Hydroxyurea (HU) has proven efficacious for treating sickle cell disease (SCD) and is recommended for most patients. However, HU is underutilized and reasons for lack of uptake are unclear. Past qualitative studies to identify barriers have been small and limited to clinical settings. Thus, to better understand barriers to HU there is a need to explore perceptions of patients and families from the broader community. We conducted a qualitative study of Facebook messages posted in an online sickle cell support group to identify barriers and facilitators to hydroxyurea use. We collected a total of 22 223 user‐generated original messages and comments posted in the SCD Unite group between January 2014 and November 2015. From these messages, we identified those containing any of the following terms: “Hydrea,” “Droxia,” “Hydroxycarbamide,” “Hydroxurea,” “Hydroxy,” “HU,” “Urea,” and “hydro*,” along with the comments associated with these posts. All nontext based posts (ie, pictures, videos, and links) were excluded. The resultant data set included 145 total original messages and 2618 associated comments. We performed content coding and analysis on all 145 original message posts followed by thematic analysis of a select subset of discussion threads, which were defined as a single original message plus their associated comments. Abstract source Full text

Purpose: To provide a focused review of sickle cell retinopathy in the light of recent advances in the pathogenesis, multimodal retinal imaging, management of the condition, and migration trends, which may lead to increased prevalence of the condition in the Western world. Methods: Non-systematic focused literature review. Results: Sickle retinopathy results from aggregation of abnormal hemoglobin in the red blood cells in the retinal microcirculation, leading to reduced deformability of the red blood cells, stagnant blood flow in the retinal precapillary arterioles, thrombosis, and ischemia. This may be precipitated by hypoxia, acidosis, and hyperosmolarity. Sickle retinopathy may result in sight threatening complications, such as paracentral middle maculopathy or sequelae of proliferative retinopathy, such as vitreous hemorrhage and retinal detachment. New imaging modalities, such as wide-field imaging and optical coherence tomography angiography, have revealed the microstructural features of sickle retinopathy, enabling earlier diagnosis. The vascular growth factor ANGPTL-4 has recently been identified as a potential mediator of progression to proliferative retinopathy and may represent a possible therapeutic target. Laser therapy should be considered for proliferative retinopathy in order to prevent visual loss; however, the evidence is not very strong. With recent development of wide-field imaging, targeted laser to ischemic retina may prove to be beneficial. Exact control of intraoperative intraocular pressure, including valved trocar vitrectomy systems, may improve the outcomes of vitreoretinal surgery for complications, such as vitreous hemorrhage and retinal detachment. Stem cell transplantation and gene therapy are potentially curative treatments, which may prevent retinopathy. Conclusions: There is lack of evidence regarding the optimal management of sickle retinopathy. Further study is needed to determine if recent progress in the understanding of the pathophysiology and diagnosis of sickle retinopathy may translate into improved management and outcome. Abstract source Full text

Children with sickle cell anemia (SCA) often exhibit nutritional deficiencies and are at high risk of dying before the age of 5 years. Ensuring adequate nutrition is a critical part of health care for such children. This study aimed to investigate the association between nutritional status, nutrient intake, and food diversity in children with SCA. A descriptive cross-sectional study was conducted on 74 children with SCA, between 24 and 71 months of age. Anthropometric measurements, food and nutrients consumption were determined. The prevalence of low weight, stunting, and overweight/obesity were 16.2%, 35.1%, and 16.2%, respectively. Mean folic acid intake was low (49.05%±51.22%), whereas the intakes of protein (426.71%±171.93%), retinol (292.97%±403.88%), phosphorus (204.55%±151.35%), magnesium (233.02%±151.14%), iron (250.76%±165.81%), and zinc (243.21%±148.40%) were high. The dietary phosphorus/protein ratio was high for 31.1% of the children, and 44.6% of the children had low dietary diversity score. No correlation was found between food diversity, nutrient adequacy, and nutritional status. Despite the adequacy of the intake of most micronutrients, diet quality was inadequate, constituting mainly ultraprocessed foods. Knowing the food consumption pattern of these children enables a more resolute nutritional intervention. Abstract source

