Leprosy

Background:

Hansens Disease

By Eric Chow and Tania Roman

M & I 103: Parasites & Pestilence

Dr. Scott Smith; Spring 2008

INTRODUCTION

Mycobacterium leprae, the bacterium that causes leprosy was discovered in 1873 by Gerhard Henrik Armauer Hansen (Bacteria Genomes 2008). Also known as Hansens disease, leprosy has a long history of associated stigma, which prevents people from seeking medical treatment and often thwarts public health intervention to curb further spread of the disease. For this reason, public health interventions should include efforts to reduce stigma via an educational awareness program and training of local health officials. Our project aims to establish special skin ailment units within existing clinics in Londrina City of Brazil with the goals of diagnosing, treating and educating the general population of the city.

BACKGROUND

Leprosy is a human disease caused by the bacillus Mycobacterium leprae (Figure 1). M. leprae is an acid-fast bacterium. As one of the slowest growing bacteria known and its inability to grow independently, successful in vitro cultivation has never been achieved. Although found in the same genus as the tuberculosis bacterium (Mycobacterium tuberculosis), the two diseases cause different symptoms.

Pathology

It is hypothesized that M. leprae infects a new host by way of skin or upper respiratory tract, but most experiments suggest the latter as the more likely possibility (Shepard 1960). M. leprae causes a chronic disease of the peripheral nerves, skin and mucosal membranes of the body and has an incubation period of about 3-5 years (Hart 2003). If initial symptoms are left untreated, then permanent damage may result in many parts of the body including the eyes and outer extremities.

Fig. 1 The above picture shows the M. leprae bacteria in human tissue.

The severity of the disease can exist on a spectrum of symptoms depending on the hosts immune response, genetics and the number of bacteria that initially infect the body. In tuberculoid or paucibacillary leprosy (PB), rashes develop on a few spots of the body. These spots are often flat and white in color with a characteristic numbness due to the nerve damage caused by the bacteria. The more severe form of the disease is known as lepromatous leprosy or multibacillary leprosy (MB) (Figure 2). The infected individual will often develop similar rashes to those with tuberculoid leprosy except that these rashes are often raised and may be characterized as bumps. Patients with this form of leprosy may also experience numbness and weakness to entire muscle groups. The third form presents a combination of symptoms intermediate to both tuberculoid and lepromatous leprosy, hence the name, borderline leprosy. Without treatment, the disease can either improve to a more tuberculoid form or advance to the lepromatous symptoms (Leprosy: Merck 2003). Skin rashes may become permanent and lead to some cases of

Fig. 2 Patient with multiple white rashes that suggest an MB leprosy infection.

face disfigurement. Further damage to the nerves may lead to severe weakening of muscle group control of the hands and feet also known as clawed hands or foot drop.

It was once thought that it was the disease that causes limbs to fall off, however this is not the case. As the disease progresses, symptoms become more severe, leading to the loss of sensation of the outer extremities due to peripheral nerve damage. Additionally, the patient cannot feel pain or temperature making cuts and burns easy to go unnoticed. Constant damage to these areas may ultimately cause the loss of these parts (Figure 3). Infection can also affect the eye, and if untreated, blindness may occur. Feet sores (Figure 4) are also common symptoms as well as damage to the nasal passages.

Diagnosis of leprosy can be easily done through the recognition of the characteristic rashes and the accompanying numbness. Blood tests and the culturing of bacterium are unsuccessful due to the inability of being able to culture these bacteria in vitro. Due to the high number of bacteria in lepromatous leprosy infected individuals, skin lesion and nasal secretion smears can be taken and viewed under the microscope. In a laboratory setting, samples of the bacteria can be amplified using polymerase chain reactions (PCR) and M. leprae specific primers to confirm the identity of the bacterial infection.

Fig. 3 Leprosy patients hands that have been repeatedly damaged due to loss of nerve sensation on the extremeties.

Treatment has been complicated due to the emergence of drug-resistant strains of M. leprae. Until recently, dapsone was used as the main drug to cure infections of M. leprae, however the World Health Organization (WHO) recommends the use of multi-drug therapy (MDT) especially for those with the MB form of the disease. In adults with PB leprosy, the recommended dosages are 600 mg of Rfampicin once a month and 100 mg of Dapsone daily for a duration of 6 months. For MB patients, the dosage is recommended at 600 mg of Rifampicin once a month, 100 mg of Dapsone daily, 300 mg of Clofazimine once a month and 50 mg of Clofazimine daily for 1 year (WHO recommended 2008). There are reported side effects to these drugs and patients should be aware that these symptoms typically diminish a couple of months after treatment has stopped.

