SCHOOL OF PUBLIC HEALTH COLLEGE OF HEALTH SCIENCES UNIVERSITY OF GHANA, LEGON EXPERIENCES OF SICKLE CELL DISEASE (SCD) AMONG ADOLESCENTS ACCESSING HEALTH CARE AT THE 37 MILITARY HOSPITAL BY DORIS DZIGBORDI AVORTRI (10239304) THIS DISSERTATION IS SUBMITTED TO UNIVERSITY OF GHANA, LEGON IN PARTIAL FULFILMENT OF THE REQUIREMENT FOR THE AWARD OF MASTER OF PUBLIC HEALTH DEGREE DECEMBER, 2022 University of Ghana http://ugspace.ug.edu.gh ii DECLARATION I hereby declare that this thesis is my own work towards the Master of Public Health and that to the best of my knowledge, it contains no material previously published by another person nor material which has been accepted for the award of any other degree in the University, except where due acknowledgement has been made in the text. …………………………… …………………… Doris Dzigbordi Avortri Date Student ……………………………. …………………… Dr. Leonard Baatiema Date (Supervisor) 29-11-2022 29-11-2022 University of Ghana http://ugspace.ug.edu.gh iii DEDICATION This work is dedicated to the almighty God for seeing me through the changing circumstances of life and the abundance of his Grace upon me thus far. To my family, my husband Mr. Richard Agbanyo, my children Seyram, Selorm and Aseye for their support and encouragement emotionally, physically and spiritually. Also, my late father and mother Mr. and Mrs. Avortri for their initial motivation towards my education. University of Ghana http://ugspace.ug.edu.gh iv ACKNOWLEDGMENT I wish to express my sincere gratitude to my supervisor, Dr Leonard Baatiema for his patience, guidance and useful suggestions which helped bring this work to a success. I am also thankful for Dr Dickson, Agbanyo Seyram and Eric Mawunyo for their time and patience spent in going through and typing the manuscript. I am also thankful to all the staff of pediatrics Out patient department of 37; especially Dr Parbie Emmanuel, Head of Department and staff nurse Hagar for their immense help in the collection of data. In addition, I acknowledge both the parents and children for their cooperation in the collection of data. Finally, but not least, I must acknowledge my indebtedness to Dr Richard Akuffo of Noguchi Memorial Institute for Medical Research for his help in diverse ways to bring this work to fruition. I must acknowledge lastly that whatever merit there is in this study derives from the invaluable contributions of all here mentioned. But I remain personally liable for all imperfections. University of Ghana http://ugspace.ug.edu.gh v Table of Contents DECLARATION ................................................................................................................................. ii DEDICATION ....................................................................................................................................iii ACKNOWLEDGMENT .................................................................................................................... iv LIST OF FIGURES ..........................................................................................................................viii LIST OF TABLES ............................................................................................................................viii LIST OF ABBREVIATIONS ............................................................................................................. x ABSTRACT......................................................................................................................................... xi CHAPTER ONE .................................................................................................................................. 1 1.0 INTRODUCTION ......................................................................................................................... 1 1.1 Background to the Study ...................................................................................................................... 1 1.2 Problem Statement ................................................................................................................................ 5 1.3 Justification of the study ....................................................................................................................... 7 1.5 Main Objective ...................................................................................................................................... 8 1.6 Research Questions ............................................................................................................................... 9 CHAPTER TWO ............................................................................................................................... 10 LITERATURE REVIEW AND CONCEPTUAL FRAMEWORK .............................................. 10 2.1 Introduction ......................................................................................................................................... 10 2.2 Burden of Sickle Cell Disease ............................................................................................................. 10 2.3 Lived Experiences of Adolescents with Sickle Cell Disease ............................................................. 16 2.4 Coping Strategies among Adolescents with Sickle Cell Disease ...................................................... 19 2.5 Existing Support Systems ................................................................................................................... 28 2.6 School Support System ....................................................................................................................... 30 2.7 Health Service Support System .......................................................................................................... 32 2.8 Community Support System .............................................................................................................. 34 2.9 Summary of the review ....................................................................................................................... 34 CHAPTER THREE ........................................................................................................................... 39 METHODS ......................................................................................................................................... 39 3.1 Introduction ......................................................................................................................................... 39 3.2 Study Design ........................................................................................................................................ 39 3.3 Setting................................................................................................................................................... 40 3.4 Study Population ................................................................................................................................. 42 3.5 Inclusion Criteria ................................................................................................................................ 42 3.6 Exclusion Criteria ............................................................................................................................... 42 3.7 Sample Size ......................................................................................................................................... 42 University of Ghana http://ugspace.ug.edu.gh vi 3.8 Quality Assurance ............................................................................................................................... 43 3.9Selection of Study Participants ........................................................................................................... 43 3.10 Instrumentation ................................................................................................................................. 44 3.11 Data Collection .................................................................................................................................. 44 3.12 Management of data ......................................................................................................................... 44 3.13 Data Analyses .................................................................................................................................... 45 3.14 Methodological Rigor ....................................................................................................................... 46 3.15 Ethical Consideration ....................................................................................................................... 47 CHAPTER FOUR ............................................................................................................................. 49 RESULTS ........................................................................................................................................... 49 4.1 Introduction ......................................................................................................................................... 49 4.2 Demographic Characteristics of Study Participants ........................................................................ 49 4.3 Summary of Study Findings ............................................................................................................... 50 4.4 Experiences of Living with Sickle Cell Disease ................................................................................ 51 4.5 Challenges Associated with Access to Health Care as an Adolescent Sickle Cell Patient. ........... 53 4.6 Social Support Systems for Adolescent Living with Sickle Cell Diseases ...................................... 56 4.7 Coping Strategies Adopted by Adolescents Living with Sickle Cell Disease ................................. 58 4.8 Measures and Strategies to Improve Access to Care and Quality of Life ...................................... 61 CHAPTER FIVE ............................................................................................................................... 65 DISCUSSION ..................................................................................................................................... 65 5.1 Introduction ......................................................................................................................................... 65 5.2 Overview of Key Findings .................................................................................................................. 65 5.3 Findings compared to previous literature ......................................................................................... 66 5.4 Experiences in Accessing Health Care .............................................................................................. 68 5.5 Existing Support Systems ................................................................................................................... 71 5.6 Suggestion by Adolescents Living with Sickle Cell Disease to Improve Health Care Delivery .... 73 CHAPTER SIX .................................................................................................................................. 76 SUMMARY, LIMITATION, CONCLUSION AND RECOMMENDATION ............................ 76 6.1 Introduction ......................................................................................................................................... 76 6.2 Summary of the study ......................................................................................................................... 76 6.3 Limitations ........................................................................................................................................... 77 6.4 Conclusion ........................................................................................................................................... 