“It Is Not Easy”: Cultural Influences of Sickle Cell Disease Management in Rural,Eastern Sierra Leone

Abstract

Sickle cell disease (SCD) is a chronic genetic disease that causes life-threatening complications and requires robust comprehensive management. Developing comprehensive SCD programs in sub-Saharan African countries requires knowledge of the cultural factors affecting health-seeking behavior. We utilized an ethnographic approach and the frameworks of Dutta and Habermas to explore cultural factors influencing SCD management in rural Sierra Leone. A purposive sample of 27 individuals with SCD and their family and professional caregivers were observed and interviewed from March 2019 to April 2019. We identified four domains (Cultural Beliefs, Cultural Values, Cultural Practices, and Dealing with SCD) of cultural influences on SCD management, and 12 sub-themes (related to collectivism; spiritual, traditional, and Western beliefs and practices; and lived experiences) that reflect the personal, social, structural, and contextual complexities of SCD management. Further research regarding roles of traditional and spiritual leaders, combinations of Western and traditional practices, and culturally centered interventions is warranted.

Keywords

culture sickle cell disease health seeking disease management health determinants qualitative ethnography sub-Saharan Africa West Africa

Sickle cell disease (SCD) represents a set of hematologic conditions arising from a mutation in the beta-globin gene, known as sickle hemoglobin (Piel et al., 2013, 2017). Global estimates suggest that nearly 300,000 children are born with SCD annually and that 80% of these births occur in sub-Saharan Africa (Piel et al., 2013, 2017). Sierra Leone represents a low-to-middle income country (LMIC) in sub-Saharan Africa for which there is a paucity of SCD management literature; however, at least 3,000 to 4,000 SCD births occur per year in this small country (Kuznik et al., 2016; Roberts et al., 2015). Early genetic sampling studies suggest that the prevalence of SCD in Sierra Leone could be as high as in Nigeria, the world leader in SCD births (Knox-Macaulay, 1983; Piel et al., 2013; Wurie et al., 1996). Mean survival ranges from 17 to 27 years in Sierra Leone (Roberts et al., 2015) and is approximately 21 years in Nigeria (Ogun et al., 2014). Unfortunately, the under-five mortality rate for children born with SCD ranges from 50% to 90% in sub-Saharan Africa (Grosse et al., 2011).

The mortality associated with SCD has been attributed to chronic hemolytic anemia, modifiable disease risks, and complications including painful vaso-occlusive episodes and high risk for infection (Makani et al., 2013; Mburu & Odame, 2019; Wirth et al., 2018). Moreover, SCD complications are influenced by environmental (weather), physiologic (nutritional status), genotypic (sickle cell variants), and social (individual, community, and system level) factors (Thompson, 2006). Most of these influences can be mitigated by prompt chronic disease management; however, SCD management in sub-Saharan countries varies based on available health care infrastructure, health policy, and human and financial resources.

To reduce morbidity and mortality, the World Health Organization (WHO, 2006) declared SCD a public health crisis in sub-Saharan Africa and recommended newborn screening for early diagnosis and prompt referral for comprehensive disease management. As is the case in many African LMICs, health care infrastructure and finance problems have hindered the development of a national screening program in Sierra Leone (Italia & Kirolos, 2019). Efforts to strengthen the health care system following the Sierra Leonean civil war and the 2014 to 2016 Ebola crisis have not addressed SCD as a national health policy issue despite the introduction of a free maternal/child preventive health care program (Berghs et al., 2019; Ministry of Health and Sanitation, 2015). To date, there have been many pilot newborn screening programs in sub-Saharan Africa, but only one country (Ghana) has established a national newborn screening and management program (Hsu et al., 2018; Kafando et al., 2009; McGann et al., 2013; Mutesa et al., 2016; Ohene-Frempong et al., 2008; Rahimy et al., 2009; Tshilolo et al., 2009). Despite infrastructural and financial impediments, researchers suggest that the successful implementation of a clinical model for comprehensive SCD management must acknowledge and address the influence of societal factors on public health initiatives (Airhihenbuwa et al., 2014; Anie et al., 2016; Ibemere, 2018; Mburu & Odame, 2019).

Culture represents an influential social factor in many African LMICs. Stigma, religion, community beliefs, and traditional medicine have been reported as key factors in individual- and community-level understanding of SCD and disease management practices (Dennis-Antwi et al., 2011, 2018; Marsh et al., 2011; Ola et al., 2016; Treadwell et al., 2015), affecting the development of context-appropriate SCD management. Fullwiley’s (2011) ethnography on SCD in Senegal reported that socio-cultural norms influence care delivery and health-seeking behavior, and therefore they deserve greater focus in global health research efforts. For example, religious and traditional leaders represent potential collaborators for the development of SCD management interventions that are tailored to acknowledge and address local beliefs and understanding. In Ghana and Nigeria, religious leaders provide SCD screening and genetic counseling in the community as part of premarital counseling (Dennis-Antwi et al., 2018; Ezugwu et al., 2019). Although highly respected, these leaders are not trained in genetic counseling; they often counsel against marriage for couples with the sickle cell trait, further stigmatizing the sickle cell trait and treating SCD as a family curse, and thus discouraging patients from seeking care (Dennis-Antwi et al., 2018; Ezugwu et al., 2019).

