sinners.
Instead, they stripped him of power and denied him burial
in the cemetery of kings. The New Testament treats lepers
Figure 1.1 These ancient masks show the deformities that many
ancient cultures had associated with leprosy. Having leprosy
caused a person to be isolated and shunned from the community.
more kindly, but still sets them apart from other sufferers.
Jesus healed the blind and deaf, but cleansed the lepers,
implying that there was some moral stigma associated with the
disease. In biblical literature, segregation and disinfection were
Historical Overview 15
SPECIAL MASS
There was a special mass performed in the Middle Ages in
Western Europe, at the site of a newly identified leper’s hut.
Sometimes referred to as a Mass of Separation, one such
version of the mass as spoken by the priest is as follows:
21
22 LEPROSY
FATHER DAMIEN
Father Damien lived from 1840 to 1889, and was originally
named Joseph Damien de Veuster. He was one of Hawaii’s
most illustrious citizens. A Belgian missionary priest, he went
to Hawaii in 1864, as a Picpus Father (priest of the Sacred
Heart of Jesus and Mary). He was ordained in Honolulu and
worked among the islanders for several years. In 1873, at his
request, he was sent to the lepers’ colony of Kalaupapa on
the island of Molokai. There, he labored until his death from
leprosy. A dedicated and driven man, Father Damien did
more than simply administer the faith. He built homes,
churches, and coffins; arranged for medical services and
funding from Honolulu, and became a parent to children
whose own parents had died. A statue of Father Damien,
created by artist Marisol Escobar, stands in Statuary Hall
in Washington, D.C.
Kalaupapa’s reputation as a leper colony is well known.
Leprosy is believed to have spread to Hawaii from China.
The first documented case of leprosy occurred in 1848. Its
rapid spread and unknown cure precipitated the urgent need
for complete and total isolation of its victims. Surrounded
on three sides by the Pacific Ocean and cut off from the
rest of Molokai by 1,600-foot (488-m) sea cliffs, Kalaupapa
provided the perfect isolated environment. In early 1866,
the first leprosy victims were shipped to Kalaupapa and
lived there for seven years before Father Damien arrived.
26 LEPROSY
TYPES OF LEPROSY
Depending on the number of lesions and the number of bacil-
lus observed on a lesion smear, leprosy can be classified into
two groups: tuberculoid and lepromatous (also discussed in
Chapter 5).
28 LEPROSY
Lepromatous Leprosy
In this type, the skin lesions appear as yellow or brown nodules
(protuberances), which are penetrated by many blood vessels.
Usually, there are multiple, poorly defined, hypopigmented
areas that affect the mucous membranes of the eyes, nose, and
throat. Multiple papules (nodular elevations on the skin) can
appear. These are usually symmetrically distributed and tend
to infiltrate (penetrate) the skin. There is a general thickening
of the skin, especially on the face and ears. Patients with an
advanced form of this disease may lose eyelashes or eyebrows.
When someone suffers from disfiguring facial features, this
condition is known as leonine facies. Lepromatous leprosy,
also called multibacillary leprosy, is the more easily spread of
the two forms of leprosy. This more severe form produces large
disfiguring nodules. The peripheral neuropathy (diseased state
of the nerves) observed in lepromatous leprosy, causes muscle
weakness and atrophy and has been associated with claw hands
and foot drops. In this form, the nerve structure is not
destroyed much, but the nodules present in the neural areas
have numerous bacteria.
According to some classifications, there is another form of
the disease, called borderline leprosy. This form is character-
ized by the presence of single or multiple skin lesions with
ill-defined or indistinct borders. Many satellite lesions emerge
around the larger ones. As suggested by the name, the nerve
structure shows an intermediate kind of pathology, with some
30 LEPROSY
31
32 LEPROSY
LEPROSY IS DIFFERENT
Compared to many other infectious diseases, leprosy is very
different. This disease causes more pain and social trauma than
even death itself. When we look at the leading causes of death
worldwide: acute lower respiratory infection, tuberculosis,
diarrhea (including dysentery), HIV/AIDS, malaria, measles,
hepatitis B, pertussis (whooping cough), neonatal tetanus, and
34 LEPROSY
35
36 LEPROSY
Figure 4.1 The bacteria that cause leprosy are rod shaped,
such as the bacteria seen in this micrograph, taken with a
scanning electron microscope and magnified 3,250 times
(top). Bacteria can also be round in shape, such as the
bacteria seen in the bottom picture (transmission electron
microscope, magnified 5,000 times).