78 6.5 Implication and Recommendations ................................................................................................... 79 6.6 Recommendations. .............................................................................................................................. 81 REFERENCES .................................................................................................................................. 83 University of Ghana http://ugspace.ug.edu.gh vii APPENDIX I: PARTICIPANTS INFORMATION ....................................................................... 88 APPENDIX II: CONSENT PROCESS ........................................................................................... 90 APPENDIX III: ETHICAL REVIEW ............................................................................................. 95 University of Ghana http://ugspace.ug.edu.gh viii LIST OF FIGURES Figure 1: Experiences of Adolescents with Sickle Cell Disease Accessing Health Care at 37 Military Hospital ................................................................................................................................................ 37 LIST OF TABLES Table 1: Demographic characteristics of study participants ......................................................... 49 University of Ghana http://ugspace.ug.edu.gh x LIST OF ABBREVIATIONS CBT Cognitive-behavioral therapy CIA Central Intelligence Agency Hb Hb Hemoglobin HRQOL Health-related quality of life NCD Non communicable disease NICU Neonatal intensive care unit SCD Sickle cell disease SCT Sickle cell trait VOC Vaso-occlusive crisis University of Ghana http://ugspace.ug.edu.gh xi ABSTRACT Sickle cell disease (SCD) is the most common inherited blood disorder throughout much of Sub- Saharan Africa affecting up to 3% of births in some parts of the continent. With the increasing occurrence of SCD in the adolescents visiting 37 Military Hospital, a basic understanding of their livelihood is of importance to medical practitioners for improved and better care delivery. This study seeks to explore the lived experiences of adolescents living with SCD as well as the challenges they face accessing healthcare at the 37 Military Hospital. It further analyses the coping strategies adopted by the SCD adolescents and existing support systems. Qualitative research methodology was employed in the study using interviews to collect data from SCD adolescents between the ages of 10 and 19 years. 25 respondents were interviewed, The study reveals adolescents experience symptoms of depression, dejection and feel stigmatized by friends. Access to health care is another challenge they experience due to inadequate specialized clinics, physicians, health insurance coverage and high cost of medical and laboratory expenses. Adolescents adopting different coping strategies including praying, physical activity, drinking of sufficient water, taking of pain killers and self-management. The study concludes that there is the need for the government to establish a free health care system for SCD patients across the country for easy access to healthcare and intensify education as well. Psychosocial support services and community-based support are also needed to improve the lived experiences of adolescent living with SCD. University of Ghana http://ugspace.ug.edu.gh 1 CHAPTER ONE 1.0 INTRODUCTION 1.1 Background to the Study Sickle cell disease (SCD) is an inherited disorder of hemoglobin caused by a mutation in the beta globin subunit of adults. This red blood cell disorder is a lifelong condition that can affect many major organs in the body. The red blood cell of sickle cell patients’ lives for a couple of weeks instead of three months as do normal red blood cells. There is therefore anemia and rapid turnover of cells. In cases of stress and hypoxia, the cells assume a sickle shape and they lock each other in the tiny blood vessels causing a blockage which then leads to the following symptoms; bone, joint, chest and abdominal pain of biting nature, Jaundice, anemia, head and foot syndrome, bone, distended abdomen, stroke, repeated infections, chronic ulcers amongst others (Porter & Kaplan, 2011). SCD affects million people all over the world, especially those of African origin, also in the Caribbean, South America, United Kingdom, France, Italy, Arab countries etc. (Zajac, 2021). Wastnedge et al (2018) reported that global meta – estimate for the prevalence of homozygous sickle cell disease is 111.91 per 100,000 births (95% CI = 100.77 – 123). Approximately 5% of the world population are healthy carriers of a gene of sickle cell disease or thalassemia. The percentage of people who are carriers of the gene is 25% in some countries (Wastnedge et al, 2018). According to World Health Organization (WHO), sickle cell disease is the most prevalent inherited disease in the African region and in many countries 10-40% of the population carry the sickle cell gene resulting in estimated sickle cell prevalence, approximately 80% of all children University of Ghana http://ugspace.ug.edu.gh 2 born with SCD are in Sub-Saharan Africa (about 200,00 babies born per year with SCD) (WHO, 2010). The mortality rate of children with SCD had been high among children under five to 23 years (about 9.3%) until the 21st century when it dropped to 2.6% in African American children. The reduction is believed to be due to the adoption of pneumococcal vaccine that was started in 2000. Although, advances in medicine and technology have caused decline in mortality rate and children with the disease, the condition is still linked to a shorter lifespan in life expectancy. These days 90% of children with sickle cell disease in developed countries survive into adulthood due to comprehensive management including new born screening, health promotion and prevention of disease complications. However, sickle cell disease in sub-Saharan Africa contribute to 5-16% of all deaths to children under 5. Most of them are undiagnosed and untreated (Gill, 2019). Misconception about SCD, the negative thought and ideas have led to stigmatization of people living with SCD. Electronic searches of medical and psychological database revealed some psychological complications in both children and adults with sickle cell disease. These include inappropriate pain coping strategies, quality of life due to restriction in daily functioning, anxiety, depression and neurocognitive impairment. School absenteeism leads to deterioration in school performance (Anie & Haematol, 2005). A study on Care giver Perception of Sickle Cell Disease Stigmatization in Ghana identified Sickle cell disease stigma is a major community health issue. The study, explored stigma and related factors for caregivers of pediatric patients with SCD in Kumasi, Ghana. Overall, participants were knowledgeable about SCD. The study revealed three themes including; blame for SCD; Caregivers were blamed as being under a curse for their evil deeds, public misconception about SCD; some people in the community considered the disease as devilish acquired as punishment of University of Ghana http://ugspace.ug.edu.gh 3 God from bad ancestors handing over from generation to generation and the shame for the financial burden of SCD. Based on these findings, it’s important for steps to be taken to explore the reduction of stigmatization in other sub-Saharan countries (Buser et al, 2021). The disease is also associated with huge economic burden worldwide. Routine and lifelong comprehensive care is needed to treat serious complications. A total of 20 studies in 10 countries between January 2014 to June 2019 on economic burden and healthcare resource utilization reveal a total direct annual cost ranged from USD 1 million to USD 3 million and the average indirect cost was found to be USD 1,293 million in the related care is negligible (Naik, Bagga & Senecai, 2019). In Congo only 60% of essential medicines for sickle cell patients for example, folic acid, multivites etc. are available in the public pharmacies. The cost of other medicines and laboratory investigations are borne by relatives (Ngolet, 2016). Management currently, includes health maintenance measures. For example, folate supplementation, hydroxyurea, broad spectrum antibiotics (for infections), analgesics and IV hydration (for Vaso occlusive pain episodes), blood transfusions and immunizations (Porter & Kaplan, 2011). Although there is no universal cure for SCD, some treatment modifications are practiced in some countries. These include; hydroxyurea which increases fetal hemoglobin level thus reducing the rate of crises’ Chronic blood transfusion, Haemopoietic stem cell transplantation, Gene therapy and L-glutamine (Porter & Kaplan). Ghana is the first to use Hydroxyurea in sub- Saharan Africa. Expensive and technologically advanced methods of care for SCD patients in UK, USA and parts of Europe are generally not affordable in Ghana and other sub-Saharan African countries. The World Health Organization, Africa in 2010 designed some intervention strategies to prevent complications of SCD and reduce frequent hospitalization (Center for disease control and University of Ghana http://ugspace.ug.edu.gh 4 prevention). This strategy provides a set of public health interventions to reduce the burden of SCD in the African region through improved awareness, disease prevention and early detection. The interventions include improvement in healthcare provisions effective clinical, laboratory, diagnostic and imaging facilities adapted to different levels of the health system, screening of newborn, training of health workers and development of protocols, genetic counselling and testing, accessibility to healthcare; establishment of patient support groups; advocacy and research. Sickle cell disease is also a significant public health burden in Ghana. According to sickle cell foundation Ghana’s annual report, about 2% (15,000) of Ghanaian new-born are affected by SCD annually. About 320 children with SCD access healthcare at 37 Sickle cell clinic every month. 395 admissions were recorded in 2019 and 192 in 2020 (37 Data and Information System, 2021). The reduction might be due to intervention by hydroxyurea although the number is still high. A study conducted at Ghana institute of Clinical Genetics Korle Bu, Accra and published in 2018 revealed that Korle Bu Teaching Hospital which is the largest teaching hospital in Ghana saw 5,451 patients with 20,788 clinic visits of children with SCD between January 2013 to December 2014 (Matutes, 2018). There are a number of traditional beliefs surrounding the origin of SCD. Ghanaians consider it as a form of rheumatism, and the names ascribed to it tend to express or describe the relentless pain suffered by those who have it Ahotutuo (Twi), Nududui (Ewe), Tswetsweetswee (Ga), Amosan (Hausa). Many consider the disease or its manifestations as inflicted upon people through witchcraft. For instance, the chronic splenomegaly experienced by some patients is thought to be a pot put in the “belly” through witchcraft. Even though SCD is ancient, it was only in 1910 that an American doctor, James B. Herrick discovered that the disease was one of red blood cells. Since this discovery, knowledge in sickle cell disease has increased and researches have led to University of Ghana http://ugspace.ug.edu.gh 5 improvement in the understanding of the pathophysiology and complications of sickle cell disease (Ohene-Frempong, 2005). 