A recent study conducted in Sierra Leone illuminates the experience of women caring for themselves and/or children with SCD in an urban and rural setting (Berghs, Dyson et al., 2020). The research team described a female culture characterized by a provision of care based on moral obligation and blame they are assigned because their children have SCD. These findings suggest that female health-seeking behaviors may be influenced by stigma experienced in the community as well as by a woman’s ability to provide care to her children safely and effectively. Although this study (Berghs, Dyson et al., 2020) has contributed to the dearth of socio-cultural literature related to SCD in Sierra Leone, a broader view of SCD management in Sierra Leone including patient, family, and provider perspectives has not yet been described. Evidence resulting from such a broad study could provide a roadmap of available resources and current SCD-related knowledge, practices, and beliefs pertinent to the development of a culturally centered, sustainable, and comprehensive SCD program to increase awareness, health care capacity, and disease management (Anie et al., 2016; Dutta, 2008; Treadwell et al., 2015).

Culture is defined as the strategies by which humans adapt to their environment or context (Dutta, 2008), so it is important to understand SCD-related strategies or behaviors as cultural in that they have developed to allow individuals to adapt to a context of inconsistent SCD care. Moreover, there are sociocultural factors associated with understanding and managing SCD in other sub-Saharan African countries that could be similar to those in Sierra Leone. Although what is known about SCD management in Sierra Leone suggests that the burden of care falls on the women in the family (Berghs, Dyson et al., 2020; Berghs et al., 2019), a more scoping perspective of these influences is needed; therefore, the purpose of this article is to describe the cultural factors influencing SCD management in the context of rural Sierra Leone from the perspectives of individuals, families, and health care professionals.

Conceptual Framework

Dutta (2008) acknowledges the development of culturally centered health care interventions require the exploration of the cultural norms, traditions, and beliefs which influence health seeking behaviors. Dutta (2008) and Habermas (1981, 1985) suggest the use of communication (communicative action) to illuminate the relationship between these concepts. The researcher and participant form a dialectic relationship where definitions of health and illness are shared to generate new culturally centered definitions of health and illness. In combination, these theoretical approaches acknowledge that definitions of health and illness are contextually based and operationalized by the agency, culture, and structures experienced by a person or community. The resulting interventions comprehensively address community level health needs.

Method

We conducted an ethnographic study informed by the theories of Dutta (2008) and Habermas (1981, 1985) to examine the emic perspectives of individuals, families, and health care professionals on cultural factors influencing SCD management. To learn about the context of definitions of health and illness in our study, we used participant observation as well as semi-structured and field interviews with patients, families, and professional caregivers conducted in the naturalistic setting (e.g., homes, clinics, and health centers).

The research team consisted of nurse scientists who have expertise in SCD research and ethnography. One member of the research team had developed relationships with the community following the 2014 to 2016 Ebola Crisis which prompted a community-based request for greater focus on SCD. A representative (research assistant) from the SCD community-based organization (CBO) assisted with recruitment coordination after receiving training and orientation to the study. This representative was well trusted in the community; was able to ensure an orderly, culturally appropriate, and strategic procedure; and possessed vast knowledge of the history of the community and SCD management.

Dr. Ibemere was formally introduced to patients, health care professionals, and community members by Dr. Jaja during a study site visit in January 2019. Observation, interview, and field data were collected in March and April 2019. This study was approved by the University of Cincinnati and the Sierra Leone Ethics and Scientific Review Committee. All participants provided written informed consent prior to interviews and participation observation. After community wide description of the study, verbal consent was obtained from participants prior to each clinical observation. Study procedures were conducted in a rural city in Eastern Sierra Leone. The community contains a regional tertiary care government hospital, a community health center with a novel pediatric SCD program, and a volunteer-run, community-based SCD organization (SCD CBO).

Recruitment and Sample

The research team purposively selected study participants using telephone eligibility screening and during a SCD CBO town hall meeting (Creswell, 2012). A home visit schedule was drafted as part of the recruitment and sampling process. Inclusion criteria required that participants be at least 18 years of age, speak English or Krio (the commonly spoken dialect), and have an SCD diagnosis verified using the SCD CBO genotype registry and/or provide care to a child or adult with SCD. General informants included adults who worked in administration for the data collection sites. Pediatric patients (under five) are covered by the national free health care initiative (WHO, 2018) and adolescent care transitions present unique barriers especially in the context of sub-Saharan Africa where formal transition programs do not exist (Inusa et al., 2020); these represent unique situations which are unique to these age groups. Therefore, we decided to focus on the adult population whose experiences would not only detail their current experience, but also provide historical references to SCD management.

Participant Observation

Field observations were conducted in the clinics of the community health center, the SCD CBO, and the local government hospital. Members of the research team attended two SCD town hall meetings. Four home visits to participants who had SCD and/or cared for someone with SCD were conducted at locations throughout the city. During the initial site visit in January, the local lead pediatric SCD provider invited research team members to attend pediatric cohort clinic hours weekly. This invitation included opportunities to attend and provide SCD education sessions for patients and families hosted by SCD CBO volunteers, to assist with triage, and to witness consultations with the consent of the patient/family. Observations and interviews at the local government hospital were conducted after preliminary data analysis suggested that more information was needed to deepen our understanding of SCD management. Over the course of 4 weeks, 90 hours of clinical participant observation were conducted in addition to time spent living on the community health center campus. Written and digitized field notes facilitated triangulation in data analysis.

