LEPROSY
A TALE OF TWO MEN

By G. Randolph Schrodt
Department of Pathology
University of Louisville School of Medicine

     “And as he was entering a certain village, there met him ten lepers who stood a far off and lifted up their voice crying, 'Jesus, Master, have pity on us.' And when he saw them, he said, 'go show yourselves to the priest.' And it came to past, as they were on their way that they were made clean.”

This passage from the gospel of St. Luke epitomizes the enigma, the controversy, the horror that surrounded the history of leprosy through the centuries. Before considering our two historical guests of this evening, we might briefly review something of the history of leprosy through the ages. There remains great controversy.

From Eastern writings there is good evidence that India, East Asia, and China are among the most ancient homes of leprosy. The earliest reference to leprosy which appears to be rather universally accepted was written in the Chou Dynasty in 6th century B.C.  In the Chinese medical classic entitled Nei Ching there are four passages which may allude to the leprous patient. If this classic was written by one Huang Ti, leprosy may have been recognized in China over five thousand years ago. The quotations from Nei Ching are rather interesting.

l. Those suffering from ‘ta feng’ have stiff joints, the eyebrows and beard fall off.

2. The winds scatters throughout the muscles and comes in conflict with the 'wei chi' or defensive force.  The channels being clogged, the flesh becomes nodular and ulcerated.  And because of the stagnant movements of this defensive force, numbness results.

3. The vital spirit degenerates and turns cloudy causing the bridge of the nose to change color and rot and the skin to ulcerate. The winds and chill lodge in the blood vessels and cannot be gotten rid of. This is called 'li feng.'

4. For the treatment of 'li feng', prick the swollen parts with a sharp needle; let the foul air out until the swelling subsides.

Even more convincing are the descriptions of leprosy found in complete secret remedies by Hua T'o born about A.D. l90. Hua T'o designates leprosy as Tai Ma Fung and notes symptoms may first appear in the skin but the poison is actually stored in the internal organs. The skin is first numb without; sensation, gradually red spots appear, and then becomes swollen and ulcerated with out any puss.  Later when the disease has extended to such an extent, the eyebrows fall out and the eyes become blind, lips deformed and the voice hoarse.  The patient may also find ringing sounds in his ears and under his feet rotted holes.  His finger joints may be dislocated and the bridge of his nose flat.

The origin of leprosy in the near east is even more controversial.

The Hebrew word saraath which was translated leprosy referred to a large number of skin conditions.  As used in the Bible, therefore leprosy did not necessarily denote Hansen's Disease as we know it today, but rather referred to any sort of stigma or blemish that marked a victim as unclean under Hebrew ritualist law. Consensus would be at least from the history of the Jewish people - that leprosy was unknown to them during their Egyptian exile, that those having leprosy in biblical times may indeed have suffered from Hansen’s disease but more likely suffered from anyone of a number of skin lesions and other diseases as well.

In the Septuagint which is the Greek translation of the Bible carried out about 200 B.C. by Jewish authors Zaroath was translated into the word

Lepra indicating a scaling skin disease perhaps akin to psoriasis. The

Greek word Lepra became the Latin word leprosus and ultimately the English leprosy. It is of interest that Hippocrates, born in 467 B.C., probably did not recognize leprosy.

Araetus, a contemporary Galen who lived about A.D. l50, has given the first accurate medical account of leprosy in Europe. Since the disease appeared unknown to Hippocrates, it is assumed that leprosy was introduced in the Mediterranean European countries sometime between 467 B.C. and A.D. l50.  From osteological studies leprosy is believed to have been present in Great Britain and France as far back A.D. 500 to 700. Between A.D, l000 and l400 the disease reached its height in Europe.  During this time it was said that France had 2000 lazar houses and Great Britain 326. Many of these lazar houses were established at the direction of or under the influence of the church.

By the end of the l8^th century, leprosy had died out in Great Britain largely due to the restrictions placed on the leprosy patients and their contact with the healthy community and to general improvement in the conditions of society.  Leprosy continued to be a problem in Norway in the l9^th century. Today there are still cases of leprosy in European countries including Spain, Portugal, the Baltic and Baltic states, European Turkey and European Russia.

It is believed that leprosy was introduced into the American continent first by Columbus soldiers and later by the slave trade from West Africa. Indigenous leprosy in this country remains in Texas, Louisiana, Florida and California.  In South America leprosy is a serious problem in Argentina

Brazil, Colombia and Paraquay.