1.2 Problem Statement One-year cohort study done in Ghana Institute of Clinical Genetics between 2013 and 2014 on ‘Burden of Sickle Cell disease in Ghana’ saw over 5451 adolescents and adults with 20788-clinic visit (Matutes, 2018). Other studies have also reported a high burden of SCD in Ghana. For example, in 2020, Poku reported a large burden of SCD with its associated complications and went further to conclude that fatigue is central to young people’s day-to-day experience and impacting significantly on their self, social and emotional development and ability to have fulfilling life (Poku, 2020). Also, research at Korle Bu, Ghana institute of clinical genetics on ‘Transition Experiences of Adolescents and Young Adults’ revealed how majority of the adolescents had challenge adjusting to their new place of care (Kwarteng-Siaw, 2019). Although limited, these studies points to a high burden of SCD among adolescents in Ghana. However, studies to comprehensively explore the lived experiences of adolescents living with SCD is scanty, thus leaving a major gap in our understanding of the nature and diversity of experiences of adolescents living with SCD. This is critical because adolescents are a unique population group with unique experiences of challenges likely to differ from other age groups. Furthermore, this stage is when major development growth of all organs in the systems of the body. Emotional stress is likely to occur in those who have delays in their physical growth. Also, the high frequency of psychological disorders at this stage compelled with chronic disease should not be ignored. Against this background, this study seeks to explore the lived experiences of SCD among adolescents accessing health care at the 37 Military Hospital in Ghana by exploring fully University of Ghana http://ugspace.ug.edu.gh 6 the experiences of sickle cell and coping strategies among adolescents living with sickle cell; Being a chronic condition, the researcher seeks to know the copping strategies they have developed over the years. The extent to which an individual is affected by a chronic illness may thus be determined by their coping responses, because dealing with its continuous demands necessitates the acquisition of new skills and changes to daily life functioning in children and adolescents” (Anie, Egunjobi, and Akinyanju, 2010). The challenges adolescents living with sickle cell disease go through in accessing healthcare might be another worrying factor. Insurance and a lack of resources to address pain impact were identified as concrete barriers. Adolescents and adults with SCD identified provider issues (lack of knowledge, implicit bias), transportation, and a lack of social support as barriers. Negative experiences with the healthcare system contributes to more adolescents and adults with SCD choosing to manage severe pain at home (Treadwell et al, 2019.). Due to its psychosocial and economic impact on society, the researcher further wants identify and understand existing support systems from families, community, schools and healthcare settings for adolescents living with sickle cell disease. SCD education is generally lacking for the general public and within the formal education sector, leaving the population largely uninformed. Outside of the provision of health insurance schemes, which do not cover the entire population, healthcare financing is extremely limited. However, adequately trained, public health professionals in both the public and private sectors have the potential to lead the effort to shift the current trend toward a service that provides essential healthcare and basic social support to SCD patients and their families (Dennis-Antwi et al, 2007). University of Ghana http://ugspace.ug.edu.gh 7 Analyzing the outcome of narratives of the respondents will inform the public, families and government understand diverse issues concerning adolescents living with sickle cell disease and how to best support and plan for their care for them to live fulfilling lives. 1.3 Justification of the study Several studies have been done in the USA and Africa on effects of SCD on adults, psychological issues with SCD, and experiences with parents with SCD and coping and adjustment in the US. Some of which are; Real stories from People Living with Sickle Cell Disease (Centre for Disease Control and Prevention, 2021), Knowledge and experiences of parents with children affected by Sickle Cell Disease in Cape Town (Niekerk, 2015), Psycho-social problems of adolescents with sickle-cell anemia in Ekiti State, Nigeria (Adegboyega, 2021). Studies done in Ghana focused more on experiences of adults living with sickle cell disease. Some of which are; - Burden of SCD at Korle-Bu (Asare, 2018), Health care provisions for people with sickle cell disease in Ghana (Powars, 1994). Controlling sickle cell disease in Ghana (Edwin, 2011), and SCD disease awareness, Depth of knowledge (Aboagye 2019). The others done in Korle-Bu; Komfo Anokye Teaching Hospital touched on other areas of adolescents. Some of which are; Quality of life of adolescents with SCD (Meyiwa, 2019), assessment of transition readiness in adolescents and (Kwarteng – Siaw, 2017). Although the above studies are important to advance knowledge and scientific research on sickle cell disease in Ghana, research on the lived experiences of sickle cell among adolescents is limited. It is against this background that this research is designed to investigate the lived experiences of sickle cell disease among adolescents accessing healthcare at 37 Military Hospital. Engaging in reflexivity by the researcher’s continuous checks on her judgement, practices and belief systems to identify biases and other characteristics that will influence the research. This will further present trustworthiness and clarity University of Ghana http://ugspace.ug.edu.gh 8 to the results of the study thereby holding the researcher accountable. In addition to advancing knowledge and scientific research, the findings of this study based on the specific objectives will inform healthcare providers, families, teachers and communities to better understand the condition ang give the necessary support to people living with sickle cell disease. Health institutions, NGOS and Government may also implement polices that will improve livelihood of people living with sickle cell disease. 1.4 Research Objectives The objectives of the study were divided into general and specific as stated below. 1.5 General Objective To explore the lived experiences of sickle cell disease among adolescents at the 37 Military Hospital. 1.5.1 Specific Objectives i. To explore the experience of sickle cell and coping strategies among adolescents living with sickle cell. ii. To understand the challenges adolescents living with sickle cell disease go through in accessing healthcare. iii. To identify and understand existing support systems from families, community, schools and healthcare settings for adolescents living with sickle cell iv. To explore the views of adolescents living with sickle cell on measures and strategies to improve access to care and quality of life. University of Ghana http://ugspace.ug.edu.gh 9 1.6 Research Questions i. What are the experiences and coping strategies adopted by adolescents living with sickle cell diseases? ii. What challenges do adolescent living with sickle cell disease face in accessing healthcare at the 37 Military Hospital? iii. What are the existing support systems for adolescent living with sickle cell disease? iv. What are the views of adolescents living with sickle cell on measures and strategies to improve access to care and quality of life? Outline of the Dissertation The presentation of the report of this study is divided into six chapters. Chapter one reveals an accurate and deep understanding into the introduction of the study. This consist of the background to the study, statement of problem, justification, objectives and research question of the study. Chapter two presents the literature review on experiences of adolescents living with sickle cell accessing health care at 37 Military Hospital. This chapter also provides studies done by other researchers on Global burden of sickle cell disease, Sickle cell disease sub-Saharan Africa, Context of sickle cell disease in Ghana, lived experiences of adolescents with sickle cell disease, Coping strategies among adolescents with sickle cell disease, Experiences in accessing health care, Existing support systems and Conceptual frame work. Chapter three provides methods used to collect empirical data for the study whiles, chapter four analysis of the results obtained from the in-delph interviews by bringing out the thematic domains that were identified. Chapter five however, discusses the findings of the study in relation to the existing literature. Chapter six presents summary of the finding, recommendation and conclusion. University of Ghana http://ugspace.ug.edu.gh 10 CHAPTER TWO LITERATURE REVIEW AND CONCEPTUAL FRAMEWORK 2.1 Introduction This chapter presents an overview of previous research on the burden of sickle cell disease. It also explores existing literature on the lived experiences of sickle cell diseases, access to healthcare among adolescent and other people living with sickle cell diseases. Works on the coping strategies adopted by adolescent living with sickle cell, existing support systems are also discussed and reported herein. 2.2 Burden of Sickle Cell Disease i. Global burden of sickle cell disease Sickle Cell Disease (SCD) is a common hematological condition that affects millions of individuals worldwide. It is particularly common in malaria-endemic areas of the tropics, where resource restrictions typically result in dismal results, with the majority of children dying before reaching maturity. As progress is achieved in lowering under-five mortality from infectious causes, noncommunicable diseases (NCDs), including SCD, have climbed to the top of the global health agenda. Regardless, the worldwide impact of SCD in terms of morbidity and death is unclear (Wastnedge et al, 2018). SCD affects around 100,000 persons in the United States, with one in every 365 Black or African-American births, one in every 16,300 Hispanic American births, and one in every thirteen Black or African-American newborns born with the sickle cell trait, according to estimates (SCT). Between 1999 and 2002, deaths caused by sickle cell disease in Black or African-American children under the age of four reduced by 42%. The advent of a vaccination against invasive pneumococcal illness in 2000 corresponded with this decrease (Centers for Disease Control and Prevention, 2022). SCD is characterized by acute pain episodes University of Ghana http://ugspace.ug.edu.gh 11 (the disease's hallmark), anemia, recurrent infections, and chronic end-organ destruction. Because the condition is lethal in the first few years of life, newborn screening, early diagnosis, and a comprehensive treatment program are According to Quinn et al. (2010), the SCD population in the United States has a longer life expectancy, with over 90% of babies born with SCD reaching adulthood. essential. life expectancy, with more than 90% of babies born with SCD reaching adulthood. ii. Sickle Cell Disease in sub-Saharan Africa Sickle cell disease (SCD) is common in Sub-Saharan Africa, affecting up to 3% of births in some countries. Sub-Saharan has become the home to almost 80% of all children born with SCD (Rees, Williams & Gladwin, 2010). Nonetheless, the disease remains a low priority for many health-care organizations. In Africa, the most common form of SCD is caused by homozygosity for the -globin S gene mutation (SS disease). Although it is widely assumed that this condition is associated with extremely high child mortality, there is no reliable data to confirm it. Reviewed available African data on SS disease mortality from published and unpublished sources, focus on two types of studies: Cross-sectional population surveys and cohort studies. The current data are insufficient to support definitive statements, and are consistent with an early-life mortality rate of 50%–90% among children born with SS disease in Africa. When there are interventions in African child survival policies and programs could benefit more children and reduce the number of deaths among SCD children (Grosse et al, 2011). Nearly 90% of the world's SCD population is concentrated in three