Interviews

Semi-structured interviews were conducted at the local community health center. Literature-based definitions of cultural values, beliefs, and practices as well as the guiding frameworks of this study were used to outline the interview guide (Dutta, 2008; Habermas, 1981, 1985). Research team members fluent in Krio, English, and Nigerian Pidgin (derivation of West African Pidgin English-like Krio) evaluated the interview guide to ensure Krio equivalence and cultural appropriateness. Questions were left in English (see Supplementary Material 1) and verbally translated by the research assistant when Krio was preferred. To reduce traveling costs, participant interviews were concomitantly scheduled with health appointments. Interviews lasted 10 to 90 minutes, and each participant was formally interviewed once. Each interview was digitally recorded and transcribed. Interviews conducted in Krio were translated into English by fluent team members and reviewed for accuracy and English equivalence. Informal field interviews were conducted with some participants to clarify previously collected interview data. Participants and the research assistant were offered water and were provided with a small gift (t-shirt and pen) in recompense for their time. Data saturation was achieved after 27 interviews.

Data Analysis

Constant comparative analysis was used to analyze the data (Lincoln & Guba, 1985). Research team debriefing of the data analysis process was employed to decrease researcher bias. Weekly research team meetings ensured trustworthiness of emerging codes, categories, and themes. Interview data were coded, and a codebook was developed using iterative line-by-line coding. Similarities and differences in code definitions were used to aggregate codes into emergent categories; similarities and differences between categories were examined and led to the generation of themes representing how participants managed SCD in context. These themes were further abstracted into domains describing cultural factors that influence SCD disease management. Data management was facilitated using NVivo 12^® software (QSR International).

Field notes from participant and home observations served to contextualize the abstractions as there were many instances noted in which participants discussed many of the emerging codes, categories, and themes related to management of SCD. Along with documents collected from the SCD CBO and community health center, the field notes served as a means of triangulating the interview transcripts during data analysis. Themes from the field notes and information found in health care pamphlets and provider education materials served as confirmatory data for analysis abstractions.

Rigor

Using Lincoln and Guba’s (1985) criteria, dependability was established using hand-written and digital field notes as well as a reflexive journal documenting the data analysis process. All data (including theoretical memos and notes) were maintained for external audit of the data abstraction process by two faculty members with expertise in SCD and ethnography. These faculty members agreed that the abstraction process produced domains and themes which accurately depicted the data. This process included research teams meetings which included systematic review of in country methods description and analysis. Triangulation between the research team, interview data, and participant observation provided confirmability in this study. Credibility was facilitated through member checking of interview analysis to ensure accuracy. Member checking was accomplished directly after interviews, categorization of codes, and theme abstraction. Participants who could be reached by telephone after Dr. Ibemere left the field observation site provided correct pronunciations, spellings, clarified connections between themes and voiced support of the final results. To achieve transferability, participant and context description as well as sampling and recruitment strategies were well documented.

Results

In total, 27 participants ranging in age from 18 to 76 years were interviewed for this study. Four groups of participants were included: familial caregivers, professional caregivers, adults with SCD, and general informants. Most of the participants were female (n = 19) and were either mothers of children with SCD, had SCD themselves, or both. Higher levels of education were noted among the male participants and those working in health care. Further demographic information is provided in Supplementary Material 2. Four domains related to the care and management of SCD were described by participants: Cultural Values, Cultural Beliefs, Cultural Practices, and Dealing with SCD were comprised of three to four themes that deepen our understanding of these domains. Figure 1 provides a diagram of the domains and themes of this study.

Figure 1.

Factors impacting sickle cell disease management.

Cultural Values

We defined values as the long-standing community-based guidelines or standards and enduring beliefs held in this community. The participants’ descriptions illuminated a sense of camaraderie, the importance of family, and the necessity of perseverance in the face of difficulty. The themes We Do This Together, Family Ties, and It Is Not Easy provide context for the cultural values influencing SCD management.

We do this together

When participants answered questions about managing SCD or providing care, they repeatedly discussed the importance of a group approach and the assistance of others. The theme of We Do This Together implied that the suffering of one person in the community spelled suffering for the whole community. Participants made it clear that knowledge held by members of the community needed to be shared to improve management and treatment of SCD across the community:

We are not the one that heard it. Someone heard it and came and told us that they have announced that sickle cell has a center in [province name] where they advise people how to maintain, how to take care of yourself, for you to secure yourself.

Many participants described “the sickle cell center” (SCD CBO) as central to the teamwork required to manage SCD and as much a part of the family-oriented approach noted in the theme Family Ties as their immediate family.

