The Sickle Cell Trait Awareness Campaign is a five-year effort launched this month by Cayenne Wellness Center, which supports people living with sickle cell disease in California. It aims to provide education and encourage success. It helps in learning about your genetics and understanding how they are incorporated into your everyday life, which is a major step in achieving personal success. They want to provide education about Sickle Cell Trait (SCT) for those affected by it and give resources that will do just that. Related content

Introduction: Vaso-occlusive pain episodes (VOE) are the leading cause of emergency department (ED) visits & hospitalization in patients with sickle cell disease (SCD). During SCD-VOE, patients develop an acute arginine (Arg) deficiency. Supplementation with Arg has shown opioid-sparing effects, improves blood pressure & cardiopulmonary function & decreases length of hospital stay. Though we have previously demonstrated that intravenous (IV) Arg improves mitochondrial (mito) function, the precise mechanisms by which Arg mediates these positive effects remain unclear. Methods: Prospective single-center double-blind randomized controlled trial (RCT) of IV Arg (TID, up to 7 days) in children with SCD age 3-21 years hospitalized for VOE requiring IV opioids. Patients with significant liver/renal dysfunction or those previously enrolled were excluded. Subjects were randomized into 1 of 3 arms: 1) 100 mg/kg/dose Arg, standard dose (SD), 2) loading dose: 200 mg/kg followed by SD or 3) placebo. Demographics, total parenteral opioid (TPO) use (morphine equivalents, mg/kg) time to crisis resolution (time of study drug delivery to last IV opioid in hours), pain scores, patient reported outcomes (PROMIS) & mito function were obtained before treatment & at discharge. The primary outcome measure was TPO use between study arms. Hypothesis tests & unadjusted & covariate-adjusted fixed & mixed effects generalized linear regression models were used, as appropriate, to compare cross-sectional & longitudinal outcomes within & between randomization arms. This protocol utilized IND#66943 (Sponsor-Morris), registered with ClinicalTrials.gov (NCT02536170). Results: 1,548 patients were screened, 266 were eligible, 114 consented, & 108 were randomized. Safety results of this RCT have been previously reported (Reyes et al, Am J Hematol 2022). Demographics & clinical outcomes of subjects randomized by treatment arm are provided in Table 1. While statistically insignificant, there was a clinically relevant decrease in TPO & time to crisis resolution in both Arg arms compared to placebo. The placebo group required 45% higher TPO & experienced >15 hours longer mean time to crisis resolution compared to combined Arg-treated groups when adjusted for HU use, continuous age and sex. Among children <17 years, the placebo group (n=33) required 80% more TPO compared to combined Arg groups (n=57; p=0.075). No differences in ED vs discharge pain scores or patient/parent PROMIS reports across arms were found. Mito Complex V activity was higher (p=0.02) & protein carbonyl levels were lower (p=0.003) at ED visit in patients on Hydroxyurea (HU). Notably mito Complex IV & V activity increased significantly in both Arg arms while there was no change in the placebo group (Fig 1, p<0.001); protein carbonyl levels in platelet rich plasma decreased in both Arg groups (Fig 1, p<0.001), suggesting a decrease in oxidative stress that increased in the placebo arm (p=0.02). Greatest mito improvement occurred with Arg loading dose. Abstract source

Introduction: All states in the US require hemoglobinopathy testing as part of the newborn screen (NBS) which incidentally identifies individuals with sickle cell trait (SCT). Each state has its own protocol for disclosure of SCT results to families (i.e., via primary care provider (PCP), public health nurse, direct mailing to family) with no standards for documentation in the electronic health record (EHR). Our past work showed that SCT is documented 53% of the expected rate within our EHR. Though gaps in documentation may not equate to lack of disclosure, such gaps demonstrate a risk for inadequate counseling and loss of data with transitions of care. Further, the majority of individuals with SCT are Black or Hispanic. Failure to disclose SCT may contribute to inequities in healthcare and hamper informed reproductive decision making. Methods: A cross sectional survey of outpatient PCPs at 43 pediatric offices within a pediatric health system in Delaware, Pennsylvania, and Florida was conducted. Using Likert scales, rating scales, and free text response, the survey assessed PCP practices surrounding NBS results, attitudes toward SCT disclosure and documentation, and ranking of implementation strategies from the "Expert Recommendations for Implementing Change” project intended to increase SCT documentation and disclosure. Recruitment and survey administration was via REDCap. Lunch was provided to offices with >75% participation. Free text items were analyzed using qualitative methods. Descriptive statistics were used for other items. The study was approved by the IRB. Results: Participants: Seventy-eight PCPs from 43 practices completed surveys (51% recruitment rate). Fourteen offices received lunch incentives. PCPs worked in their offices a mean of 7.1 years (SD 6.8y). Most (76%) identified as female and White (66%), with 17% Asian, 4% Black, and 12% Hispanic. Practice locations varied in percentages of patients insured by Medicaid (3-76%) or who identify as Black (2-91%). Abstract source