countries: Nigeria, India, and the Democratic Republic of Congo. Estimates are difficult to come by due to the lack of federal newborn screening programs; however, approximately 700,000 births occur each year, University of Ghana http://ugspace.ug.edu.gh 12 and the prevalence of SCD in newborns was 3% in a regional newborn screening program (DeBaun & Galadanci, 2021). Despite the fact that this disease has a high mortality rate in Africa, little is known about it. The lack of large-scale early-life screening has been a major impediment to progress. The majority of SCD deaths in Africa are likely to occur before cases are even diagnosed (Oron et al, 2020). In rural Tanzania, 999 neonates were examined between February and September 2019, with 31.6 percent (315/999) having sickle cell trait and 3.9 percent (39/999) having SCD. Hemoglobin C was found to be absent. Few parents were aware that their children had sickle cell anemia (0.3 percent). In rural northern Tanzania, the birth prevalence of SCD is extremely high, with 3.9 percent of newborns having SCD and 31.6 percent having sickle cell trait. It was the highest prevalence ever recorded in Tanzania and one of the highest in Sub-Saharan Africa (Eastburg et al, 2021). Nigeria has close to half of the more than 300,000 babies born globally each year with severe hemoglobin disorders. The World Health Organization (WHO) estimates that 5% of the world's population carries trait genes for hemoglobin disorders, primarily sickle cell disease and thalassemia. According to the World Health Organization, more than 300,000 babies with severe hemoglobin disorders are born globally each year, with Sub-Saharan Africa bearing 75% of the burden, with Nigeria bearing 66% of the burden in the region. According to a World Health Organization (WHO) report, 24% of the Nigerian population carries the mutant gene, and the prevalence of sickle cell anemia is around 20 per 1,000 births. The prevalence ranges from 20 to 30% in Cameroon, the Republic of Congo, Gabon, Ghana, and Nigeria, and is as high as 45% in some parts of Uganda. Meanwhile, according to a previous study, "Nigeria has the highest sickle cell disease birth prevalence in the world, with an estimated 150,000 annual births of babies with University of Ghana http://ugspace.ug.edu.gh 13 sickle cell anemia, the most common form of sickle cell disease." According to the WHO (2014), "at least 100,000 babies die from the disorder in Nigeria every year." In other Sub-Saharan African countries, the inherited blood disorder is thought to be the underlying cause of about one in every twelve newborn deaths (Alabiand & Adebowale, 2021). A 2018 study on the 'Prevalence of Complications of Sickle Cell Disease' by Umm Al-Qura University in Makkah, Kingdom of Saudi Arabia used 145 medical files of pediatric patients with SCD ranging in age from one day to 14 years, with a mean SD of 7 3.6 years. Sickle cell anemia with Vaso-occlusive crisis (VOC) was diagnosed in cases who lived outside of Makkah, and 47.1 percent of those who lived in Makkah were diagnosed with VOC on admission. VOC SCA patients were hospitalized once on average, with 25.5 percent being admitted numerous times. Acute splenic sequestration crisis occurred in 3.1 percent of Saudi patients compared to 12.8 percent of non-Saudi patients. The team reported that VOC was the most prevalent complication of SCD, accounting for 55.9% of cases, followed by infection (9 percent). A health care practitioner with competence in SCD, ideally a hematologist, should be consulted for the prevention of acute SCD problems, according to 41.4. Furthermore, difficulties are minimized by starting penicillin prophylaxis in the neonatal period and receiving proper vaccinations, blood transfusions for those at risk of stroke (Alkot et al, 2018). Despite the low cost of many preventive SCD interventions, insufficient resources have been allocated, and progress in reducing SCD burden has lagged behind other African public-health efforts. Announcement of new huge funding recently for research into curative SCD therapies is encouraging in the long run, but it is doubtfully to help Africa's SCD children notably over the next few decades (Uyoga et al, 2020). University of Ghana http://ugspace.ug.edu.gh 14 iii. Context of Sickle Cell Disease in Ghana Every year, approximately 15,000 (2%) of Ghanaian newborns are diagnosed with SCD (Matutes 2018). An earlier study from Ghana by Ohene-Frempong et al (2008) found that “55 percent of sickle cell disease children born in Ghana have the homozygous form. Ages 13 to 44 years revealed a 2:1 ratio of hemoglobin (Hb) SS to hemoglobin SC; this reversed after 44 years, possibly due to higher mortality seen in HbSS patients, who have been documented to have a more severe form of the disease” (Asare et al. 2018). A pilot newborn screening program in one of Ghana's regions revealed a prevalence of 1.8%, (approximately 15000) newborns with SCD are born in Ghana each year. It appears that with proper use of basic medical facilities, more children with SCD are now surviving into adulthood, with the oldest patient in the cohort currently in her late twenties.’ If Ghana wants to impact on SCD care, they must prioritize multidisciplinary care, including adequate primary care, as well as the development and implementation of a national sickle cell disease policy that includes, but is not limited to, universal newborn screening” (Asare et al.2018). People with sickle cell disease (SCD) are frequently undetected until they are four years old and during a pain crisis in Ghana, a country in Sub-Saharan Africa with the world's highest SCD prevalence (Inacio 2021). Despite having the world's highest prevalence of sickle cell disease (SCD), according to Sims et al. (2021), no nation in Sub-Saharan Africa has a universal SCD screening program. Parents in Accra, Ghana, were interviewed about SCD diagnostic trends (age at diagnosis, manner of diagnosis, and age of first pain crisis). A total of 354 people were investigated. During the neonatal period, just a few persons were diagnosed with the condition through SCD testing. Only 44% of University of Ghana http://ugspace.ug.edu.gh 15 children with SCD had been identified by the age of four, and 46% had had a pain crisis. The vast majority (66%) were diagnosed as a result of a pain crisis.either in an acute (49%) or primary care (17%) setting. Children were diagnosed with SCD at a younger age (74 percent by the age of four), and adults were identified at a later age (30 percent by four years old). The findings confirmed the necessity of newborn screening programs, which are lacking in Ghana. According to the study, patients are "likely being excluded from life-saving preventative therapy." Children in Accra, Ghana, are at risk of disease complications and mortality due to the lack of a comprehensive newborn screening program for SCD. People in the study were diagnosed with SCD in an acute care setting, as well as toddlers, school-aged children, or later. Meaning that they are prevented from critical preventive care. Acknowledging the existing SCD diagnosis patterns will aid efforts to improve SCD diagnosis early and reduce disease-related mortality and morbidity in this high-prevalence community (Sims et al, 2021). A community-based cross-sectional survey of 938 children aged 1–12 years from three districts in the Volta region revealed an overall frequency of sickle cell disease of 2.0 percent, with 16.0 percent of sickling screening positive. The HbSF genotype was the most common among sickle cell disease individual genotypes (0.9 percent vs. 0.2 percent; HbSS, 0.6%; HbSC and 0.3%; HbSCF).. However, no discernible link could be found between sickle cell disease and the growth and development of the children who were screened (Oppong et al. 2020). Over the years, SCD has been overlooked as a disease of public health significance. In Ghana, statistics on deaths from major childhood diseases such as malaria, acute respiratory infections, and malnutrition-related issues have buried the health status of children with catastrophic genetic disorders such as SCD (Ohene-Frempong, 2007). According to the Central Intelligence Agency (CIA),2007). Ghana is heavily reliant on international financial and technical University of Ghana http://ugspace.ug.edu.gh 16 assistance, making it not possible to implement effective and adequate services, including SCD healthcare (CIA, 2007). Basic information on SCD was first included in the national child health growth monitoring plan in 2007 to raise awareness of SCD and the need for testing among mothers (Dennis-Antwi, 2007). SCD patients are treated as general outpatients, reporting to health institutions on a daily basis, with the exception of Accra, Kumasi, and a few governmental and private treatment centers primarily in the south, such as Tema, Koforidua, Agogo, and Sunyani. Ghana, has yet to implement comprehensive SCD care. assistance (Okpala et al, 2002). The vast majority of these patients are admitted to hospitals with serious illnesses and are not always diagnosed with SCD (Orji et al, 2002). 2.3 Lived Experiences of Adolescents with Sickle Cell Disease A study on the illness experiences of young people with sickle cell disease (SCD) looked at adolescents with SCD who were experiencing fatigue. In Ghana, 24 adolescents participated in narrative and picture-elicitation interviews. The narratives were used to build a grounded theory, "body as a machine." Fatigue was the most limiting and disruptive aspect of growing up with SCD. Its source of meaning and value was indicated by what it indicated. Fatigue was a socially undesirable and stigmatizing trait in youth due to the demands of high physicality. As a result, fatigue was a significant threat to "normalcy." The social value of the physical body and its capabilities influenced adolescents' fatigue experiences. The lives of the adolescents were dominated by the need to manage exhaustion in order to construct/maintain socially acceptable identities. As a result, acknowledgment is required if there is a need for a recognition of the significance of fatigue to adequately support young people growing up with it” (Poku, Caress & Kirk, 2020). Another integrative narrative review of literature on Adolescents' experiences of living with sickle cell disease found 683 studies, of which 40 met the inclusion criteria, and 9 University of Ghana http://ugspace.ug.edu.gh 17 broad themes emerged: knowledge and understanding of the condition, symptom experiences, self-management, attitude to treatment, healthcare experiences, social relationships, difference and striving for normalcy, school experiences, and emotional wellbeing and coping. The study concluded that Sickle cell disease has an impact on a variety aspect of an adolescent’s life. As a result, nursing care and research should place a greater emphasis on the developmental well-being of adolescents, as well as promote peer support networks among adolescents with the condition (Poku, Care & Kirk 2018). A study on the Quality of Life of Adolescents with Sickle Disease was conducted in 2019 at the Ghana Institute of Clinical Genetics, Korle Bu Teaching Hospital. “Quality of life (QOL), a social construct measuring a person's overall well-being has been shown to be lower in adolescents with SCD due to the disease's effects. The study's goal was to assess the quality of life of adolescents attending a Sickle Cell clinic at the Ghana Institute of Clinical Genetics in Korle Bu. The study took a qualitative approach, employing the phenomenological technique. In-depth interviews were conducted with 15 adolescents with Sickle Cell Disease who were attending the Institute's Sickle Cell Clinic, and the audio was recorded and transcribed. To generate themes for data analysis, thematic analysis was performed by coding and recoding. The study found that the quality of life of adolescents with Sickle Cell Disease