Oculocutaneous albinism is an autosomal recessive disorder characterized by a lack of pigment in the hair, skin, and eyes. Albinism is caused by defective or absent tyrosinase, an enzyme necessary for melanogenesis. Although rare in the western world, albinism is quite common in sub-Saharan Africa, likely as a result of consanguinity. Albinism has long been associated with stigma and superstitions, such as the belief that a white man impregnated the mother or that the child is the ghost of a European colonist. Recently, a notion has emerged that albino body parts are good-luck charms or possess magical powers. These body parts may be sold for as much as $75,000 on the black market. As a result there have been over 100 albino murders in Tanzania, Burundi, and other parts of Africa in the past decade, which is now beginning to garner international attention and thus prompting novel legislation. To ameliorate the plight of individuals with albinism in Africa, a coordinated effort must be organized, involving medical professionals (dermatologists, ophthalmologists, oncologists), public health advocates and educators, social workers, human rights and antidiscrimination activists, law-enforcement agencies, and governmental support groups. The main issues that should be addressed include skin cancer prevention education, stigma and discrimination denouncement, and swift prosecution of albino hunters and their sponsors.
Originating from the word “albus,” meaning white in Latin, albinism is a genetic disorder resulting in a decrease or absence of pigmentation in the hair, eyes, and skin. The prevalence of oculocutaneous albinism (OCA) is about 1:37,000 in the United States and 1:20,000 in most populations in the world. The prevalence in regions of sub-Saharan Africa is estimated to be much greater than these figures; for example, it is estimated at about 1:4000 in Zimbabwe and in Tanzania it is 1:1429. Albinism most commonly arises from mutations in genes encoding for proteins involved in the synthesis or transport of melanin by melanocytes. As well as giving skin its color, melanin protects the skin from the deleterious effects of the sun’s ultraviolet radiation (UVR) and, although present, melanocytes in OCA are dysfunctional, predisposing individuals to cutaneous and ocular pathologic conditions. This is particularly problematic for albinos living in South Africa who suffer both emotionally and physically from dermatologic malignancies and, more recently, must cope with being hunted for their body parts as well. Thus, it is imperative to inform the medical community and the general national and international public about the tragedies faced by albinos to protect them from skin cancer and ritualistic murders by individuals seeking wealth through clandestine markets perpetuating witchcraft.
Demystifying albinism myths and misconceptions
Throughout Africa, an indeterminate number of individuals with albinism, especially children, have been the victims of brutal attacks and murder in the name of witchcraft, superstition, and wealth ( Box 1 ). Most recently, the atrocities committed against albinos has received widespread attention because of various crimes reported, such as infanticide, kidnapping, amputations, and decapitations, committed for purposes of supplying highly valued body parts used for amulets, which are then sold in underground witchcraft markets. For example, up to $75,000 may be offered for a set of arms, legs, ears, and genitals from an individual with albinism. Thus, albinos must live in a constant state of guilt and angst, often forced to flee their homes and live in solitude to avoid the albino hunters.
Weaving albino hair into a net improves the chances of catching fish
Albino body parts worn as amulets bring good luck, fortune, and health
Albino body parts are a necessary ingredient for witchdoctor potions
Albinos have magical superpowers and can cure diseases
Intercourse with an albino lady will cure human immunodeficiency virus (HIV) infection
Spitting on an albino prevents the condition in one’s family
Mother of albino child was laughed at by an albino during pregnancy
Albinism is caused by a missing top layer of skin
Albinos and their mothers are possessed by evil spirits
The devil stole the original child and replaced it with an albino
Albinism is very contagious and spread through touching
Albinos are housed by ghosts of European colonists
Albinos have low brain capacity and cannot function at the same level as others
Mother of albino was impregnated by a white man
The main driving forces underlying these profiling crimes are ignorance, myth, and superstition, such as the belief that individuals with albinism possess superpowers or that their body parts bestow fortune and health. Thus, the stigma and atrocities affecting the albino population may be attributed to lack of familiarity and education about albinism coupled with ignorance. Compared with albinos in developed countries, many albinos in sub-Saharan Africa suffer from a lack of access to health care and awareness within their communities of their condition. This may manifest itself in a higher and earlier incidence of skin cancer, mortality, and stigmatization.
