Poor Sleep Quality in Jamaican Adults With Sickle Cell Disease: Prevalence, Risk Factors, and Association With Quality of Life.

Poor sleep and chronic illnesses have a bidirectional relationship where presence of one can worsen the other. Sickle cell disease (SCD) is associated with significant morbidity and early mortality. In this study, we examine sleep quality, its predictors, and its association with quality of life in Jamaican adults with SCD. This cross-sectional study evaluated 177 well adult SCD patients for sleep quality using The Pittsburgh Sleep Quality Index (PSQI) and quality of life using the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me). Multiple linear regression models examined the predictors of poor sleep quality. The mean global PSQI score was 6.9 (SD 4.2) with 56.5% having poor sleep quality. Women had significantly worse scores for sleep efficiency (p 0.005), sleep latency (p 0.03) and higher use of sleeping medications (p 0.02). Those overweight/obese had significantly worse subjective sleep quality (p 0.001) and sleep efficiency (p 0.05). In multivariate regression analysis, overweight individuals had poorer sleep quality (OR: 2.9; 95% C.I.: 1.07, 7.88) than those with normal weight whereas those unemployed and looking for a job had lower prevalence of poor sleep quality (OR 0.2; 95% C.I.: 0.05, 0.77) compared to employed individuals. Participants with good sleep quality had significantly better functioning in all 5 domains of the ASCQ-Me. In conclusion, persons with SCD who are overweight or obese are at increased risk of poor sleep which can negatively affect quality of life. Patient populations and healthcare providers will need to manage the emerging burden of overweight/obesity.

Telehealth: Navigating the COVID-19 Pandemic and Beyond: The Sickle Cell Unit Experience.

Introduction: To examine the use of telehealth for delivery of health care in persons with sickle cell disease in a resource-constrained country during the COVID-19 pandemic. Methods: This study was a retrospective review of patient encounters at the Sickle Cell Unit (SCU), Jamaica during a 3-year period, March 10, 2019 to March 9, 2022 and a comparison of endpoints between 1 year before and 2 years during the pandemic. Primary endpoints of registration numbers, day-care admissions, and study visits were obtained from logbooks and the electronic medical records. Additional endpoints included well visits, hydroxyurea (HU) visits, and bone pain crisis. Results: Patients registered at the clinic on 17,295 occasions, with 7,820 in the pre-pandemic year decreasing by 43.8% and 35% in the 2 subsequent pandemic years. Overall, study visits increased by 4.9% and 1.3% in the pandemic years. They increased in adults by 13.1% and 8.9% but fell by 3.2% and 6.2% in children. Fewer people were seen in the pandemic years, with children showing a 20.7% decline in numbers. Tele-visits accounted for 31.4% of all study visits during the pandemic years and increased by 23.6% between the pandemic years. There were more well-visits and HU visits, but fewer pain visits and day-care admissions in the pandemic years. Conclusions: The SCU maintained health care delivery for a high-risk population during the pandemic, with tele-visits mitigating the short-fall from in-person visits. Tele-visits may be more acceptable to adults with a chronic illness and may be a suitable alternative for delivering health care.

Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth.

Globally, the majority of persons born with sickle cell disease do not have access to hydroxyurea or more expensive interventions. The objectives were to estimate the survival in homozygous sickle cell disease, unbiased by symptomatic selection and to ascertain the causes of death in a pre-hydroxyurea population. The utility of early life biomarkers and genetically determined phenotypes to predict survival was assessed. A cohort study based on neonatal diagnosis was undertaken at the Sickle Cell Unit, a specialist clinic delivering care to persons with sickle cell disease in Jamaica. Screening of 100,000 deliveries detected 315 babies with homozygous sickle cell disease of whom 311 have been followed from birth for periods up to 43 years. Pneumococcal prophylaxis and teaching mothers splenic palpation were important, inexpensive interventions. Anticipatory guidance, routine care and out-patient acute care were provided. Each participant was classified as alive, dead, or defaulted (usually emigration). Causes of death were ascertained from clinical records and/or post-mortem reports. Survival was assessed using the Kaplan-Meier function. Sex-adjusted Cox semi-parametric proportional hazards and Weibull modelling were used to assess the effects on survival of biomarkers. Survival to 40 years was 55.5% (95% CI 48.7% to 61.7%). Acute Chest Syndrome (n = 31) and septicemia (n = 14) were significant causes of death at all ages. Acute splenic sequestration (n = 12) was the most common cause of early deaths. Survival was significantly shorter in those with lower hemoglobin at 1 year, high total nucleated count at 1 year, and a history of dactylitis ever. In these hydroxyurea naïve patients, survival into midlife was common. Causes of death were often age specific and some may be preventable. Early life biomarkers predictive of decreased survival in SS disease identify a patient group likely to benefit from close clinical supervision and potentially high risk therapies.