was good, based on their subjective meanings and interpretations of their health at this stage of life. This study's findings included the adolescent patients' perceived general, emotional, physical, social, and cultural health and well-being. Family support and clinical visits were discovered to have a significant impact. on the quality of life of SCD adolescents It was discovered that culture had no effect. There were no differences in the quality of life of male and female SCD adolescents. The researcher concluded that, in general, the adolescents' perceived quality of life was good; however, emotional health was found to be lacking in some younger University of Ghana http://ugspace.ug.edu.gh 18 female adolescents. Positive social factors associated with QOL should be encouraged. It is also recommended that the emotional health of younger adolescents, particularly females, be considered and prioritized in SCD care” (Meyiwa, 2019). An empirical qualitative study of six adolescents with sickle cell disease conducted in Kingston, Jamaica revealed that the majority of the adolescents had a positive self-concept. They reported feeling accepted because of strong family, school, and peer support. All were actively involved in social activities such as parties, but found it difficult to participate in sporting activities. It was thus concluded that Sickle cell disease can be extremely difficult for adolescents, but with a positive self-concept and increased social support, particularly from family and peers, these adolescents were able to effectively cope with their condition (Brown et al, 2020). A study carried out in Zambia on lived experiences of adolescent learners with SCD generated several themes that highlighted the experiences that students go through while attending school. The study documented the experiences of adolescent learners as well as their parents and teachers. Based on interviews conducted with students suffering from SCD. The following themes emerged: Being sick frequently; repeating grades; studies and extra lessons; exemption from strenuous activities and Teachers do not understand the situation, as evidenced by a number of issues raised during interviews with mothers. These highlighted their experiences. Children with sickle cell disease were used as students. The following themes emerged: children with SCD miss school; children with SCD require more attention in class; Teachers do not understand sickle cell disease and repeat extra lessons and classes. The themes that emerged from the interviews with teachers revealed teachers' perspectives on students with sickle cell disease. The following are the themes: Parents and students do not want the SCD condition to be known; Teachers' lack of understanding of the SCD; Individualized University of Ghana http://ugspace.ug.edu.gh 19 attention and adequate support are required for children with SCD (Ngomah, Moraes & Munsaka, 2019). 2.4 Coping Strategies among Adolescents with Sickle Cell Disease An empirical study conducted in Kingston, Jamaica on "The lived experiences of adolescents with sickle cell disease" by (Forrester et al., 2015) revealed that “the majority of the participants reported a positive self-esteem during the interviews. Three participants stated unequivocally that they did not feel different from others. In the presence of SCD, they felt good about themselves. Due to delayed menarche, female participants were extremely anxious. Some participants also expressed frustration, sadness, and depression, particularly during painful crises. The results were the same for both male and female participants. They were afraid of dying at times, especially during severe painful crises, hospitalizations, or when a friend or peer died as a result of SCD complications. All participants stressed the importance of staying healthy in order to avoid painful SCD crises and complications. Some health-maintenance strategies included avoiding social activities and adhering to strict dress codes. They believed that following medical and dietary regimens would help them improve their health and live longer lives. "I," one participant exclaimed. make sure I have all of my medications, drink plenty of water, eat properly, and exercise, stay fit, and get my regular check-ups." Respondents relied heavily on prayer and spiritual activities as coping mechanisms. They reportedly believed in God and prayed frequently, especially when their conditions worsened. They engaged in spiritual activities such as fasting and attending church prayer meetings”. Hurtig and White conducted another study on Psychosocial Adjustment in Children and Adolescents with Sickle Cell Disease (Hurtig & White, 1986). The study looked into how chronic illness-related stress affected adjustment. A group of 50 children and adolescents with sickle cell University of Ghana http://ugspace.ug.edu.gh 20 disease were chosen from a Sickle Cell Center over a long period of time, with symptoms ranging from mild to severe. The severity of the illness was assessed in terms of perceived pain, hospitalizations, and emergency room visits, as well as the nature of the presenting symptoms and levels of adjustment. The findings revealed that There were issues with a variety of adjustment variables, especially for adolescent males, and most notably in the areas of behavior problems and social adjustment. There were implications for adaptive coping with adolescent developmental processes (Anie et al., 2010). Kofi Anie's 2010 online study investigated the psychosocial impact of sickle cell disease in 408 adolescents and adults attending three hospitals in Lagos, Nigeria. “The majority of participants believed that society as a whole had a negative perception of SCD, and they reported negative perceptions and attitudes. The findings also revealed that depressive feelings were prevalent. Even though feelings of anxiety or self-hatred were uncommon, they were experienced by nearly half of the study population” (Anie, Egunjobi & Akinyanju, 2010). In another development, adolescents (all students) with sickle cell disease who attended sickle cell outpatient clinics at Lagos University Teaching Hospital, Gbagada General Hospital, Massey Street Children's Hospital, and the National Sickle Cell Centre were recruited opportunistically over two months for a study on the psychosocial impact of SCD in a Nigerian population. The typical clinic Attendance at the three hospitals ranged from 30 to 45 people. It was discussed that “the impact of chronic illness, such as SCD, on individuals can be divided into three categories: adjusting to symptoms and incapacities, maintaining adequate relationships with health-care providers, and managing the emotional and social consequences of the illness. The extent to which an individual is affected by a chronic illness may thus be determined by their coping responses, because dealing University of Ghana http://ugspace.ug.edu.gh 21 with its continuous demands necessitates the acquisition of new skills and changes to daily life functioning in children and adolescents” (Anie, Egunjobi, and Akinyanju, 2010). Kliewer and Lewis (1995) investigated the role of parenting and family variables in the overall coping processes of 39 sickle cell disease children and adolescents (SCD). Parents provided information about their children's health history, coping suggestions for their children, their own coping strategies, and family cohesion during home interviews. Children were asked to rate their general coping strategies and their level of hope. Each of the three models of family influences tested in this study received partial support. After taking into account the effects of age, gender, Children's hope was positively associated with active coping suggestions by parents, regardless of family structure or type of SCD. A cohesive family environment was associated with children's active coping, and avoidance coping was predicted by less parental use of restructuring coping and more parental use of active coping strategies. The study as a whole, provided evidence for the impact of parental coaching and modeling, as well as the family environment, on coping processes in children with SCD (Kliewer & Lewis, 1995). A study looked at the relationships between pain, pain coping, and sleep, as well as the factors that affect pain, coping, and sleep in children with sickle cell disease (age, gender, frequency, and intensity of pain) (SCD). “An electronic visual analog scale Pain Coping Questionnaire and the Pittsburg Sleep Quality Index were completed by 66 adolescents aged 11 to 16 years SCD. Approximately two-thirds of the adolescents reported pain in the previous month, but there were no significant associations between pain and age, gender, pain intensity, or frequency. The majority of them dealt with pain by seeking information, problem solving, social support, and making positive self-statements. In males, there were significant negative correlations between worse Pain severity, behavioral distraction, and internalizing or catastrophizing are all factors to University of Ghana http://ugspace.ug.edu.gh 22 consider. The vast majority (91.2 percent) suffered from mild to severe sleep disturbances, with 18.2 percent requiring sleeping medication three or more times per week. It was determined that children with SCD experience pain, which affects their sleep patterns and their ability to cope with pain. Nurses must assess pain, coping, and sleep simultaneously, and promote sleep and hygiene for positive coping strategies during pain episodes” (Graves & Jacob, 2014). Coping in Sickle Cell Disease Adolescents, a pilot study was conducted to determine how adolescents use religious and spiritual beliefs to cope with a chronic illness such as sickle cell disease (SCD). Another study that looked at coping, spirituality, and health-related quality of life in 48 adolescents with SCD found that they had high rates of religious attendance and belief in God, prayed frequently, and had high levels of spirituality (e.g., finding meaning/peace in their lives and deriving comfort from faith) (Cotton et al., 2009). For symptom management, 35% of adolescents reported praying once or more per day. Adolescents' most common positive religious and spiritual coping strategies were that 73% asked forgiveness for their sins and sought God's love and care. For adolescents with SCD, spiritual coping, particularly prayer, was important (Cotton et al., 2009) A related study conducted during the 2007 and 2008 Sickle Cell Research and Education Day annual events held at Cincinnati Children’s Hospital Medical Center (CCHMC) in Cincinnati, Ohio among Adolescents age 11-19 years with SCD via letter and flyer identified Prayer and spiritual activities as a major coping strategy for the respondents. They reportedly believed in God and prayed often, especially when there was an exacerbation of their conditions. They participated in spiritual activities such as fasting and going to church for prayer meetings Other coping strategies that were also used were diversional activities applied during periods of exacerbation of their illness as it helped take their minds off their condition and to feel good about themselves. University of Ghana http://ugspace.ug.edu.gh 23 Such activities included watching television or talking with close friends and families (Cotton et al., 2009). A study on Coping and Coping Assistance among Children with Sickle Cell Disease and Their Parents discovered that “how a family copes with the physical and psychosocial challenges of SCD influences the functioning of the child and family. It was also discovered that children face a variety of stressors related to SCD and its treatment, including but not limited to pain. Families use a variety of approach and avoidance-oriented coping strategies to deal with these stressors. In complementary and distinct ways, quantitative and qualitative assessments contributed to a better understanding of coping processes in children with SCD and their parents” (Hematol, 2015). Another study looked at the outcomes of a cognitive-behavioral therapy (CBT) intervention for pain in children with sickle cell disease using smartphones as a novel delivery modality (SCD). 