Project Materials

PSYCHOLOGICAL IMPACT OF LIVING WITH SICKLE CELL ANAEMIA

Chapter one

INTRODUCTION

1.1 Background of the Study

Sickle cell disease affects roughly 100,000 Americans and has been estimated to afflict one out of every 365 Black or African-American births, with one in every 13 Black or African-American newborns being born with sickle cell trait (SCT) (WHO, 2014).

Sickle cell disease is a genetic blood disorder that affects haemoglobin within red blood cells and has been found to cause death among Black or African-American children under the age of four, with a 42% decrease from 1999 to 2002 (WHO 2014).

This significant drop was due to the introduction of a vaccine in 2000 that protects against invasive pneumococcal illness in individuals (WHO 2014).

Sickle cell disease has not received appropriate acknowledgement from the Nigerian government in recent years, despite the efforts of various nongovernmental organisations to raise awareness about the disease.

In people with SCD, red blood cells that are normally flexible and spherical become rigid and sickle-shaped. The sickled red blood cells clump and plug small blood arteries, impeding the normal flow of blood. This vascular blockage results in acute painful crises, which are characteristic of SCD.

These painful vaso-occlusive episodes are the most common condition experienced by people with SCD, accounting for 91% of hospital admissions (Brozovic, 1987). Other problems of SCD include infection risk, acute chest syndrome, anaemia, and stroke.

The condition can also have an effect on adolescents’ physical appearance. Small stature and delayed puberty are SCD problems that can have an impact on self-esteem and peer interactions (Charache, Lubin, & Reid, 1989).

A recent study focus is on determining why some children and adolescents with SCD suffer psychological issues while others do not.

Family functioning, such as the family’s ability to manage with the illness, is one set of indicators that researchers and doctors can use to determine which children with SCD are more likely to develop psychological disorders.

Family competence refers to the family’s ability to manage the stressors they face, which includes competent emotion regulation and problem solving.

By collecting data on children with a variety of adaptational outcomes to SCD, researchers may study children and families who are adapting successfully to the condition and apply those lessons to children who are having difficulty adjusting. A variety of models seek to explain why some chronically unwell children have good psychological well-being while others do not.

For example, Wallander and colleagues’ disability-stress-coping model (Wallander & Varni, 1992; Wallander, Varni, Babani, Banis, & Wilcox, 1989) incorporates risk and resistance elements that aid in adaptation.

Resistance elements that influence perceptions and coping in response to stresses associated with chronic diseases include the family environment, social support, adaptation by family members, and utilitarian resources.

Similarly, Thompson and his colleagues’ transactional stress and coping model (Thompson, Gil, Burbach, Keith, & Kinney, 1993) aims to account for differences in children’s adjustment to chronic disease.

A variety of factors influence child adjustment, including mother adjustment, which is affected by assessments 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 view SCD as a stressor or a condition that causes stressors that families must adapt to.

Although different models emphasise family functioning to varying degrees, they all accept the home environment’s ability to influence children’s psychological adjustment. Kliewer, Sandier, and Wolchik (1994) revealed alternative mechanisms by which families influence children’s stress response.