What Causes Leprosy? 37
Each family has many genera, and each genus can have many
species. As an example, Mycobacteria is a genus name. It
belongs to the family Mycobacteriaceae. This family belongs
to the class called Actinomycetales. The bacteria that causes
leprosy is called Mycobacterium leprae, with leprae being the
species name (Figure 4.2).
MYCOBACTERIUM LEPRAE
The Mycobacterium leprae bacillus, also known as Hansen’s
bacillus, ranges from 2 to 7 micrometers (1 micrometer
=1/1000 of a millimeter) in length and 0.3 to 0.4 micro-
meters in width. Like many other bacteria, M. leprae has
38 LEPROSY
TUBERCULOSIS
In 1882, Robert Koch identified the organism that causes
human tuberculosis (TB), Mycobacterium tuberculosis.
At the time, TB was rampant, causing one-seventh of all
deaths in Europe. Even today, the disease remains a global
health problem, with around 1 billion people (over 20% of the
world’s population) infected worldwide. In the United States,
the disease occurs most commonly among the elderly,
malnourished people, alcoholics, impoverished males, and
Native Americans. This bacterium is primarily transmitted
through contact with the nasal or oral secretions of the
infected patients.
Disease symptoms include fever, fatigue, and weight
loss. The disease is generally characterized by a persistent
cough. In many countries, individuals, especially infants, are
vaccinated against this bacteria by BCG (Bacilli Calmette
Guérin). The disease is preventable by maintaining better
public health standards and improving social conditions,
for example, reducing homelessness and drug abuse. Once
someone is infected, the disease can be fully cured under
supervised medical treatment, especially if detected in
early stages.
40 LEPROSY
45
46 LEPROSY
INNATE IMMUNITY
The innate or natural immune system is that part of the
immune system that we are born with. The innate immune
system keeps out invading organisms (pathogens), such as
viruses, bacteria, and fungi through a number of mechanisms.
These mechanisms include physical barriers, such as the
skin and the mucous layers; fever to overheat the invaders;
pathogen destroying enzymes secreted in mucous layers
and elsewhere; and acidic bodily secretions, which bacteria
do not like. Other mechanisms through which the immune
system keeps out invading bacteria include the comple-
ment—a system of plasma proteins that principally attack
bacteria; the inflammatory response, which involves the
action of several biochemicals and immune cells to destroy
pathogens, prepare other cells to resist attack, and regulate
downstream steps of the immune response; and non-
specific white blood cells, called effector cells, principally
the macrophages that gobble up pathogens. Innate immu-
nity is not specifically geared toward any particular kind of
organism. It is a nonspecific array of defense mechanisms
to prevent any organism from getting into or thriving in
our systems.
ACQUIRED IMMUNITY
Also known as specific or adaptive immunity, acquired immu-
nity focuses on those features of the immune system that are
“learned” during a person’s lifetime, rather than the immunity
an individual is born with. This part of the immune system
deals with specific invaders and learns to recognize them
by exposure to them. Acquired immunity can function at
different levels and in different forms, called humoral and
cell-mediated immunity.
Host-pathogen Interactions 47
After M.leprae gain entry into the body, they can provoke
one of three possible, responses, contingent upon the measure
of cell-mediated immunity (CMI) in the host. To begin with,
they could be ingested by the macrophages and completely
digested, if CMI is present. This action can take place at the
point of entry or at the lymph nodes to where they may be
transported inside the large defense cells (macrophages). This
type of action is believed to take place in most people infected
by M.leprae, many of whom may never know they have been
invaded by the leprosy bacilli. Approximately 90 to 95% of
people exposed to leprosy experience this defensive action
of the body.
