More than 20 years ago, I prescribed hydroxyurea (HU) for the first time. Derrick was 17 and had been admitted more than 50 times for severe pain crises. Brought in severe pain to the hospital by ambulance, he would receive intravenous fluids and narcotics for several days and then finally…
How Many People with Sickle Cell Disease Are Receiving Hydroxyurea? Hydroxyurea is used widely in the United States and Europe as a treatment for sickle cell disease (SCD). Hydroxyurea was approved by in the United States Food and Drug Administration for the treatment of adults with severe sickle cell anemia…
Young adults with sickle cell disease must navigate a difficult road to independence once they age out of pediatric care. The anxiety surrounding transition, the challenges of medical complications, and chronic psychosocial stressors are obstacles to a seamless transition to adult medical care. The two cases presented here demonstrate that…
Objectives: Poor oral health can have a negative impact on overall health. This is especially concerning for individuals with sickle cell disease (SCD), an inherited blood disorder that affects hemoglobin and can lead to an increased risk of infection and hyperalgesia. Because the majority of individuals with SCD have Medicaid…
Sickle cell disease (SCD) is an inherited red blood cell condition common among people of African, Middle Eastern and Asian descent. SCD causes anemia, severe pain episodes, and damage to tissues and organs, which can lead to premature death. It is estimated that more than 2 million individuals living in…
The Southern Medical Journal and its editorial board are pleased to feature the important clinical topic of sickle cell disease (SCD) in this special issue. In addition, I appreciate the leadership of the guest editors, Dr Rosalyn Stewart of the Departments of Medicine and Pediatrics at the Johns Hopkins University…
Sickle cell disease (SCD) is the most common hereditary disorder and affects 30 million people worldwide. Advances in science have improved overall survival in patients with SCD and as such, more patients are reaching reproductive age and are becoming pregnant. SCD in pregnancy leads to multiple complications that put both…
Sickle cell disease (SCD) is a heterogeneous inherited disorder of hemoglobin that causes chronicm hemolytic anemia, vaso-occlusion, and endothelial dysfunction. These physiologic derangements often lead to multiorgan damage in infancy and throughout childhood. The most common types of SCD are homozygous hemoglobin S (HbSS disease), hemoglobin SC disease, and sickle…
There is little specific research on common psychiatric conditions in sickle cell disease (SCD). Conditions involving chronic pain are known to co-occur with depression and anxiety to a high degree, and greater chronicity and severity of pain tend to be linked more closely with both depressive symptoms and the diagnosis…
Community health workers (CHWs) are laypersons who work one to one with individuals to improve health outcomes and healthcare system use.1 They have many different titles: lay health worker, outreach worker, health advocate, promotora de salud, navigators, and guides. Typically, CHWs work with complex, less adherent, underserved people to increase…
Objectives: Alloantibody formation secondary to transfusion in patients with sickle cell disease (SCD) is a well-known phenomenon. Pretransfusion testing (eg, “antibody screening”) protects patients from receiving incompatible red blood cell transfusions. Because alloantibodies have a tendency to evanesce (ie, become undetectable over time), however, this phenomenon puts patients at risk…
Sickle cell disease (SCD) is an inherited red blood cell disorder that affects approximately 100,000 people in the United States.1 The hallmark of SCD is vaso-occlusive crisis, which often results in emergency department (ED) visits and hospitalization. Rapid assessment and treatment of vaso-occlusive crisis, close monitoring, social services support, and…
Sickle cell disease (SCD), the most common inherited blood disorder, is characterized by defective oxygen transport. Every part of the eye can be affected by microvascular occlusions from SCD; however, the major cause of vision loss is proliferative sickle cell retinopathy (PSR). Although individuals with the HbSS genotype of SCD…
Objectives: The purpose of this study was to compare the diameters of the dural venous sinuses (DVSs) in children with sickle cell disease (SCD) with healthy controls and determine whether the size has any correlation to history of cerebral infarct among children with SCD. Methods: A retrospective review compared demographics,…
Hospital care for pain crises has been the most studied phenomenon in sickle cell disease (SCD) and is exceptionally costly to the healthcare system.1–4 Hospitalizations likely represent a small subset of the total burden of SCD pain, however. Close investigation of the pain experience of adults with SCD has revealed…
All infants born in Maryland are tested for sickle cell disease (SCD) and other hemoglobinopathies via blood spot screening by the Newborn Screening Program (NBS). An NBS nurse(s), upon receiving a positive blood spot for SCD, contacts the pediatrician of record to inform him or her of the results, requests…
Objectives: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN…
Bone marrow necrosis with subsequent embolization of the fat and necrotic tissues into the systemic circulation causing fat embolism syndrome and multiorgan failure is a rare complication of patients with hemoglobinopathies. The exact etiology of this condition is not known. Because it occurs more often in patients with compound heterozygous…
Objectives: Neurocognitive dysfunction is an important complication of sickle cell disease (SCD), but little is published on the utility of screening tests for cognitive impairment in people with the disease. The purpose of this study was to evaluate the Montreal Cognitive Assessment (MoCA) as a screening tool and identify predictors…
Objectives: Transcranial Doppler ultrasound (TCD) effectively identifies children with sickle cell anemia (SCA) who are at increased risk of stroke. We evaluated a low-cost quality improvement (QI) intervention to increase the proportion of children screened by TCD. Methods: We measured the proportion of children with SCA receiving appropriate TCD screening…
Many patients with sickle cell disease (SCD) will require surgical intervention during the course of their lifetime. Common surgeries include orthopedic and abdominal procedures. Perioperative complications occur commonly and can be related to the surgical procedure or the underlying hemoglobinopathy. The complication rate may be reduced by preoperative optimization of…
Objectives: Sickle cell disease (SCD) is associated with a high level of emergency department and hospital utilization, as well as a high rate of hospital readmissions. At Johns Hopkins Hospital, as at other institutions, SCD accounts for a large proportion of readmissions. Our study examined patient and hospitalization factors involved…
Sickle cell disease (SCD) is a genetic disorder of the blood that leads to severe morbidity and early mortality. The best known burden of SCD is the vaso-occlusive crisis (VOC). These acute, excruciatingly painful events are the leading cause of hospital and emergency department (ED) utilization1 and can be associated…
Walter Clement-Noel, a dental student from Grenada, reported the first case of sickle cell disease (SCD) in Chicago in 1910. The hallmark of this genetic disorder is vasoocclusive crises associated with severe pain. SCD occurs in 1 of every 365 black or African American births and 1 in 16,300 Hispanic…
It is well established that the communicative relationship between patients and healthcare providers is essential for the provision of high-quality care.1 Strong and affirmative communication skills not only enhance the patient–provider relationship but also are known to be significantly associated with treatment efficacy, patient commitment to treatment regimens, and a…
Osteonecrosis is one of the most devastating musculoskeletal manifestations of sickle cell disease and most commonly affects the femoral head. Although the exact pathophysiology of this condition in patients with sickle cell disease is unknown, it is suggested that red cell sickling and repetitive vaso-occlusion may be associated with tissue…
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