- Email: [email protected]
Rehabilitation of leprosy-affected people: An overview
Clinics in Dermatology (2016) 34, 66–69
Rehabilitation of leprosy-affected people: An overview J. Wim Brandsma, PhD ⁎ Independent leprosy—hand rehabilitation consultant, Hoevelaken, 3871 HG-18, The Netherlands
Abstract Leprosy is mainly a disease of the skin and nerves. The involvement of nerves may lead to impairments that have, contributed to the fact that leprosy is often still seen as a disease to be feared. In non–leprosy-endemic Western countries, beliefs continue to prevail about the inevitability of deformities and the lack of a cure. I review the pathogenesis of deformity and to present a discourse on how deformities can, to a very large extent, be prevented and corrected. © 2016 Published by Elsevier Inc.
Terminology In this paper, the terminology of the World Health Organization on International Classification of Function (ICF) will be adopted1 (Table 1). Impairments are problems in body function or structure, such as a significant deviation or loss. Impairments as a result of leprosy can include vision loss, paralysis, joint contractures, and ulcers. Activity limitations are difficulties an individual may experience in executing activities. Leprosy-affected people may, for example, not be able to eat or dress in a culturally acceptable manner. Participation problems are problems an individual may experience in maintaining involvement in day-to-day situations. Leprosy-affected people may, not be allowed to go to school, to work, or to participate in community activities.
⁎ Corresponding author. Tel.: +31 343 411533. E-mail address: [email protected] (J. Brandsma). http://dx.doi.org/10.1016/j.clindermatol.2015.10.015 0738-081X/© 2016 Published by Elsevier Inc.
Disability is the umbrella term for impairments, activity limitations, and participation restrictions. For the latter item, rather than restriction, the preferred term participation problem is increasingly used. Deformity is defined as a structural–anatomic deviation from the norm, such as absorption or contracture. Leprosy-affected people is the preferred term used in this paper and may refer to patients undergoing treatment and persons released from multidrug therapy (MDT—medical treatment), who still experience the primary and secondary consequences of nerve function impairment (NFI) and may need continued attention and care (Figure 1).
Etiology of impairments and deformity Most impairments and deformities can be attributed to transient or permanent loss of nerve function. There are a number of noticeable, predominantly facial impairments and deformities, such as the collapse of the nose. These impairments and deformities can be prevented by timely
Rehabilitation of Leprosy-Affected People: An Overview
Fig. 1
67
Primary and secondary impairments due to nerve function loss.
diagnosis and adequate treatment. The consequences of NFI can often be prevented by early diagnosis and adequate treatment of leprosy neuropathy after diagnosis. 2
it may need to be repeated more frequently if patients are undergoing a specific drug treatment for neuropathy.3
Nerve function assessment and evaluation
Nerve function assessment: Voluntary muscle testing and monofilament testing
Regular nerve function assessment (NFA) is an important tool in the prevention of permanent NFI. Patients may already present with NFI at time of diagnosis. If the infection is of relatively short duration (less than 6 months), adequate treatment may reverse any nerve impairment. Some patients may develop NFI while on treatment and others may develop NFI after MDT. Most leprosy programs now undertake a baseline NFA at the time of diagnosis. It is recommended that NFA be repeated at regular, monthly intervals after diagnosis, although
Basic manual muscle strength tests are used to assess the integrity of motor fiber function. Likewise, tests for touch perception are used to assess and monitor the function of sensory fibers. The best test to assess sensory function makes use of monofilaments. It should be kept in mind that significant subclinical decrease in nerve function may be present before NFI can be objectively assessed with voluntary muscle testing and monofilament testing. In an ongoing, prospective, double-blind, randomized, placebo-controlled trial, patients with subclinical NFI are
68 receiving prednisolone for 5 months to determine if a course of prednisolone may prevent the onset of clinical “reactional” episodes resulting in nerve function loss.4
Primary and secondary impairments due to nerve function loss The primary or direct results of nerve function loss are loss of the ability to sweat, loss of (protective) sensation, and paralysis. These primary impairments may result in secondary impairments as illustrated in Figure 1. The overall objective in leprosy treatment is the prevention of nerve function loss, which can, to a very large extent, be achieved by early diagnosis of the disease and timely detection and adequate treatment of neuropathy.
