ABSTRACT

This study investigated the coping mechanisms adopted by individuals living with sickle cell disease at Lagos University Teaching Hospital, Idi-Araba, Lagos. Data were collected from both primary and secondary sources. The primary data were obtained through a structured questionnaire, with 105 copies distributed randomly to people living with sickle cell disease in hospitals, schools, churches, and mosques. Secondary data were gathered from relevant and recent journal publications accessed through the internet. The information collected from these sources was analyzed using frequency distribution tables and simple percentage analysis to interpret and present the study findings.

The findings revealed that the respondents possessed an adequate level of knowledge and understanding of the psychosocial effects of sickle cell disease, as well as its management and treatment strategies. This was demonstrated by the high proportion of respondents who agreed with the coping options presented, including engaging in intimate relationships, maintaining commitment, ambition and industriousness, sustaining a positive and cheerful attitude towards their circumstances, communicating personal concerns to others, seeking social support through organized activities, and consulting professional advisers such as counsellors.

Furthermore, the global burden of sickle cell disease continues to increase, with considerable implications for the quality of life of affected individuals. The simultaneous occurrence of psychosocial challenges among people living with sickle cell disease and their caregivers was identified as a concern capable of negatively affecting both the patients and their families. Consequently, the study emphasizes the importance of considering parents within the broader context of their family relationships and circumstances. Healthcare professionals should provide appropriate psychological care and support to affected individuals and their caregivers to improve the effectiveness of disease management and treatment strategies. In view of these findings, policymakers and non-governmental organizations should collaborate in organizing public lectures, seminars, and awareness programmes to discuss appropriate measures for reducing the burden and occurrence of sickle cell disease in society and promoting healthier communities.

CHAPTER ONE

INTRODUCTION

1.1 Background of the Study

More than seven million babies are born annually with structural or functional abnormalities, many of which result from the inheritance of defective genes (Piel et al., 2013). Sickle cell anaemia (SCA) is an inherited disorder transmitted from parents to their children. It develops when a child inherits the gene responsible for sickle haemoglobin (HbS), a structural variant of normal adult haemoglobin (HbA). Haemoglobin is the protein contained in red blood cells, which are normally disc-shaped and responsible for transporting oxygen throughout the body. Inherited genetic information is obtained from both parents, with the genetic composition of an individual reflecting contributions from each parent (Piel et al., 2013).

Similarly, Yawn, Buchanan, Ballas, Hassell, and James (2014), as well as Ohijoungbe and Burnett (2013), described sickle cell anaemia (SCA), also known as drepanocytosis, as a hereditary blood disorder characterized by an abnormality in the haemoglobin molecule responsible for carrying oxygen in red blood cells. This abnormality causes red blood cells to become rigid and assume a sickle-like shape under certain conditions. Sickle cell disease develops when an individual inherits two abnormal copies of the haemoglobin gene, one from each parent. The specific form of the disease depends on the mutations present in the inherited haemoglobin genes. Individuals who inherit only one abnormal copy of the gene generally do not experience the symptoms of the disease and are described as having sickle cell trait. Such individuals are also known as carriers.

Brass (2011) explained that sickle cell disease results from a mutation in the beta-globin gene responsible for producing haemoglobin. This mutation causes the replacement of glutamic acid with valine at the sixth position of the beta-globin chain, resulting in the production of abnormal haemoglobin known as haemoglobin S (HbS), rather than the normal haemoglobin A (HbA). The altered physicochemical properties of haemoglobin S cause its molecules to polymerize and precipitate, thereby changing the shape of red blood cells and making them sickle-shaped. This process also increases blood viscosity through the formation of tactoids.

Brass further explained that sickle cell anaemia is inherited through an autosomal recessive pattern involving both parents. Individuals who carry only one affected gene are generally asymptomatic and are described as heterozygous carriers. When a child inherits an affected gene from each parent, the child may develop the homozygous HbSS form of sickle cell anaemia.

During fetal development and the early stages of life after birth, the effects of the HbSS genotype are limited by the production of fetal haemoglobin (HbF). This fetal haemoglobin reduces the effects of sickling by diluting the concentration and influence of haemoglobin S. As the bone marrow produces increasing numbers of red blood cells containing higher amounts of haemoglobin S and decreasing amounts of fetal haemoglobin, the manifestations of sickling gradually become apparent. Consequently, infants may begin to exhibit symptoms of the disease from approximately six months of age, when fetal haemoglobin levels start approaching those found in adults (Brass, 2011).