Background: The transition from the pediatric setting to adult care is a well-described period of morbidity and mortality for persons with sickle cell disease (SCD). We sought to measure the feasibility and effectiveness of providing skill-based educational handouts on improving self-management and transition readiness in adolescents with SCD. Methods: This was a single-center study in which participants completed a self-assessment, the Adolescent Autonomy Checklist (AAC), to assess transition readiness and self-management skills at baseline. After results were reviewed by the study coordinator, participants were provided with skill-based handouts on noted areas of deficit. The AAC was subsequently completed at a follow-up visit. All data were stored electronically and transferred into SAS for statistical analyses. Results: Sixty-one patients completed the AAC at baseline and postintervention. At baseline, patients reported needing the most help with skills in money management, living arrangements, vocational skills, and emergency and healthcare skills. Postintervention, statistically significant improvements (P < 0.05) occurred in skills related to laundry, housekeeping, healthcare, and sexual development. A regression model exploring the time to follow-up showed that most improvements could not be attributed to maturation alone. Conclusion: This study showed that educational handouts are a readily implementable and well-accepted intervention among adolescents with SCD who identify challenges with skills necessary to successfully transition to adult care. Distinguishing which transition needs are best improved with this type of intervention will help to strengthen the multidisciplinary approach necessary to support adolescents and young adults with SCD as they matriculate to adult care. Abstract source

Benefits of identifying sickle cell disease (SCD) carriers include detection of at-risk couples who may be informed on reproductive choices. Studies consistently report insufficient knowledge about the genetic inheritance pattern of SCD among people with sickle cell trait (SCT). This study explored perspectives of adults with SCT on the information needed to make an informed reproductive decision and the recommendations for communicating SCT information. Five focus groups (N = 25) were conducted with African Americans with SCT ages 18-65 years old. Participants were asked about their knowledge of SCT, methods for finding information on SCT, impact of SCT on daily living, and interactions with healthcare providers. An inductive-deductive qualitative analysis was used to analyze the data for emerging themes. Four themes emerged, highlighting the unmet information needs of African American sickle cell carriers: (a) SCT and SCD Education; (b) information sources; (c) improved communication about SCT and SCD; and (d) increased screening strategies. Future studies are needed to determine effective strategies for communicating SCT information and to identify opportunities for education within community and medical settings. Identifying strategies to facilitate access to SCT resources and education could serve as a model for meeting unmet information needs for carriers of other genetic conditions. Abstract source

Objectives: Perceived stress is associated with sickle cell disease (SCD) pain; however, little is known about psychological mechanisms that may clarify this link among adult patients. This study explored whether anxiety and depression symptoms explained the relation between perceived stress and SCD pain episode frequency among 70 African-American adults (51.4% women, mean age 35.6 years). Methods: Participants completed measures of perceived stress, pain, and psychological symptoms in an outpatient clinical setting. Results: A serial multiple mediation model showed that psychological symptoms collectively reduced the association between perceived stress and SCD pain frequency (b = 0.116, P = 0.141). However, only the indirect effect of stress on pain frequency through anxiety symptoms was significant (b = 0.089). Conclusions: Anxiety but not depression symptoms best explain the association between stress and SCD pain. Further research is needed to identify the specific components of negative affect that drive the experience of SCD pain. Abstract source