Second, in persons with no resistance (lack of CMI), M.leprae
freely multiply, even within the very cells (macrophage) that
SCHWANN CELLS
Various support cells are associated with neurons, most
typically, Schwann cells. A neuron is made up of a dendrite
that receives the impulse (from another nerve cell or from
a sensory organ), the cell body (numbers of which side-by-
side form gray matter) where the nucleus is found, and
the axon which carries the impulse away from the cell.
Wrapped around the axon are the Schwann cells, and the
spaces/junctions between Schwann cells are called nodes
of Ranvier. Collectively, the Schwann cells make up the
myelin sheath (numbers of which side-by-side form white
matter). Schwann cells wrap around the axon. Having an
intact myelin sheath and nodes of Ranvier are critical to
proper travel of the nerve impulse. Diseases that destroy
the myelin sheath (demyelinating disorders) can cause
paralysis or other problems. Schwann cells are analogous
to the insulation on electrical wires, and just as electrical
wires short out if there’s a problem with the insulation, so
too, can neurons malfunction when myelin sheaths are not
intact (Figure 5.3).
Host-pathogen Interactions 53
56
(small pieces of tissue) are taken from the skin and then
stained to detect bacteria. The density of bacteria observed is
recorded logarithmically, as the bacterial index (BI). Although
this test can confirm the presence of the disease, it does have a
limitation. If the amount of bacteria is too low, they will not
show up in the test.
In addition to microscopic detection, other methods are
used, as well. The most widely used serological test works by
detecting antibodies against the phenolic glycolipid-1 (PGL-1).
Antibodies are produced by our body against the bacterial
proteins, if there is an infection. These tests also have a
limitation. They can identify 90% of the severely infective or
lepromatous form of the disease, but only 40 to 50% of mildly
infective or paucibacillary patients.12 Such antibodies are
also found in 1 to 5% of healthy individuals living in infected
areas. Diagnosis using polymerase chain reaction (PCR) also
has similar limitations. It can diagnose 97% of multibacillary
patients, but only 44% of paucibacillary patients.13 PCR is only
useful clinically to support a diagnosis of leprosy when
atypical signs or characteristics are present. It was used to
detect the spread of disease in ancient times, as discussed in
Chapter 3. Ultimately, effective diagnosis in a person with
very low bacterial loads is still a challenge.
DRUG DEVELOPMENT
As we learned in Chapter 1, devising a proper curative treat-
ment for leprosy took time. Over many decades, and through
lots of trial and error, scientists eventually reached a situation
of greater confidence. Further study, in order to promote
greater understanding, is still ongoing, however.
Toward the end of 19th century (as is still true today),
this disease was still affecting more human lives in India than
anywhere else in the world. Initial attempts at drug therapy
were the result of early information about herbal therapy.
Meanwhile, research continued to improve on the chaulmoogra
57
58 LEPROSY
oil treatment, which was anything but the ideal therapy. Taken
orally, it produced nausea, while injections of the thick oil were
quite painful. In 1937, after a distinguished career, Wellesley
Bailey died, but not before seeing real hope for the patients he
had come to love and serve. Hope came in the form of the
discovery of one of the most revolutionary drugs of its day. As
early at 1908, German Chemist Gerhardt Domack had made
successful attempts to produce what eventually became the
parent chemical in the sulphone family of drugs. Out of this
research, evolved diamino diphenyl sulphone, (Dapsone, or
DDS), although at the time, no one associated DDS with a
possible cure for leprosy. It was considered too toxic a drug to
be used on humans. In 1941, American physician Dr. Faget was
courageous enough to use the parent sulphone drug, with
encouraging results (Figure 6.1).
Even then, as a bacteriostatic drug, DDS did not actually
kill the leprosy bacilli. It only prevented their multiplication.