Family ties

Another cultural value identified during participant interviews and observation was Family Ties. Family was defined as a network of biological and non-biological members who helped a child or an adult, especially those who had SCD. Participants described living situations and amorphous relationships between family members, clarifying how individuals were related only when directly asked to do so. Participants commonly reported living with extended family. Most of the caregivers described in these Family Ties were female, and the female study participants expressed clearly that they were responsible for keeping their children alive and safe from “the sick” (the commonly used name for illnesses like SCD):

They say it is the suckling mothers that actually do work. Suckling mothers, anything that bothers [the family], it is you that it will affect. When your man doesn’t have anything, you [suckling mother] that is at home, you are the one that must try so you all will get something . . . I think it is me that gave birth to the children, I strain.

These relationships ensured that a person requiring immediate care or suspected of having “the sick” would at least seek health care; in one case, this entailed a more than 300-mile drive on an uncle’s motorcycle from Guinea to Sierra Leone to receive health care. The journey, as described by the participant, was not easy.

It is not easy

Like a song refrain, It Is Not Easy resounded in daily conversation to explain living with and managing SCD while living in a low-resource context. After reporting the difficulties in their lives, community members and participants often voiced It Is Not Easy, but they also shared stories that revealed the ingenuity, industry, grit, and tenacity they exhibited to withstand the challenges of SCD in this context and with available resources. As a general informant explained, “They say one man can eat an elephant, but [a] small bit at a time.” For participants living with SCD, this adage meant developing ways to maintain their dignity and continue working without causing SCD exacerbations. One participant shared that she was “doing garden work” growing plasas (hearty, green potato leaves), which allowed her to feel useful without “overworking” her body: “I make a small garden and from there I can get some money so I can come take my medicine.” Professional caregivers of the SCD CBO discussed the difficulties of resource management and the necessity of creativity for disease management, including managing a large day clinic in a small office space as well as providing acute nursing care (e.g. including heated massage and over-the-counter pain medication) with limited medical supplies (Observations on April 2019).

Cultural Beliefs

Cultural beliefs represent thoughts or opinions that are strongly accepted as explanations for why situations (in this case, disease) occur. Various causes, names, and cures for SCD emerged from the data analysis and were confirmed by field notes of participant observations at the two clinical sites. Older participants described the historical impact of SCD in this community. The symptoms of the disease had become accepted words in the commonly spoken Krio and native languages, referred to as “bone hurt,” “bone sickness,” bone pain, and/or SCD “attacks” in the Kono (mengeh/kamaku), Fula (binta), and Mandingo (kurudumeh) languages as noted by participants. Cultural beliefs stemmed from at least three different perspectives regarding how one develops SCD.

Native sense

Participants used the phrase Native Sense to describe supernatural causes of SCD symptoms. These beliefs were rooted in powers that did not possess a positive connotation (e.g., witches, wizards, or devils). Participants shared stories of people with SCD being possessed by witches or being called witches, and they described a combination of poisonous powers could cause SCD symptoms. Participants felt the Native Sense explained the abdominal swelling, yellowing of the sclera, and pain that people with SCD experience. As one participant explained, “They have a witch in their stomach, that is what is bothering them.” Another remarked, “Some say that it was bad people that birthed me. Witch, they call me a witch. Some people say that it was bad poison I was given, they said a lot.” In some cases, participants also described SCD as “a sick that kills.” There were instances in which participants believed that SCD could be cured in the Native Sense. Participants recounted that a person with SCD who lives past the age of 21 or a woman who lives with SCD and gives birth “will never die of sickle cell.”

Western sense

Professional caregivers or those receiving education from the SCD CBO explained the causes of SCD as related to SCD pathophysiology and used a biomedical, genetics based perspective. SCD symptoms were believed to be caused by infection, dehydration, cold, and physical activity. The Western Sense did not afford a cure for SCD but followed the prescribed medication regimen to minimize “attacks” or to “cool down” the symptoms of SCD. Many lay participants recited the information they had learned over the years from the volunteers of the SCD CBO:

Well, according to what I see and what they tell me, the sickle cell will always come in a child when the papa has small [amount] and the mama has small [amount]; when they have a child, that child will have sickle cell.

These sentiments were confirmed during multiple clinical participant observations (April 2019) of SCD education that emphasized genetic inheritance as well as health maintenance and promotion (i.e., penicillin and malaria prophylaxis, vitamins), including activities to avoid (i.e., running, jumping, playing rough sports, immersion in or drinking cold water). The Western Sense, however, did not preclude some from seeking a higher power for assistance.

Spiritual sense

A sense of spirituality characterized participants’ descriptions of beliefs that the hand of God or Allah was apparent in SCD management and treatment. Most participants expressed that God or Allah had the power or capability to help a person through their SCD experiences if they believed, prayed, fasted, attended church, and/or sought guidance from a leader in their religious sect. The Spiritual Sense represented a belief system that helped participants understand their SCD symptoms. Omnipotent beings were deemed to be so powerful that participants sought their assistance for treatment when they or someone they knew exhibited SCD-like symptoms. As one participant noted, “I look to God, I hope to God. To say that if God can allow this in our body, and so God can also make it go out of our body so we will be well.”

These sentiments were evident on the premises of the community health clinic in daily devotion for professional caregivers, the call to prayer for patients practicing Islam, Sunday church services, and informal conversations that participants had about their lives (Observation on April 7, 2019). The Spiritual Sense guided many participants, and spiritual gathering places offered them an opportunity to gain advice about what to do next.

