the leprosy problemin the world

17
Bull. Org. mond. Sante 19663, 34, 811-826 Bull. Wld Hith Org. 1 The Leprosy Problem in the World * L. M. BECHELLI I & V. MARTfNEZ DOMfNGUEZ 2 There is at present a lack of accurate data on the prevalence of leprosy in the different countries of the world, primarily because case-finding has not reached the desired level in many of them. The authors have attempted to provide more realistic figures, using infor- mation obtained from several sources and various criteria for calculating estimated pre- valence rates. In all there are 2 831 775 registered patients and 10 786 000 estimated cases; the latter figure may well be an underestimate. The number of treated patients is about 1 928 000, some 68% of the registered cases and 18% of the estimated. About 2097 million people live in areas with prevalence rates of 0.5 per 1000 or higher; in these areas nearly one million new cases of leprosy can be expected within the next five years. The estimated number of disabled patients is 3 872 000, of whom 1 961 000 are in disa- bility grades 2-5 (excluding anaesthesia to pain). The data represent an attempt, made with many reservations, to give an indication of the magnitude of the leprosy problem throughout the world. GEOGRAPHICAL DISTRIBUTION OF LEPROSY: REGISTERED AND ESTIMATED NUMBER OF PATIENTS The data available on the prevalence 3 of leprosy in most countries do not represent the real situation, because case-finding has not reached the desired level in many countries. As a result, whereas for some countries the official rates indicate the approxi- mate prevalence of leprosy, for the majority the rates of registered and estimated cases are likely to be too low. On the other hand, the rates quoted for regis- tered and estimated cases may well be for an earlier date than that of the most recent population census: many countries have only a rough estimate of their populations. Furthermore, in some countries, the apparent prevalence is reduced because patients are discharged from case-lists as soon as the disease becomes ar- rested. In many others, inactive cases, especially if tuberculoid, are maintained on the active list for too * This article will also be published, in Spanish, in the Boletin de la Oficina sanitaria panamericana. 1 Chief Medical Officer, Leprosy, Division of Communi- cable Diseases, World Health Organization, Geneva, Swit- zerland. ' Medical Officer, Leprosy, Division of Communicable Diseases, World Health Organization, Geneva, Switzerland. ' Prevalence means the number of cases of a disease at a given date (point-prevalence) or in a given period (period- prevalence). The data given in Table 1 refer to point-pre- valence, per 1000 inhabitants. long. These facts and the different criteria used for releasing patients from control make it even more difficult to obtain accurate data on leprosy pre- valence. In presenting leprosy rates we have tried to obtain more realistic figures for the estimated number of cases and rates for each continent and each country, which are shown in Table 1. We wish to stress that these data represent only an attempt, made with many reservations, to give an idea of the magnitude of the problem throughout the world. The sources of information were as follows: (1) information given by various countries and WHO Regional Offices in reply to a questionnaire, (2) reports of visits to countries by WHO Head- quarters' staff and short-term consultants, (3) reports of the WHO Leprosy Advisory Team on the assessment of certain leprosy control projects, (4) data available in the literature and official reports published by governments or international organizations, and (5) reports of leprosy conferences and seminars organized by WHO. The method of estimation was as follows. The findings of the WHO Leprosy Advisory Team (LAT) in Africa (Northern Nigeria; North, Central and 1754 -811-

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Page 1: The Leprosy Problemin the World

Bull. Org. mond. Sante 19663, 34, 811-826Bull. Wld Hith Org. 1

The Leprosy Problem in the World *L. M. BECHELLI I & V. MARTfNEZ DOMfNGUEZ 2

There is at present a lack of accurate data on the prevalence of leprosy in the differentcountries of the world, primarily because case-finding has not reached the desired level inmany of them. The authors have attempted to provide more realistic figures, using infor-mation obtained from several sources and various criteria for calculating estimated pre-valence rates.

In all there are 2 831 775 registered patients and 10 786 000 estimated cases; the latterfigure may well be an underestimate. The number of treated patients is about 1 928 000,some 68% of the registered cases and 18% of the estimated. About 2097 million peoplelive in areas with prevalence rates of0.5 per 1000 or higher; in these areas nearly one millionnew cases of leprosy can be expected within the next five years.