J. Wim Brandsma footwear and exercises, they should take safety precautions when cooking, insulating cooking utensils and keeping a distance from open fires. They also need to rehydrate dry skin with loss of autonomic nerve function via soaking and oiling. They should restrict walking to short distances, sitting and resting their feet whenever possible.
Surgical rehabilitation The need for surgery for leprosy-affected people is often seen as an indication of “failure:” Failure to diagnose early, before the onset of neuropathy; failure to detect and treat neuropathy; and failure to teach principles of self-care and self-management in patients at risk with extremities demonstrating loss of protective sensation. There are generally three distinct areas for reconstructive surgery in leprosy.5
Protection of hands and feet with loss of protective sensation Nerve surgery Once nerve function loss, and in particular loss of protective sensation, has taken place, it remains a lifelong challenge to keep the patient’s hands and feet free of injury. Hands and feet with loss of protective sensation can be protected, but adherence to lifestyle changes is necessary.5
Decompression of nerves (neurolysis) has been undertaken over the past decades, but meta-analysis has found that there is little evidence of its effectiveness in leprosy neuropathy, although it does provide pain relief in chronic painful nerves and can be used to evacuate nerve abscesses.6
Footwear Feet with loss of protective sensation are prone to injury. This is especially the case in leprosy-endemic countries, where people customarily walk barefoot. Footwear with a hard under sole and soft, cushioned insoles are required. When the foot is severely structurally deformed due to previous ulcers, fitted shoes may be necessary.
Exercises Patients must undertake specific exercises to prevent (or overcome) contractures of paralyzed hands. Contractures are unnecessary secondary complications that can be avoided by daily exercises. The leprosy-affected person with paralyzed eyelid muscles needs to develop the habit of attempting regular eye closure. During attempted eye closure, the eyeball moves under the upper eyelid (Bell phenomenon) and is moistened, washing foreign bodies and dust from the eye surface.
Self-care Persons with sensory deprived extremities must understand the dangers that are intrinsic to the condition. In addition to
Ulcer surgery Hands and feet with loss of protective sensation are at risk for burns, mostly of the hands, and ulceration, mostly of the feet. For every ulcer injury, the underlying thermal/ biomechanical mechanisms must be explored with the patient to prevent reulceration and to promote healing; nevertheless, when feet are developing new ulcers, surgery may prevent further breakdown and facilitate healing.
Reconstructive surgery Reconstructive surgery for leprosy generally involves tendon transfers to correct imbalance and restore function. Procedures have been developed for all paralytic conditions that may develop due to NFI of the nerves that are commonly affected in leprosy: intrinsic minus hand (ulnar nerve–claw hand), loss of opposition (median nerve), foot drop (common peroneal nerve), and lagophthalmos (facial nerve). The reader is referred to the literature for description of the various techniques.5 Pre- and postoperative therapy, physiotherapy, and occupational therapy need to be available to obtain maximum benefits of tendon transfer surgery.
Rehabilitation of Leprosy-Affected People: An Overview
Socioeconomic rehabilitation Many people affected by leprosy still experience the consequences of the attitudes that prevail in societies and cultures that are ignorant about the disease. They are shunned, dismissed from school or work, and not allowed to attend public gatherings. Community education and changes in legislation are still needed in many leprosyendemic countries to ensure equal rights and opportunities for people affected by leprosy. Whereas in the past leprosy-affected people sometimes had the opportunity to work in sheltered workshops, this is no longer practiced or implemented in many leprosy-endemic countries. Leprosyaffected people have organized in self-help groups through community-based rehabilitation programs, sometimes consisting of people with diverse physical and/or mental impairments.7–9
Conclusions The consequences of the disease and leprosy neuropathy can, to a very large extent, be prevented or corrected. Expertise in the medical management of this disease and rehabilitation of leprosy-affected people needs to be available for the foreseeable future. Governments in leprosy-endemic countries and international and national nongovernmental organizations supporting leprosy work should remain alert in their fight against leprosy. They should ensure that expertise on leprosy remains available
69 and that medical and allied health professionals and the general public know that leprosy can be cured; is not a disease to be feared; and can, in most instances, be easily recognized and diagnosed when early signs and symptoms are known.
