Central sensitization (CS) has been identified as a leading culprit responsible for maintaining pain in several chronic pain conditions. It may explain differences in the symptom experience of individuals with Sickle Cell Disease (SCD). Quantitative sensory testing (QST) can be used to examine CS and identify individuals that may have a heightened CS profile. The present study identified and categorized SCD patients based on QST into a high or low CS phenotype and examined clinical pain, vaso-occlusive crises, psychosocial factors, and sleep continuity (i.e. sleep duration, sleep fragmentation, and sleep efficiency) differences between groups. Thirty-four (34) adults with SCD who completed daily morning (sleep) and evening (pain) diaries over a three-month period and underwent QST were included in these analyses. Participants were divided into CS groups (i.e. No/Low CS vs. High CS), based on thermal and mechanical temporal summation and after-sensations, which were norm-referenced to healthy, matched controls. Sixteen participants exhibited No or Low CS while 18 exhibited High CS. Participants in the High CS group were significantly older (mean age 43.5 years vs. 33.9 years, p = 0.02), but there was no significant difference in sex between groups. During the average of 74 days of diary monitoring, participants in the High CS group on average experienced significantly higher clinical pain (mean difference = 24.0/100 points), more vaso-occlusive crises (mean difference = +15%), more catastrophizing, greater negative mood, and poorer sleep continuity (p’s <0.05), however, there was no difference in sleep duration between groups (p = 0.78).We demonstrated individual differences in central pain processing among individuals with SCD. In general, greater central sensitization was associated with higher clinical pain, more crises, worse sleep, and more psychosocial disturbances. Future analyses should investigate whether sleep and psychosocial disturbances mediate the relationship between central sensitization and pain outcomes. Abstract source

The review of sickle cell disease by Piel et al. (April 20 issue) is timely and highlights the need to address the lack of research about this disease in sub-Saharan Africa. The authors rightly state that in the past two decades, childhood mortality has been reduced in sub-Saharan Africa, but the survival data cited by Piel et al. were derived from a single-site study performed almost four decades ago. Two-year follow-up data from a pilot cohort study in Nigeria show that survival among children with sickle cell disease remains poor in sub-Saharan Africa. There are no conclusive data to support the use of chemoprevention in addition to insecticide-treated bed nets for prophylaxis against malaria in patients with sickle cell disease. With regard to Figure 3 in the review by Piel et al., multiple data suggest that the Cameroon haplotype of the β-globin gene (HBB) is associated with a more severe phenotype than the Benin haplotype; thus, in the figure, the Cameroon haplotype should have been to the right of the Benin haplotype. In addition to fetal hemoglobin (HbF)-promoting loci and the coinheritance of α-thalassemia that are established genetic modifiers of sickle cell disease, data also provide support for genetic risk markers of renal dysfunction in APOL1 and HMOX15 and of cholestasis in UGT1A1. Abstract source Full text

OBJECTIVE: To identify characteristics of pediatric sickle cell disease (SCD) hospitalizations and to examine admission demographics and medical expenditures. METHODS: Admissions with SCD were identified from the 2009 and 2012 releases of the Healthcare and Cost Utilization Project's Kids Inpatient Database. Disease-specific secondary diagnoses including acute chest syndrome (ACS), vaso-occlusive pain crisis (VOC), splenic sequestration, and stroke/transient ischemic attack were analyzed for patient and hospital demographics. Analytical endpoints included total healthcare expenditures and mortality. RESULTS: We reviewed 75,234 inpatient hospitalizations with a diagnosis of SCD. Over $900,000,000 was spent annually in associated healthcare expenditure. The median length of hospitalization stay (LOS) for all admissions was 3 days (interquartile range [IQR] 2-5 days). VOC was the most frequent secondary diagnosis, recording 48,698 total hospitalizations and a median LOS of 3 days (IQR 2-6 days). Of the 8,490 hospitalizations with ACS, the infant population had a significantly higher mortality rate compared to other age groups (2% vs. 0.3%, P < 0.001). Cerebral vascular accidents incurred the second highest median hospitalization cost ($18,956), behind ACS ($22,631). A high proportion of Caucasian patients died during hospitalization for VOC (0.4% vs. 0.1%, P = 0.014) and ACS (4% vs. 0.2%, P < 0.001) when compared to non-Caucasians. CONCLUSION: Inpatient hospitalizations for secondary manifestations of pediatric SCD were associated with significant healthcare expenditures. Patients with an increased statistical risk for death during hospitalization included Caucasians with SCD complications of ACS and VOC, and patients <1-year-old with ACS. Further research is needed to substantiate the associated clinical significance of these findings. Abstract source Full text