JOSEPH DE VEUSTER

Joseph De Veuster was born on January 3,l840 in the Flemish district at Ninde-lez-Tremello in Belgium about l9 miles southeast of Antwerp.  Dr. De Veuster was the next to last of eight children born of Francis and Catherine De Veuster.  Little is known about his early life. He completed his initial schooling at the age of thirteen and for the next four years remained a helper on the family farm.  When he was eighteen it was decided that he take up the marketing of grain as did his father before him at Tremello.  Before beginning his apprenticeship, however, it was decided that he should learn french and he was sent to a school at Wallonia for this purpose.  During this time, Joseph De Veuster decided to enter the religious life of the Roman Catholic Church. On February 2,l859 he entered the congregation of Picpus and changed his christian name to Damien. Completing a four year period of training in the seminary the now Brother Damien applied for missionary work in the Hawaiian Islands.  On November 2, l863 he embarked from Bremerhaven on the R.W. Wood, a three-masted ship flying the Hawaiian flag.  After the customary sailing dangers of those days were encountered and overcome - including a voyage around Cape Horn, the R.W. Wood arrived in Honolulu March l9, l864.

De Veuster was ordained a priest on May 2l of that year. Shortly thereafter he was assigned to the district of Kohala on the Island of Hawaii.

Damien’s parish in the district of Kohala was 80 miles long and 30 miles wide.  During the eight years he spent there, he exhibited a remarkable degree of activity and endurance which would serve him well in his next assignment on the Island of Molokai.

Before considering the work of Damien among the lepers of Molokai, it might be of some interest to briefly review the history of leprosy in the Hawaiian Islands.  The exact date when leprosy first appeared in the

Islands cannot be exactly determined. Mai Pake, or Chinese sickness, is the word in the Hawaiian vocabulary used to define leprosy and it is generally believed that the Chinese introduced the disease into the islands.  Chinese did visit Hawaii in various ships before the year l830 and the first Chinese coolie immigrants from Hong Kong arrived in Honolulu in l852. One of the earliest description of leprosy in Hawaii is that given by the

Reverend Charles Samuel Stewart, who landed at Honolulu in l823.  An entry in his journal dated May 2l, l823 notes “not to mention the frequent and hideous marks of scourge, which more clearly than any proclaims the curse, of a God of Purity, and which while it annually consigned hundreds of these people to the tomb, converts thousands while, living into walking sepulchres.  The inhabitants generally are subject to many disorders of the skin; the majority are more or less disfigured by eruptions and sores found many are as unsightly as lepers.”

Honolulu was officially declared a city on August 3, 1850 and on

December l3, l850 the first Board of Health was organized by order of King Kamehameha III.  However, it was not until 1863 that the subject of leprosy came to be discussed by the Board of Health.  At that time it was noted that

Mai Pake, the Chinese disease, was rapidly spreading on Oahu.  On June l0, 1865 a suitable location for incurable cases of leprosy came up for discussion and the suitability of land on the north side of Molokai was considered.  The first shipment of lepers landed at Kalupap January 6,l866, the beginning of segregation and banishment of lepers to the leper settlement.  To this leper colony, Damien arrived seven years later, on May 10, l873, with a Bishop Maigret who was head of the catholic missions in the Hawaiian Islands.  Damien was 34 years of age when he began his work among the lepers.  Dr. A. Mouritz, who was a physician to the leper settlement on the island of Molokai, from l884 to l888 described Damien as active and vigorous, measuring 5’ 7" and weighing l64 pounds.  Interestingly enough, he notes that his chest measurements was 41 inches.

DAMIEN ON MOLOMI

Molokai lies at the center of the Hawaiian Archipelago.  On the north shore of Molokai the promontory of Kalawao pushes into the sea.  It was to this area of Molokai that the Hawaiian authorities chose to sequester the lepers.  From l866 to l873, 797 lepers were exiled. Three hundred and eleven of these had died by l873 when Damien arrived.

The number of lepers at Molokai during the years l873 to l889 ranged from 294 to 1166.  The number of deaths ranged from l0l per annum to 236.  When Damien arrived in Molokai, the plight of the lepers was miserable.