Based primarily on its major pattern of autosomal recessive inheritance, the prevalence, morbidity, and mortality of albinism could be diminished with the education of school-aged children, particularly emphasizing the consequences of marrying relatives. Although many of the genetic causes and inheritance patterns of albinism have been established, many communities are uninformed and unaware of the implications of such consanguineous matrimonial arrangements. Thus, the high incidence of OCA in certain regions of sub-Saharan Africa might be due to encouragement of consanguineous alliances. For instance, some of the coastal communities in Tanzania “are matrilineal. Children of a maternal uncle belong to the clan of the wife of that uncle and therefore to a different clan from the children of his sisters and brothers. First cousins may therefore marry, enhancing the chances of albino offspring. Compounding this is the influence on coastal people of the Arab tradition of marrying among relatives, so that family property remains within the clan.”
These practices inevitably increase the incidence of albinism amongst children of related parents. Nevertheless, the traditional consanguineous marriage arrangements are not the only reason for the high prevalence of albinism in certain regions, as “communities may have fewer cases to show because many areas still practice infanticide. Traditional midwives may sometimes kill off albino children and the case is then presented as a stillbirth.” Such evidence further highlights the prevalence of severe stigmas and misconceptions associated with albinism and the need for improved awareness.
Tanzanians as well as other sub-Saharan Africans believe in numerous myths and superstitions regarding the etiology of albinism, unaware of its genetic cause, and further contributing to its high incidence. For example, Lund surveyed 138 schoolchildren (average age 14.4 years old) with albinism in Zimbabwe about the cause of OCA, and found that 70 (50.7%) had no knowledge of why they lacked skin coloration and were different from their classmates, 15 (10.9%) favored a biologic reason, 19 (13.8%) believed God was responsible, and 13 (9.4%) stated other incorrect causes, such as witchery, punishment for a family member mocking an albino and “top layer of skin missing.” Other misconceptions regarding the cause of albinism include the belief that the mother was impregnated by a white man or that the devil replaced the African child with an albino.
Furthermore, although more than 50% of albinos in Tanzania have an albino relative, most do not recognize its genetic cause. In a study assessing albino attitudes and beliefs, McBride and Leppard demonstrated that although 59 participants with OCA had an albino relative, only 13 believed that the condition was inherited. In some regions, the misconceptions about the cause of albinism might extend into the health care field to include physicians and nurses, who may be under the impression that albinism is contagious and therefore might avoid physical and social contact with those affected.
In addition to the distinct physical features of albinism, the social structure and superstitious belief system provides the grounds for further prejudice and social rejection. In Zimbabwe, albinos are called “sope,” which suggests that they are inhabited by evil spirits; in Tanzania, they are also ridiculed and called “nguruwe,” which means pig; “zeru,” which means ghost; or “mzungu,” which means white person. Beginning in childhood, their marked and dissimilar exterior appearance compared with “normally pigmented family members and the rest of the black community, results in problems of acceptance and social integration for those affected.”
Lund reported that albino students in a school in Zimbabwe were not only called names and ridiculed, but were also beaten and avoided by peers who would not eat or play with them. Some participants in this study reported that they were mocked and avoided by family members, who would not share food or clothes with their albino relatives. Many albino children were not allowed to visit their parents’ workplaces and were sometimes placed in the sun to become accustomed to UVR. Thus, it is not surprising, that one 15-year-old albino confessed, “I cannot see the blackboard clearly. I cannot work in the open doing manual work. I don’t like walking long distances to interschool sports. I am always being humiliated by others calling me names.” Although some albinos may benefit from a good family support system, once they leave home many encounter rejection by employers in what some have described as “apartheid in reverse.” The superstitions surrounding albinism may also place mothers of albinos in a vulnerable position, subject to stigmatization and harassment, and resulting in severe psychosocial distress.