Fig. 4 Patient developing a foot sore caused by the damage from leprosy infection.

Epidemiology

Very little is known about the disease in its natural habitat. It is suspected that the armadillo may be a reservoir for the disease however studies have shown that the bacteria can also infect non-human primates and mice. Leprosy is endemic worldwide however most of todays cases of leprosy infections are found in the developing countries, with the highest prevalence in India. Brazil, Myanmar and Nepal also continue to have a large

Fig. 5 Map of the prevalence of leprosy worldwide in 2007.

number of cases. At the beginning of 2006, the WHO recorded approximately 219,826 new cases of leprosy worldwide, which is a dramatic shift from 1993 when the number of new cases that year was about 590,933 (New case detection 2005). In Brazil, there were 43, 933 newly detected cases of leprosy in 1998 with a total of 72,953 total cases (Cassandra, 2002). Continued public health efforts have decreased the prevalence worldwide, however there continues to be locations that have not been reached.

Leprosy and Stigma

One of the salient characteristics of leprosy is its associated stigma and the effects it has on early detection and adherence to treatment. Since Medieval Europe, leprosy has been the cause of the outcast of many of its carriers, which were forced into leprosy isolation centers. Many centuries after, in contemporary Brazil the stigma and belief system surrounding leprosy is still very well ingrained in society. Because our proposed health intervention aims at reducing the stigma associated with leprosy, it is important to understand the elements of Brazilian culture that influence patient experience and shape popular knowledge and belief about leprosy.

In Brazil, there a number of commonly held beliefs about leprosy, particularly in the way it is transmitted. Many people believe that leprosy is highly contagious and that it can be acquired through casual contact, from having visited a leprosarium or from a sexual encounter. Other beliefs associate leprosy with unsanitary conditions, dogs, or eating fish. Leprosy is also commonly known as the falling of limps disease and is thought to be incurable. Moreover, the Catholic church and the Evangelical religions strongly reinforce the idea that leprosy is a form of divine punishment. On the other hand, Afro-Brazilian religions look at leprosy as the result of sorcery or witchcraft (White 2002). Also, poverty increases the susceptibility to leprosy since a high level of inequality and population growth gives rise to over-crowding, which facilitates aerosol transmission of M. leprae. In Brazil, a significant percentage of leprosy patients live in favelas, or slums, thus perpetuating the association of leprosy with poverty and augmenting the stigma associate with this condition (White 2005).

Given the negative connotations that many of the folk beliefs about leprosy carry, many people are extremely hesitant to either seek treatment once they suspect leprosy, or to continue treatment once they start experiencing side effects that might expose their condition to the public. This has serious medical implications because this is the main cause of morbidity amongst leprosy patients since the loss of sensation results in

Fig. 6 During or after MDT, many leprosy patients experience a leprosy reaction, which is not a side-effect but the bodys own natural immune response to MDT. Nonetheless, this reaction often times discourages people from continuing treatment.

inevitable injuries to the body. Furthermore, stigma strongly influences compliance with treatment, especially since Multi-drug therapy has many side effects, which indicate that the person has leprosy. The drug Clofazamine, for example, causes the skin to become darker on in darker people, it acquires a darker reddish color, which is easily identified as leprosy. For these reasons, health interventions that address the issue of stigma caused by leprosy in a cultural specific context will be highly efficacious in dealing with the factors that preclude early leprosy detection and adherence to treatment (White 2007). Leprosy Control Precedence

Due to leprosys high associated mortality and morbidity, there have been a number of collaborative efforts geared towards its control and abatement. One of the most recent steps towards this goal was in 1991, when the World Health Assembly called for a global effort to eliminate leprosy as a public health problem by the end of the second millennium (Visschedijk et al, 2000). Elimination was defined as a level of prevalence below one case per 10,000 people. One important element of this worldwide effort was the Leprosy Elimination Campaigns (LECs), which trained health workers in case finding, educated communities in order to increase awareness, and performed active case finding and patient treatment.

These campaigns had considerable success in many countries, especially since the diminishing stigma associated with leprosy resulted in a better outlook for patients (Naff, 2006). In 1997 and 1998 LECs were held in 29 Indian States and Union Territories. More than 500,000 health workers were involved and 454, 290 new cases were detected (Visschedijk et al, 2000). Similarly in Nepal more than 11, 000 new cases were identified during the 1998 national campaign, while the incidence number the previous year was around 7,500 (Ministry of Health Nepal Department of Health Services 1998).

