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BACKGROUND OF THE STUDY
Lonergan, Cline, Abbondazo (2001) defined sickle cell anaemia as a chronic, genetic and hemolytic disease peculiar to the negro race due to homozygous inheritance of an abnormal haemoglobin and resulting in a variation in the structure of the globins. According to Claster and Vichinsky (2003), “Sickle Cell disease is an inherited blood disorder that affect the red blood cells”. People with sickle cell disease have red blood cells that contain mostly haemoglobin ‘S’, which is an abnormal type of haemoglobin. Sometimes, these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels.
Umeh (1996) explained further that when, sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged because; the function of the normal red blood cell is to transport oxygen with the help o f haemoglobin. Claster (2004) disclosed that sickle cell trait (AS) is an inherited
condition in which both haemoglobin A and S are produced in the red blood cells, always more of A than S sickle cell trait is not a type of sickle cell disease, people with sickle cell trait are generally healthy.
As stated in Sarojini (1998), the sickle haemoglobin is inherited according to Mendelian laws as autosomal recessive character. The homozygous individual for sickle cell haemoglobin are designated as “SS” while the heterozygous individuals are designated as “AS” and are said to have the sickle cell trait or are carriers. Homozygous individual for normal haemoglobin are designated as “AA”.
According to Mendelian laws as cited in vermar (2004), if carriers (AS), marries each other they will probably produce a sickler (SS) in line with the Mendelian ratio 1(SS): 2(AS): 1(AA). In Umeh (2004), “for a person to become a sickler, he must inherit the gene for the sickle cell in a double recessive condition”. A person who inherits the recessive gene from both parents suffers from sickle cell anaemia. In this disease condition, the red blood cells contains less haemoglobin and therefore carry less volume of oxygen to the living cells and hence the loss of energy in the sufferers. The distorted shape of the red blood cells hiders free flow of blood in the vessels resulting in severe pain experienced by sufferers. Mak and Davies (2003), explained that the sickled cells are rapidly destroyed, the patient becomes anaemic, the bone marrow becomes over active in an attempt to build new red blood cells. This causes severe pains on the bones, severe anaemia causes the weakness of the heart; this could lead to heart failure. Umeh (1996), decleared that generally, there is a poor, physical development and the individual is usually very weak, life is miserable and where there is no proper medical attention the patient dies in his youthful age. According to styles and Wright (2005), the sickle cells also block the flow of blood through vessels resulting in lungs tissue damage (acute chest syndrome), pain episodes (arms, legs, chest and abdomen). It also causes damage to most organs including the spleen kidneys and liver. Damage to the spleen makes sickle cell disease patients especially young children easily overwhelmed by certain bacterial infections.
So far there seems to be no known cure for sickle cell anaemia this disease can only be prevented by giving the individual who wish to marry, genetic counselling. Blood tests are usually carried out to determine the genotype of couples wishing to marry. Such tests are usually done in hospitals. A carrier of the gene “SS” can protect his children by marrying an “AA” individual. In this case, their children are either AA or AS all of whom are healthy. Umeh (2004).
In search for a substance that can prevent red blood cells from sickling without causing harm to other parts of the body, Hydroxyurea was found to reduce the frequency of severe pain, acute chest syndrome and the need for blood transfusion in adult patient with sickle cell disease. Droxia, the prescription form of hydroxyurea was approval by the FDA in 1998 and is now available for adult patient with sickle cell anaemia. Studies will now be conducted to determine the proper dosage for children. Taylor, Carter. Poulose (2004).
STATEMENT OF PROBLEM
In some secondary schools, students believe that sickle cell anaemia is a curse, which some children inherited from their parents. Some explained that this disease could be caused due to the existence of witches and wizards (Ogbanje) in the societies. Some students also said/explained further, that the sins of the forefathers are the causes of sickle cells anaemia, that any child who suffers from such disease has to be avoided so as to infect them with the disease. This study has the problem of ascertaining the perception of the secondary school students of sickle cell anaemia and awareness of the implication of sickle cell anaemia on married couples.
PURPOSE OF STUDY
The general aim of this study is to establish the level of perception of sickle cell anaemia and the effect on couple among the secondary school in Enugu south Local Government Area of Enugu State. Specifically, the study will;
a) Access how far the students know of the existence of sickle cell anaemia.
b) Access, how much the students know on the disease as regards, what the disorders are, how it is acquired and how it is prevented or cured.
c) Determine how far the students know their genotype and whether it would affect marital relationship and birth of children with the diseases.
d) Access the awareness of the students about the implications of sickle cell anaemia on married couples.
SIGNIFICANCE OF THE STUDY
There are benefits obtainable from this study if the result of the study is properly utilized because this research work will educate the societies on the checking of genotype among individual to prevent sickle cell anaemia in the society. The study will also help in the reduction of the number of divorces by creating awareness of checking of genotype before marriage. This will also help in reduction of pre-mature death of children as a result of sickle cell disease and also help in the choice of life partners.
These research work will educate the secondary school students on the disease, by creating awareness on how the disease called sickle cell anaemia came about, how it can prevent and exposing them on checking their genotype and also this will make them to know if they are carrier or not.
These stretches out to investigate on all the secondary schools in Enugu South Local Government Area of Enugu State. A total number of 5 (five) schools will be investigated on their perception of the effects of sickle cell anaemia on couples.
The following guided the study
v To what extent are the students aware of the existence of sickle cell anaemia?
v To what extent do student know how sickle cell is acquired?
v To what extent has the student know about the management of the disorder?
v To what extent are the students aware of the implication of sickle cell anaemia to married couples?