References 1. World Health Organization. International Classification of Functioning, Disability and Health. Geneva, Switzerland: WHO. 2001. 2. Brandsma JW. Prevention of disability in leprosy: The different levels. Ind J Lepr. 2011;83:1-8. 3. Van Brakel WH. Nerve function assessment. In: Schwarz R, Brandsma JW, eds. Surgical Reconstruction and Rehabilitation in Leprosy and Other Neuropathies. Kathmandu: EKTA; 2004. 4. Wagenaar I, Brandsma JW, Post E, et al. Two randomized controlled clinical trials to study the effectiveness of prednisolone treatment in preventing and restoring clinical nerve function loss in leprosy: The TENLEP study protocols. BMC Neurol. 2012;12:159. 5. Schwarz R, Brandsma JW, eds. Surgical Reconstruction and Rehabilitation in Leprosy and other Neuropathies. Kathmandu: EKTA; 2004. 6. van Veen NH, Scheuders TA, Theuvenet WJ, et al. Decompressive surgery for treating nerve damage in leprosy. A Cochrane review. Lepr Rev. 2009;80:3-12. 7. Van Brakel HW, Sihombing B, Djarir H, et al. Disability in people affected by leprosy: The role of impairment, activity, social participation, stigma and discrimination. Glob Health Action. 2012;5. 8. Roosta N, Black DS, Rea TH. A comparison of stigma among patients with leprosy in rural Tanzania and urban United States: A role for public health in dermatology. Int J Dermatol. 2013;52:432-440. 9. Finkenflugel H, Rule S. Integrating community-based rehabilitation and leprosy rehabilitation services into an inclusive development approach. Lepr Rev. 2008;79:83-91.
Rehabilitation of leprosy-affected people: An overview J. Wim Brandsma, PhD ⁎ Independent leprosy—hand rehabilitation consultant, Hoevelaken, 3871 HG-18, The Netherlands
Abstract Leprosy is mainly a disease of the skin and nerves. The involvement of nerves may lead to impairments that have, contributed to the fact that leprosy is often still seen as a disease to be feared. In non–leprosy-endemic Western countries, beliefs continue to prevail about the inevitability of deformities and the lack of a cure. I review the pathogenesis of deformity and to present a discourse on how deformities can, to a very large extent, be prevented and corrected. © 2016 Published by Elsevier Inc.
Terminology In this paper, the terminology of the World Health Organization on International Classification of Function (ICF) will be adopted1 (Table 1). Impairments are problems in body function or structure, such as a significant deviation or loss. Impairments as a result of leprosy can include vision loss, paralysis, joint contractures, and ulcers. Activity limitations are difficulties an individual may experience in executing activities. Leprosy-affected people may, for example, not be able to eat or dress in a culturally acceptable manner. Participation problems are problems an individual may experience in maintaining involvement in day-to-day situations. Leprosy-affected people may, not be allowed to go to school, to work, or to participate in community activities.
⁎ Corresponding author. Tel.: +31 343 411533. E-mail address: [email protected] (J. Brandsma). http://dx.doi.org/10.1016/j.clindermatol.2015.10.015 0738-081X/© 2016 Published by Elsevier Inc.
Disability is the umbrella term for impairments, activity limitations, and participation restrictions. For the latter item, rather than restriction, the preferred term participation problem is increasingly used. Deformity is defined as a structural–anatomic deviation from the norm, such as absorption or contracture. Leprosy-affected people is the preferred term used in this paper and may refer to patients undergoing treatment and persons released from multidrug therapy (MDT—medical treatment), who still experience the primary and secondary consequences of nerve function impairment (NFI) and may need continued attention and care (Figure 1).