According to Brass (2011), sickle cell anaemia is one of the most recognized hereditary haematological disorders affecting humans. The author estimated that approximately 30,000 children are born annually with sickle cell anaemia worldwide, making it an important genetic and epidemiological health concern in Brazil and other parts of the world. The disease was described as originating in Africa and spreading to the Americas through the forced migration of enslaved Africans. Its occurrence is more common in populations with a higher proportion of African ancestry, including parts of northeastern Brazil and the states of São Paulo, Rio de Janeiro, and Minas Gerais.

In these Brazilian regions, the cited study reported the occurrence of new cases of sickle cell disease among live births and the presence of sickle cell trait among a proportion of the population. It further estimated that approximately 2,500 children are born annually with sickle cell disease in Brazil (Brass, 2011).

The non-white population of Brazil was estimated to constitute 44.66% of the population according to the 2000 population census. Among this population, approximately 1% to 6% were reported to carry the HbS gene, thereby contributing to the continued occurrence of sickle cell anaemia. Brazilian scientific literature consequently identifies the condition as a significant public health concern.

In Nigeria, the prevalence of sickle cell disorders has been described as particularly high in comparison with that of several other African countries. Available estimates cited in the original study indicate that more than 100,000 Nigerian children are born with sickle cell disorders annually. Children affected by the condition experience a higher frequency of illness than the general population and face an increased risk of premature death, particularly during the first five years of life.

Statistical information cited in the original study also indicates that more than 40 million Nigerians carry the sickle cell gene, commonly referred to as the ‘S’ gene. This represents a substantial number of carriers compared with the populations of several other affected African countries. Despite the considerable number of individuals affected by sickle cell disorders in Nigeria, negative perceptions and unfavourable attitudes towards the condition remain evident in society (WHO, 2006).

The psychosocial consequences of sickle cell disease can be distressing for affected children, their parents, other family members, and caregivers. Children living with the condition may experience difficulties adjusting to different aspects of everyday life. In particular, sickle cell disease has been associated with emotional and behavioural difficulties, poor self-concept, challenges in interpersonal relationships, restricted participation in athletic activities because of health-related limitations, and poor academic performance (Noll et al., 2007).

The effects of the disease also extend to the family, especially those responsible for providing care. Caregivers of children living with sickle cell disease may experience emotional distress, psychological pressure, increased family stress, and additional financial responsibilities. These challenges are partly associated with the unpredictable nature of painful crises and the demands involved in managing the condition (Moskowitz, 2007).

During sickle cell crises, affected children may experience severe pain throughout the body, particularly in the legs and back. Their caregivers are responsible for supporting various aspects of disease management, including encouraging preventive health practices, managing painful episodes, teaching appropriate coping skills, and ensuring adequate nutrition and hydration. Parents and other caregivers may also experience inadequate support from relatives, friends, and other members of the community when a child experiences a crisis. Such circumstances can contribute to frustration, emotional distress, and feelings of hopelessness among caregivers.

The original study further explains that a definitive cure was not available at the time of its writing and identifies prevention and appropriate management as important approaches to addressing the condition. Preventive measures include genetic counselling for prospective or intending couples and guidance from healthcare professionals on healthy living practices. Such information may be disseminated through electronic and print media to increase public awareness and encourage informed decisions.

At the family level, the psychosocial effects of sickle cell disease may include financial hardship, insufficient social support, and limited assistance for parents caring for affected children. Children living with the disease may experience recurrent pain, frustration, inadequate care and support during crises, difficulties with social adjustment, visual impairment, and the loss or disruption of friendships. The condition may also affect their access to affection, educational opportunities, employment prospects, and broader psychosocial well-being.

These challenges demonstrate the importance of understanding the coping mechanisms adopted by individuals living with sickle cell disease. Appropriate coping strategies, combined with psychological support, family involvement, and effective healthcare services, may help affected individuals manage the demands of the condition and improve their quality of life.

1.2 Problem Statement

In Nigeria and other parts of the world, numerous studies have investigated sickle cell disease, particularly its causes and the factors contributing to its occurrence in society. However, according to the researcher, there has been insufficient comprehensive investigation into the psychosocial effects of the condition on individuals living with it. The present study therefore seeks to address this gap by examining the psychosocial experiences of people affected by sickle cell disease and the coping mechanisms they adopt.