Cultural Practices

Cultural Practices referred to approaches or techniques that were grounded in cultural beliefs and used for health maintenance. Participants often reported using native or traditional practices, biomedically based approaches, or a combination of these to manage SCD.

The country way

Participants used the phrase Country Way or “Country Medicine” to describe practices that employed traditional medicine and healers. These practices aimed to extract the cause of SCD from the body. The Country Way was prescriptive as participants provided almost identical descriptions of these practices. Almost all discussed the use of native leaves, heating or steaming of the body, and the use of tying (with rope) affected or swollen body parts:

If you ask the children, they will tell you, “It is native medicine they are giving me, country medicine.” Native medicine is country medicine. “That is what I have been drinking, they rub it on me, they tie it and put it on me.” That is, they tie the medicine. “They tie my feet and my hand and put some here and band it.”

Many participants expressed that they no longer practice certain aspects of The Country Way due to poor outcomes. One participant shared, “oh, I tried I failed. That made me not to even mention it. I tried herbalist, other people. When he went to the bush, he brought some leaves, put them in a pot, so that the boy, in fact we left on that treatment. I was very lucky the place was more tolerable otherwise I would have lost that boy.” Another participant explained, “we used to tie them, if you tie the arm, the blood will not circulate. So, the blood will settle, that arm will swell until it bursts. That’s what was happening.”

However, certain parts of The Country Way endure. Massage with steamed leaves (i.e., potato leaves) or mentholated emollients were common and frequently described as effective pain-relieving practices associated with The Country Way. Participants also consumed and encouraged the consumption of native leaves due to their high vitamin and mineral content, which they believed would keep those with SCD healthy. One participant recalled, “I make it, I drink it. That is what has been maintaining me to how you see me today.”

The western way

This set of practices was generally embraced by those participants (and community members) who held The Western Sense beliefs about the origin, symptoms, and treatment of SCD. All participants (health system and community based caregivers, adults with SCD, and general informants) described the process below in a similar prescriptive fashion as was noted in The Country Way:

We give them the antibiotic that they should be taking to avoid infections. We give them sample and that of the folic acid that we give them all. We also give them the vitamin C all, and we give them the analgesic for the pain . . . We have the ibuprofen 200 mg, we have the tabs Diclofenac, I think 200mg, 50mg also for the age categories . . . the paracetamol 500 mg tablets and also the 250 mg tablets according to the age range.

A number of participants living with SCD and family care givers described improved health as a result of The Western Way; a participant reported, “we continue to take the medicine and the advice, and the child now does not get sick like she was before.”

The hybrid way

Many participants also described a combination of The Country Way and The Western Way. Participants, particularly professional caregivers, discussed their pharmaceutical treatment as only one means of managing SCD. As one participant explained, it is “not everybody that we give medicine, not everybody because the medicine is not enough.” A participant explained that a professional caregiver used massage to treat her young child, who could not walk for 4 years due to contractures following repeated, untreated SCD crises. A cultural practice informational session was provided to SI on common massage emollients used in this community. This practice relied on multilevel intervention as one participant noted, “They give me medicine. They rub rub my body. They give me medicine. They rub rub our bodies. They care for me well.” A young woman was also brought to the SCD CBO after a failed trial of tying up an affected limb, and the use of both biomedical and traditional means of disease management were witnessed (Observations on April 2, 3, and 8, 2019).

One of the main features of The Hybrid Way was an emphasis on counseling the patient and their family about SCD. Genetic, individual, and family counseling integrated topics such as avoidance of triggers for vaso-occlusive episodes (e.g., dehydration, poor nutrition, changes in body temperature) and methods for initiating discussions about SCD with community members. As one participant explained,

When you meet me the first thing is to reassure you that this is not the end of your life. Having sickle cell doesn’t mean you are going to die today, tomorrow, or the day after. Give you assurance that with all the management and all the advices that we are going to give you, if you work with it, that you will live like any other people.

The Hybrid Way included home visits, especially for frequently ill clients. A professional caregiver explained that these home visits allowed the SCD CBO to assess both living conditions and home management of SCD:

We always visit them [participants], talk to them . . . Sometimes we check if they are eating on time, if they are drinking water. When we have funding, we used [it] to buy small, small provision, we carry to the ones, visit them.

Dealing With SCD

Participants reported that Dealing with SCD was an experience of physical ailment but also of discovery entailing personal, social, and financial challenges as well as an ultimate decision to accept or reject their diagnosis of SCD. It was not an individualistic experience, as many participants described the importance of previously highlighted Cultural Values; however, this domain suggested that the communal spirit was also used to impose stigma and isolation on those living with SCD and their families. This domain highlights the experiences and perspectives of those living with or caring for someone with SCD in this community.

Experiencing the sick

Participants often first described the pain experience of a person living with SCD. Many participants described a flare of SCD symptoms as an “attack” by “the sick.” Some stated that the disease was “standing up” in their body or would “cool down,” terms suggestive of the cyclical nature of SCD symptoms. Physical pain was not the only experience reported. Discussing their birthing experience, mothers reported “being short of fluid” to describe SCD-related anemia. Many participants discussed the psychological turmoil that a person with SCD goes through due to both the disease and community beliefs.

