CHAPTER ONE

INTRODUCTION

1.1   Background of the Study

Sickle cell disease affects approximately 100,000 Americans and has been established to affects about 1 out of every 365 Blacks or African-American birth, about 1 in 13 Black or African-American babies is born with sickle cell trait (SCT) (WHO, 2014). Sickle cell disease is a genetic blood disorder that affects the haemoglobin within the red blood cells and has been found to cause death among Blacks or African-American children, younger than 4 years of age which fell by 42% from 1999 through 2002 (WHO 2014), this significant drop was because of the advent of vaccine in 2000 that protects against invasive pneumococcal illness in individuals (WHO 2014). Sickle cell has not been given adequate recognition by the Nigerian Government in recent time, despite several nongovernmental organizations creating awareness of this illness.

In individuals with SCD, the red blood cells, which are usually flexible and round, become rigid and sickle-shaped. The sickled red blood cells aggregate and block small blood vessels, preventing the normal flow of blood. This vascular occlusion causes intense painful crises, which are the hallmark of SCD. These painful vaso-occlusive episodes are the most frequent problem encountered by individuals with SCD, resulting in 91% of hospital admissions (Brozovic, 1987). Other complications of  SCD include susceptibility to infections, acute chest syndrome, anemia, and stroke. The disease can also affect the physical appearance of adolescents. Small stature and delayed puberty, complications of SCD, can affect self-esteem and peer relationships (Charache, Lubin, & Reid, 1989). A current research focus is to understand why some children and adolescents with SCD develop psychological problems while others do not. Family functioning, such as competence of the family to cope with the illness, is one domain of variables that may help researchers and clinicians understand which children with SCD are most at risk for psychological problems. Family competence refers to the ability of the family to manage the stressors they confront, including both effective emotion management and problem management. By gathering data on children with a range of adaptational outcomes to SCD, researchers could study more carefully children and families who are adapting well to the disease, and those lessons could be applied to children with adjustment difficulties. A number of models attempt to explain why some children with chronic illness enjoy good psychological well-being while others do not. For example, the disability-stress-coping model described by Wallander and his colleagues (Wallander & Varni, 1992; Wallander, Varni, Babani, Banis, & Wilcox, 1989) includes risk and resistance factors that contribute to adaptation. The family environment, social support, family members’ adaptation, and utilitarian resources are examples of resistance factors that influence appraisals and coping in response to stressors associated with chronic conditions. Similarly, the transactional stress and coping model developed by Thompson and his colleagues (Thompson, Gil, Burbach, Keith, & Kinney, 1993) attempts to account for variations in the adjustment of children with chronic illness. Child adjustment is influenced by a number of factors, including maternal adjustment, which is influenced by appraisals and coping efforts as well as family functioning. When applied to SCD, both of these models, as well as other perspectives (e.g., Slaughter & Dilworth-Anderson, 1990), would conceptualize SCD as a stressor or as a condition that produces stressors that necessitate adaptation by families. Although different models emphasize the role of family functioning to a greater or lesser extent, each acknowledges the potential of the family environment to affect children’s psychological adjustment. Kliewer, Sandier, and Wolchik (1994) identified alternative pathways by which families influence children’s adaptation to stressors. Parents both model competent responses to stressors and shape the family context in which coping in response to stressors occurs. Parents are responsible for establishing family routines, for creating a sense of cohesion or disengagement among family members, for setting norms regarding communication in the family, and for regulating contact with peers, other adults, and extended family members. Thus, family competence at coping with stressors largely is influenced by parental behavior. Families who are more competent may enhance psychological adaptation to the disease process in several ways. First, competent families do a better job of making children feel cared for and safe. Feelings of security and belonging, in turn, may reduce children’s sense of threat in response to disease related and other stressors. Second, competent families convey to their children that they matter, which may enhance self-esteem and again reduce harmful threat appraisals. Third, more competent families may influence children’s adjustment by conveying the notion that support is available, and thus may affect children’s evaluations of their resources to manage stressors. In the above ways competent families affect children’s sense of threat, which has been linked theoretically and empirically to adjustment (e.g., Kliewer et al., 1994; Kliewer, Fearnow, & Walton, 1998; Sheets, Sandier, & West, 1996). Competent families also affect children’s coping efforts by modeling adaptive coping strategies, facilitating approach-oriented versus avoidant coping efforts, and providing access to coping resources both inside and outside the family. Some of these notions are echoed by Kazak and her colleagues (Kazak, Segal-Andrews, & Johnson, 1995), who argue for the importance of a family systems approach in understanding children’s adjustment to illness.

Presence of patients with SCA will lead to stress, emotional, and social disturbances among families (Burlew et al., 1989). In general, negative perception was reported from community toward sicklers regarding attitude, health problems, educations, and psychological issues (Anie et al., 2010). The complications of sickle cell disease (SCD), whether physical, psychological, school achievements, or emotional, were well documented (Grove et al., 2013). On considering the quality of life of sicklers, one should never ignore social, emotional, and psychological aspects of the disease (Asnani, 2010). In view of the foregoing, this study sought to assess the psychological impact of living with the much-dreaded sickle cell Anaemia Disease.

1.2     Statement of the Problem

The World Health Organization noted the highest prevalence of hemoglobin AS in Africa to range between 10 and 40 % of the population in some areas. A percentage high enough to necessitate an investigation into the disadvantages of living with sickle cell Anaemia. There is a dearth of information on the psychological impact of living with the condition. Hence, the need for this research.

1.3       Objectives of the Study

The main objective of this study is to determine the psychological impact of living with sickle cell anaemia. Specific objectives include;

1. To determine the impact of sickle cell anaemia on the cognitive and psychosocial behaviour of people living with the condition.
2. To determine how sickle cell anaemia affects the central nervous system of people living with the condition.

1.4       Research Questions

1. Does sickle cell anaemia have any significant impact on the cognitive and psychosocial behaviour of people living with the condition?
2. Does sickle cell anaemia affect the central nervous system of people living with the condition?

1.5       Research Hypotheses

H01: Sickle cell anaemia does not have any significant impact on the cognitive and psychosocial behaviour of people living with the condition.

H02: Sickle cell anaemia does not affect the central nervous system of people living with the condition

1.6       Significance of the Study

This study will be of immense benefit to other researchers who intend to know more on this study and can also be used by non-researchers to build more on their research work. This study contributes to knowledge and could serve as a guide for other study.