Winter storms and cold temperatures can be dangerous. Stay safe and healthy by planning ahead. Prepare your home and vehicles. Prepare for power outages and outdoor activity. Check on older adults. Although winter comes as no surprise, many of us may not be ready for its arrival. If you are prepared for the hazards of winter, you are more likely to stay safe and healthy when temperatures start to fall. Full text

The Sickle Cell Disease Partnership is a multi-sector collaboration of healthcare stakeholders committed to advancing actionable federal healthcare policies that will improve the lives of patients living with Sickle Cell Disease. The Partnership’s main objective is to work collaboratively across the Sickle Cell Disease community to translate the recommendations of the National Academies’ foundational report into legislative and administrative actions in Congress and the Executive Branch. Their vision is a day when every individual with Sickle Cell Disease in the United States lives to his or her fullest potential because the individual has timely, sustained access to high-quality, equitable, coordinated care and treatment.   Official website

Dr. Charity I. Oyedeji treats patients with non-malignant (non-cancerous) blood disorders like sickle cell disease, thalassemia, iron deficiency anemia, and immune thrombocytopenia. Several of her patients have blood disorders that have been present since birth. Others have blood disorders that they acquire later in life due to nutritional deficiencies or autoimmune causes. She has a special focus on treating adults with sickle cell disease. She became interested in sickle cell disease because several of her family members have died from the condition, while others are thriving while living with it. She chose to become a hematologist because she wanted to improve care for adults with sickle cell disease and enjoy helping improve the lives of all patients with non-malignant blood disorders. She enjoys cooking plant-based, vegan foods because she believes good food is a vital part of maintaining good health and building healthy blood cells.   Representative Publications: Improving the Quality of Care for Adolescents and Adults With Sickle Cell Disease—It’s a Long Road Characterising the Prevalence of Overweight and Obese Status Among Adults With Sickle Cell Disease Adherence to Iron Chelation Therapy With Deferasirox Formulations among Patients with Sickle Cell Disease and β-thalassemia Geriatric Assessment for Older Adults With Sickle Cell Disease: Protocol for a Prospective Cohort Pilot Study Online profile

Dr. Sharl Azar is a hematologist and clinical investigator at the Massachusetts General Hospital and Harvard Medical School. He received his medical degree from Oregon Health & Science University where he also completed his residency in internal medicine and his fellowship in hematology and medical oncology while also serving as Chief Fellow. He went on to complete an advanced fellowship in hemostasis and thrombosis at the University of California, San Diego. Dr. Azar is passionate about the care of patients with Sickle Cell Disease, Thalassemia, and other hemoglobin disorders along with patients with Hemophilia, von Willebrand disease, and bleeding disorders. He also sees patients with other blood disorders including diseases of clotting, red cell and platelet function, and hemolysis.   Online profile

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Objectives: The aim of the present cross-sectional study was to investigate the association between periodontal inflamed surface area (PISA) and serum inflammatory biomarkers in patients with sickle cell anemia. Design: Patients with sickle cell anemia (n = 80) and systemically healthy individuals (n = 80) were enrolled in the study. Crisis episodes were recorded and blood samples were collected from patients with sickle cell anemia. Clinical periodontal parameters and PISA values were calculated from all patients. Ferritin and high sensitivity C-reactive protein (hs-CRP) levels were analyzed biochemically. Results: In sickle cell anemia group, presence of periodontitis (p < 0.001) was more frequent than periodontal health (p < 0.001). All clinical periodontal recordings and PISA values were higher in the sickle cell anemia group compared to controls (p < 0.001). Patients with PISA > 776 mm² had 6.06-fold greater chances of having hs-CRP levels above 10 mg/L (OR = 6.06; 95 % CI: 1.9-19.26) and had 31.41-fold greater chances of having ferritin levels above 1000 ng/L (OR = 31.41; 95 % CI: 6.62-149.16). Also, they had 3.27-fold greater risk of having crisis frequency above three times per year (OR = 3.27; 95 % CI: 1.05-10.23) after adjusting for confounders. Conclusion: In patients with sickle cell anemia, positive association was evident between PISA values and serum acute phase biomarkers levels as well as the frequency of acute painful crisis. Patients with increased inflammatory burden may have a higher likelihood of developing periodontitis in the presence of sickle cell anemia. PISA value could be a candidate disease activity indicator in patients with an underlying hematological condition. Abstract source

Cheat Codes: A Sickle Cell Podcast, is led by expert hosts Dr. Ahmar Zaidi and Dr. Mike Callaghan from the Comprehensive Sickle Cell Center at the Children's Hospital of Michigan, and produced by BloodStream Media. Each episode of Cheat Codes: A Sickle Cell Podcast brings listeners a series of segments packed with critical education and research information that patients and families need to know, as well as updates and clarifications from the social media "buzz" around sickle cell. Cheat Codes also features interviews with leading experts, moments from major conferences, and contributions from people living with sickle cell, all while Dr. Z and Dr. Mike keep the show fresh, fun, and off-the-cuff! Enjoy, share, and subscribe to Cheat Codes: A Sickle Cell Podcast.   Abstract source