For many years, it did seem that Dapsone would eventually
help in eliminating leprosy—that is, until resistant organisms
began to appear. Fortunately, new and more potent drugs, such
as Rifampicin, became available. Rifampicin, in combination
with other drugs (multi-drug therapy, or MDT), has given real
hope that, sometime in the future, the elimination of leprosy
could possibly become a reality. Rifampicin also kills the very
well-known cousin of M. leprae, M. tuberculosis. This drug acts
by stopping the synthesis of messenger RNA from DNA, by
affecting the enzyme (RNA polymerase) important for this
process (Figure 6.2). Rifampicin is specific to the bacterial
enzyme, and does not harm our enzyme function.
In 1981, the World Health Organization started recom-
mending multi-drug therapy. Three drugs are taken in combi-
nation: Dapsone, Rifampicin (or Rifampin), and Clofazimine.
Treatment takes anywhere from six months to a year or more,
depending on the extent and character of the disease. Multi-drug
treatment has brought new hope to millions of leprosy patients,
Controlling the Disease 59
64
the bacteria refuse to grow artificially, scientists must find
another way to study it.
Of course, one way to accomplish this is to isolate bacte-
ria from the patients and then use them for experiments.
E. coli
Escherichia coli (E. coli, for short) is a bacterium that
normally lives in the intestines of humans and other animals.
Most types of E. coli are harmless, but some can cause
disease. Disease-causing E. coli are grouped according to the
different ways by which they cause illness. Enterotoxigenic
Escherichia coli, or ETEC, is the name given to a group of
E. coli that produce special toxins which stimulate the
lining of the intestines causing them to secrete excessive
fluid, thus producing diarrhea.
The organism can be found on a small number of cattle
farms and can live in the intestines of healthy cattle. Meat
can become contaminated during slaughter, and organisms
can be thoroughly mixed into beef when it is ground. Bacteria
present on the cow’s udders or on equipment may get into
raw milk.
Eating meat, especially ground beef, which has not
been cooked sufficiently to kill E. coli, can cause infection.
Contaminated meat looks and smells normal. Although the
number of organisms required to cause disease is not known,
it is suspected to be very small. Among other known sources
of infection are consumption of sprouts, lettuce, salami,
unpasteurized milk and juice, and swimming in or drinking
sewage-contaminated water.
Bacteria in diarrheal stools of infected persons can be
passed from one person to another if hygiene or hand washing
habits are inadequate. This is particularly likely among toddlers
who are not toilet trained. Family members and playmates of
these children are at high risk of becoming infected.
65
66 LEPROSY
GENOME SEQUENCING
We owe a lot to armadillos, with respect to our understanding
of the bacteria that causes leprosy. All organisms remain a
mystery, until we have deciphered their genes and functions,
at international levels, with help from many different groups,
scientists, and institutions. Scientists want to know about all
of the genes an organism has. This is achieved by genomic
sequencing (sequencing all DNA) from any organism in
question. Bioinformatics, the application of computer science
to the interpretation and management of biological data, has
provided many software programs and databases, which help
in quick, informative analyses of the obtained sequences. It is
important to know the DNA sequence, as the DNA holds all
the information about an organism—what it is, how it works,
and how it behaves.
Scientists have made a similar attempt to sequence M. leprae.
The bacteria for such a vast project were obtained from an
70 LEPROSY
72
Ongoing Reforms and the Future 73
to recover from the disease and live with dignity and respect.
Many of these patients, however, come from poor, uneducated
families and societies, where they will have a difficult time
being accepted. The institute continues helping patients with
the following message: “Leprosy is a poor man’s disease, treat
the patients like other human beings.” 17
IS A VACCINE A POSSIBILITY?
During an invasion by a foreign organism, the body’s defense
mechanisms become quite active as they attempt to evade the
74 LEPROSY
78
Humoral immunity—Type of immunity that principally operates through a
type of white blood cell called a B cell, which originates in bone marrow
and the spleen. During humoral immune responses, proteins called anti-
bodies, which can stick to and destroy antigens, appear in the blood and
other body fluids.