The estimated number of disabled patients is 3 872 000, of whom 1 961 000 are in disa-bility grades 2-5 (excluding anaesthesia to pain).

The data represent an attempt, made with many reservations, to give an indication ofthe magnitude of the leprosy problem throughout the world.

GEOGRAPHICAL DISTRIBUTION OF LEPROSY:REGISTERED AND ESTIMATED NUMBER OF PATIENTS

The data available on the prevalence 3 of leprosyin most countries do not represent the real situation,because case-finding has not reached the desiredlevel in many countries. As a result, whereas forsome countries the official rates indicate the approxi-mate prevalence of leprosy, for the majority the ratesof registered and estimated cases are likely to be toolow. On the other hand, the rates quoted for regis-tered and estimated cases may well be for an earlierdate than that of the most recent population census:many countries have only a rough estimate of theirpopulations.

Furthermore, in some countries, the apparentprevalence is reduced because patients are dischargedfrom case-lists as soon as the disease becomes ar-rested. In many others, inactive cases, especially iftuberculoid, are maintained on the active list for too

* This article will also be published, in Spanish, in theBoletin de la Oficina sanitaria panamericana.

1 Chief Medical Officer, Leprosy, Division of Communi-cable Diseases, World Health Organization, Geneva, Swit-zerland.

' Medical Officer, Leprosy, Division of CommunicableDiseases, World Health Organization, Geneva, Switzerland.

' Prevalence means the number of cases of a disease at agiven date (point-prevalence) or in a given period (period-prevalence). The data given in Table 1 refer to point-pre-valence, per 1000 inhabitants.

long. These facts and the different criteria used forreleasing patients from control make it even moredifficult to obtain accurate data on leprosy pre-valence.

In presenting leprosy rates we have tried to obtainmore realistic figures for the estimated number ofcases and rates for each continent and each country,which are shown in Table 1. We wish to stress thatthese data represent only an attempt, made with manyreservations, to give an idea of the magnitude of theproblem throughout the world.The sources of information were as follows:

(1) information given by various countries andWHO Regional Offices in reply to a questionnaire,

(2) reports of visits to countries by WHO Head-quarters' staff and short-term consultants,

(3) reports of the WHO Leprosy Advisory Teamon the assessment of certain leprosy control projects,

(4) data available in the literature and officialreports published by governments or internationalorganizations, and

(5) reports of leprosy conferences and seminarsorganized by WHO.

The method of estimation was as follows. Thefindings of the WHO Leprosy Advisory Team (LAT)in Africa (Northern Nigeria; North, Central and

1754 -811-

Page 2: The Leprosy Problemin the World

L. M. BECHELLI & V. MARTINEZ DOMINGUEZ

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Page 11: The Leprosy Problemin the World

THE LEPROSY PROBLEM IN THE WORLD 821

TABLE 2GEOGRAPHICAL DISTRIBUTION OF REGISTERED, ESTIMATED

AND TREATED PATIENTS

Leprosy patients

Continent TreatedNo. No.

registered estimated Number | of % ofregistered estimated

Africa 1 712 132 a 3 868 000 1 062 527 b 62.0 27.5

America 177 813 358 000 95 804 c 53.9 26.8

Asia 915 525 d 6 475 0yJ e 755 334 f 82.5 11.7

Europe 16 624 9 52 000 9 973 h 60.0 19.2

Oceania 9 681 33 000 4291 44.3 13.0

Total 2 831 775 10 786 000 1 927 929 ] 68.1 ] 17.9

a No information about 12 countries (see Table 1).b No information about 26 countries (see Table 1).c Information about 16 countries only (see Table 1).d Information about 26 countries only (see Table 1).c No information about Mongolia.f Information about 22 countries only (see Table 1).0 No information about Romania.h No information about Romania and USSR.No information about New Guinea.

