This study sought to ascertain the knowledge of utilization of brain CT in management of sickle cell disease. It also assessed doctors; their knowledge of SCD possible brain complications, and established the need for enlightenment in the indispensable role of CT in management of SCD patients. The survey study involved 222 medical doctors in UNTH. Data was collected using a structured questionnaire which in addition to demographic data collected information on knowledge of the utilization of brain CT in SCD brain complication. The data was analyzed using descriptive statistics of frequency and percentage. The result revealed that there’s 100% knowledge of possibility of brain complication in SCD patient. But, there is limitation in the knowledge of the brain complication that can arise with epidural haematoma, increased intracranial pressure and cerebral aneurism having the least score of 2.3%, 2.3% and 3.6% respectively. Knowledge of the Utilization of brain CT is 40.1% which is below half of the population. There is also poor knowledge of when to refer/ utilize of brain CT SCD patient with shock and headache 2.7% and 11.7% respectively, 68.5% on emergency only, 59.9% can be waived/put aside, 74.8% financial constraint, 7.2% time wastage, ignorance and availability, 18% effect of radiation and about 2.3% have no regard for life at all.
LIST OF TABLES
Table 1 Gender distribution
Table 2 Duration of Clinical Practice
Table 3 Area of specialization
Table 4 Personal Engagement in medical Research
Table5 Possibility of Brain complications
Table 6 Possible Brain Complications of Sickle Cell Patients
Table 7 Brain CT as a Diagnostic Tool for SCD Brain Complications
Table 8 Clinical Picture That Can Warrant Referral for Brain CT
Table 9 Effects of radiation on the brain of SCD using brain CT
Table 10 Management of SCD Patient with short life span
Table 11 Comparison between area of specialization and most appropriate time to refer sickle cell patients for brain CT
Table 12 Comparison between years of practice and can brain CT be Waived for sickle cell patients
LIST OF FIGURES
Figure I: Time for referral
Figure II: Factors hindering referral
Figure III: Negligence of brain CT
TABLE OF CONTENTS
Title
Approval Page
Certification
Acknowledgement
List of Tables
List of Figures
Abstract
Chapter One
Introduction
1.1 Background of Study
1.2 Statement of Problems
1.3 General Objectives
1.4 Specific Objectives
1.5 Significance of Study
1.6 Scope of study
1.7 Literature Review
Chapter Two
Theoretical Background
2.1 The Anatomy and Physiology of the Brain
2.1.1 Major Blood Vessels of the Brain and Complication
2.2 Relationship between Sickle Cell Anaemia (SCA),
Sickle Cell Disease (SCD) and Sickle Cell Trait
2.3 All about Sickle Cell Disease (SCD)
2.4 Computed Tomography (CT)-Head
Chapter Three
Research Methodology
3.1 Research Design
3.2 Population, Sample and Sampling Procedure
3.3 Sample Technique
3.4 Method of Data Collection
3.5 Procedure for Data Collection
3.6 Validity of Data
3.7 Reliability of Data
3.8 Data Analysis
Chapter Four
4.1 Data Presentation and Discussion
4.2 Discussion
Chapter Five
5.1 Summary of Findings
5.2 Recommendations
5.3 Limitation of Study
5.4 Areas of Further Research
References
Appendix
INTRODUCTION
Sickle cell disease involves an altered gene that produces abnormal haemoglobin. Sickle Cell Disease (SCD) and its variants are genetic disorders resulting from the presence of mutated form of hemoglobin, hemoglobin S (HBS) 2. Red blood cells (RBC) with sickled haemoglobin that have too little oxygen becomes C-shape in addition to becoming stiff and sticky. Sickle Cell Disease (SCD) is the most common inherited haemoglobinopathy that has claimed a lot of lives even up till date. Is an extremely important and serious health care issue in Saudi Arabia, some other Middle East countries, certain regions in Africa (including Nigeria), USA and India1? SCD originated in West Africa [mostly in blacks] where it has the highest prevalence. It is also present to a lesser extent in India and the Mediterranean region. From statistics available from the Centers for Disease Control and the National Institute of Health3;
Sickle cell anaemia is the most common inherited blood disorder in United States. More than 70,000 people in the United States have SCD.