Reports of stigma and isolation at the hands of friends, family, community members, and health care professionals emerged repeatedly in the data analysis. Participants (both male and female) narrated their experiences of being called witches, demons, or “premature” because of their appearance (i.e., swollen abdomen, yellow eyes, skinny). Many women described family altercations that placed blame on them for the health of their children. One shared, “You know when a woman is sick too much and the child she births is sick too much, the man will take off.” Obtaining an education as a person with SCD often involved teasing and corporal punishment. As one participant recounted, “If you say I am not well, then they go and flog you.” Participants shared that they had received assistance to resolve school concerns through the SCD CBO but felt that more support was needed to protect children living with SCD while they were at school (Observations on April 8 and 10, 2019).

The difficulties of disease management were amplified by socioeconomic challenges as noted by one participant: “At that time when you go into crisis if there is no money and then you don’t know about the medication at all, then normally the child will die of that.” When describing the experience of being sick, many participants discussed a prohibitive “fee-at-time-of-service” scheme used in the local hospital facility. The range in socioeconomic status was furthered illuminated during four home visits (Observation on April 10, 2019): One home was an abandoned fishery with no electricity or running water; another was a resettlement home built by foreign diamond mining companies adjacent to an expanding diamond mine. Participants described varying levels of financial stress related to SCD management in this context.

Diagnosing the sick

To determine the cause of SCD symptoms, many participants narrated their discovery of their SCD diagnosis. Participants reported relying on informal and formal referral systems that direct a person with symptoms to be screened at the SCD CBO or the local government hospital. Participant observations (April 2019) indicated that the SCD CBO had a limited number of genotyping tests and the local government hospital was only able to perform sickling tests (which do not confirm genotype). The local government hospital often referred persons with a positive sickling test to the SCD CBO for confirmatory genotyping, if it was available, and vice versa:

They wrote a paper for me and said for me to go to the government hospital to do a test for me. So I went to the government hospital. They did a test for me. They told me it is sickle cell that is in my body.

Owning or disowning the sick

Participants described two approaches to the diagnosis of SCD. Some participants discussed feeling disbelief, sadness, or denial upon diagnosis, which resulted in their disowning the treatment plan. As one participant noted, “Some of us suckling mothers are stubborn. We don’t take advice.” The psychological burden of diagnosis and lack of SCD awareness often increased the stigma that women faced in this setting. Some women recounted convincing their male partners to be screened. Another participant explained that her husband was afraid: “I tried to force him so he will come to do the test, he said he didn’t want to, he said he didn’t want to have a spoiled [broken] heart.” Other participants narrated a process of acceptance, beginning treatment, and even becoming SCD community advocates. Many owned the diagnosis by “following the law” (to maintain their health) that is taught by the SCD CBO. Acceptance also allowed many to stand up to the stigma of an SCD diagnosis:

The particular side where I am, many of them their children fall down and die in their hands because of this sickness. But since I have been encouraging many people, they come. Even when I leave here (the SCD CBO building), when I go, they ask me, what did they tell you? I give them the advice, the advice they give me, the teaching, medicine, I direct them how they should be. Many of their children they live happy.

Discussion

The results of this study highlight the importance of understanding the beliefs, values, and practices of a rural Sierra Leonean community to inform future clinical management of SCD. The findings of this study show that SCD management is influenced by the patient’s understanding of the disease but also by external forces from their family, community, spiritual and traditional systems, and social determinants of health. Most apparent in the domain of Cultural Values, the influence of the communal nature of daily life was discussed by many participants. Participants explained We Do This Together because in order for the community to thrive, each community member was dependent on assistance from and the health of other members; however, this value seemingly contrasts with descriptions of Experiencing the Sick that describe physical pain, economic struggles, and psychological battles of living with SCD or caring for someone with SCD that result from multilevel stigmatization. Although many different belief systems were discussed, The Native Sense and The Spiritual Sense demonstrate an external cause of SCD rather than a genetic cause (as seen in The Western Sense). Some practices stemmed from long-standing traditional beliefs about supernatural or witchcraft-based causes of disease while others were developed from evidence-based treatment guidelines. Participants described The Hybrid Way as a practice that acknowledged traditional, spiritual, and biomedical beliefs to address disease management. Participants also described that an SCD management experience could be isolating and burdensome.

Several findings of this study support extant literature on SCD management in LMICs and high-income regions. The participants’ descriptions of the physical pain and symptoms they experience in this rural city are comparable to findings reported in the literature about Nigeria (Ikefuna et al., 2009), Ghana (Hammond et al., 2015), and the United States (Adegbola et al., 2012). The financial strain experienced by the patients, their families, and their community due to costs of treatment and health maintenance has been described in the literature as a common occurrence due to “fee-at-time-of-service” health system models (Amendah et al., 2013; Dennis-Antwi et al., 2011; Mathew & Nambiar, 2020). These types of fee models further marginalize vulnerable, impoverished groups and often result in people deciding to delay care. These delays in care usually have a negative impact on patient health outcomes as a person’s condition deteriorates. Moreover, the increased acuity of a person’s condition results in higher care costs, perpetuating an often vicious cycle of health disparities in LMICs (Mathew & Nambiar, 2020).