South Cameroon); Asia (the Philippines; KhonKaen, Thailand; and Myingyan and Shwebo, Burma)in random-sample surveys showed that, even incountries with a fairly good case-finding programme,new cases amounting to 75 % of the number of regis-tered cases were detected. Clearly, this proportionwould be much higher in countries with poor case-finding. For this reason, and in order to makeallowances for unknown cascs, when we estimatedthe number of cases we added 75 %, 150% or 300%to the number of registered cases for countries with,respectively, satisfactory, fair or poor I case-findingprogrammes. In the few countries of Europe whereleprosy is still endemic, 25% was added to accountfor undetected cases.For countries for which information was incom-

plete or non-existent, the estimate was based on theprevalence rates of neighbouring countries.

In those countries in which one or more areas have

1 Satisfactory: case-finding with an adequate coverage ofthe population.

Fair: case-finding with partial coverage of the population.Poor: case-finding inadequate, or with very limited

coverage of the population, or in the initial phase, or non-existent.

been randomly surveyed by the LAT, we have takeninto account the prevalence rate obtained. We havealso accepted some estimates proposed by govern-ments, short-term consultants and others. Therefore,in Table 1 each figure for the estimated number ofcases also bears an indication of how the estimatewas obtained (see footnote to Table 1).The rate obtained for 1959, for instance, was then

related to the population of the country for 1963 asgiven in the Demographic Year Book of the UnitedNations. In this way the estimated number is up todate and refers to 1963 (unless otherwise stated inthe table).

It is fully realized that, for many countries, amargin of error is very likely. However, the attempthad to be made to give an idea of the magnitude ofthe problem in the world, thus allowing us to con-sider the population at risk of infection and thefrequency of disabilities, in order to provide a betterapproach to the leprosy problem from the epidemio-logical, human and socioeconomic aspects.With these reservations in mind, the number of

registered cases and the estimated prevalence in thecontinentswere calculated; these are shown inTa ble 2

Page 12: The Leprosy Problemin the World

822 L. M. BECHELLI & V. MARTINEZ DOMINGUEZ

It will be seen that there are, in all, 2 831 775 regis-tered patients and 10 786 000 estimated cases; thelatter may well be an underestimate. To give onlyone example, in 1938 Sorel estimated the number ofcases in French West Africa at 30 000, while in 1963the number of registered patients for the countriespreviously forming French West Africa reached550 384. A review of data published on manycountries led to a similar observation.About 2097 million people are living in areas of

the world with prevalence rates of 0.5 per 1000 orhigher and eventually they may be at risk of infection,which is obviously greater in the hyperendemic areas(see Table 3).

It is known that, among those exposed to leprosy,about 20% are particularly susceptible and a variablenumber of them will contract leprosy. Actually, manyare already infected and, because of the long incu-bation period, will show signs of the disease invariable periods of time. For example, of the childhousehold contacts of lepromatous patients studiedby Dharmendra (1962) in Chingleput, 14% developedleprosy within four years. In the Philippines the ratewas 2.2% for all ages in a five-year period; the inci-dence in the age-group 5-14 years was twice as high(Guinto et al., 1954). This means that within fiveyears, among child household contacts of lepro-matous patients, a proportion between, say, 4% and14% can be expected to show signs of leprosy. Thusthousands of children will, in the near future, beleprosy sufferers.

According to a WHO estimate, the number ofleprosy cases expected in the next five years incountries with a prevalence rate of 0.5 per 1000 orhigher is 995 000, with the following distribution:

Africa 312 000America 26 000Asia 650 000Europe 3 000Oceania 4 000

The method of estimation was as follows:

" Data from the Philippines (Cordova and Talisay,Cebu Province; Doull et al., 1942) show that the pre-valence rate (all types of leprosy) is 15 per 1000, theincidence rate among household contacts is 5.33 per 1000person-years and the incidence among non-contacts is0.83 per 1000 person-years.We may assume that the incidence rate among house-

hold contacts does not vary according to the prevalencelevel of leprosy in the community but that the incidencerate among non-contacts is proportional to the prevalencerate of infectious cases.

About 25% of leprosy cases in Africa are infectious,while about 30% of leprosy cases in other continents areinfectious.[']"The computations are as follows:

1) For countries outside Africa:Contacts: estimated number of patients x (average

household size - 1) x 0.02665Non-contacts: (population- estimated no. of patients

rx average household size) x

- x 0.00415

(where r = estimated prevalence rate).2) For countries in Africa:

Figures obtained by the formulae above should bemultiplied by 5/6.For a number of countries information on the average

household size was not available. In such cases theinformation from similarly situated countries has beenused to arrive at the estimate."