Furthermore, case studies have shown that community health education programs that focused on reducing stigma and increasing acceptance of leprosy are effective in promoting more favorable attitudes towards leprosy. For example, in Bangladesh lower levels of prejudice towards leprosy were observed in a rural community which had received community education as compared to the another rural community without the health education program. In the village that did not receive health education, over 75% of the families reported that they would not share a meal with a leprosy sufferer and 94% cited that they would not permit their son to marry a girl who is cured of leprosy (Wong and Subramanian, 2003).

Also, culture-specific health education programs, such as the one implemented in Malaysia in the late 1980s, have been found to increase acceptance of leprosy messages and improved the publics knowledge and attitudes towards leprosy. Other countries have been working with traditional and religious healers to provide for leprosy patients, most likely because many patients consult them first before seeking treatment from western health-care systems. In fact, a study in Nigeria showed 59% of the patients consulted the folk-medicine sector as the first step in their health seeking routine. Moreover, use of the mass media to change attitude of the masses towards leprosy increase case detention in Sri Lanka in 1995 (Wong and Subramanian, 2003).

Other health interventions took place in India, when after the advent of the Multi-drug Therapy (MDT) in 1982, the National Leprosy Eradication Program (NLEP) was launched in 1983 with the objective of arresting the disease in all known cases of leprosy. However, coverage with MDT remained low due to a range of organizational issues, and a fear of the disease and associated stigma (NCMH, 2005). Nonetheless, other national efforts in India, such as The Bombay Leprosy Project, which was launched more than 20 years ago, have been successful in using therapeutic management for the prevention of disabilities. The projects administrators attributes its success to its reduction in the social stigma in the patients families and the education of the community (Ganapati, 2003).

These case studies shed light on the importance of reducing stigma associated with leprosy in order improve the effectiveness of other health interventions. Innovated approaches, like the extensive training of community health workers, need to be implemented to improve community awareness and participation. In this manner, suspected leprosy cases do not fear stigma and report to a health facility at the earliest for timely diagnosis and prompt treatment (NCMH, 2005). Project Location

Because a reduction in stigma associated with leprosy would be extremely beneficial in an area with high leprosy endemicity such as Londrina, located in the state of Parana in Southern Brazil, we chose this city as our project location. Londrina is the second largest city in the state and counts with about half a million habitants. Most importantly, descriptive study aimed to evaluate the leprosy control program in Londrina from 1997 to 2001 showed that these programs lacked and failed to provide information about leprosy, and had a significant patient dropout rate (Barro, 2004). Therefore a pilot program would be helpful in addressing the weaknesses of previous health interventions via a cultural sensitive approach.

PROJECT DESIGN

The project will initially begin with a special skin ailment clinic in Londrina City, Brazil. We will gather information by creating a focus group in order to gain a better understanding of commonly held beliefs surrounding leprosy. From this information, the initial group will be able to fine tune the project to meet the cultural needs of the people. Additionally, local health officials and volunteers will be trained in diagnosing and prescribing the medication necessary to treat patients so as to ensure the future sustainability of the program.

Clinic

In order to eliminate the stigma that might result from establishing a clinic only treating leprosy, we will open a special skin ailment unit aimed at joining one of the existing community health centers or hospitals in the area. The clinic will be run by the medical personnel that have already been trained in diagnosing leprosy. Although leprosy is not a skin disease, its early clinical signs consist of superficial rashes of the dermis. Thus by opening a general skin clinic, patients can feel at ease when seeking medical care without fear of going into a clinic specific for leprosy. This clinic will provide free diagnoses and treatment for those with leprosy. MDT is provided for by the WHO at no cost.

To better integrate the program into the local public health services and to encourage community involvement, we will train local health officials and volunteers as the primary caregivers for the skin unit. Training will be feasible to be achieved because the diagnosis of leprosy does not require special tools or skills. Often times, the disease can be recognized through the initial skin rashes and lesions and treatment can be prescribed thereafter. Thus training will not only involve the WHOs PowerPoint presentation on the diagnosis of leprosy, but also clinical training by shadowing the group of health professional and medical providers during daily rounds.

By the end of this initial pilot program, we hope to use the information gathered from the focus group to better cater towards the needs of the community. What we will learn in these initial 6 months of the program will allow us to better answer misguided questions about leprosy and further reduce stigma by reaching out to people throughout the entire city. The information gathered from our research may also allow us to personalize our teaching techniques allowing for a more thorough impact on the community at large. Through the education and continued training of local volunteers, such a campaign to eliminate leprosy can be sustained. In the future, we hope that we will be able to expand these skin ailment units so that people will have easy and free access to have their leprosy infections diagnosed and treated.