Our study confirms findings by Berghs, Dyson and colleagues (2020) that SCD management falls on the shoulders of women. Stigma and potential harm from their husbands (and their families) loomed over female participants in a context where it is considered to be a woman’s obligation to ensure their child’s health. The women of the current study recounted that assistance from the SCD CBO helped to convince their husbands to be screened for SCD. The ability to shift blame, psychological burden, and risk through screening changed many lives and suggests that increased awareness of disease inheritance is desperately needed at community and provider levels (Berghs, Dyson et al., 2020). Denying or suppressing a SCD diagnosis allowed participants to avoid stigma and community isolation, a finding also reported in studies in Ghana (Dennis-Antwi et al., 2011), Nigeria (Anie et al., 2010), Kenya (Marsh et al., 2011) and Jamaica (Anderson & Asnani, 2013).

The Spiritual Sense was pertinent to managing and coping with SCD for the individual, their family, and the community (Bediako et al., 2011; Dennis-Antwi et al., 2018). As in countries like Ghana, religious leaders and houses of worship can serve as facilitators or obstacles to prompt SCD management as these existing systems represent community contact points to increase SCD awareness and improve screening or enable stigmatization. Nzewi (2001) reported that SCD was thought to be Malevolent ogbanje, or evil reincarnation in Igbo culture, as was reported in the current study. The Native Sense depicts a historical referent of SCD within native languages that describes SCD as “bone hurt” or “bone sickness” which may be alleviated using some traditional practices such as massage. Although massage is recognized in the National Heart, Lung, and Blood Institute (NHLBI, 2014) expert guidelines, the evidence base is too limited for practice recommendation. These findings suggest that renewed research efforts are warranted to study the effectiveness of massage as a nonpharmacological treatment for vaso-occlusive episodes (Williams & Tanabe, 2016). Western Sense and The Western Way were named as other means of understanding and managing SCD. These beliefs and practices align with the National Heart, Lung, and Blood Institute’s (NHLBI, 2014) recommendations for infection prevention and health maintenance in individuals living with SCD. Emphasis is placed on pharmacological management of SCD following newborn screening; however, disease-modifying treatment like hydroxyurea was unavailable in Sierra Leone at the time of this study. The Western Way was therefore heavily focused on infection prevention, vitamin supplementation to reduce nutritional causes of anemia, and home-based pain management.

This study addresses the current literature gap by describing the context of SCD management in rural, eastern Sierra Leone. Highlighting the existing systems for disease management illuminates the potential for adaptation and application to other contexts. Adaptation of these SCD management systems can be further investigated using an implementation science framework to examine appropriateness, adaptation, feasibility, and effectiveness of community-based health care organization practices in other regions. Moreover, these findings suggest that interventions to increase SCD management must be holistic in scope, including not only a biomedical focus but also strategies to address socio-cultural and political determinants of health (Berghs, Ola et al., 2020). Further research should include existing patient and provider education and counseling programs within systems that acknowledge the rural, Sierra Leonean context. As noted by Dutta (2008), discussions of health and health communication are not limited to the presiding biomedical paradigm; other social factors may have greater influence on the ability to manage a disease process. Participants described the well-known physiological complications of SCD and suggested pharmaceutical treatments; however, they reported many social determinants that defined their ability to maintain health in this context suggesting that further health policy development and research are warranted in this context. These concepts are similar to Diefenbach and Leventhal’s (1996) Common Sense Model in that beliefs provide the individual a way to label their health threat. According to the Common Sense Model, participants appraised their the severity of their symptoms based on the effectiveness of available cultural practices and cultural/psychological inputs. However, it should be noted that culturally-centered care and the assessment of that care recognize that culture and context are not inputs but constitute the decision-making environment.

In this study, participants living with SCD, their family members, and professional caregivers provided in-depth explanations of their experiences with SCD management that had not previously been reported. The provision of this level of detail allowed a compilation of data on individual- and community-level systems, contributing to deeper knowledge about coping with SCD in the Sierra Leonean context. Guided by the theoretical frameworks of Dutta (2008) and Habermas (1985) which suggest communicative action can generate culture-centered health and illness definitions, the design of this study adds a critical perspective to the extant literature. As this type of study requires knowledge about and recognition of the needs of the community, travel to Sierra Leone was required to establish rapport with the community prior to conducting any research activities. The partnership between the researchers and the local SCD providers increased the efficiency and effectiveness of the research strategy used in this study.

The results of this study provide data on the landscape of the existing health care infrastructure in this community which highlights three overall health care delivery systems. Future practice and research implications include the development of culture-centered educational and disease management interventions for health-system based and family caregivers, and those living with SCD. This study focused on what would be deemed outpatient care, future research should focus on the inpatient experience. In combination with the data gathered from the current study, decision support tools for SCD management could be developed. Cost-benefit analysis in this context greatly needed to understand the economic burden associated with SCD which can inform health policy. With support from the Ministry of Health and Sanitation, expansion of The Hybrid Way across this region will extend access to primary care interventions for SCD management.