Hyperpigmented—Areas of skin that become darker or redder than the rest
of the skin.
Hypopigmented—Areas of skin that get discolored or lose their normal
color.
Laminin—A protein specifically found in nerve tissue.
Leonine facies—Lion-like facial features.
Leprologist—A physician experienced in the study and treatment of leprosy.
Lepromatous leprosy—The more easily spread of the two forms of leprosy.
This more severe form produces large disfiguring nodules.
Macrophages—Specialized immune cells that can engulf other cells or
pathogens.
Major histocompatibility complex (MHC)—A set of cellular surface proteins/
antigens that are specific for an organism and play a major role in identi-
fying similar tissue and rejecting tissue from other organisms.
Morbidity—Illness or suffering caused by a disease.
Mortality—Deaths caused by a disease or any other agent.
Multibacillary leprosy—See lepromatous leprosy.
Neuropathy—A disease or abnormality of the neural system.
Nucleus—The eucaryotic cell organelle that is surrounded by a double
membrane and contains all of that cell’s genetic material.
Obligate intracellular parasite—A parasite which cannot survive outside a
living cell.
Osmotic lysis—Rupturing of a cell when placed in a dilute environment.
Paleobiologists—Scientists who study the biology of fossil organisms.
Paleo-epidemiologists—Scientists who study disease in fossil organisms.
Papule—A small, solid, often inflammed elevation of the skin that does not
contain pus.
79
Glossary
Pasteurization—The process of heating milk and other liquids to destroy
microorganisms that can cause spoilage or disease.
Paucibacillary leprosy—Type of leprosy in which the nerve architecture is
destroyed and in which there can be formation of granulomas in nerves.
Peptidoglycan—A large polymer composed of long polysaccharide chains
that are linked to each other. They provide much of the strength and
rigidity of the bacterial cell walls.
Phagocytosis—The engulfing, and usually the destruction of particulate
matter by phagocytes.
Polymerase chain reaction (PCR)—An in vitro technique used to synthesize
large quantities of specific nucleotide sequences from small amounts of
DNA.
Prokaryote—Organisms that lack a true membrane bound nucleus.
Pseudogenes—Parts of DNA; similar to genes of other organisms but are
not functional.
Ribosome—The organelle where protein synthesis occurs.
Schwann cell—A type of glial cell of the peripheral nervous system that helps
separate and insulate nerve cells.
Thymus—A small glandular organ located behind the breastbone.
Transfer RNA (tRNA)—A small RNA that binds an amino acid and delivers it
to the ribosome for protein synthesis.
Tuberculoid leprosy—The more benign type of leprosy that affects the
nerves; often leads to numbness (usually of the extremities). Affects the
peripheral nerves and, sometimes, the surrounding skin on the face, arms,
legs, and buttocks.
80
Notes
CHAPTER 1: 11 World Health Organization
HISTORICAL OVERVIEW International. “Global Leprosy
1 “Leprosy: History and Incidence,” Situation.” Available online at
Encyclopedia.com. Available online at http://www.cefpas.it/fad/fad/
http://www.encyclopedia.com/html/sec 1global.htm
tion/leprosy_HistoryandIncidence.asp
CHAPTER 6:
2 The Leprosy Mission International, CONTROLLING
Available online at http://www THE DISEASE
.leprosymission.org 12 “Leprosy research in the post-genome
3 Leprosy Sufferers Need Compassion, era”. Available online at http://www
“Forum on Leprosy.” Available online .lepra.org.uk/review/Mar01/pp8-22.pdf
at http://www.webspawner.com/ 13 Ibid.
users/LEPROSY/
14 American Cancer Society.
CHAPTER 3: “Thalidomide Makes News Again:
LEPROSY AROUND New Research on How Thalidomide
THE WORLD May Help the Cancer Patients.”