46 children with SCD were given CBT coping skills training using a randomized, waitlist control design. The intervention included one CBT session and eight weeks of home practice with cellphones. Using pre-post questionnaires, the Coping Strategies Questionnaire was used to assess changes in active psychological coping and negative thinking between the randomized groups. The use of CBT skills was associated with decreases in next-day pain intensity and increases in same-day functional ability. According to daily diaries kept by the entire sample during treatment, activity A comparison of the pre- and post-groups revealed that, according to the pre-post group comparison, youngsters increased active psychological coping attempts. According to daily diary data, when children used CBT skills on days with more pain, the next day's pain intensity was reduced. It was then concluded that coping skills training using cognitive behavioral therapy (CBT) and smartphone technology can improve coping, reduce stress, and University of Ghana http://ugspace.ug.edu.gh 24 reduce pain severity in children with SCD; however, changes to the study protocols may be required (Pain, 2015). Experiences in Accessing Health Care Among Adolescent’s Sickle Cell Patients Health care access is the ability to obtain healthcare services such as prevention, diagnosis, treatment, and management of diseases, illness, disorders, and other health-impacting conditions. For healthcare to be accessible it must be affordable and convenient. (Gulliford & Morgan). Many people including adolescents with sickle disease in Ghana do not have access to adequate healthcare hence are unable to live quality lives. Kanter et al (2020) surveyed 440 sickle cell disease (SCD) adolescents and adults in Virginia, Southeast America, using 7 comprehensive programs widely recognized as leaders in SCD care. The survey results indicated “significant gaps and dissatisfaction with care, particularly in the emergency department. Patients' experiences are strongly linked to their perceptions of the quality of care. All participants expressed dissatisfaction with care for a variety of reasons, including perceived racial discrimination, access issues, and a lack of clinician confidence and knowledge in managing SCD complications, particularly pain. Furthermore, 53 percent of SCD patients who complained of discrimination more likely to disregard medical treatment and recommendations Mistrust in health-care professionals has been linked to non-adherence to medical treatment and avoidance of the emergency room in times of crisis, both of which have been linked to increased mortality. Clinician comfort with caring for SCD patients is a barrier to improving care quality, particularly in the ambulatory setting. Another survey of primary care physicians (PCPs) at Johns Hopkins Community Physicians revealed that PCPs are hesitant to care for patients with SCD. University of Ghana http://ugspace.ug.edu.gh 25 They are most uneasy when dealing with SCD-specific issues, such as prescribing hydroxyurea and managing pain crises. Prior experience caring for SCD patients and knowledge gained during residency are linked to increased confidence. Patients with SCD in the United Kingdom reported in a focus group receiving poor quality care from their PCPs, owing to a perceived lack of confidence and knowledge about SCD-specific disease management, a lack of communication, and accessibility challenges Seven (7) patients still believed that their primary care physicians played an important role in their overall health management”. The United States recognized the need to invest significant resources in expanding comprehensive sickle cell centers and improving SCD self-efficacy. Efforts must be directed toward smaller communities and rural areas in order to overcome the negative impact of social determinants of health, which can be barriers to high-quality care (Oyedeji & Strouse, 2020) A needs assessment based on pervasive disparities in the community The University of California San Francisco, Berkeley investigated the disparities in health resources and barriers to facilitators of care faced by adolescents and adults with sickle cell disease (SCD) in order to identify opportunities to support the implementation of evidence-based interventions aimed at improving care. 51 health care providers, administrators, and community-based organizers used triangulated qualitative and quantitative data from 58 adolescents and adults with SCD. The following findings were discovered during group interviews: Insurance and a lack of resources to address pain impact were identified as concrete barriers. Adolescents and adults with SCD identified provider issues (lack of knowledge, implicit bias), transportation, and a lack of social support as barriers. Negative experiences with the healthcare system contributed to 84 percent of adolescents and adults with SCD choosing to manage severe pain at home. Providers concentrated University of Ghana http://ugspace.ug.edu.gh 26 on structural barriers such as a lack of access to care guidelines, comfort with and knowledge of SCD management, and poor care coordination. It was concluded that strategies for improving access to compassionate, evidence-based quality care, as well as strategies for reducing the burden of having SCD, are warranted for a medically complex population (Treadwell et al, 2019.). According to a survey of 13 adolescents and 5 young adults in a rural community, 62% of adolescents had difficulty leaving their pediatrician. They were worried about finding an adult doctor, arranging transportation, preparing to use an adult hospital, and dealing with financial problems. They proposed services such as a transition program to provide information about adult providers, ways to take control of one's own care, and assistance in helping adult providers learn more about the disease. In a rural community, adolescents with SCD identify potential barriers to adult health care. Several barriers are similar to those reported by young adults. Adolescents with sickle cell disease require accessible self-management interventions to better cope with their disease, improve health outcomes and health-related quality of life, and promote successful transition to adult health care services. However, there haven't been many comprehensive self-management and transitional care programs established. Internet and mobile phone technologies can increase the accessibility and acceptability of interventions to promote self-management in sickle cell disease adolescents (Eysenbach, 2018). Adolescents, parents, and health care providers described the ongoing psychosocial and stigmatization challenges that youth with sickle cell disease face. Participants saw the value in addressing some of these issues with a digital self-management tool. They suggested that “an effective digital self-management program provide appropriate sickle cell disease–related education, guidance on developing self-advocacy and communication skills, empower adolescents with information for future planning, offer options for social support, and be designed to be engaging for both adolescents and parents to University of Ghana http://ugspace.ug.edu.gh 27 use”. A digital platform that provides these elements is an accessible and acceptable way to address adolescent self-management and transitional care needs (Kulandaivelu et al., 2018). Adolescents with SCD frequently miss clinic appointments due to competing activities involving school or peers. One adolescent stated that some participants use aids such as calendars or planners to keep track of appointments. Attendance appeared to be influenced by the adolescent's health status as well. Some focus group participants stated that they do not want to go to the clinic when they are feeling well. Participants reported that if their parents/caregivers were present, they were more likely to attend a clinic appointment. The study also discovered a number of factors related to Adolescents' interactions with the hospital system as influences on clinic attendance One of the most important factors mentioned was the relationship with providers (clinic/hospital personnel). They expressed their dissatisfaction with staff members who they felt were insufficiently trained to perform medical procedures on SCD patients. Another issue was seeing different doctors on each visit, and the providers repeatedly asking the same questions to multiple people. Adolescent patients reported difficulty understanding physician instructions and being uneasy with treatments. Patient satisfaction with care appeared to influence attendance as well. In general, comments indicated a desire for appointment scheduling flexibility (Crosby et al., 2009). “The goals of psychosocial interventions in chronic illnesses such as SCD in the global context are to help reduce negative thoughts and feelings about the condition and to encourage the acquisition or maintenance of coping strategies. Psychological therapies, in addition to routine medical treatment, should be offered as standard care in the management of SCD, where studies of these therapies have shown encouraging results. The overall goal is to assist patients in better coping, fulfilling roles, and achieving a higher quality of life. These interventions should be age- University of Ghana http://ugspace.ug.edu.gh 28 appropriate and accessible in both hospital clinics and community-based settings” (Drews et al., 2010). 2.5 Existing Support Systems Family Support System. An investigator investigated the role of parenting and family variables in the overall coping processes of 39 sickle cell disease children and adolescents (SCD). Interviewed parents provided information about their children's health history, coping suggestions for their children, their own coping strategies, and family cohesion. Children were asked to rate their general coping strategies and their level of hope. Each of the three models of family influences tested in this study received partial support. A cohesive family environment was associated with children's active coping, and avoidance coping was predicted by less parental use of restructuring coping and more parental use of active coping strategies. This study provides evidence for the impact of parental coaching and modeling, as well as the family environment, on coping processes in children with SCD (Kliewer & Lewis, 2005). “A study looked at the relationship between coping and family functioning and health outcomes in adolescents with SCD, taking into account sociodemographic and psychosocial risk factors. At a baseline assessment, 41 adolescents and their families (41 primary caregivers, 9 secondary caregivers, and 15 healthy siblings) completed paper-and-pencil measures of coping and family functioning (time 1). Medical file reviews were used to determine disease severity, SCD complications, healthcare utilization, and average hemoglobin level at time 1 and time 2 (1 year later). Time 1 disease-related parenting stress predicted time 2 health outcomes, but no significant associations for coping were found. Teens with increased disease severity and higher healthcare University of Ghana http://ugspace.ug.edu.gh 29 utilization were found in families who reported lower family functioning. Efforts should be made to investigate specific mechanisms by which adaptive family functioning contributes to health outcomes in adolescents with SCD, with a particular focus on addressing the potential role of multiple sociodemographic and psychosocial risk variables” (Barakat et al, 2007). Another study, by Barakat et al (2008), used teen- and parent-report