Furthermore, previous investigations have often concentrated on explaining the causes of sickle cell disease and the factors responsible for its continued prevalence in Nigeria. Comparatively less attention has been given to the psychosocial consequences experienced by parents and other caregivers of children living with the condition. This study intends to contribute to addressing that gap by examining the effects of the disease on caregivers and the challenges associated with providing care.

Although some studies have considered the financial burden of sickle cell disease on affected families and caregivers, less attention has been devoted to the role of family support, social welfare services, and self-help programmes in reducing the psychosocial burden of the condition. These forms of assistance may be important in improving the quality of care provided to people living with sickle cell disease and in reducing the difficulties experienced by their families.

The limited attention given to these issues creates a need for further investigation into the experiences of affected individuals, the psychosocial challenges they encounter, and the methods they use to cope with the condition. It is also important to examine the support available to caregivers and the ways in which such support may contribute to better disease management.

In response to these concerns, the researcher undertakes this study to investigate the psychosocial effects of sickle cell disease and the coping strategies adopted by people living with the condition at Lagos University Teaching Hospital, Idi-Araba, Lagos.

1.3 Purpose and Objectives of the Study

Main Objective

The main objective of this study is to examine the coping mechanisms adopted by individuals living with sickle cell disease at Lagos University Teaching Hospital, Idi-Araba, Lagos.

Specific Objectives

The specific objectives of the study are to:

1. Assess respondents' knowledge of the causes of sickle cell disease.

2. Examine the incidence of sickle cell disease among the respondents.

3. Assess the psychosocial effects of sickle cell disease among the selected respondents.

4. Investigate the psychosocial effects of sickle cell disease on parents.

5. Identify and examine the coping strategies adopted by individuals living with sickle cell disease.

1.4 Research Questions

The study seeks to answer the following research questions:

1. What does sickle cell disease mean to the respondents?

2. What is the incidence of sickle cell disease within the local government area?

3. What psychosocial effects do people living with sickle cell disease experience?

4. What psychosocial effects does sickle cell disease have on caregivers?

5. What specific coping strategies are adopted by people living with sickle cell disease?

1.5 Research Hypotheses

The following null hypotheses guide the study:

1. There is no significant difference between the psychosocial effects of sickle cell disease and the coping strategies adopted by the respondents.

2. There is no significant relationship between respondents' knowledge of the causes of sickle cell disease and their demographic characteristics.

3. There is no significant relationship between respondents' knowledge of the causes of sickle cell disease and the coping strategies they adopt.

1.6 Significance of the Study

An investigation into the perceived psychosocial effects of sickle cell disease and the coping strategies adopted by people living with the condition at Lagos University Teaching Hospital, Idi-Araba, will contribute to the existing body of knowledge on the experiences and coping practices of affected individuals. The study is expected to be useful to people living with sickle cell disease, their parents and caregivers, healthcare providers, and society at large by improving understanding of the psychosocial challenges associated with the condition and the strategies used to manage them.

The findings may also highlight the importance of introducing and strengthening social welfare initiatives and self-help group programmes for affected individuals and their families. Such programmes may provide opportunities for emotional support, information sharing, and assistance with the challenges of living with sickle cell disease. By addressing some of the difficulties experienced by patients and their caregivers, these interventions may contribute to improved quality of life and reduced caregiving burdens.

In addition, the findings of the study may support the practical application of appropriate disease-management strategies and preventive measures. Improved awareness, timely healthcare, and suitable preventive interventions may contribute to reducing the burden of sickle cell disease within the population.

The study may also benefit prospective couples by emphasizing the importance of genetic counselling and routine haemoglobin genotype testing before marriage or conception. A better understanding of haemoglobin genotypes and the inheritance of sickle cell disease can help individuals make informed reproductive decisions and understand the potential genetic risks to their children.

Finally, it is anticipated that improved knowledge among healthcare professionals, parents, and guardians, combined with public education and counselling initiatives undertaken by organizations such as the Sickle Cell Club of Nigeria and other relevant medical bodies, will strengthen awareness of the condition. Such efforts may encourage informed decision-making, improve support for affected individuals and families, and contribute to reducing the burden of sickle cell disease in society.