The results of this study must be understood within the context of its limitations. There were many recruitment challenges. Initially, the research team created a recruitment flyer with a contact phone number for interested participants, but it became clear that asking potential participants to call or text would not be feasible given the cost of phone credits. Attempts to enroll participants during an SCD clinic day with the SCD CBO proved to be logistically challenging given the number of potential participants present; therefore, the research team relied on the assistance of the research assistant for participant recruitment. The relationship that this volunteer has with the community may not be transferred easily to other contexts.

The context of this study is limiting in that the tribes and ethnicities of this location may not mirror those in other parts of Sierra Leone or sub-Saharan Africa; however, in future studies, this limitation could be remedied with an increased sample size that spans the country to increase the transferability of findings. The length of time Dr. Ibemere spent in the country is another limitation of this study as a longer immersive experience would have yielded further rich, dense interview and observation data to strengthen the findings, which demonstrate that it is important to gain a better understanding of the role of the traditional healer and religious leader in relation to SCD health maintenance. Further ethnographic as well as longitudinal studies are warranted as there is a dearth of information available on SCD treatment and management in Sierra Leone.

Conclusion

The cultural influences on SCD care and management were the foci of this study. Findings from this study demonstrated that SCD care and management were influenced by cultural beliefs, values, practices, and the experience of SCD. Participants described many difficulties and obstacles to understanding and managing SCD for themselves, their loved ones, or their patients, including experiences of seeking guidance from God or Allah, traveling long distances to meet a traditional healer or witch doctor, paying fees for medication and treatment, trying to manage at home, or finding assistance through the auspices of community-level health care resources. Further research focused on the role of religious and traditional leaders, the combination of traditional and Western methods of SCD management, and the development of culturally appropriate educational interventions for patients, caregivers, and community members is needed.

Supplemental Material

sj-docx-1-qhr-10.1177_10497323211006384 – Supplemental material for “It Is Not Easy”: Cultural Influences of Sickle Cell Disease Management in Rural, Eastern Sierra Leone

Supplemental material, sj-docx-1-qhr-10.1177_10497323211006384 for “It Is Not Easy”: Cultural Influences of Sickle Cell Disease Management in Rural, Eastern Sierra Leone by Stephanie O. Ibemere, Donna Z. Shambley-Ebron, Paula Tanabe and Cheedy Jaja in Qualitative Health Research

Supplemental Material

sj-docx-2-qhr-10.1177_10497323211006384 – Supplemental material for “It Is Not Easy”: Cultural Influences of Sickle Cell Disease Management in Rural, Eastern Sierra Leone

Supplemental material, sj-docx-2-qhr-10.1177_10497323211006384 for “It Is Not Easy”: Cultural Influences of Sickle Cell Disease Management in Rural, Eastern Sierra Leone by Stephanie O. Ibemere, Donna Z. Shambley-Ebron, Paula Tanabe and Cheedy Jaja in Qualitative Health Research

Footnotes

Acknowledgements

The authors would like to acknowledge and thank every participant who shared their story with us. We would also like to acknowledge the leaders and staff of the Sickle Cell Carers Awareness Network and Adama Martha Memorial Community Health Center. A special thanks to Drs. Rita Masese, Dominique Bulgin, Beverly Hittle, and Donnalee Frega for their keen eyes, thoughtful comments, and thorough review of this manuscript.

Declaration of Conflicting Interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This work was supported by the University of Cincinnati College of Nursing Research/Scholarly Activities Council PhD Dissertation Award; however, the supporting source had no involvement in research activities or restrictions regarding publication.

ORCID iD

Stephanie O. Ibemere

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Supplemental Material for this article is available online at . Please enter the article’s DOI, located at the top right hand corner of this article in the search bar, and click on the file folder icon to view.

Author Biographies

Stephanie O. Ibemere is currently a postdoctoral researcher at Duke University School of Nursing with interests in ethnography, community-engaged participatory research, and implementation science. Her research program focuses on building healthcare provider capacity to deliver high quality and effective care for chronic disease symptom management in low and high resource contexts with a special interest in sickle cell disease.

Donna Z. Shambley-Ebron is Associate Professor Emerita and former Program Director of the PhD program for University of Cincinnati College of Nursing with extensive experience in health disparities, transcultural care, ethnography, and community-based participatory methods. Her research program has focused on developing and testing culturally and developmentally appropriate interventions for historically marginalized groups, such as African American women, focused on risk reduction, sexual health, and HIV prevention.

Paula Tanabe is the Laurel B. Chadwick Distinguished Professor in the Schools of Nursing and Medicine and Vice Dean for Research in the Duke School of Nursing. As a clinical and health services researcher, her program of research aims to improve healthcare systems and patient health outcomes for persons living with sickle cell disease with particular focus on pain management in the emergency department during a vaso-occlusive crisis.

Cheedy Jaja is an associate professor in the University of South Carolina College of Nursing and board certified Psychiatric and Mental Health Nurse Practitioner with expertise in health systems strengthening and nurse capacity building. His program of research focuses on addressing issues of social justice, health disparities, and improving health outcomes for marginalized and vulnerable populations with a particular focus on sickle cell disease and pharmacogenetics in sickle cell pain management.

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