assessment of available interventions for improving QOL

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1. ASSESSMENT OF AVAILABLE SUPPORT AND INTERVENTIONS FOR IMPROVING THE QUALITY OF LIFE OF ADOLESCENTS WITH SICKLE CELL DISEASE

2. BACKGROUND TO THE STUDY Sickle cell disease is a genetic blood disorder causing anaemia, pain crises, and organ damage (CDC, 2025b). UNICEF reports high childhood mortality in sub-Saharan Africa due to limited access to care. Globally, sickle cell disease cases rose to 7.74 million in 2021 with 515,000 neonates diagnosed (“Global, Regional, and National Prevalence and Mortality Burden of Sickle Cell Disease, 2000–2021,” 2023). Africa bears 75% of the burden with Nigeria reporting the highest cases (Ally & Balandya , 2023). In Kenya, sickle cell disease is a major public health concern with 12,000 births annually especially in Kilifi county ( Uyoga et al., 2019).

3. BACKGROUND TO THE STUDY… Adolescence is a period of transition between childhood to adulthood encompassing ages 10 to 19. The period is characterized by intellectual, emotional, psychological, social, and physical development ( Reavley et al., 2017) . Adolescence with sickle cell disease often encounters developmental challenges giving them a low pace development unlike their peers (Hoyt et al., 2022). Adolescents with sickle cell disease often face challenges which require comprehensive support services and some are vital for transitioning to adult care.

4. STATEMENT OF THE PROBLEM According to World Health Organisation, the ideal situation for sickle cell disease involves a multifaceted intervention that encompasses comprehensive, multidisciplinary and patient-centred approach to enhance quality of life of adolescents with sickle cell disease. Interventions include early diagnosis through newborn screening programs, access to essential medications like hydroxyurea , regular follow up in specialised clinic, availability of blood transfusion and born marrow transplant therapies. A multidisciplinary team involving doctors, nurses, haematologist, social workers, patient educators and psychologist that ensures continuity during the transition to adulthood (World Health Organization, 2025)

5. STATEMENT OF THE PROBLEM… The adolescent with sickle cell disease face significant challenges globally as the real-world situation contrast with the ideal standards set by World Health Organization. In Sub Saharan Africa sickle cell disease contributes to early childhood mortality with an estimate of 50-90% of affected children die before their fifth birthday (CDC, 2025b). Interventions aimed at improving health outcomes through neonatal screening and comprehensive care programmes still remain limited and unavailable in some healthcare settings In Kenya, the burden of sickle cell disease is substantial with the high prevalence from western and coastal region where Kilifi County lies

6. RESEARCH OBJECTIVES The main aim of the study is to assess the available supports and interventions for improving the quality of life of adolescents with sickle cell disease in Kilifi County. Specific objectives are to:

7. RESEARCH QUESTIONS What are the currently available supports and interventions aimed at improving the quality of life of adolescents with sickle cell disease in Kilifi County? What proportion of adolescents with sickle cell disease in Kilifi County have benefited from the available support and intervention services ? How do existing support systems and interventions impact the quality of life of adolescents with sickle cell disease in Kilifi County? What key challenges and barriers hinder adolescents with sickle cell disease from accessing interventions and support services in Kilifi County? What are the lived experiences of adolescents with Sickle Cell Disease (SCD) in Kilifi County, particularly concerning the impact of SCD on their daily lives and needs for support beyond what is currently available?

8. HYPOTHESIS There is no significant relationship between the utilization of available support and intervention services and the quality of life of adolescents with sickle cell disease in Kilifi County.

9. SIGNIFICANCE OF THE STUDY Adolescents Researchers Healthcare providers Policymakers Community organizations General public NGOs

10. SCOPE AND DELIMITATION The scope of this study is centered on assessing available interventions and support on the quality of life of adults with Sickle Cell Disease in Kilifi County, which is geographically defined by Kilifi County, Kenya. The primary population under investigation comprises adolescent aged 1 2 and 19 year s who have a confirmed diagnosis of sickle cell disease . The core focus remains on these adolescent residing within Kilifi county

11. OPERATIONAL DEFINITIONS Adolescent Anaemia Haemoglobin I nterventions Sickle cell disease Support Quality of Life

12. LITERATURE REVIEW Search strategy Conceptual review Empirical review Theoretical framework Application to the study Summary

13. PHILOSOPHY: PRAGMATISM The research philosophy guiding this study is primarily pragmatism, which allows for a flexible and practical integration of positivist (quantitative) and interpretivist (qualitative) approaches. This is appropriate for assessing both measurable outcomes and lived experiences related to support and interventions for adolescents with sickle cell disease (Shan, 2023).

14. RESEARCH DESIGN This study will utilize a cross-sectional mixed-methods approach, combining both quantitative and qualitative data to comprehensively address its objectives: quantitative methods are used to map available support services, proportion of adolescents benefitting from the services, and assess perceived impacts, while qualitative methods explore adolescents’ lived experiences and barriers to accessing care (R et al., 2025). A parallel convergent design facilitates the simultaneous collection of both data types, enabling their integration during the interpretation stage to maximize the breadth and depth of understanding ( Abdulshakoor , 2023)

15. SETTING Kilifi County, situated on Kenya's coast and covering 12,246 km² with a population of approximately 1.44 million (2019 census), features a predominantly rural setting where adolescents (10–19 years) make up about 22% of inhabitants ( Ssewanyana et al., 2021). The region is marked by high poverty (58–67% live below the poverty line), limited education, and elevated adolescent pregnancy and school dropout rates ( Ssewanyana et al., 2018).