As the general medical term implies, OCA also affects the eyes; some studies have indicated that up to 100% of albinos have some form of visual impairment. Ocular disorders associated with OCA include hypopigmentation of the iris and retina, hypoplastic fovea, hyperopia, strabismus, photophobia, loss of stereoscopic perception, and nystagmus. Such visual handicaps hinder some of the major components of learning, such as reading and seeing the blackboard. These obstacles place albino students at a disadvantage, as many cannot afford ophthalmic care or appropriate visual aids. Visual impairments, poverty, lack of access to health care, and that lack of educational provisions inevitably result in poor educational outcomes and continued frustration. Consequently, this fosters a cycle that prevents albinos from succeeding and condemns them to manual outdoor labor, such as that in sisal plantations, further increasing the risk for skin damage.
Because of a lack of melanin, which serves as the skin’s own sunscreen against UVR, albinos are predisposed to various types of solar skin damage, such as actinic cheilitis, actinic keratoses, and various skin cancers. The likelihood of damage increases in tropical climate regions, as the amount of clothing worn is typically minimal while the daily exposure to UVR is maximal. Studies have shown that the risk for dermatologic malignancy in sun-exposed areas (such as the face, ears, neck, and shoulders) is enhanced in individuals with OCA, who may present with solar keratoses as early as 8 years of age.
A study amongst individuals with OCA in Tanzania revealed that 100% exhibited skin damage by the first year of life and advanced symptomatic cancers were observed in 50% of those between 20 and 30 years of age, with 1 case of skin cancer in a 9-year-old child. In a Nigerian study, no albinos older than 20 years of age were free of subclinical malignant skin damage and in the 1980s, less than 10% of albinos living around Dar es Salaam survived beyond 30 years of age. Within Tanzania, less than 2% of albino children were expected to reach 40 years of age. A more recent study conducted in northern Tanzania reported that although albinos previously died between 20 and 30 years of age, today they live considerably longer because of preventive sun protection. Nevertheless, in regions afflicted by poverty, sunscreens are unaffordable and only available to a limited portion of the population; thus, strategies should focus on sun avoidance and other means of protection early in childhood.
During the 1980s in Dar es Salaam, individuals suffering from albinism had about 4 years between the presentation of irreversible skin pathology and metastatic disease, mainly caused by squamous cell carcinoma (SCC) in the head and neck region. Lack of access to medical care or neglect might be one of the reasons why skin cancer has a deadlier course in the albino population near the Equator than in other developed countries. Mortality from SCC is low amongst fair-skinned individuals in developed, equatorial nations with high levels of UVR, but amongst albinos in Nigeria and Tanzania, it is very high.
The low life expectancy in the albino population is clearly multifactorial. Those suffering from albinism are often unable to succeed in school. One study reported that only 12 of 350 adults with OCA worked indoors, suggesting that outdoor occupations are contributing to the lower life expectancy for albinos. As previously mentioned, albinos are often discriminated against and the system in place does not have the educational provisions to address their poor eyesight and allow them to succeed and compete with the nonalbino population. For example, in his editorial article, “White skin, black souls”, Kuster indicates that in Malawi, “it is a common belief that albinos have low brain capacity and are unable to function at the same level as ‘normal people’.” Kuster further notes than an educated albino woman never held a job in Zimbabwe because “employers always said, more or less, during the interview that an albino secretary would hurt the company’s reputation” because of the fear that albinism is contagious.
The first step in assisting individuals with albinism in sub-Saharan Africa would be to educate the population as a whole about the cause of albinism and decrease prevalence by increasing awareness of the implications of consanguineous relationships ( Box 2 ). Poverty and illiteracy in some parts of Africa may result in a lack of understanding among individuals that marriage between cousins can increase the chances of acquiring certain genetic disorders. Raising awareness through radio broadcasts and schools’ curricula might be helpful in promoting integration, albeit challenging, given that albinism is steeped in superstition and misconceptions that lead to fear and misunderstanding. Albinos and parents of albino children must understand the causes of albinism, its many medical and psychosocial implications, and how to appropriately protect themselves from UVR. In particular, the benefits of sun protection and the consequences of solar injury should be stressed, as studies have shown that many albinos are not familiar with this information. For example, in a study by McBride and Leppard, 10% of albinos surveyed stated that they only applied sunscreen at night and some did not wear sun-protective clothing, such as wide-brimmed hats, because of fashion and cultural concerns. It is also imperative that mothers of newborns with albinism fully understand the cause before returning home from the hospital, as members of the family and community may assume that the infant is of uncertain ancestry, resulting in condemnation of the mother and rejection of the child. Teachers must be informed that children with albinism are as capable as other students, but have difficulties with vision and reading, thus requiring enlarged text textbooks and seating closer to the blackboard.