4 Sharon Lerner. “Cases of leprosy on October 6, 1998. Available online at
the rise in U.S.,” The New York Times, http://www.cancer.org/docroot/NWS/
February 20, 2003, available online at content/NWS_1_1x_Thalidomide_
International Herald Tribune, Makes_News_Again.asp
http://www.iht.com/articles/
CHAPTER 7:
87291.html
UNDERSTANDING THE
5 World Health Organization M. LEPRAE BACILLUS
International. “Elimination of Leprosy 15 San Francisco State University,
as a Public Health Problem.” Available Department of Geography. “The
online at http://www.who.int/lep/ Biogeography of the Nine-Banded
6 American Leprosy Missions. “Angola: Armadillo,” December 7, 1999.
Facts.” Available online at http://www Available online at http://bss.sfsu.edu/
.leprosy.org/PROJangola.html geog/bholzman/courses/fall99projects/
7 Ibid. armadillo.htm
8 World Health Organization 16 Cole ST et al. “Massive gene decay in
International. “Global Leprosy the leprosy bacillus” Nature 2001, 409:
Situation.” Available online at 1007–1011
http://www.cefpas.it/fad/fad/
1global.htm CHAPTER 8:
ONGOING REFORMS
9 Ibid. AND THE FUTURE
10 World Health Organization 17 “Baba Changing the Lives of
International. “Weekly epidemiological Ostracized Lepers.” Available online at
record.” January 4, 2002. Available http://www.brightindia.com/mymite
online at http://www.who.int/ .html
docstore/wer/pdf/2002/wer7701.pdf
81
Bibliography
American Cancer Society. “Thalidomide Makes News Again: New Research
on How Thalidomide May Help the Cancer Patients.” October 6, 1998.
Available online at http://www.cancer.org/docroot/NWS/content/NWS
_1_1x_Thalidomide_Makes_News_Again.asp
American Leprosy Missions. “Angola: Facts.” Available online at http://www
.leprosy.org/PROJangola.html
“Baba Changing the Lives of Ostracized Lepers.” Available online at
http://www.brightindia.com/mymite.html
“Leprosy: History and Incidence.” Encyclopia.com. Available online at
http://www.encyclopedia.com/html/section/leprosy_HistoryandIncidence
.asp
Leprosy Mission International. Available online at http://www.leprosymission
.org
“Leprosy research in the post-genome era.” Available online at http://www
.lepra.org.uk/review/Mar01/pp8-22.pdf
Leprosy Sufferers Need Compassion. “Forum on Leprosy.” Available online at
http://www.webspawner.com/users/LEPROSY/
Lerner, Sharon. “Cases of leprosy on the rise in U.S.” The New York Times,
February 20, 2003, available online at International Herald Tribune,
http://www.iht.com/articles/87291.html
San Francisco State University. Department of Geography. “The
Biogeography of the Nine-Banded Armadillo.” December 7, 1999.
Available online at http://bss.sfsu.edu/geog/bholzman/courses/fall99
projects/armadillo.htm
World Health Organization International. “Elimination of Leprosy as a Public
Health Problem.” Available online at http://www.who.int/lep/
———. “Global Leprosy Situation.” Available online at http://www.cefpas.it/
fad/fad/1global.htm
———. “Weekly epidemiological record.” January 4, 2002. Available online at
http://www.who.int/docstore/wer/pdf/2002/wer7701.pdf
82
Further Reading
ADe Mallac, M.J. Hansen’s Disease: The Shared Paradigm: East Sussex,
England: Book Guild, Ltd, 2001.
Donnelly, Karen. Leprosy. New York: Rosen Publishing Group, 2001.
Farrow, John. Damien the Leper. Image, 1998.
Gaudet, Marcia. Carville: Remembering Leprosy in America. Jackson, MS:
University Press of Mississippi, 2004.
Job, C.K. Leprosy: Diagnosis and Management. New Dehli, India: Indian
Leprosy Association, 1975.
Kappor, P. Guide to Leprosy and Leprosy Control. India: J.M. Mehta, 1977.
Stewart, Richard. Leper Priest of Moloka’i: The Father Damien Story.
