Patients who understand their own health conditions and partner with their clinical teams to adopt personalized treatment plans are happier and achieve better outcomes. This practical toolkit provides clinicians with materials and resources to engage with patients and educate them about sickle cell disease. Use the toolkit with patients individually or in group education settings to help build a strong foundation for shared decision-making regarding treatment goals and preferences, transitions of care, and individualized treatment plans. Included in this toolkit: • Patient-friendly slide deck with helpful speaker notes on living with sickle cell disease, the newest options for treatment, and factors influencing therapy selection • Tips for engaging patients in collaborative learning so they become active participants in their own care • Resources that support shared decision-making, self-management, and treatment adherence in your practice   Abstract source

In patients with sickle cell disease (SCD) and diabetes mellitus (DM), hemoglobin A1c (HbA1c) is unreliable and the American Diabetes Association recommends monitoring long‐term glycemia by measuring serum glucose, but use of serum fructosamine (SF), a measurement independent of red cell lifespan, has been reported. SF as a screen for DM in SCD, however, is not standardized and its relationship to serum glucose has not been validated. Further, screening for DM was not adequately addressed in the 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for SCD management. Blood transfusions, an important treatment for some patients with SCD, can also impact HbA1c. We present a case of a patient with SCD and cystic fibrosis‐related diabetes on monthly chronic transfusions therapy (CTT) who had well‐correlated “steady state” HbA1c and SF levels over time, suggesting for the first time these markers may actually be useful when following long‐term glycemic control in patients with SCD on CTT programs. Abstract source

Sickle cell disease (SCD), an inherited hemoglobinopathy that most commonly affects persons of African ancestry, is estimated to affect 1 in 365 Black persons in the United States . Persons with SCD produce abnormal hemoglobin that causes erythrocytes to become rigid and deform under low oxygen conditions, leading to ischemia–reperfusion injury in the microvasculature with subsequent organ damage and pain. SCD affects nearly every organ system; average life expectancy estimates of affected persons are 43–54 years. Persons with SCD are at increased risk for pulmonary disease and pneumonia. Previous studies have shown that influenza severity and hospitalization rates are higher among persons with SCD than those without SCD. Thus, persons with SCD could be at higher risk for development of severe disease if infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of coronavirus disease (COVID-19). Although empirical data are limited, 16 cases of COVID-19 pneumonia have been reported in persons with SCD , and a study of COVID-19 intensive care unit (ICU) admissions reported 2 (4%) of 48 children had SCD . We describe a large series of COVID-19 cases and associated deaths among persons with SCD in the United States. Abstract source

Background/Objectives: Among sickle cell disease (SCD) patients, vaso-occlusive crises (VOCs) are recurrent and unpredictable attacks of acute pain. These pain crises are often treated with analgesics, including opioids, which have been associated with misuse and overdose. The aim of this study was to examine the association between VOC events and opioid use and assess the association between opioid prescriptions and health care resource utilization among SCD patients. Methods: This was a retrospective cohort study using Texas Medicaid medical and prescription claims between September 2011 and August 2016. The index date was the first SCD diagnosis. Patients (2–63 years) with at least one inpatient or two outpatient SCD diagnoses, who were continuously enrolled during 12 months postindex, were included in the study. The primary outcome was number of opioid prescriptions, while the independent variable was number of VOC events. Covariates included age, gender, nonopioid medication use, nonstudy SCD-related medication (penicillin and folic acid) use, evidence of blood transfusions, number of SCD-related complications, number of SCD-related comorbid conditions, and Charlson Comorbidity Index score. Negative binomial regression analysis was used to address study objectives. Results: Of 3368 included patients, 1978 (58.7%) had at least one opioid prescription with a mean of 4.2 (SD=7.2). Overall, 2071 (61.5%) had at least one VOC event with an average of 2.9 (SD=4.4). The results from the negative binomial regression showed that for every increase in VOC events, the number of opioid prescriptions increased by 9.5% (Incidence rate ratio=1.095, 95% CI: 1.078–1.111; P ≤ 0.0001). Other significant covariates associated with higher opioid use included age (13 and older compared to 2–12) and increase in the number of nonopioid pain medications, nonstudy SCD-related medications, and SCD-related complications. Conclusions: The majority of SCD patients had at least one VOC event and were prescribed opioids during the 12-month study period. We found that each VOC event was associated with a 9.5% increase in the use of opioids. SCD guidelines recommend opioids for the treatment of VOC-related pain. Payers and providers should be aware of opioid use in this population, consider appropriate VOC prevention measures, and provide SCD patients with access to appropriate pain management. Abstract source