TREATED CASES IN THE WORLD

According to the information set out in Table 1and summarized in Table 2, the number of treatedpatients in the world is about 1 928 000. This figurewould probably be much larger if information frommany countries were not lacking and it probably doesnot include patients already released from control.It has been the experience of WHO staff in manycountries that inclusion of cases under the heading" treated patients " very often does not mean thatthe patients are actually under treatment or are beingtreated regularly; in many countries it indicates thatpatients have received treatment over a varyingperiod.With these reservations, it can be concluded that

the number of patients treated in Africa and Americais relatively high in relation to the number of casesregistered (62.0% and 53.9 %, respectively) andreaches 27.5% and 26.8 %, respectively, of the numberof estimated cases. In Asia the number of cases

1 We should point out that the lepromatous rate in somecountries of Asia is higher than that in parts of Africa(4.58 per 1000 in Thailand; 7.11 per 1000 in Shwebo and6.28 per 1000 in Myingyan, Burma; 2.40 per 1000 in Came-roon and 2.08 per 1000 in N. Nigeria). " However, the pre-sence of high prevalence rates of leprosy combined with lowlepromatous rates in certain countries cannot be explained bythe assumption that lepromatous cases are the only sources ofinfection; the borderline group, reactional tuberculoid andsome indeterminate cases evidently have a certain degree ofinfectiousness" (WHO Expert Committee on Leprosy,1960). In the non-lepromatous cases this status may be onlytemporary. The proportion of lepromatous cases in tropicalAfrica is about 10%. " The continued maintenance of highprevalence rates in hyperendemic areas where tuberculoid-type leprosy constitutes as much as 90% of the total casesmay be attributed, at least in part, to the usually unde-termined proportion becoming " open " during periods ofreaction " (W-HO Expert Committee on Leprosy, 1966).

Page 13: The Leprosy Problemin the World

DISTRIBUTION OF LEPROSY IN THE WORLD

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Page 14: The Leprosy Problemin the World

THE LEPROSY PROBLEM IN THE WORLD 823

treated is high in relation to those registered (82.5 .),but low in relation to the estimated number of cases(11.7 %O). The proportion is also low in Oceania, butis higher in Europe. Of the total number of estimatedcases, at least 17.9% have been treated, regularly orotherwise.

TOTAL POPULATION OF COUNTRIES GROUPEDACCORDING TO THE ESTIMATED PREVALENCE OF

LEPROSY (0.5 PER 1000 AND HIGHER)

On the basis of the estimated prevalence of leprosyin each country (Table 1), we have provided ananalysis, in terms of prevalence rate (for rates of 0.5per 1000 and higher), country by country (Table 3)and for total population (Table 4) (see also the map).

DISABILITIES IN LEPROSY PATIENTS

The problem of disabilities in relation to leprosycontrol has increased in importance and has receivedspecial attention in recent years. Apart from thehuman aspect, it is also realized that patients withdisabilities represent a significant loss of manpowerfor many countries where leprosy is endemic and,as is known, prevalence rates are usually higher indeveloping countries, which are in particular need ofcapable men to raise the economic level, the standardof living and the level of education of the community.Again with many reservations, we have attempted

to estimate the number of disabled patients all overthe world in order to provide figures that will allowan appraisal of the socioeconomic implications ofleprosy. To estimate the number of disabled patientsthe following methods have been used.In those countries in which the Leprosy Advisory

Team has worked, the percentage of disabilities'found in a representative sample of patients has beenapplied- namely,

Argentina (1964) 35.85%Burma (1963) 48.7 %Cameroon (1961) 35.6 %Nigeria (1960) 23.4 %Philippines (1963) 32.22%Thailand (1962) 41.46%

In some countries, e.g., India and Spain,2 forwhich data on the frequency of disabilities are

I Disabilities were classified according to the recom-mendations of the WHO Expert Committee on Leprosy(1960), details of which are given in the annex.