Albuminuria is considered to be a relevant biomarker for the detection of early glomerular damage in patients with sickle cell disease (SCD). Improvements in our understanding of the pathophysiological processes and molecular mechanisms underlying albuminuria are required, because increasing numbers of patients with SCD are developing chronic kidney disease. The early recognition of sickle cell nephropathy (SCN) and studies of the natural course of this emerging renal disease are therefore crucial, together with identification of the associated clinical and biological risk factors, to make it possible to initiate kidney-protective therapy at early stages of renal impairment. The pathophysiological process underlying SCN remains hypothetical, but chronic haemolysis-related endothelial dysfunction and the relative renal hypoxia triggered by repeated vaso-occlusive crises have been identified as two potential key factors. The optimal preventive and curative management of albuminuria in the context of SCD is yet to be established, but recent studies have suggested that hydroxyurea therapy, the cornerstone of SCD treatment, could play a key role in reducing albuminuria. The place of conventional kidney-protecting measures, such as renin-angiotensin system inhibitors, in the treatment of SCD patients also remains to be determined. Abstract source Full text Full text (PMC)

Organisation for Sickle Cell Anaemia Relief & Thalassaemia Support (OSCAR Birmingham) is a voluntary organisation and registered charity established in 1974 primarily to promote greater awareness of sickle cell and thalassaemia disorders (SCD’s). OSCAR Birmingham aims to support individuals affected by sickle cell and thalassaemia disorders who may be socially disadvantaged due to social exclusion or lack of support. The Mission of OSCAR Birmingham is to educate, support and provide relevant information through sign posting and advocacy to those with Sickle Cell and Thalassaemia disorders, their families and carers. Aims and Objectives: • To reduce social isolation through the provision of opportunities for social interaction of clients and their families. • To educate clients, their families and carers to help manage their conditions more effectively and to maximise their quality of life, i.e. to help people “live as positively as possible” with these conditions. • To advocate: on behalf of individuals and their families. • To raise awareness, education, publicity and campaign on matters relating to Sickle Cell and Thalassaemia and Disorders associated with these conditions i.e. Stroke, Diabetes etc. Official website

The Diggs-Kraus Sickle Cell Center at Regional One Health has cared for more than 51,000 sickle cell patients. Medical procedures developed here have been adopted nationwide. The mission of the Diggs-Kraus Sickle Cell Center (SCC) is to provide primary outpatient medical care to SCD patients in a holistic way that is sensitive to the pressures that this catastrophic disease brings to bear on patients and their families. The center offers regular preventative and maintenance care which includes routine lab work at least twice a year, an annual eye exam, immunizations, and general health and nutritional information. Each program is designed around the individual patient and may include specialists from several areas. With the multi-disciplinary approach, fewer patients face medical crisis. The process is designed to get patients back on their feet and to prevent as many crises as possible Official website

The III & Long Foundation was founded by Santonio Holmes in the fall of 2011. The mission of the III & Long Foundation is to raise awareness to help fight Sickle Cell Disease (SCD) and provide financial support and treatment options for families affected by the disease. The III & Long Foundation works in partnership with local SCD organizations to provide grants that enable families to receive proper treatment and education. The foundation is dedicated to building awareness about the disease through programs, merchandise, and fundraising events. These endeavors support families and raise funding for research on SCD. III & Long Foundation produces special programs and educational services that unite and support the SCD community, including: • Tutoring services for children during hospital treatment • Educational seminars for parents • Social media awareness campaign • Thanksgiving and Christmas holiday community events • Annual Strikes for Sickle Cell Bowling event Official website

East Carolina University Comprehensive Sickle Cell Center is one of the largest centers in the United States. Through decades of participation in research and education opportunities, the center has contributed immensely to better understanding and advancing treatment of this condition. East Carolina University Comprehensive Sickle Cell Center’s patients and their families are the center’s backbone; their strength, resilience, optimism, and grace teach important life lessons every day. Individuals living with sickle cell disease are more than their diagnosis. They have hopes, dreams and experience daily joys and challenges. They aspire to excel in school, sports, and in their careers. The goal of this center is to be part of these daily and lifelong journeys while promoting empowerment and self-advocacy until a definitive cure is discovered. Official website

Embrace Kids Foundation was founded in 1991 with a vision to raise funds for children with cancer and blood disorders. Embrace Kids is a tight community comprised of patient families, donors, volunteers and students who all share similar values and support each other. It exists to lighten the burden, maintain normalcy and improve the quality of life for families whose children are facing cancer, sickle cell and other serious health challenges. Embrace Kids addresses the spiritual, emotional and financial needs of patient families in the New Jersey/New York City metropolitan area. A partial list of the programs and services offered by the foundation includes the Embrace Kids Learning Center, Emergency Financial Assistance, Tackle Sickle Cell, Family Services, Inspirational Events, Social Services and Counseling. Official website

Sickle Cell Warriors of Wisconsin is a volunteer based, non-profit agency providing educational programs and community outreach for individuals and families in the greater Milwaukee metropolitan area. It forms partnership with local hospitals and social service agencies. Goals: • Advocacy: Commitment for elevating the quality of life of those impacted by sickle cell disease, while also creating a platform for their voices to be heard. • Educate: To educate the community in the areas of signs and symptoms, treatment, disease management, research, and resources. • Support: To look to provide those impacted by sickle cell disease with a support system and resources to aid in getting through difficult times. Mission: • Raise awareness: to bring sickle cell awareness to the world stage. • Support families: to supply much needed resources for families in need during crisis. • Guide to resources: to provide necessary resources all in one place. Official website