16. TARGET POPULATION: QUANTITATIVE Adolescents aged 10-19 years living with sickle cell disease in Kilifi County, parents or caregivers for younger adolescents (aged 10–14 years) or those who may have difficulty expressing their experiences independently Inclusion criteria Adolescents with confirmed clinical diagnosis of SCD Received outpatient/inpatient care for SCD within the past 12 months Exclusion criteria Unverified diagnosis of SCD Critically ill/currently hospitalized cases Severe cognitive impairment

17. TARGET POPULATION: QUALITATIVE Inclusion criteria Adolescents with confirmed clinical diagnosis of SCD Caregivers who have provided support to adolescents with SCD within the past 12months Coordinator of KEMRI outpatient SCD clinic Exclusion criteria Severe cognitive or emotional challenges that would hinder meaningful engagement. Critically ill Caregivers not actively involved in care of SCD patients within the past 12 months

18. SAMPLE: QUANTITATIVE The study will use Cochran’s formula below: (Cochran & Cochran, 1977) n= Where: n = sample size Z = Z-value 1.96 for 95% confidence level p = estimated prevalence = 0.008 (0.8%) ( Uyoga et al., 2019) d = desired margin of error (precision), typically 0.02 (2%) due to the low prevalence (~0.8%) and need for higher accuracy   =76.32=77 Then add 10% for non-response=77+(0.1*77) =77+7.7=84.7=85 n= 85 participants  

19. SAMPLE: QUALITATIVE The sample size will be determined using the principle of data saturation, which occurs when no new themes, categories, or insights emerge from additional in-depth interviews . Based on existing literatures, the saturation can be reached after 30-70 interviews (Squire et al., 2024).

20. SAMPLING TECHNIQUE Quantitative: Purposive sampling In the qualitative phase , purposive sampling w ill used to select adolescents with sickle cell disease who c an provide rich, detailed insights into their lived experiences, perceptions of support services, and challenges encountered in accessing the services . Parents will be recruited concurrently with the adolescents during routine clinic visits, after obtaining informed consent from both parties. Sampling will continue until data saturation is achieved .

21. INSTRUMENT Quantitative: First, a researcher-developed checklist and secondly a structured questionnaire Qualitative: Semi-structured interview

22. VALIDITY OF THE INSTRUMENTS Content v alidity of the study instruments is ensured by research supervisor and expert review where necessary adjustments will be made based on their comments and recommendations. Th e semi-structured interview guide achieves validity through pilot testing. Face validity is enhanced by clear, participant-friendly language.

23. RELIABILITY OF THE INSTRUMENTS Reliability is ensured through the use of validated tools with proven internal consistency, such as the PedsQL ™ (α = 0.74–0.90) and BCQ (α = 0.95). The structured questionnaire and checklist will be pilot-tested for consistency, while the interviews follow standard procedures to ensure dependability and data trustworthiness.

24. DATA COLLECTION Quantitative data will be collected by a trained research assistant using a structured questionnaire and checklist over a period of three months Qualitative data will be collected through in-depth interviews with purposively selected adolescents living with sickle cell disease and, where appropriate, their caregivers.

25. DATA ANALYSIS Quantitative data: SPSS Descriptive statistics such as frequencies, percentages, means, and standard deviations will be used to summarize demographic characteristics, service utilization, and availability of support and intervention services . Inferential statistics such as Chi-square tests will examine associations between the use of support services and perceived quality of life. A significance level of p < 0.05 will be used to determine statistical significance. Qualitative data from in-depth interviews will be transcribed and analyzed using thematic analysis . Transcripts will be coded inductively using NVivo software, and emergent themes will be identified through reading, coding, clustering of codes, and interpretation.

26. DATA INTEGRATION

27. ETHICAL CONSIDERATION Introductory letter Informed consent Confidentiality and anonymity

28. REFERENCE Abdulshakoor , E. M. (2023). SICKLE CELL DISEASE SELF-MANAGEMENT AMONG LATE ADOLESCENTS AND THEIR FAMILIES IN SAUDI ARABIA: A MIXED-METHOD STUDY [University of Rhode Island]. https://hdl.handle.net/20.500.14154/71402 Ally, M., & Balandya , E. (2023). Current challenges and new approaches to implementing optimal management of sickle cell disease in sub-Saharan Africa. Seminars in Hematology , 60 (4), 192–199. https://doi.org/10.1053/j.seminhematol.2023.08.002 Uyoga , S., Macharia , A. W., Mochamah , G., Ndila , C. M., Nyutu , G., Makale , J., Tendwa , M., Nyatichi , E., Ojal , J., Otiende , M., Shebe , M., Awuondo , K. O., Mturi , N., Peshu , N., Tsofa , B., Maitland, K., Scott, J. A. G., & Williams, T. N. (2019). The epidemiology of sickle cell disease in children recruited in infancy in Kilifi , Kenya: A prospective cohort study. The Lancet. Global Health , 7 (10), e1458–e1466.