to clarify associations between pain, psychological adjustment, and family functioning with health-related quality of life (HRQOL) in a sample of adolescents with sickle cell disease (SCD). “Forty-two adolescents (aged 12 to 18) with SCD and their primary caregivers completed paper- and-pencil pain, psychological adjustment, and HRQOL measures. Primary caregivers also completed a measure of disease-related parenting stress. A review of medical records determined the severity of the disease. Pearson correlations revealed significant inverse relationships between pain frequency and physical and psychosocial HRQOL domains as rated by the teen and primary caregiver. Internalizing symptoms (such as anxiety and depression) and disease-related parenting stress were also found to be significantly related to lower HRQOL. A series of regression analyses on possible mediator models confirmed that disease related parenting stress served as a mediator between pain frequency and physical and psychosocial HRQOL. Findings for mediation models involving internalizing symptoms were less consistent. Parent-rated teen depression and teen anxiety served as mediators of the association between pain frequency and HRQOL in these studies. In the context of SCD management, efforts to improve HRQOL should focus on internalizing pain symptoms as well as parenting stress” (Barakat et al., 2008). A Family-Based Randomized Controlled Trial of Pain Intervention for Adolescents with Sickle Cell Disease determined the efficacy of a family-based cognitive-behavioral pain management intervention for adolescents with SCD, reducing pain and improving health-related variables as University of Ghana http://ugspace.ug.edu.gh 30 well as improving psychosocial outcomes. Each adolescent and a family support person were assigned at random to either a brief pain intervention (PAIN) (n = 27) or a disease education attention control intervention (DISEASE ED) (n = 26). At baseline (prior to randomization), post- intervention, and one-year follow-up, primary pain and health-related variables (health service use, pain coping, pain-related hindrance of goals) and secondary psychosocial outcomes (disease knowledge, disease self-efficacy, and family communication) were assessed. At either time point, there was no significant difference in outcome change by group. According to preliminary findings, comprehensive interventions that address a wide range of skills related to disease management and adolescent health concerns may be more effective in assisting teens during the healthcare transition. (Hematol, 2010). 2.6 School Support System “Teasing and bullying are common complaints among school-aged children with SCD, according to anecdotal evidence from adolescent students aged 14 to 18 years. This was reported by 23% of the study participants. Other major psychosocial problems experienced by young people with SCD during their school years include fear of dying too soon, fear of discussing the condition with friends and teachers, embarrassment about bedwetting and reluctance to participate in school trips as a result, teasing by colleagues due to jaundice and associated discoloration of their eyes, and anger if ill-informed staff consider the child lazy and wanting to avoid school activities. Anxiety that young people with SCD experience at school may lead to the formation of a negative image of themselves, teachers, and school personnel” (Anie, Egunjobi & Akinyanju, 2010). A researcher assessed adolescents with SCD's perceptions of school performance, SCD interference, and acceptability of educational support strategies. The relationship between school performance, SCD interference, and demographics was also investigated in order to identify University of Ghana http://ugspace.ug.edu.gh 31 potential risk factors. Thirty adolescents between the ages of 12 and 20 filled out demographic and SCD school performance questionnaires. Although approximately 37% of participants reported receiving special education services, more than 60% reported that SCD interfered with their academic performance. Females reported that SCD had a greater impact on their schooling than males. The study findings provide important insights into demographic risk factors and support the need for adolescents with SCD to have individualized health and educational plans. Almost half of the participants (46.7%) reported participating in a school-sponsored extracurricular activity (e.g., sports team, band), and 16.7 percent reported participating in a school-sponsored work activity (e.g., internship, work-study job). Outside of school, 43.3 percent of participants were involved in a group activity (e.g., church youth group, sports team), 30 percent took lessons (e.g., dance, music), and 23.3 percent volunteered or performed community service. More females participate in school or community extracurricular activities. Over half of the participants agreed that educational support strategies were necessary (Crosby et al, 2016). “On January 9, 2015, researcher advisors developed a guide to support young people with sickle cell disease at school in response to research reports indicating that schools struggle to support young people with sickle cell disease. A new law (Section 100 of the Families Act 2014) requires schools and academies to make plans to support students with medical conditions” (A guide to school policy, 2015). Schools struggle to support sickle cell patients, but the Sickle Cell Society can provide assistance and advice. "Teachers are exposed to a wide range of medical conditions, and it is unreasonable to expect them to remember the specifics of all of them." At the same time, young people with sickle cell disease dislike initiatives that highlight their differences from their peers," said Professor Simon Dyson of De Montfort University, who led the research University of Ghana http://ugspace.ug.edu.gh 32 team. "What was required was a policy that supported sickle cell students while remaining in the background and not overloading teachers with information." The Guide to School Policy for Young People with Sickle Cell Disease is based on best practices and includes a template for developing an individual health care plan. It is completely free to download, print, or post on the school's website. 2.7 Health Service Support System “Ghana is heavily reliant on international financial and technical assistance, making it difficult to implement effective and adequate services, including SCD healthcare (CIA, 2007). Understandably, the situation is different in most of Europe and the United States, where SCD screening programs are widespread on both a universal and pilot basis. SCD screening and management are governed by specific guidelines. Ghana, like most Sub-Saharan African countries, lacks a national policy, management guidelines, or national statistics on SCD, despite the fact that nearly 25% of the population, or one in every four, is a carrier and 2% of all babies born, or 20 births per 1000 live births, have a form of SCD” (Dick, 2006; OheneFrempong, 2007). So far, Ghana has begun discussions about establishing a national SCD program. Children who live in urban and peri-urban areas are more likely to have access to better diagnostic services, which are frequently based on solubility tests, which are not sensitive enough to detect high levels of hemoglobin S. (HbS). Solubility testing is a simple test that is frequently used in clinical care laboratories in Sub-Saharan Africa to detect the presence of HbS in blood. A chemical is added to the patient's blood sample, which reduces the amount of oxygen carried by the blood. Some HbS will be present in those who carry even one sickle cell gene. These tests, however, produce inconclusive results because they do not distinguish between SCD and sickle cell trait University of Ghana http://ugspace.ug.edu.gh 33 (Anionwu and Atkin, 2001, American Association for Clinical Chemistry, 2006; Tshilolo et al, 2008). “Most SCD patients in Ghana receive medical care through the existing static health facilities found within the national, regional, and district healthcare systems. Except for Accra, Kumasi, and a few public and private treatment centers in Tema, Koforidua, Agogo, and Sunyani, SCD patients are seen as general outpatients, reporting daily at health facilities. Ghana and the majority of Sub- Saharan African countries have yet to establish comprehensive SCD care services” (Okpala et al, 2002). The vast majority of these patients present to the hospital with acute illness without having been diagnosed with SCD (Orji et al, 2002). In the United Kingdom and the United States, widespread universal or selective newborn screening programs ensure early diagnosis and comprehensive management with optimal outcomes (Hilliard et al, 2004). “SCD has not been recognized by African governments as a disease of public health importance. So far, patient care has been straightforward. Few centers provide special healthcare services for SCD patients, leaving a large proportion of affected families to attend general outpatient services in district hospitals and health centers with limited knowledge of specialist SCD care. There are few support groups to offer affected patients and families the much-needed psychosocial support. Although charismatic churches have policies for premarital testing, which raises some issues, genetic counselling services are generally lacking. SCD education is generally lacking for the general public and within the formal education sector, leaving the population largely uninformed. Outside of the provision of health insurance schemes, which do not cover the entire population, healthcare financing is extremely limited. However, if adequately trained, public health professionals in both the public and private sectors have the potential to lead the effort to shift the current trend toward a service that provides essential University of Ghana http://ugspace.ug.edu.gh 34 healthcare and basic social support to SCD patients and their families. The final section of this paper proposes establishing a model for SCD provision in Africa, with a focus on Ghana” (Dennis- Antwi et al, 2007). 2.8 Community Support System A study looked into the psychological effects of SCD in a Nigerian population. The findings support the idea that attitudes and perceptions in society have a psychosocial impact on people with SCD. According to the study's findings, “a large proportion of respondents believe that SCD as an illness that is underappreciated in society and that more awareness is needed, and that if society was more aware of people with SCD, it would be more accepting of those with the condition. As a result, appropriate psychosocial interventions must be developed in a global context. As a starting point, more public education about SCD should be provided in order to change beliefs, attitudes, and stigma. This should be followed by other interventions that are tailored to different levels and settings of access to health care. In developing countries like Nigeria, the emphasis would be on initiating basic psychosocial interventions by nonspecialized health workers in a primary care team at a Community Health Post (Level 1) or Primary Health Centre (Level 2), where psychologists or social workers are not available. This would be consistent with the World Health Organization's current priority of primary care” (Anie. 2010). 