SCD occurs in 1 in every 500 African Americans.
About 8% of Africans Americans are carriers of SCD.
Two million people have sickle cell trait
Approximately 1 in 12 African American has the trait
SCD causes significant morbidity and mortality, particularly in people of African and Mediterranean ancestry. Morbidity, frequency of the crises, degree of anaemia and the organ system involved vary considerably from individual to individual4.
For decades, complications of SCD produced the highest mortality rate in the first 3years of life 5. Complications of SCD are due to chronic haemolysis of fragile red blood cells (RBC) or secondary to vascular occlusion by sickle RBC with subsequent tissue infarction. Approximately half of the individuals with (SCD) experience vaso-occlusive crises. The frequency of crises is extremely variable. Infarction is common through the body in patient with SCD, and it’s responsible for the earliest clinical manifestation. In the brain, white matter and gray matter infarcts are seen, causing impairment and functional neurologic deficits8. Approximately more than 50% causes of high morbidity and mortality of SCD patients are as a result of brain complications like acute ischemia, lesion in the brain tissue, cerebral aneurysms.10
Approximately 25% of all patients will have a neurological complication over their life time12 and stroke occurs by the age of 20 in 11% of the patients13.Stroke is a common complication of sickle cell disease and can lead to learning disabilities, lasting brain damage, long-term disabilities, paralysis, or death.14
Because of an increase incidence of adenoid hypertrophy18 .Sidman & Fry19 reported complete remission of frequent vaso-occlusive crisis due to obstructive sleep apnea following tonsillectomy and adenoidectomy. They believe that oxygen desaturation during periods of apnea was the cause of the frequent vaso occlusive crisis. Thus physicians should be aware of possible exacerbation of SCD by sleep apnea syndrome, so definite diagnosis and proper treatment should be employed.
Cerebrovascular complications of SCD are common and are a major source of concern to the hematologist and neurologist. Based on Kotb etal research result, cerebral atrophy is the most common imaging finding followed by small vessel disease and then by cortical infarction. An unusual finding was an epidural haematoma associated with skull bone infarctions and scalp edema that was successfully managed conservatively.20
Traditionally, plain film radiography has been mainstay in the assessment of patients with SCD but computed tomography [CT] is being used and considered as the initial tests of choice for stroke assessment and detection of cerebral pathology in SCD.6,11,14 There are several reports describing brain MRI & CT findings in SCD patients from the western hemisphere15. There are prevalence of infarction, small vessel disease, atrophy, silent stroke and diploic thickening in brain MRI, CT, or both in SCD patients based on the clinical picture like Cerebro-Vascular Accident (CVA), seizures or headaches, eye symptoms, meningitis-like symptoms, shock, fainting, focal numbness etc16.Let’s note that headache which may be severe[migraine] is a frequent symptom of SCD16,17. SCD patient with intracranial haemorrhage/subarachnoid haemorrhage as a result of ruptured aneurysms may present with focal neurological deficits, severe headache, increased intracranial pressure, or coma. Immediate mortality is as high as 50%.This is a frequent cause of sudden unexpected death at home9, of which would have being prevented or managed if patient has gone for brain CT examination. Thus, early detection of cerebral pathology, as well as prevention of overt stroke are important goals in management of SCD11.
After physical and neurological examination are performed the most common next step is a cranial computed tomography (CT-Scan) without contrast which is sensitive for intracranial haemorrhage and will show brain ischemia after 2-3 hours and all kinds of CNS infections.9
Reported in the literature, emphasis that the non-surgical conservative approach in treating combined skull infarction and epidural hematoma should be attempted first to avoid complications of surgical intervention15 and the infarction and epidural hematoma are diagnosed with CT.
Existing guidelines and policy statements for the management of children with sickle cell disease do not provide the specificity required for quality assessment and improvement. Children with SCD continue to be at risk for morbidity and early mortality7. There is need to highlight the need for a study to address the use of imaging(specifically brain CT) in diagnosis and management of cerebro-vascular and other brain complications of SCD patients20.