Honolulu: HI: University of Hawaii Press, 2000.
83
Websites
American Leprosy Missions
http://www.leprosy.org/
World Health Organization—Leprosy Information
http://www.who.int/lep/
Centers for Disease Control—Hansen’s Disease (Leprosy)
http://www.cdc.gov/ncidod/dbmd/diseaseinfo/hansens_t.htm
84
Index
Acid-fast, 39 Cell wall Humoral immunity,
Acquired immune defi- components, 38–43 46–47, 49, 79
ciency syndrome function, 40 Hyperpigmented, 56
(AIDS), 33 Cholera, 23 Hypopigmented, 56
discovery of, 9, 37 Clofazimine, 58 lesions, 23, 28
Acquired immunity, 46–47 Cytotoxic, 49
Adaptive immunity, 46 Immune system
AIDS. See Acquired Danielsen, Daniel components of, 47–51
immune deficiency Cornelius, 13 functions, 45–46
syndrome DDS. See Diamino response, 27, 45–49,
Anaesthetic, 23 diphenyl sulphone 51–55, 60, 75–76
Antibodies, 47, 49, 57 De Veuster, Damien weakened, 37
Antigen, 45, 47 Joseph (Father Immunity
presenting cells, 51 Damien), 23, 25 to leprosy, 9, 23, 53–54
receptor, 49–51 Diagnosis, leprosy, 10, types, 45–49
Apoptosis, 54 22, 56–57, 59 Indian Council for
Armadillos Diamino diphenyl sul- Medical Research, 73
advantages of, 66–70 phone (DDS), 58 Innate immunity, 46, 48
bacteria in, 31, 43, Diphtheria, 75 Infectious diseases
66–67, 70 Domack, Gerhardt, 58 types, 9, 33–34, 72, 75
Autoclave, 70 Influenza, 23
Endemic International Leprosy
Bacillus Calmette Guérin of leprosy, 10–11, 17, Elimination Program,
(BCG), 31, 75 33, 69 73
Bacteria Epidemic
colors, 42–43 of leprosy, 16, 33 Koch, Robert, 13, 39
and disease, 8–9, Escherichia coli, 64–65 Kushtha. See Leprosy
11–13, 23, 25, 27,
31, 35, 37, 39, 42, Faget, Dr. Guy, 58–59 Laminin, 51
44–46, 48, 51, 53, Leonine facies, 29
57, 60, 63–66, Gangrene, 9 Leper Act (1898), 19
69–71, 75–76 Genome Leprologist, 51
resistant, 41 sequencing, 69–71 Leprosy
structures, 35, 38–42, Gram, Christian, 42 around the world, 21,
70 Gram staining method, 31–34, 73
Bailey, Alice Grahame, 39, 42–43 causes, 8, 12, 19, 23,
18–19 Granuloma, 29 31, 35–44, 56, 69
Bailey, Wellesley, 18–19, characteristics, 33–34,
58 Hansen, Gerhard 57
BCG . See Bacillus Armauer complications, 11
Calmette Guérin and the discovery of control of, 56–63
Bioinformatics, 69 leprosy, 12–13, 19 discovery of, 13
Biopsy, 56–57 Hansen’s disease. See history of, 8–20
Borderline leprosy, 29, 54 Leprosy social stigma, 8, 10, 12,
Hemorrhagic fever, 34 14–17, 21, 33–34,
Cell mediated immunity Host-pathogen interac- 60, 63, 72–73, 77
(CMI), 46–47, 52, 54–55 tions, 45–55, 70, 76 types, 27–30, 53–54
85
Index
Leprosy Mission Pasteurization, 37 risk, 72
International, 19 Paucibacillary leprosy, 29 unsanitary conditions
Lepromatous leprosy, 27, PCR. See Polymerase and, 9, 23
29–30, 53–54 chain reaction Treatment, leprosy, 8, 13,
Little Flower Institution, Peptidoglycan, 40, 43 72
72–73 Pertussis. See Whooping chaulmoogra oil, 17–18,
Lymphocytes, 50 cough 20, 57–58
