The World Health Organization defines quality of care as “the extent to which health care services provided to individuals and patient populations improve desired health outcomes.” To achieve this quality, the health care must be safe, effective, timely, efficient, equitable, and centered on people. Kanter et al surveyed 440 adolescents and adults with sickle cell disease (SCD) through 7 comprehensive programs widely recognized as leaders in the care of people with SCD. The results of the survey suggested significant gaps and dissatisfaction with care, especially in the emergency department. Patient experience has a strong association with the perception of quality of care. Both pediatric and adult SCD populations reported dissatisfaction with care for reasons such as perceived racial discrimination, difficulties with access, and lack of clinician confidence and knowledge in managing SCD complications, especially pain. In the survey described, participants with severe pain or 4 or more pain episodes in the previous 6 months were much less likely to be satisfied with their scheduled nonacute appointments. Abstract source

The United States and countries around the world face a major public health concern with the current outbreak of the novel (new) coronavirus (COVID-19). In an effort to serve its members, ASH is maintaining this webpage as a medium to exchange information to assist hematologists in navigating the COVID-19 public health crisis. While ASH aims to provide a forum for the exchange of information that is helpful, accurate and up-to-date, the relevant health information and data concerning the disease, as well as the various governmental actions and responses to the disease, are changing constantly, and ASH makes no representation, warranty, or guaranty whatsoever as to any information that may be exchanged through this webpage.   Abstract source

How do people with sickle cell disease (SCD) do with COVID-19? Patients with SCD often have underlying cardiopulmonary co-morbidities that may predispose them to poor outcomes if they become infected with SARS-CoV-2. Data are being collected by the international COVID-19 sickle cell disease registry and by the ASH Registry, and providers are encouraged to report their SCD patients with COVID-19. The Sickle Cell Disease Association of America updates its recommendations weekly about best practices for the care of SCD patients in the era of COVID-19. Below, we address FAQs that arise most commonly from providers less familiar with SCD. How should I evaluate respiratory symptoms in children and adults with an active COVID-19 infection? There is significant overlap in presenting symptoms between COVID-19, acute chest syndrome (ACS) and other infectious causes of pneumonia. Providers should test for COVID-19 and other infectious pathogens and have a low threshold for imaging. COVID-19 most commonly presents with a more diffuse ground glass appearance, versus more localized infiltrates consistent with pneumonia or ACS.   Abstract source

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Chronic pain affects 50% of adults with sickle cell disease (SCD). Although inflammation is thought to contribute to the pathogenesis of chronic pain, no studies have examined the differences in circulating cytokines between patients with SCD with and without chronic pain. We performed an observational cohort study using blood and urine samples from adults with SCD with and without chronic pain at their usual state of health. We tested the hypothesis that, compared to those without chronic pain, those with chronic pain would have significantly higher baseline circulating proinflammatory cytokines. A total of 61 adults with SCD, 40 with chronic pain and 21 without chronic pain were tested. When SCD patients with chronic pain were compared to those without chronic pain, no significant differences in cytokine levels were noted. The variables most associated with the diagnosis of chronic pain in this population were opioid dose and subject age. Abstract source

Sickle cell disease is an inherited hemoglobinopathy, with relatively high prevalence in African American and some Hispanic populations within the United States. Those with SCD have increased risk for multisystem comorbid conditions as well as shortened lifespan compared to the general population. Pain is the most commonly occurring symptom of SCD, with patients experiencing both acute pain episodes as well as chronic pain that persists beyond exacerbations. Pain accounts for over half of hospital admissions among children with SCD. While acute pain has historically been a focus of research and clinical care, chronic pain is an increasingly recognized problem in SCD. Diagnostic criteria for chronic pain for patients with SCD is currently defined as pain on more than half of days that is not associated with active disease presence, lasting longer than 6 months. Using these criteria, an estimated 30–40% of adults and adolescents with SCD have chronic pain. Abstract source

Global Alliance of Sickle Cell Disease Organizations (GASCDO) represents:

• Patients with SCD;
• Parents, carriers, and families of patients with SCD;
• National and regional SCD organizations;
• Health care professionals;
• Educators and advocates;
• Community representatives;
• Others not limited to individuals, organizations, institutions (including centers of excellence, research and care provider organizations); and the industry.

The Global Alliance of Sickle Cell Disease Organizations (GASCDO) was inaugurated in Amsterdam, Netherlands on January 10, 2020. It is the first established entity representing individuals living with sickle cell disease (SCD) globally. In cooperation with other stakeholders in the field ; the organization’s vision is to reduce global incidence of SCD and ensure equitable access to optimal care and treatment by all people with SCD worldwide. Official website

COVID-19 is a new disease and there is limited information regarding risk factors for severe illness. Based on currently available information and clinical expertise, older adults and people of any age who have serious underlying medical conditions might be at higher risk for severe illness from COVID-19. Reduce Your Risk of Getting Sick With COVID-19 • Continue your medications and do not change your treatment plan without talking to your doctor. • Have at least a 2-week supply of prescription and non-prescription medications. Talk to your healthcare provider, insurer, and pharmacist about getting an extra supply (i.e., more than two weeks) of prescription medications, if possible, to reduce trips to the pharmacy. • Talk to your healthcare provider about whether your vaccinations are up-to-date. People older than 65 years, and those with many underlying conditions, such as those who are immunocompromised or with significant liver disease, are recommended to receive vaccinations against influenza and pneumococcal disease.   Abstract source

The novel coronavirus, and the disease that it causes, COVID-19, is causing significant challenges for millions of Americans living with rare diseases. People with underlying conditions are at risk of more severe illness from COVID-19, and rare disease patients, their caregivers and family members are seeing their lives disrupted in numerous other ways. The National Organization for Rare Disorders conducted a community survey in early April to help understand and bring these issues to light and to support the development of resources that address concerns raised by respondents. It is clear from the survey results that the community is overwhelmingly concerned and impacted by COVID-19; 98% are worried and of those, 67% are very or extremel worried. In addition, 95% have been impacted to some degree at a cost to their immediate and long-term health and well-being.   Abstract source

The objective of this study was to analyze acute care utilization of sickle cell disease (SCD) and sickle cell trait (SCT) in children and identify trends in emergency department (ED) visits and inpatient admissions over a 10-year period. This is a retrospective population-based study of SCD- and SCT-related ED visits and admissions from 2006 to 2015. Data were acquired from the Healthcare Cost and Utilization Project (HCUP), National Inpatient Sample (NIS), and National Emergency Department Sample (NEDS) database. Cost-to-charge and estimated professional fee ratios were applied to approximate costs. Over 80% of medical expenditure on HbSS is through ED-based admissions. There is a statistically significant increase from 2006 to 2015 in the direct hospital admissions associated with patients less than 18 years of age who have been diagnosed with SCT. Conclusion: Among patients less than 18 years of age with HbSS, inpatient admissions through the emergency department accounted for the largest medical expenditure of the SCD subtypes. Abstract source