Sun protection education
Sun protection products
Eye care and corrective lenses
Wide-brimmed hats, sunglasses, scarves
Educational provisions
Magnifying glasses, braille machines
Opaque clothing and high socks
Regular full body skin checks
Indoor job placement assistance
Genetic transmission education
Enhanced protection from criminals
Swift justice for captured albino hunters
Awareness campaigns to debunk myths
In the clinical setting, individuals with albinism should be provided with dermatologic examinations, guidelines on how to shield themselves from the sun, and sun protection products such as sunscreen, sunglasses, opaque clothing that covers most of the skin, scarves, high socks, and wide-brimmed hats. Given that most albinos are unemployed, they cannot afford sun-protective gear, which is expensive in Africa, thus it should be encouraged that society establish measures to support albinos and their families. Governmental endeavors may include assistance with indoor job placement, supplying adequate amounts of sun-protective products and funding for organizations involved in albinism awareness and support. Most of the educational provisions necessary for students with albinism are not being met, and in Tanzania, unlike Zimbabwe, there are no provisions for children with visual problems or handicaps. In the current circumstances, little can be accomplished without the government’s support and international assistance. An unsupportive social environment hinders the progress of individuals with albinism, stagnating their intellectual and physical potential, despite their desire to progress.
Awareness campaigns
Recently, various organizations dedicated to increasing albinism awareness and assisting albinos in attaining proper medical care and appropriate sun protection have been established throughout the world ( Table 1 ). For example, the Regional Dermatologic Training Center (RDTC) at the Kilimanjaro Christian Medical Center (KCMC) in Moshi, Tanzania, has created an albino assistance program and mobile skin care clinic. The primary mission of the RDTC has been to significantly improve the living conditions of albinos living in Tanzania. A physician and nurse in a 4-wheel drive vehicle regularly visit 10 villages within a100-km 2 area in Arusha and Kilimanjaro. During these visits, skin checks are performed and sun protection information and supplies are provided.
| Albinism Organizations | Contact Information |
|---|---|
| National Organization for Albinism and Hypopigmentation (NOAH) | http://www.albinism.org |
| Ghana Association of Persons Living with Albinism (GAPA) | http://www.gapagh.org/stigmatization.html |
| Tanzania Albino Center (TAC) | http://www.tanzaniaalbino.org/ |
| Stichting Afrikaanse Albino (SAA) | http://www.afrikaansealbinos.nl |
| Assisting Children in Need (ACN) | http://www.assistingchildreninneed.com |
| Regional Dermatologic Training Center (RDTC) | http://gc21.inwent.org/ibt/site/rdtc/ibt/main.html |
| Albino Association of Malawi (TAAM) | http://www.fedoma.net/members/taam.php |
| Albinism Fellowship United Kingdom | http://www.albinism.org.uk/ |
| Asante Mariamu | http://www.asante-mariamu.org |
| Tanzania Vision Support | http://www.thefoundation-tz.org |
| Positive Exposure | http://www.positiveexposure.org |
| Albinism Trust New Zealand | http://www.albinism.org.nz/home.html |
| Cordaid | http://www.cordaid.nl/ |
| Under the Same Sun | http://www.underthesamesun.com/home.php |
| Albinism Fellowship of Australia | http://www.albinismaustralia.org/ |
| ALBA (Asociación de Ayuda a Personas con Albinismo) | http://www.albinismo.es/ |
| WHO Intersun Program | http://www.who.int/uv . |
| BaiChina (Kids from China with Albinism) | |
| Chicago Connection for Minorities with Albinism (CCM) | |
| Norwegian Association for Albinism (NFFA) | http://www.albinisme.no/ |
| The Hermansky-Pudlak Syndrome (HPS) Network | http://www.hpsnetwork.org/ |
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