Food rations were minimal, housing deplorable, morale poor and medical care minimal.  Not surprisingly, this missionary priest devoted his energies not only to the spiritual care but to the temporal welfare of his leper flock. Until l878 the lepers were not provided with any doctors.  Only then did they put in occasional appearances remaining a few days among the sick and then leaving only to return several weeks later.  In the interim Damien served as hospital attendant and doctor.  The drugs used in the treatment of the disease were probably without effect, although administered with hope and enthusiasm. In l878 Damien heard of a drug called Hoang Nan Pills, a chinese remedy extracted from species of ivy.  In l878 he wrote to a fellow missionary “I received with joy beyond words the box of Hoang Nan Pills as well as your friendly letter, so full of encouragement and confidence.  I read it in translation to a large audience of lepers whom it filled with joy and hope. They have discussed the matter and raised a subscription to purchase

Hoang Nan in quantity."  Damien was a better missionary than a scientist. Fifteen months after beginning use of Hoang Nan Pills, he wrote “I did not wish to write to you sooner for I wish to be sure that the improvements due to your providencial medicament would be lasting. It has prolonged the life of many; it has restored the use of their limbs to others.”

DAMIEN STRICKEN WITH LEPROSY

It is not known precisely when Damien first developed symptoms of leprosy.  Beginning in l876, his arms and pack were covered with dry yellowish spots.  In the autumn of l881, he developed pain in his left foot and later an area of insensibility in his left foot.  Thereafter, the progress of the disease became very rapid.  Tubercles appeared on his left ear which became inordinately large, his eyebrows fell out and his hands and face were covered with swellings and sores.  Damien De Veuster died April l5, l889 and was buried at the foot of the tall pandanus tree on Molokai where he slept when he first arrived among the lepers.  In l936 the corpse of the leper priest was exhumed, transported first to Honolulu and then to Louvain, Belgium.

Damien De Veuster was a controversial person. He gained an unusual amount of worldwide notoriety for his pioneer missionary work in the care of lepers.  He was, in general, beloved by his leper friends and by people with whom he was closely associated.  He was however, described as brusque and obstinate, particularly when he spoke in behalf of the interest of the lepers. Among some of his detractors were his very superiors. Some of the comments made by his superiors were "He is a man almost completely wanting in judgement", "He needs a guardian", "He is ineffective as a priest.”   A succeeding provincial wrote, "He is an overbearing man, capricious and proud. He has led people to regard him as the consoler and the nurse, etc. of the lepers and he is nothing of the sort."  And again in l888 "The worst of all our missionaries was Father Damien who has been lifted so high by adulation that no one henceforth will succeed in getting him down to earth again.”

Perhaps the worst of Damien's detractors was another clergyman and Doctor of Theology, one C.M. Hyde, who on August 2, l889 wrote an open letter to Reverend H.B. Gage concerning Father Damien. The letter is so vitriolic that perhaps its worth reading in its entirety.

Dear Brother:

In answer to your inquiries about Father Damien, I can only reply that we who knew the man are surprised at the extravagant newspaper laudations as if he was a most saintly philanthropist.  The simple truth is he was a coarse dirty man, headstrong and bigoted.  He was not sent to Molokai but went there without orders, did not stay at the leper settlement before he became one himself, but circulated freely over the whole island, and he came often to Honolulu. He had no hand in the reforms and improvements inaugurated which were the work of our Board of Health, as occasion required and means were provided. He was not a pure man in his relations with women and the leprosy with which he died should be attributed to his vices and carelessness.  Others have done much for the lepers, our own ministers, the government physicians, etc. but never with a catholic idea of meriting eternal life.

Yours, etc.

C.M. Hyde  

After this letter, many came to the defense of Damien but none more notable than Robert Louis Stevenson.  Stevenson had not known Damien but had visited Molokai after Damien's death and talked with those who knew the missionary priest well.  Upon reading Hyde's letter, Stevenson addressed himself to Hyde February 25, l890 in a letter defending Damien. Stevenson's letter is lengthy and pointed. Several quotes from it bear witness to his admiration for the leper priest.  He addresses in this fashion: "for it is in the interest of all mankind and of the cause of public decency in every quarter of the world, not only that Damien should be righted but that you and your letter should be displayed at length in their true colors to the public eye.  To do this properly, I must begin by quoting you at large.  I shall then proceed to criticize your utterance from several points of view, divine and human in the course of which I shall attempt to draw again and with more specification the character of the dead saint whom it has pleased you to villify.  So much being done, I shall say farewell to you forever. And again your church and Damien's were in Hawaii upon rivalry to do well, to help, to edify, to set devine examples.  You having in one huge instance failed and Damien succeeded, by marvel it should not have occurred to you that you were doomed to silence, that when you had been outstripped in that high rivalry and set in glorious in the midst of your wellbeing, in your pleasant room and Damien crowned with glories and horrors toiled and rotted in that pigsty of his under the cliffs of Kalawao, you the elect who would not, were the last man on earth to collect and provicate gossip on the volunteer who would and did.”

