CHAPTER ONE

INTRODUCTION

1.1 Background of Study

Initial thalassaemia screening was first carried out in 1975 by Silvestroni and colleagues in Latium, Italy, as part of school prevention programme (Sylvestroni E et al., 1978). Screening for sickle cell anaemia began before this, in Virginia in 1970. Nationwide screening programmes also began in Canada, Cyprus, Greece, Italy and the UK during the 1970s, with proven success (Neal Copper et al., 1988). Until this point, the genetic causes of haemoglobinopathies were understood but little had been done to prevent them in newborns (Tosun F et al., 2015)

Premarital Screening (PMS) is defined as a panel of tests in which couples that are going to get married are tested for genetic, infectious and blood transmitted diseases to prevent any risk of transmitting any disease to their children. Nowadays premarital screening is one of the most important strategies for prevention of genetic disorders, congenital anomalies and several medical, psychological marital problems (Al Sulaiman A et al.,2014).

Premarital Counseling (PMC) is also the most appropriate procedure as it is generally acceptable from the religious and ethnical point of view as well as its minimal health and economical requirements (El-Hamzi MA 2019). The majority of counseling concerns haemoglobinopathies (Sickle cell anaemia and thalassaemias). These diseases are a major public health problem in the Mediterranean area, the Middle East, the Indian subcontinent including Bangladesh, South East Asia, tropical Africa and the Caribbean.

According to WHO approximately 250 million people are heterozygous for inherited haemoglobinopathies including thalassaemias and sickle cell disease (WHO 2013). In Kingdom of Saudi Arabia (KSA) and other Muslim countries, the pattern of marriage encourages consanguineous and other forms of relative marriages, leading to an increase in the occurrence of recessive genetic disorders (Mitwally H.H et al., 2013) .

Genetic disorders especially thalassaemias and haemoglobinopathies are highly prevalent in general population of these countries including Bangladesh (WHO, 2019) . Hereditary disease specifically haemoglobin E disease, thalassaemia, double heterozygous haemoglobin E -Beta thalassemia and to a lesser extent haemoglobin D disease or sickle cell disease are present with a high prevalence in South East Asia including Bangladesh and cause greater suffering of the population in these areas (WHO, 2015).

According to a study carried out by Precious Kalamba Gbeneol et al., 2015 in Nigeria on the title “Knowledge, Attitude and Uptake of Premarital Screening for the Sickle Trait Among Married Couples in a Semi-Urban Community in South-South Nigeria” revealed that more than 24% of Nigerians are carriers of the sickle cell gene, while about 2% of all the newborns in Nigeria are born with the sickle cell disease.

The disease is a lifelong cause of severe morbidity that often require prolonged hospital admission; even as the patients have 92% excess mortality. The prevention of the disease is therefore very important, especially in Nigeria whose contribution to the global total has been projected to increase. Premarital screening for the sickle cell gene is considered one of the best ways of preventing the sickle cell disease. A total of 290 questionnaires were administered and retrieved.

The respondents had an average age of 30.55 ±4.1 years; majority (65.86%) had a tertiary education and they were all Christians. All the respondents knew about the sickle cell disease, while 84.83% were aware of the premarital screening for the disease. All had positive attitude towards the premarital screening, with 88.97% recommending that the screening be made compulsory for all intending couples. Most (72.76%) of the respondents carried out the premarital screening when they got married. The age, educational attainment and the religious denomination of the respondents significantly influenced the uptake of the screening (p-value > 0.001).

A total of 9.00% of the respondents were of the same HbAS genotype as their intended spouses; out of which 21.05% went ahead and got married, citing as reasons the difficulty of jettisoning their spouses (25.00%); and the power of God to prevent a child with sickle cell disease (75.00%). The knowledge, attitude and uptake of premarital screening for sickle cell disease are high in the study, even as the screening programme is not driven by the government. The input of the government is however required in providing health education, genetic counselling and prenatal diagnosis.

1.2 Statement of the Problem

According to WHO approximately 250 million people are heterozygous for inherited haemoglobinopathies including thalassaemias and sickle cell disease (WHO 2013). In the Kingdom of Saudi Arabia (KSA) and other Muslim countries, the pattern of marriage encourages consanguineous that is descending from the same ancestors and other forms of relative marriages, leading to an increase in the occurrence of recessive genetic disorders (Mitwally H.H et al., 2013).

Hemoglobinopathies, genetic disorders of hemoglobin, are the most common inherited disease in humans (Chatterjee T et al., 2015). So many youths today rush into marriage without knowing if they are compactible medically or not which later cause many issues in their marriage since some are unable to bear children, others give birth to children with illnesses like sickle cell anemia, hemophilia which could have been prevented.

All these with personal observation from a couple who have been married for more than six years now with no child but series of miscarriages which is as a result of blood group incompatibility actually prompted the researcher to  carry out this research.

1.3 Objectives

1.3.1 General Objectives

To assess youths (18-35years) knowledge and the factors affecting their willingness to undertake premarital screening in the Muea community Buea.

1.3.2 Specific Objectives

1. To assess the level of knowledge youths (18-35years) have on premarital screening in the Muea community Buea.
2. To identify the factors hindering youths (18-35years) willingness to undertake premarital screening.
3. To ascertain the consequences of not doing premarital screening among youths of the Muea community

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