Quality of Life in Saudi Arabian Sickle Cell Patients: Expanded Follow-up Survey
Authors: Sewaralthahab Sarah , Malhan Hafiz Mosa , Al Zayed Abdullah Hasan , Alrumaih Ibrahim , Alotaibi Ghazi S , Alshalati Fatimah , Algahtani Farjah , Aleem Aamer , Alsaleh Khalid 
Published: December 2024

Introduction
Sickle cell disease (SCD) is a common hematological condition affecting many worldwide. SCD has a high prevalence rate in Saudi Arabia affecting 20,000 people per 1,000,000. Two distinct haplotypes exist in the Saudi Arabia resulting in phenotypic variations. In the Eastern Province, the Arab-Indian (AI) haplotype is predominant while in the Southwestern Province the Benin haplotype predominates. Limited data exist regarding the influence of SCD on the quality of life (QoL) across the country, specifically from the perspective of patients. This study was conducted to evaluate the accuracy and consistency of different QoL indicators in a heterogeneous group of individuals with SCD by examining the influence of SCD on the overall QoL.
Methods:
This is a prospective observational study; participants were SCD patients aged 18 years and older receiving care at one of three participating tertiary care centers in the central province (Riyadh), Eastern province (Qatif) and Southern province (Jazan). Patient recruitment was done in hematology outpatient clinics. Data were collected regarding QoL indicators, particularly on the participants' education, mental health, professional health, and overall health. In addition, demographic and clinical information, such as age, disease severity, gender, frequency and intensity of pain, hospital admissions, and treatments received, were also gathered.
Results:
A total of 494 patients participated in the study (Female= 230; Male= 264) with a mean age of 33.6 ± 9.2. Geographically, participants were from: Qatif (n=117; 23.68%), Riyadh (n=112; 22.67%), and Jazan (n=265; 55.64%). Overall health status was positive (excellent, very good) in 44% of patients and negative (somewhat good, OK, bad) in 56% of patients with SCD. When asked about their health compared to the previous year, 30% of participants indicated that their health was almost the same, 53% of participants indicated their health was better (somewhat better 28%, much better 25%), and 17% indicated their health was worse (13% somewhat worse, 4% much worse). About 40% of participants noted feeling exhausted, depressed or anxious. The effects of SCD on domains of activities of daily living was restrictive in all participants by more than 50%, except for showering which was at 30% Of the cohort, 40% experienced limitations due to strenuous activities, 18.6% were constrained by moderate-intensity work, and 15.6% encountered significant constraints while carrying supplies. Over 55.6% of the respondents noted that their capacity to perform tasks at work and beyond was diminished; 40% of respondents responded negatively when asked about feeling energetic. Of important note, more than 60% of participants considered their employer not to be understanding to their disease and/or limitations.
Conclusion:
In Saudi Arabia, SCD has significant effects on various aspects of the QoL in its patients. These effects are noted across the country where different SCD genotypes and phenotypes are known to be predominant. Both mental and physical aspects of life were affected substantially in patients and a large number of patients expressed lack of understanding from their employers when it comes to their disease and resulting limitations. To our knowledge, this is the largest cohort of SCD patient evaluated for QoL in Saudi Arabia with participants from 3 of the major regions in the country.
Findings from this study will be utilized to develop targeted interventions to improve employer awareness and work culture towards SCD, as well as initiate longitudinal studies to track QoL changes over time and the impact of new treatment protocols. Additionally, effectiveness of multidisciplinary care models in improving both mental and physical aspects of health for patients with SCD will be investigated.