Background: Sickle cell disease (SCD) is an inherited blood disorder associated with acute pain crisis and other complications that lead to frequent emergency department (ED) visits. To improve outcomes, the National Heart, Lung and Blood Institute (NHLBI) published recommendations for management of acute pain crisis. NHLBI also funded eight centers across the United States to participate in the Sickle Cell Disease Implementation Consortium. This six-year effort consists of two phases. Phase one involved conducting needs assessments of barriers and facilitators to SCD care. The aim of this study was to describe challenges and facilitators to caring for SCD from the perspective of ED providers in central North Carolina (NC). Methods and Findings: We conducted a needs assessment survey with ED providers throughout NC. We also conducted focus groups and an interview with ED providers from three healthcare facilities in central NC. One hundred and eleven surveys (60.6% physicians, 26% registered nurses, 13.5% physician assistants) were completed and 13 providers participated in 3 focus groups and 1 interview. Slightly more than half (50. 4%) utilized individualized dosing protocols to treat sickle cell pain. Only 32.4% of the providers were aware of the NHLBI SCD recommendations. Barriers to care from the survey included: patient behavior (67.57%), the opioid epidemic (67.57%), overcrowding (64.86%), and concern about addiction (49.55%). Perceived barriers to care identified in the focus groups and interview included: high patient volumes, lack of SCD care protocols, poor communication among providers and stigma. Facilitators to care included: individualized pain plans, comfort prescribing opioids and electronic medical records. Conclusion: SCD care is influenced by many factors. Our results illuminate the need for increased use of the NHLBI SCD recommendations, individualized pain protocols, and use of electronic medical records and other care-interventions, specifically geared towards improving provider knowledge and mitigating provider bias. Abstract source Full text

Sickle cell disease has been largely an invisible global health issue, especially in regions of high incidence mainly due to lack of awareness among both the local health policy makers and the public. Public health interventions, such as screening of newborns, provision of prophylaxis against bacterial infections, and immunizations against pneumococcal infections can have the greatest impact. Family education on assessment of spleen size and subsequent detection of splenic sequestration and promptness to seek medical attention for a febrile child is also important in the control of the morbidity and mortality of children with SCD living in resource-poor countries. In addition to these affordable interventions, hydroxyurea therapy is necessary to decrease both the acute and chronic complications of sickle cell anemia. Sickle cell disease has been recognized to have global health significance by key institutions including the World Health Organization in 2006 and the United Nation is 2008. In 2010, the WHO released national health care management goals and set targets to be achieved by the countries in sub-Saharan Africa for the control and management of SCD. These are yet to be translated into action. To do, this would require active and sustainable public-private partnerships for sustainable program development in these regions. Effective interventions should be integrated into existing health systems, the best examples linking primary healthcare facilities to specialized sickle cell disease centers in regional and tertiary healthcare institutions. Abstract source Full text

Sickle cell anemia is a heritable disease caused by a mutation in the gene that provides instructions for the production of hemoglobin. Hemoglobin is a protein found in red blood cells that carries oxygen from the lungs to tissues and organs. The sickle cell mutation causes the red blood cells to deform into a sickle shape. These deformed blood cells cannot travel through blood vessels as well as normal cells and are also more likely to form clots. Eye Disease in Sickle Cell Anemia Eye disease in sickle cell anemia patients can be divided broadly into two conditions: anterior eye disease and posterior eye disease. Anterior eye disease involves the chamber of the eye, while posterior eye disease involves the retina, optic nerve, and the subretinal tissues, the system of blood vessels and cells that provide nutrients to the retina. Current research indicates that early problems can be seen in the eye prior to vision problems. A dilated eye test can be used to determine whether these issues are present and evaluate if treatments are working to reduce or prevent vision problems.   Abstract source

Sickle cell disease is a disorder that causes red blood cells to become sickled (banana-shaped), as well as sticky and rigid. These sickle cells can block blood flow in small blood vessels of the body. The hemoglobin in blood cells carries oxygen to all areas of the body. Sickle cells can block the small blood vessels in the eye depriving the eye of oxygen and causing damage. This is called sickle retinopathy (SR). What are Some of the Symptoms of SR? Retinopathy (re ti NAH poh thee) is a disorder that can continue to get worse. It can occur in one or both eyes. Early damage does not usually affect the person’s ability to see. Retinopathy can go undetected for years without an eye exam by a trained eye doctor (ophthalmologist). The eye doctors in the St. Jude Eye Clinic have been treating people with sickle cell disease for many years, and they are familiar with SR. Sickle retinopathy can progress to severe proliferative sickle cell retinopathy (PSR). PSR can cause bleeding into the eye or detachment of the retina. It can lead to vision changes and, in rare cases, blindness.   Abstract source