GERHARD HENRIK ARMAUER HANSEN

Hansen was born in Bergen, Norway on July 29, l84l, the eighth of a family of l5 children.  His father, Claus Hansen was a wholesale merchant until the severe contraction of credit of l848 through l85l drove him into bankruptcy.  He then worked as a cashier in a bank.  Hansen in l859 began his medical studies of the University of Christiania (now Oslo).  He helped pay his tuition by first teaching at a girls school and spending one year as a prosector in the Institute of Anatomy.  In l866 he passed his degree with honors and completed his internship at the National Hospital in Christiania. That was followed by serving as a medical officer to the Lofoten Fishing Fleet, a group of islands off northern Norway.  In l868 Hansen entered the service of the Leprosy Hospitals in Bergen taking an appointment as a physician at the nursing home for lepers No. l. In August of the same year he accepted the post of assistant physician at the Lungegaard Hospital and later that of physician at the St. Jorgens Hospital.  The physician in charge of the Lungegaard Hospital was Daniel Cornelius Danielssen, a renowned leprosy researcher of the time.  Among Danielssen's notable works was one he co-authored with C.W. Boeck, published in l847, entitled "Om Spedalsked”.  Due in large part to the prevelance of leprosy in Norway at this time and to the notoriety of Danielssen and Boeck, Bergen was the leading center for leprosy research in Europe at this time.

When Armauer Hansen began his career in the leprosy hospitals of Bergen, 

 Opinions varied as to the cause of this disease. Many scientists including Danielssen were convinced that Leprosy was hereditary.  Others maintained that it was contagious.  Hansen soon took the position that Leprosy was contagious and thus stood in opposition to his master, Danielssen.  The story goes that on one encounter with Danielssen, Hansen said “and I may as well tell you that in my opinion your views on leprosy are altogether wrong.  You think the disease is hereditary, not contagious, whereas the truth is that it is contagious and not hereditary.”  While this outbreak might predictably have ended Hansen’s career it in fact enhanced it.  For although Danielssen still disagreed with his protege, he supported him.  It is of interest that shortly afterwards Hansen married Danielssen's daughter.

In 1870 Hansen spent a year in Bonn and Vienna applying himself to the study of pathologic anatomy and microscopy.  Returning In l87l to Bergen, he received a grant from the Doctor Egsberg Foundation permitting him funds to inquire into the causes of leprosy.  He spent two summers in the western part of Norway in areas with a high incidence of leprosy and then continued his research and experiments at the Lungegaard Hospital and the No. One Nursing Home for lepers.

The culmination of this work was the publication entitled "Investigations Concerning the Etiology of Leprosy" in which he described in detail the appearance of the lepra bacilli.

Hansen's methodology was simple.  His microscopic preparations were made from material removed from leprous nodules.  Most of his preparations were examined without benefit of stain although apparently with some osmic acid was used. The more refined stains of Koch, Weigert and Neisser were yet to be produced.  Quoting from Spedalskhedens Arsager, Hansen says "Depending on the age of the nodule, there are obtained preparations in which there are found besides blood either only round cells, fragments of capillary vessels, and small bundles of connective tissue or also larger cells and large and small brown elements. If the preparations are examined without any admixture there can be detected here and their rod shaped bodies, either at rest or at slightly oscillating movement.  When the cells are preserved entire, their number is small.  If now a drop of water is added to the preparation the rods move more quickly and little by little more and more rods appear; the older the nodule is the more numerous the rods become. He goes on to say "If a piece of fresh nodule is teased in a drop of one percent osmic acid or if the nodule is preserved in osmic acid and examined the next day or on the second day (later the preparations become less clear because of the strong staining), the rods lying within the cells which are colored more intensely by the osmic acid, then the remaining contents of the cells are detected with far greater ease than in fresh preparations.”

The significance of this simple scientific report was not immediately appreciated.  Probably not until Neisser confirmed these observations in l879-l880 with the use of more refined staining techniques did Hansen's finding gain any degree of notoriety.