Etiology of impairments and deformity Most impairments and deformities can be attributed to transient or permanent loss of nerve function. There are a number of noticeable, predominantly facial impairments and deformities, such as the collapse of the nose. These impairments and deformities can be prevented by timely
Rehabilitation of Leprosy-Affected People: An Overview
Fig. 1
67
Primary and secondary impairments due to nerve function loss.
diagnosis and adequate treatment. The consequences of NFI can often be prevented by early diagnosis and adequate treatment of leprosy neuropathy after diagnosis. 2
it may need to be repeated more frequently if patients are undergoing a specific drug treatment for neuropathy.3
Nerve function assessment and evaluation
Nerve function assessment: Voluntary muscle testing and monofilament testing
Regular nerve function assessment (NFA) is an important tool in the prevention of permanent NFI. Patients may already present with NFI at time of diagnosis. If the infection is of relatively short duration (less than 6 months), adequate treatment may reverse any nerve impairment. Some patients may develop NFI while on treatment and others may develop NFI after MDT. Most leprosy programs now undertake a baseline NFA at the time of diagnosis. It is recommended that NFA be repeated at regular, monthly intervals after diagnosis, although
Basic manual muscle strength tests are used to assess the integrity of motor fiber function. Likewise, tests for touch perception are used to assess and monitor the function of sensory fibers. The best test to assess sensory function makes use of monofilaments. It should be kept in mind that significant subclinical decrease in nerve function may be present before NFI can be objectively assessed with voluntary muscle testing and monofilament testing. In an ongoing, prospective, double-blind, randomized, placebo-controlled trial, patients with subclinical NFI are
68 receiving prednisolone for 5 months to determine if a course of prednisolone may prevent the onset of clinical “reactional” episodes resulting in nerve function loss.4
Primary and secondary impairments due to nerve function loss The primary or direct results of nerve function loss are loss of the ability to sweat, loss of (protective) sensation, and paralysis. These primary impairments may result in secondary impairments as illustrated in Figure 1. The overall objective in leprosy treatment is the prevention of nerve function loss, which can, to a very large extent, be achieved by early diagnosis of the disease and timely detection and adequate treatment of neuropathy.
J. Wim Brandsma footwear and exercises, they should take safety precautions when cooking, insulating cooking utensils and keeping a distance from open fires. They also need to rehydrate dry skin with loss of autonomic nerve function via soaking and oiling. They should restrict walking to short distances, sitting and resting their feet whenever possible.
Surgical rehabilitation The need for surgery for leprosy-affected people is often seen as an indication of “failure:” Failure to diagnose early, before the onset of neuropathy; failure to detect and treat neuropathy; and failure to teach principles of self-care and self-management in patients at risk with extremities demonstrating loss of protective sensation. There are generally three distinct areas for reconstructive surgery in leprosy.5
Protection of hands and feet with loss of protective sensation Nerve surgery Once nerve function loss, and in particular loss of protective sensation, has taken place, it remains a lifelong challenge to keep the patient’s hands and feet free of injury. Hands and feet with loss of protective sensation can be protected, but adherence to lifestyle changes is necessary.5
Decompression of nerves (neurolysis) has been undertaken over the past decades, but meta-analysis has found that there is little evidence of its effectiveness in leprosy neuropathy, although it does provide pain relief in chronic painful nerves and can be used to evacuate nerve abscesses.6
Footwear Feet with loss of protective sensation are prone to injury. This is especially the case in leprosy-endemic countries, where people customarily walk barefoot. Footwear with a hard under sole and soft, cushioned insoles are required. When the foot is severely structurally deformed due to previous ulcers, fitted shoes may be necessary.
Exercises Patients must undertake specific exercises to prevent (or overcome) contractures of paralyzed hands. Contractures are unnecessary secondary complications that can be avoided by daily exercises. The leprosy-affected person with paralyzed eyelid muscles needs to develop the habit of attempting regular eye closure. During attempted eye closure, the eyeball moves under the upper eyelid (Bell phenomenon) and is moistened, washing foreign bodies and dust from the eye surface.
