CHAPTER ONE

Background to the Study

Sickle cell disease (SCD) is one of the most common genetic disorders in the world, affecting millions of people globally. It is caused by a change in the gene responsible for producing hemoglobin, the protein that helps red blood cells carry oxygen throughout the body. In people with SCD, red blood cells lose their round shape and become sickle-like, which causes them to stick together, block blood flow, and break down quickly. This leads to problems such as severe pain, anemia, organ damage, and even death. Every year, about 300,000 children are born with SCD worldwide, with most of these cases occurring in sub-Saharan Africa, where the condition is a significant public health concern (World Health Organization [WHO], 2020).

Globally, SCD remains a challenge despite advances in medical care. In developed countries, early diagnosis through newborn screening and access to effective treatments, like blood transfusions and medications, have significantly improved the lives of people with SCD. However, in low- and middle-income countries, where healthcare systems are weaker, most children born with SCD do not survive past their fifth birthday. This high mortality rate is largely due to a lack of awareness, limited access to healthcare, and the high cost of treatments (Piel et al., 2017). The global fight against SCD has also been hampered by insufficient public health focus compared to other diseases, leading to gaps in funding, research, and policy interventions.

In Africa, SCD has a much higher prevalence than in other parts of the world. The condition is particularly common in regions where malaria has historically been widespread. This is because people with the sickle cell trait—who inherit one abnormal hemoglobin gene—have some protection against severe malaria. However, when two individuals with the sickle cell trait have children, there is a 25% chance that the child will inherit two abnormal genes, resulting in SCD. According to the WHO (2020), Africa accounts for more than 75% of global SCD cases, with over 200,000 babies born with the disease annually. Most of these children lack access to the medical care they need, such as regular health check-ups, vaccines, and basic medications like antibiotics to prevent infections. As a result, complications such as stroke, severe infections, and acute pain episodes are common, often leading to early death (Makani et al., 2018).

In Cameroon, SCD is a significant public health issue, affecting thousands of children and their families. Studies estimate that about 20% of the population carries the sickle cell trait, and 1 in 50 children is born with SCD each year (Nguefack et al., 2021). The condition is one of the leading causes of childhood illness and death in the country. Many parents and caregivers have little or no knowledge about SCD, its causes, or how to manage it. This lack of awareness is often compounded by cultural beliefs and stigma, which prevent families from seeking medical help. For example, some communities view the symptoms of SCD, such as recurrent pain and swelling, as signs of curses or supernatural forces. Such misconceptions discourage affected individuals from accessing proper care and treatment.

Healthcare facilities in Cameroon face significant challenges in managing SCD. While hospitals like Mount Mary Hospital in Buea provide some level of care, the resources available are often insufficient to meet the needs of patients. There is a shortage of trained healthcare professionals who specialize in SCD, and many hospitals lack the necessary equipment for accurate diagnosis and treatment. For children aged 5 to 18 years, managing SCD requires regular medical check-ups, medications to reduce pain and prevent complications, and psychosocial support to help them cope with the condition. However, these services are often inaccessible to families living in rural areas or those who cannot afford the high cost of treatment (Mbanya & Mbanya, 2018).

Efforts to improve the management of SCD in Cameroon have gained momentum in recent years. The government has partnered with non-governmental organizations to raise awareness about the condition through community education programs. These programs aim to inform the public about the causes of SCD, the importance of genetic counseling, and the available treatment options. Schools and churches have also been involved in spreading awareness and supporting affected families. Additionally, some healthcare facilities are working to provide more affordable treatment options, including the use of hydroxyurea, a drug that has been shown to reduce pain episodes and improve the quality of life for people with SCD (Nguefack et al., 2021).