It is of some interest that the lepra bacilli were probably seen by Danielssen back in the l850's as part of a “granular masses” associated with leprous nodules.  Danielssen, however, did not recognize these as bacteria. In l859 he showed the visiting Rudolph Virchow the brown granules obtained from the leprous nodules.  Virchow was not impressed and interpreted them as mere clumps of degenerating fat.  So it may be that if Rudolph Virchow had not visited Bergen in l859, leprosy today might have been called Danielssen's Disease instead of Hansen's Disease.

      Hansen for years attempted to infect a variety of experimental animals with leprosy and to cultivate the bacterium on artificial media-as we know without success.  Both he and Danielssen attempted to inoculate themselves periodically from material obtained from leprous patients, also without success.  In l879 without the recipient’s consent, he inoculated leprous material from one patient into the conjunctival sac of another patient afflicted with the maculo anesthetic type of leprosy.  When the recipient realized that she had been operated on by way of experiment without prior consent she complained to the authorities.  As a result Hansen was dismissed May 3l, l880 from his post as senior physician at the No. One Nursing Home for lepers.  However, he was allowed to continue as chief medical officer for leprosy in Norway.

 In time, honors came to Hansen for his memorable discovery.  He was elected honorary chairman of the First International Conference on Leprosy held in Berlin in l897 and was President of the second conference held in Bergen in l909.

Hansen suffered first symptoms of heart disease as early as l900 and in the following years he had several severe heart attacks.  During the periods of relatively good health he continued to travel around Norway.  In February he made such a trip to Floro, a fishing area north of Bergen and it was there that he died February l2, l9l2.

LEPROSY TODAY

Leprosy continues today to be a major health problem in some areas of the world.  Precise prevalence figures are difficult to obtain, especially in countries such as Russia and China, but there are an estimated twelve million to fifteen million cases in the world.  The great majority of these patients are in Asia and Africa but there are substantial numbers in South America.  In the United States, 244 new cases of leprosy were reported in l98l.  Most of these involve infections that were acquired outside the United States

Mycobacterium leprae was the first bacillus identified as the cause of a chronic disease in man and is the only bacillus causing human disease that has not been cultivated in vitro.  The organism can be grown in mouse footpads and in the armadillo.  More recently, a spontaneous case of leprosy has been detected in a sooty magabey, “ a non human primate", at the Delta Regional Primate Center in New Orleans.  The sooty mangabey is now the first non-human primate model for leprosy studies.  The use of the first two of these animal models has been of use in evaluation of therapy.

Dapsone, introduced in the late l940’s and early l950’s, has been a

most effective drug in the control of leprosy.  Now, however, there are an increasing number of reports of Dapsone resistance both primary and secondary. As a result, the chemotherapeutic regimes for leprosy are changing and now multiple drug therapy is the treatment choice.  For pauci-bacillary infection Dapsone and Rifampin are used.  For multibacillary infections Rifampin, Dapsone and Clofazimine are used.

It has long been appreciated that there are two polar forms of leprosy with several intermediate forms.  At one extreme is the lepromatous form characterized by anergy to the lepra antigen and a failure of cell mediated immunity.  At the other end of the spectrum is the tuberculoid form of the disease characterized by hypergy to the lepra antigen and intact cell mediated immune system to the bacillus.  In the lesions of lepromatous leprosy helper T-cells are absent or markedly decreased.  While in the tuberculoid form, the predominant T cell in the lesions is the helper T cell.  There is a predominant view today that the particular variety of leprosy appearing in a given patient is genetically determined.

REFERENCES

1. Mouritz, A. (1943) A Brief World History of Leprosy. Published by A.      Mouritz 

2. Cochrane, R. G. and Davey, T.F. (l964) Leprosy in Theory and Practice, 2nd Edition, Baltimore: The Williams and Wilkins Company.

3. Engelbert, O. (1977) The Hero of Molokai.  Boston:  St. Paul Editions

4. Feldman, W.H. Gerhard Henrik Armauer Hansen. What did he see and when?  Intl. J. of Leprosy 33:412-416 (1965)

5. Rokstad, I., Gerhard Henrik Armauer Hansen. Intl. J. of Leprosy 32:64-70 (l964)

6. Baillie, R.A. and Baillie, E.E. Biblical leprosy as compared to present day leprosy.  Southern Med. J. 75:855-857 (l982)

7. Binford, C.H., Meyers, W.M. and Walsh, G.P. Leprosy JAMA 247:2283-2292 (1982)

8. Shepard, C.C. Leprosy today (Ed.) N. Eng. J. Med. 307: 1640-1641 (1982)