CHAPTER ONE
INTRODUCTION
1.1 Background
Sickle cell disease is an autosomal recessive genetic red blood cell disorder characterized
by sickling, a condition in which RBCs become crescent or sickle shape as against the
normal disc-shaped that is flexible enough to move easily through the blood vessels
(Johannes et al ., 2010). The sickle shape occurs due to a mutation in the Hb gene, and
this decreases the cells’ flexibility. When the abnormal haemoglobin S appears in
homozygous form (Hb SS), it is referred to as sickle cell anaemia, and it can also affect
individuals in combination with other abnormal haemoglobins such as sickle cell
haemoglobin C disease (Hb SC) and sickle cell β-thalassaemia (Hb Sβ-Thal) (Okpala et
al.., 2004).Due to its severe adverse effect on patients mortality, sickle cell disease (SCD)
has been identified as a set of diseases of public health concern. The high mortality rate
among patients with SCD in Africa has been attributed to several factors including suboptimal management and care. African has been associated with the highest prevalence of
the sickle cell trait, with figures suggesting that between 10% and 40% of the entire
population may be affected (Ana A .Baumann et al., 2018).Citation10 According to
WHO, the incidence of sickle cell trait ranges from 20% to 30% in Cameroon (WHO,
2006), the Democratic Republic of the Congo, Gabon, Ghana, and Nigeria. A high
prevalence of 45% has been reported in some parts of Uganda.Citation11 Availableevidence suggests that about 90% of the world’s sickle cell disease population lives in
Nigeria, India, and the Democratic Republic of the Congo, where the disease affects up to
2% of the population (Khanolkar et al…, 2018).Citation12 Evidence suggests Nigeria
has the largest population of persons affected with sickle cell disease
globally.Citation13,Citation14 (Caroline, 2014). In spite of this, there is only few
specialized centre for SCD in the DOUALA. Prenuptial testing is available but few come
forward for testing since health promotion to increase its awareness is still lacking. Also,
care for people with SCD is suboptimal as majority of sufferers lack financial resources
(Nini Yesih Ngwengi et al …, 2020). Although this aspect of control of the disease has
not been given enough emphasis, prevention through public education, genetic counselingand screening is a better economic strategy especially in resource limited settings (Obi
Peter Adigwe et al …, 2022) . These preventive methods are reported to be effective in
the control of thalassemia in some few parts of the world. Nevertheless, appropriate
knowledge regarding SCD is required for individuals, especially carriers to make
informed decisions about their life style. Consequently studies are necessary to elucidate
the epidemiology of SCD and the knowledge, attitude and practices towards SCD in
Cameroon (Maymard, 2014). To enable them to make positive decisions in the
management of their crisis as such the government of Cameroon has created several
centers to educate sickle cell patients on the effect of their condition and how to manage it
in other to modify their life style to suit their condition (Bernard Nabe et al …, 2024).
Thus this study is aim at investigated the Patients’ knowledge, and compliance in the
management of SCD among Sickle cell patients at the SCU in Laquintinie District
Hospital Douala Cameroon.
1.2 Problem statement
SCD is one of the most common monogenetic diseases worldwide (BMC Health, 2018).
The prevalence of patient suffering from SCD is between 20% to 30% in Cameroon
(WHO, 2006).the management of SCD in the comm.+unity continues to present
significant challenges to patients due to lack of partnerships between SCD clinicians and
researchers in high and low income countries in order to further research and improve
clinical care globally (Isaac Odame et al…, 2010). Cameroon being one of the WHO
regions and developing country is not left out, the researcher during his clinicalexperience at laquintinie district hospital had inadequate knowledge and negative
attitudes towards its management. For this reason, it pushes and motivated the researcher
to carry out research on the Patients’ knowledge regarding the management of SCD at the
SCU in Laquintinie Hospital Douala.
1.3 Purpose
To increase Patients’ knowledge and compliance towards the management of sickle cell
disease in the sickle cell unit at the Laquintinie Hospital Douala.1.5 Research question
1. What is the Patients’ knowledge regarding the management of sickle cell disease in
the sickle cell unit at the Laquintinie Hospital Douala?
2. What are the compliances of patients toward the management of sickle cell disease in
the sickle cell unit at the Laquintinie Hospital Douala?
3. What are the barriers faced by patients at the center for the management of sickle cell
disease at the Laquintinie Hospital Douala?
1.6 Research hypothesis
A statement of expectation or prediction which is tested by research. It is divided into two
sections; null and alternative hypothesis.
1.6.1 Null hypothesis (HO).
Patients at Laquintinie Hospital Douala have inadequate knowledge on the management of
sickle cell disease.
1.6.2 Alternative hypothesis (H1).
Patients at Laquintinie Hospital Douala have good knowledge on the management of sickle
cell disease.