Self-care Persons with sensory deprived extremities must understand the dangers that are intrinsic to the condition. In addition to
Ulcer surgery Hands and feet with loss of protective sensation are at risk for burns, mostly of the hands, and ulceration, mostly of the feet. For every ulcer injury, the underlying thermal/ biomechanical mechanisms must be explored with the patient to prevent reulceration and to promote healing; nevertheless, when feet are developing new ulcers, surgery may prevent further breakdown and facilitate healing.
Reconstructive surgery Reconstructive surgery for leprosy generally involves tendon transfers to correct imbalance and restore function. Procedures have been developed for all paralytic conditions that may develop due to NFI of the nerves that are commonly affected in leprosy: intrinsic minus hand (ulnar nerve–claw hand), loss of opposition (median nerve), foot drop (common peroneal nerve), and lagophthalmos (facial nerve). The reader is referred to the literature for description of the various techniques.5 Pre- and postoperative therapy, physiotherapy, and occupational therapy need to be available to obtain maximum benefits of tendon transfer surgery.
Rehabilitation of Leprosy-Affected People: An Overview
Socioeconomic rehabilitation Many people affected by leprosy still experience the consequences of the attitudes that prevail in societies and cultures that are ignorant about the disease. They are shunned, dismissed from school or work, and not allowed to attend public gatherings. Community education and changes in legislation are still needed in many leprosyendemic countries to ensure equal rights and opportunities for people affected by leprosy. Whereas in the past leprosy-affected people sometimes had the opportunity to work in sheltered workshops, this is no longer practiced or implemented in many leprosy-endemic countries. Leprosyaffected people have organized in self-help groups through community-based rehabilitation programs, sometimes consisting of people with diverse physical and/or mental impairments.7–9
Conclusions The consequences of the disease and leprosy neuropathy can, to a very large extent, be prevented or corrected. Expertise in the medical management of this disease and rehabilitation of leprosy-affected people needs to be available for the foreseeable future. Governments in leprosy-endemic countries and international and national nongovernmental organizations supporting leprosy work should remain alert in their fight against leprosy. They should ensure that expertise on leprosy remains available
69 and that medical and allied health professionals and the general public know that leprosy can be cured; is not a disease to be feared; and can, in most instances, be easily recognized and diagnosed when early signs and symptoms are known.
References 1. World Health Organization. International Classification of Functioning, Disability and Health. Geneva, Switzerland: WHO. 2001. 2. Brandsma JW. Prevention of disability in leprosy: The different levels. Ind J Lepr. 2011;83:1-8. 3. Van Brakel WH. Nerve function assessment. In: Schwarz R, Brandsma JW, eds. Surgical Reconstruction and Rehabilitation in Leprosy and Other Neuropathies. Kathmandu: EKTA; 2004. 4. Wagenaar I, Brandsma JW, Post E, et al. Two randomized controlled clinical trials to study the effectiveness of prednisolone treatment in preventing and restoring clinical nerve function loss in leprosy: The TENLEP study protocols. BMC Neurol. 2012;12:159. 5. Schwarz R, Brandsma JW, eds. Surgical Reconstruction and Rehabilitation in Leprosy and other Neuropathies. Kathmandu: EKTA; 2004. 6. van Veen NH, Scheuders TA, Theuvenet WJ, et al. Decompressive surgery for treating nerve damage in leprosy. A Cochrane review. Lepr Rev. 2009;80:3-12. 7. Van Brakel HW, Sihombing B, Djarir H, et al. Disability in people affected by leprosy: The role of impairment, activity, social participation, stigma and discrimination. Glob Health Action. 2012;5. 8. Roosta N, Black DS, Rea TH. A comparison of stigma among patients with leprosy in rural Tanzania and urban United States: A role for public health in dermatology. Int J Dermatol. 2013;52:432-440. 9. Finkenflugel H, Rule S. Integrating community-based rehabilitation and leprosy rehabilitation services into an inclusive development approach. Lepr Rev. 2008;79:83-91.