Despite these efforts, much work remains to be done to address the challenges posed by SCD in Cameroon. For instance, there is a need for more comprehensive policies that prioritize SCD in the national healthcare agenda. This includes integrating SCD screening into routine health services, training more healthcare workers on SCD management, and increasing funding for research and treatment. It is also important to address the social and cultural barriers that prevent families from seeking care. By educating communities about the scientific causes of SCD and promoting early diagnosis and treatment, significant progress can be made in reducing the disease’s impact.

Mount Mary Hospital in Buea plays a critical role in addressing SCD in the region. The hospital provides services such as diagnosis, counseling, and treatment for children and their families. It also works to raise awareness about SCD within the community, encouraging early detection and proper management of the disease. For children aged 5 to 18 years, the hospital focuses on providing holistic care, which includes not only medical treatment but also emotional and psychological support. This approach helps children manage the physical and emotional challenges of living with SCD, improving their overall quality of life.

In summary, SCD is a serious health issue that affects millions of people worldwide, with a particularly heavy burden in Africa and Cameroon. While progress has been made in understanding and managing the disease, significant gaps remain in awareness, access to care, and resources. By focusing on children aged 5 to 18 years, institutions like Mount Mary Hospital in Buea can help reduce the suffering caused by SCD and improve the lives of affected families.

STATEMENT OF THE PROBLEM

Sickle cell disease (SCD) is a serious health problem that affects many children around the world, especially in Africa. Children living with SCD often experience severe pain, anemia, infections, and other complications that affect their growth, education, and quality of life. In many cases, families do not have enough knowledge about SCD, its causes, and how to properly manage it. This lack of understanding can lead to delays in diagnosis and treatment, making the condition worse for affected children (Makani et al., 2018).

In Cameroon, SCD remains a major challenge for children and their families. Many parents are unaware that SCD is caused by the inheritance of abnormal hemoglobin genes from both parents. Because of this, they often do not seek genetic counseling before having children. Additionally, myths and cultural beliefs about SCD, such as seeing it as a curse, create stigma and prevent families from seeking medical care (Mbanya & Mbanya, 2018). These challenges are even more pronounced in rural areas, where access to healthcare services and trained medical staff is very limited.

Hospitals like Mount Mary Hospital in Buea try to help children with SCD by offering medical care and education, but they face many difficulties. These include a lack of resources, like medications and equipment, and the high costs of treatment. For children aged 5 to 18 years, who are in school and trying to live normal lives, managing SCD can be especially difficult. Without regular care, these children are at risk of severe health complications, poor academic performance, and even early death (Nguefack et al., 2021).

The problem is made worse by the limited awareness campaigns in communities. Families and schools are not equipped with the right information to support children with SCD. This gap in knowledge and support creates unnecessary suffering for both the children and their families. There is an urgent need to educate the community, improve access to healthcare, and address the myths and stigma surrounding SCD. Without these steps, many children with SCD in Cameroon will continue to face unnecessary pain, poor health, and limited opportunities for a better life (WHO, 2020).

Research Questions

Main Research Question

What is the level of knowledge about sickle cell disease, its causes, and management among children aged 5 to 18 years at Mount Mary Hospital, Buea?

Specific Research Questions:

What do children aged 5 to 18 years and their caregivers know about the causes of sickle cell disease?

How do children with sickle cell disease and their caregivers manage the condition at home and in school?

What challenges do children aged 5 to 18 years face in managing sickle cell disease in the Mount Mary Hospital, Buea?

What role does the hospital play in educating children and their families about the management of sickle cell disease?

Research Objectives

Main Objective:

To assess the knowledge, causes, and management of sickle cell disease among children aged 5 to 18 years at Mount Mary Hospital, Buea.

Specific Objectives:

To evaluate the knowledge of children and their caregivers about the causes of sickle cell disease.

To identify the strategies used by children and their caregivers in managing sickle cell disease at home and in school.

To examine the challenges faced by children aged 5 to 18 years in the management of sickle cell disease.

To explore the role of Mount Mary Hospital in providing education and support for managing sickle cell disease.