T cells, 49, 51, 54–55 Phagocytosis, 49 drugs, 10–11, 21, 31,
Polymerase chain reaction 58–60, 63, 67,
Macrophages, 46, 48–49, (PCR), 34, 57 76–77
51–52 Prevention, leprosy history, 17–20
Major histocompatibility education, 72–74 isolation, 23
complex (MHC), 51 research, 75–77 multi-drug, 58–59, 63,
Malaria, 6, 33–34 Prokaryote, 35 75
Meningitis, 6 Promin, 59 and recovery, 22
MHC. See Major histo- Pseudogenes, 70 and research, 57–63,
compatibility complex 67, 69–71, 73
Morbidity Ribosomes, 35 Tuberculoid leprosy,
of leprosy, 77 Rifampicin, 58 27–30, 53–54
Mouat, Dr. J.F., 18 Tuberculosis, 33–34, 39,
Multibacillary leprosy. See Schwann cells, 51–54, 70–71 75
Lepromatous leprosy Signs and symptoms, causes and transmis-
Mycobacterium leprae leprosy, 8, 21, 57 sion, 8–9, 13, 35,
in the body, 12, 45, anaesthesia, 9, 11, 23, 37, 39
51–55 29 symptoms and treat-
characteristics, 37–39, blindness, 27 ment, 39
42–44, 64–71 deformities, 9, 27, 31, Typhoid fever, 75
discovery, 13, 31 59–60, 63, 72, 77
and leprosy, 8, 18–19, hypopigmented spot, Vaccines
22, 31, 37–39, 63, 11, 23–24 and leprosy, 31, 44, 67,
69–71, 75 muscle weakness, 25, 71, 73–76
targets of, 51 27, 29 Viruses, 45, 47
nerve damage, 9, 24–25,
National Institute of 27, 29–30, 55–56, Whooping cough
Health (NIH), 73 63, 77 (Pertussis), 33
Neuropathy, 29 rash, 10–12, 27–29, 60 WHO. See World Health
Organization
Obligate intracellular Thalidomide, 60 World Health Organiza-
parasite, 44 Thymus, 47 tion (WHO)
Osmotic lysis, 40 Transmission, leprosy, efforts of, 11, 31–33,
22–23, 69 58, 73, 75
Paleobiologists, 41 history, 16
Paleo-epidemiologists, 41 infected soil, 23 Zeihl-Neelsen staining
Papule, 29 respiratory, 8–9, 11, 22 method, 39
86
Picture Credits
14: © Werner Forman/Art Resource, NY 43: © Jack Bostrack/Visuals Unlimited
20: National Library of Medicine 48: © Peter Lamb
24: © CORBIS 50: © Dr. David M. Phillips/Visuals Unlimited
26: © Emmanuel Kwitema/Reuters/CORBIS 53: © Peter Lamb
28: © WHO/P. Virot 59: © Bettmann/CORBIS
36: (top and bottom) © Dr. Dennis Kunkel/ 61: © Peter Lamb
Visuals Unlimited 62: © Bettmann/CORBIS
38: Courtesy Public Health Image Library 68: © Philip Gould/CORBIS
(PHIL), CDC 71: © Peter Lamb
40: © Peter Lamb 74: © Najlah Feanny/CORBIS SABA
87
About the Author
Alfica Sehgal, Ph.D., is currently completing her postdoctoral research at
Johns Hopkins Medical School in Baltimore Maryland. She received her
Ph.D. in molecular parasitology from Tata Institute of Fundamental Research
in Mumbai, India. She received her first postdoctoral research training at Yale
University School of Medicine in New Haven, Connecticut. During her Ph.D.
and post-doctoral training, she performed extensive molecular level research
on parasites like Plasmodium and Toxoplasma. Sehgal is interested in study-
ing the basic biological mechanisms in different organisms and applying that
knowledge to improve pharmacological and diagnostic methods.
88