'India: Wardekar (1962)-20% disabilities.Spain: Gay Prieto & Contreras Duenas (unpublished,

1959)-15.4 % disabilities.

available in the literature, the percentage of disabledpatients found by national leprologists has beenapplied to each country. Since these percentagesincluded only disabilities in Grades 2-5, we haveadded the percentage for Grade I (anaesthesia topain) found in the LAT surveys (17.69% on average).

In African, Asian and American countries, theaverage percentage of disabled patients found by theLAT in the respective areas (29.5%, 40.79% and35.85 %) has been used. In European countries wehave adopted the same percentage as that found inSpain (33.09 %), and in Oceania the percentage foundby the LAT in the Philippines (32.22 %).On this basis, the disability figures for the five

continents are as shown in Table 5.Therefore, of the total estimated number ofpatients

in the world, 3 872 000 could have disabilities, ofwhom 1 961 000 might have them in Grades 2-5(excluding anaesthesia to pain). These figures couldbecome higher if early diagnosis and regular treat-ment are not carried out. It is not difficult to realizewhat this would mean in terms of loss of manpower.A decrease of effort in leprosy control projects or theinterruption ofsupport would substantially aggravatethe situation and cause distress to millions of humanbeings.

HUMAN, SOCIAL AND ECONOMIC IMPLICATIONSOF LEPROSY

The seriousness of the endemicity of leprosy inrelation to other diseases cannot be evaluated onlyin terms of the total number of patients or prevalencerates; the duration of the disease, the disabilities thatit causes and the human and social consequences tothe leprosy patients and their families must also betaken into account.The long duration of the disease, especially in

lepromatous cases, the frequency and persistence ofdisabilities, the normal life-span of tuberculoid,indeterminate and lepromatous cases without re-action, and the high cost of reconstructive surgerygive to leprosy a special position among diseases.Govemments are obliged to establish costly long-term programmes. Other economic implications ofleprosy have also been mentioned in the section ondisabilities. To complete the picture of the humanand social impact of leprosy the age-old prejudiceagainst the disease must be added; in human andsocial consequences, no other disease causes such areaction in the community and so much distress andunhappiness to patients and their families. This

2

Page 15: The Leprosy Problemin the World

824 L.- M. BECHELLI & V. MARTfINEZ DOMfNGUEZ

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Page 16: The Leprosy Problemin the World

TE LEPROSY PROBLEM IN THE WORLD

TABLE 4TOTAL POPULATION OF COUNTRIES GROUPED

ACCORDING TO ESTIMATED PREVALENCEOF LEPROSY a

Prevalence (per 1000) Total population

0.5- 1 153 757 000

1- 5 1 204 764 000

5-10 582 223 000

10-20 80 448 870

20-30 34 937 500

30-40 25 780 000

40-50 10 240 300

> 50 5 804 500

Total 2 097 955170

a For prevalence rates of 0.5 per 1000 or higher.

anxiety may follow leprosy patients and relativesthroughout their lives and cast a permanent shadow

TABLE 5ESTIMATED NUMBER OF DISABILITIES

DisabilitiesContinent

Grades 1-5 Grades_2-5

Africa 1 107 000 423 000

America 128 000 61 000

Asia 2 609 000 1 463 000

Europe 17 000 8 000

Oceania 11000 6 000

Total 3872 000 [ 1961 000

over their families and professional and social activi-ties. Fortunately, the situation is gradually changing- and WHO has been playing an important role inthis direction. Nevertheless, in varying degreesaccording to country and differing in rural and urbanareas- prejudice still persists which is not found toa comparable degree with any other disease.

FinexCLASSIFICATION OF LEPROSY DISABILITIES

(WHO Expert Committee on Leprosy, 1960)

A. Hands

Grade 1 - Anaesthesia to pain., 2- Mobile claw hand. Useful thumb.

3 - Intrinsic paralysis involving fingers andthumb, or fingers only but with con-tracture.

, 4- Partial absorption of the fingers but withuseful length remaining.

5 - Gross absorption. Stumps only left.

B. FeetGrade 1 - Anaesthesia.

2- Trophic ulceration (present or past).3 - Paralysis (foot drop or claw toes).

, 4- Partial absorption of the foot (up toone-third of surface areas of the solelost).

Grade 5 - Gross absorption (more than one-thirdof the foot lost).