Work Referenced

1. Bacteria Genomes MYCOBACTERIUM LEPRAE. EMBL-EBI Website.2008. Available at: http://www.ebi.ac.uk/2can/genomes/bacteria/Mycobacterium_leprae.html Accessed: 18 May 2008.

2. Barro, M.P.A.A. Assessment of the status of leprosy in the city of Londrina-PR from 1997-2001: epidemiological operational and organization aspects. 2004. Hansenologia Internationalis. 20:2:110-117.

3. Bhattacharya S.N., Sehgal V.N. Leprosy in India. 4 March 1999. Clinics in Dermatology. 17:2:159-170.

4. Burden of Disease in India. NCMH Background Papers. Ministry of Health & Family Welfare. Available at: www.who.int/macrohealth/action/NCMH_Burden. Accessed 12 May 2008.

5. Ganapati R, Pai VV, Kingsley S. Disability and management in leprosy: A field experience. Indian Journal of Dermatology, Venereology and Leprology. 69:6:369-374.

6. Feenstra, Pieter. Strengths and weaknesses of leprosy-elimination campaigns. 17 June 2000. The Lancet. 355:9221:2089-2090.

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13. Leprosy: Management of Complications. World Health Organization Website. 2005. Available at: http://www.wpro.who.int/sites/leprosy/treatment/treatment_complication.htm. Accessed: 18 May 2008.

14. Naafs, Ben. Viewpoint: Leprosy after the year 2000. June 2003. Tropical Medicine and International Health. 5:6:400-403.

15. New case detection trends in leprosy. World Health Organization Website. 2005. Available at: http://www.who.int/lep/situation/NCDetection2006.pdf. Accessed: 19 May 2008.

16. Shepard, Charles. Acid-fast bacilli in nasal excretions in leprosy, and results of inoculation of mice. 1960. Am J Hyg 71: 147-57.

17. Smith, D Scott. Leprosy. eMedicine Website. 2006. Available at: http://www.emedicine.com/med/topic1281.htm. Accessed: 19 May 2008.

18. Visschedijk j, Broek J, Eggens H, Lever P, Beers S, Klatser. Review: Mycobacterium leprae-millenium resistant Leprosy control on the threshold of a new era. June 2000. Tropical Medicine and International Health. 5:6:388-399.

19. White Cassandra. Explaining a Complex Disease Process: Talking to Patients about Hansens Disease (Leprosy). 2005. Medical Anthropology Quaterly. 19:3:310-330

20. White, Cassandra. Iatrogenic stigma in outpatient treatment for Hansens disease (leprosy) in Brazil. Health Education Research-Theory & Practice. 17 Jan 2007.

21. White, Cassandra. Sociocultural considerations in the treatment of leprosy in Rio de Janeiro, Brazil. 28 August 2002. Lepr Rev 73:356-365.

22. WHO recommended MDT regimens. World Health Organization Website. 2008. Available at: http://www.who.int/lep/mdt/regimens/en/index.html. Accessed: 19 May 2008.

23. Wong M.L, Subramaniam P. Social-Cultural Issues in Leprosy Control and Management. Available at: www.aifo.it/english/resources/online/apdrj/apdrj202/leprosy.pdf. Accessed 12 May 2008.

Hansens Disease Treatment. National Park Service Website. 2008. Available at: HYPERLINK "http://www.nps.gov/kala/historyculture/hansens2.htm" http://www.nps.gov/kala/historyculture/hansens2.htm. Accessed: 20 May 2008.

Smith, D Scott. Leprosy. eMedicine Website. 2006. Available at: HYPERLINK "http://www.emedicine.com/med/topic1281.htm" http://www.emedicine.com/med/topic1281.htm. Accessed: 19 May 2008.

Smith, D Scott. Leprosy. eMedicine Website. 2006. Available at: HYPERLINK "http://www.emedicine.com/med/topic1281.htm" http://www.emedicine.com/med/topic1281.htm. Accessed: 19 May 2008.

Smith, D Scott. Leprosy. eMedicine Website. 2006. Available at: HYPERLINK "http://www.emedicine.com/med/topic1281.htm" http://www.emedicine.com/med/topic1281.htm. Accessed: 19 May 2008.

Leprosy: Global Situation. World Health Organization Website. 2005. Available at: HYPERLINK "http://www.who.int/lep/situation/en/" http://www.who.int/lep/situation/en/. Accessed: 18 May 2008.

Leprosy: Management of Complications. World Health Organization Website. 2005. Available at: HYPERLINK "http://www.who.int/lep/situation/en/" http://www.wpro.who.int/sites/leprosy/treatment/treatment_complication.htm. Accessed: 18 May 2008.

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