CHAPTER ONE
INTRODUCTION
1.0        Introduction
The problem of sickle cell anaemia is in different faces. In this work we are looking at the social aspect of the sickle cell anaemia or the psychological assault of people living with sickle cell anaemia. People does not see them as normal, majority of the people see people with sickle cell anaemia  as a  working corpse that can die at any time.
1.1       Background of study
The condition of having sickle cell disease will be defined as occurring in one or the other of the two following states: (1) sickle cell anemia-a severe, uncorrectable, and often fatal anemia with many clinical manifestations, and (2) sickle cell trait--a relatively benign condition with symptomatology occurring only under extraordinary circumstances.
Hemoglobin functions as the oxygen carrying substance of the blood, and is responsible for the pigmentation of the erythrocytes or red blood cells. The condition of sickle cell anemia results from the presence of an abnormal hemoglobin, known as Hb-S, in the erythrocytes. The presence of this hemoglobin is hypothesized to have been an asset in protecting· its bearer from the effects of malaria in those countries where the occurrence of malaria is common. In 1964, A. C. Allison (Levitan and Montagu 1971), proposed, that the· heterpgygote AS was less susceptible· than the normal homozygotes to Falciparum malaria, an often fatal· malaria constantly reinfecting its victims with severe and prolonged disease.
However, for that segment of the population carry in the abnormal hemoglobins, but residing in no malaria environments, the advantage of hemoglobin S disappears.
In the United States, sickle cell anemia is found predominantly Wintrobe (1967), the "frequency of occurrence of sickle cell trait is 8.5 per cent of the black population. Approximately one out of four hundred blacks (National Sickle Cell Prevention Act, 1971) experiences the anemia itself. The incidence of the disease in other races is quite rare, and when it does occur in members of other races, it is usually experienced by persons of Greek, Italian, East Indian, South American and Middle Eastern origins.
Clinical Manifestations
Sickle cell anemia has been referred to as "the great mimicry" due to the 'multiphasic manifestations and complexities of its symptoms. Consequently, the risk of incorrect early diagnosis is high. (Lin-Fu 1965)
In infants, symptomatology, particularly in the first few months of life, is bizarre and non-specific'. Major complaints 'usually include colic, failu~e to thrive, jaundice, nausea and vomiting, recurrent fever, and swelling in hands and feet.
In older children, periodic occurrences of acute episodes with various manifestations, facilitate recognition of sickle cell anemia. These episodes, known 8S "crises"
(Lin-Fu, ibid.), are brought about when the erythrocytes or red blood cells begin to sickle. Sickling is the term used to describe the change that occurs within the cells under conditions of reduced oxygenation. The cells elongate and twist into distorted shapes that prohibit free flowing movement of the blood. It is proposed that "logjams" of these rigid cells form, resulting in impeded circulation in the smaller vessels, thus inhibiting the flow of oxygen to the affected part of the body. This blood flow obstruction causes tissue death, which produces acute, incapacitating pain in any part of the body, fever, and many other non-specific symptoms. This symptom complex comprises the sickle cell crisis."
Other manifestations include necrosis, which might occur resulting in chronic leg ulcers; however, this is less common among those children afflicted wit~ sickle cell anemia than for adults. Incipient blindness from retinal detachment might also result from sickling and reduced. Oxygenation in the cells of the eyes. The result is an overall retarded growth and development, and increased susceptibility to infections and pneumonia are further manifestations of the anemia. The childhood victim of this illness is usually characterized by a. barrel-shaped chest, enlarged protruding abdomen; and thin extremities.