C. FaceType I - A permanent mark or stigma of leprosy

not amounting to ugliness (loss of eye-brows, deformity of the ear).

2 - Collapse of nose.3 - Paralysis of the eyelids, including lago-

phthalmos or paralysis of the facialnerve.

4- Loss of vision in one eye or dimness ofvision in both eyes (can count fingers).

5-Blindness.

D. MiscellaneousType 1 - Gynaecomastia.

2 - Involvement of the larynx.

825

Page 17: The Leprosy Problemin the World

826 L. M. BECHELL & V. MARTINEZ DOMINGUEZ

ACKNOWLEDGEMENTS

The valuable co-operation of Mr K. Uemura and Mr T. Sundaresan of Health Statistical Methodology,Division of Health Statistics, World Health Organization, Geneva, Switzerland is greatly appreciated, particularlyin relation to the method of estimation of the expected number of leprosy cases within the near future (p. 822 et seq.).

RISUMtI

La situation r&elle de la lepre dans la plupart des paysne correspond pas a ce qu'indiquent les informationsrecueillies ou publiees concemant la pr6valence de I'affec-tion, le depistage n'ayant pas encore atteint partout leniveau souhaitable. Les auteurs ont tent6 d'etablir deschiffres refletant davantage la r6alit6 dans chaque conti-nent et chaque pays. Ils ont utilise a cet effet les informa-tions foumies par chaque pays et chaque bureau regionalen reponse ai un questionnaire, les rapports d'enqueteseffectu6es sur place par des membres du Secr6tariat del'OMS et des consultants ai court terme, les rapportsetablis par 1'Equipe consultative de l'OMS pour la 1lprerelatifs a 1'evaluation de certains projets de lutte contrela lepre, les donn6es puisees dans la litt6rature et dansles rapports officiels publies par les gouvemements etles organisations internationales, et enfin les rapports desconf6rences et des seminaires de l'OMS sur la lepre.

Les estimations ont tenu compte des observations del'Equipe consultative de l'OMS pour la lepre, en Afrique,en Asie et en Amerique, au cours d'enquetes portant surdes 6chantillons de population. 11 est certain cependantqu'une marge d'erreur est vraisemblable pour certainspays et les auteurs soulignent qu'en presentant les chiffresmentionn6s dans ce travail ils nWont fait qu'essayer dedonner une idee de l'ampleur du probleme dans le monde.En tenant compte de ces reserves et en se basant sur

certains criteres - c'est ainsi que 1'estimation du nombrede cas existant dans un pays a ete obtenue en multipliant

le nombre de cas d6clares par un coefficient variant sui-vant que ce pays a un programme de d6pistage satisfaisant,passable ou mediocre - le nombre de cas d6clar6s dansle monde est de 2 831 775 et celui des cas estim6s de10 786 000. Des tableaux presentent les chiffres de chaquecontinent et de chaque pays. Les nombres de cas d6clar6set estimes sont respectivement de 1 712 133 et 3 868 000en Afrique, 117 813 et 358 000 dans les Am6riques,915 525 et 6 475 000 en Asie, 16 624 et 52 000 en Europe,9681 et 33 000 en Oc6anie. Selon les informations reques,1 927 929 malades sont soign6s dans le monde. Mais denombreux pays n'ont pas fourni d'informations et lesmalades qui ont ete rayes du contr8le ne sont sans doutepas comptes; il est donc probable que les chiffres devraientetre beaucoup plus 6lev6s. Si l'on consid6re les cas declareset les cas estimes, les proportions de malades soign6s sontrespectivement de 68,1 % et 17,9%. Environ 2097 000personnes vivent dans des regions oui les taux de pr6va-lence sont egaux ou superieurs a 0,5 pour 1000. On pr6voitdans ces pays, au cours des cinq prochaines ann6es,995 000 nouveaux cas de lpre: 312 000 en Afrique, 26 000dans les Ameriques, 650 000 en Asie, 3000 en Europe et4000 en Oceanie.

Les auteurs ont 6galement tente d'estimer le nombre decas infirmes dans le monde; 3 872 000 malades presente-raient des infirmites qui chez 1 961 000 d'entre eux attein-draient les degres 2-5 (non compris I'anesthesie a ladouleur).

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