2.9 Summary of the review The review of previous studies revealed sickle cell disease as still being the major inherited blood disorder globally. The disease has huge economic burden on countries especially sub-Saharan Africa and families. Psychosocial and health care issues remain one of the challenges facing adolescents with the disease in Africa including Ghana. In developed countries like United State of America, sickle cell disease been one the inherited health conditions that has gained recognition University of Ghana http://ugspace.ug.edu.gh 35 and attention by government civil societies and communities. Psychologists, social workers physicians, health centers support services are spread out and are readily available to help those living with sickle cell disease in these developed nations. Researchers have and are still carrying out investigations on the disease and to help sufferers live better lives and policies implemented for them. The United States recognized the need to invest significant resources in expanding comprehensive sickle cell centers and improving SCD self-efficacy. (Oyedeji & Strouse, 2020). In the United Kingdom and the United States, widespread universal or selective newborn screening programs ensure early diagnosis and comprehensive management with optimal outcomes (Hilliard et al, 2004). According to Quinn et al. (2010), the SCD population in the United States has a longer life expectancy, with over 90% of babies born with SCD reaching adulthood. essential. life expectancy, with more than 90% of babies born with SCD reaching adulthood. However, in sub–Saharan Africa including Ghana people are now gaining awareness and health implications of sickle cell disease. Due to lack of funds and the heavy economic burden it carries governments in sub–Saharan Africa have not been able to provide adequately for the needs of adolescent with sickle cell disease. Despite the fact that this disease has a high mortality rate in Africa, little is known about it. The lack of large-scale early-life screening has been a major impediment to progress. The majority of SCD deaths in Africa are likely to occur before cases are even diagnosed (Oron et al, 2020). Only few researches have been done in Ghana on adolescents living with sickle cell disease. Psychosocial problems, challenges in accessing health care with minimal or no social support are some of their lived experiences. Over the years, SCD has been overlooked as a disease of public health significance in Ghana (Ohene-Frimpong, 2007). University of Ghana http://ugspace.ug.edu.gh 36 Moreover, qualitative instead of quantitative approach adopted in most of the previous study was employed in this study. The chapters that follow present the Methods, Findings, Discussions, Conclusion and Recommendations. University of Ghana http://ugspace.ug.edu.gh 37 Conceptual Framework Conceptual framework is that aspect of research process that study the subject ideas of a topic. It is an analytical tool used to make conceptual distinctions that bring together different ideas that lead to the realization of the intended objectives. It is a structure the researcher uses to best explain the natural progression of the phenomenon to be studied (Camp, 2020). It is also, a visual representation that provides a picture of the phenomenon, clearly defined relationship between different variables and their relationship to each other. Figure 1: Experiences of Adolescents with Sickle Cell Disease Accessing Health Care at 37 Military Hospital Authors’ Conceptual Framework University of Ghana http://ugspace.ug.edu.gh 38 The conceptual frame work above is structural organization of the study i.e The Experiences of Adolescents with Sickle Cell. As chronic inherited condition, adolescent with sickle cell disease undergo serious psychosocial challenges of depression, stigmatization and loss of dreams and goals. There is the need therefore to establish a form of support for them in their families, schools and the hospital. The impact of the disease and frequent crises make them to develop their own coping strategies like, drinking of sufficient water, exercising, praying, and taking of pain killers. The condition also requires them to seek frequent health care in a specialized health facility but there are various factors that influence access to health care. These are; individual, community and health system factors. University of Ghana http://ugspace.ug.edu.gh 39 CHAPTER THREE METHODOLOGY 3.1 Introduction This chapter presents the research methodology of the study. The chapter describes source and how data was collected. The research design and the estimation technique proposed for the study. the Population size, sampling technique used to arrive at a sample size was also justified. Finally, the methods used for the data analysis are also thoroughly discussed. 3.2 Study Design Philosophical Assumptions Usually, quantitative and qualitative approaches are employed in research. The fundamental difference in these approaches depends on philosophical assumptions, According to Creswell and Poth (2018), Whether being aware, or not, always certain beliefs and philosophical assumptions shape and influence how researchers find answers to questions through information which also serve as basis of evaluation of a study. These assumptions are Ontology, which is the way researchers welcome multiple ideas of realities and report by exploring multiple realities from different people. The second is Epistemology. In this. The researchers get close contact with participants to get evidence on the views of each individual on the field. Anxiology which is the third considers both the researcher and the information gathering from the field, reporting values and biases and fourthly Methodology which are the methods used in research. Quantitative research stems from positivism which believes in single reality and contend to only one truth (Yilmaz, 2013). While qualitative research is based on interpretivism and constructivism and believes that realities are multiple, holistic, constructed socially (Creswell & Poth, 2018; University of Ghana http://ugspace.ug.edu.gh 40 Yilmaz, 2013). In addition, quantitative research is deductive, explains phenomena by numerical data with well-organized data collection, test hypothesis of measurable variables by means of statistical analysis (Yilmaz, 2013).A qualitative study design was considered over quantitative for this study because in qualitative research inductive, interpretive and naturalistic approaches are used to study people cases, phenomena, social situations and processes in their natural settings (Denzin& Lincoln, 2011)It is more experiential by nature and focuses on capturing people's feelings and perspectives. It has advantages of tracking shifting views among a target group, can be used to provide context and possibly explain something that numbers alone cannot disclose. Qualitative research allows for a lot more flexibility. It provides a much more flexible approach. If useful insights are not being captured researchers can quickly adapt questions, change the setting or any other variable to improve responses. Qualitative data capture allows researchers to be far more speculative about what areas they choose to investigate and how to do so. It allows data capture to be prompted by a researcher’s instinctive or ‘gut feel’ for where good information will be found (Vaughan, 2021). It is therefore more convenient carrying out qualitative research on adolescents living with sickle cell disease at a setting their familiar with thus, their clinic at the 37 Military Hospital to gain insights into their lived experiences, collecting extensive data on the variables in the research topic over a period of time. 3.3 Setting The study was conducted at 37 Pediatric Out Patient Department which takes care of children and adolescents with SCD. The 37 Pediatric Out-Patient Department runs seven special clinic including neuro, asthma, renal, HIV, NICU, and sickle cell clinics. This teaching hospital is the 37th built under the British colony in West Africa and situated about 4km from Accra International University of Ghana http://ugspace.ug.edu.gh 41 Airport in the Ayawaso East sub-locality in the Accra metropolis. Accra metropolis, the capital city of Ghana is an urban district and one of the five main districts in the Greater Accra region. It has a population of 1,779,165 with a population growth rate of 3.1%, the land capacity of 185km² and consists of 46.1% of the total population in the whole region making it densely populated (Ghana Statistical Service, 2010). The main ethnic groups in the district are Akans, Ga- Dangmes and Ewes with the indigenes involved in fishing and trading as the main occupation. The metropolis has a lot of schools including tertiary institutions with the premier university, University of Ghana being situated in the district. In relation to health care facilities, there are a lot of clinics, health posts, registered traditional health centers and hospitals which are either private, public or quasi-government institutions. The 37 Military Hospital, a major military- based hospital located in Ayawaso East sub-locality in the Accra metropolis, is a specialist hospital in the entire region after KorleBu Teaching Hospital. It was established in 1941 by the British Military Officer, General George Griffard to provide medical services to troops from the 2nd world war. However, in 1956, the facility was opened to the general public and since then it has been providing specialized care to the military, the general public, United Nations and other foreigners in the sub-locality (Addae, 1997). The hospital has a nursing training school that trains both civilians and military in post- basic nurse anesthesia, health assistants, general nursing and midwifery. The hospital also provides postgraduate residency programs for physicians. The hospital’s healthcare personnel are either civilian or military with a bed capacity of 400 (Duncan- Wesley, 2015). The majority of the healthcare services are provided to the general public (Addo, 2016). The hospital has set up a Sickle Cell clinic that runs every Thursday at the pediatric outpatient department. Admissions SCD patients are done from the emergency unit or the Sickle Cell clinic into the pediatric ward of the facility. University of Ghana http://ugspace.ug.edu.gh 42 3.4 Study Population The population of the study included all adolescents by WHO’s definition (10 – 19 years) attending sickle cell clinic at 37 Military Hospital. An estimated number of about 540 adolescents comprising males and females with SCD are presently accessing routine care at the 37 Military Hospital. 3.5 Inclusion Criteria All adolescents aged between 10 years and 19 years who access health care at 37 Military Hospital and who were present during the period of data collection. 3.6 Exclusion Criteria All children below the age of 10 years accessing health care at 37 Military Hospital present or not during the period of data collection including adolescents who were not willing to participate. their routine visit, were 3.7 Sample Size This study used the convenience sampling approach to select participants (male & female) between the ages of 10 years and 19 years (WHO definition of adolescent) for the qualitative study. Convenience sampling is collecting samples that are conveniently located around a location (Edgar, 2017). This approach was used because there is only one clinic day in a week. The respondents were selected as they came for their routine visit, were available and agreed voluntarily to participate in the study. In qualitative study, the sample size is guided by data saturation in which no new information is discovered, and this redundancy signals to researchers that data collection may cease (Faulkner, Stormy & Trotter, 2017). Saturation also means that a researcher can be reasonably assured that further data collection would yield similar results and University of Ghana http://ugspace.ug.edu.gh 43 serve to confirm emerging themes and conclusions. When researchers can claim that they have collected enough data to achieve their research objectives. Data collection will stop at any time when saturation is reached. In this study, saturation was reached with 25 respondents. 3.8 Quality Assurance The researcher maintains quality assurance by ensuring that the research data and reports are accurate, complete and transparent. This allows f