SMJ articles in the category "sickle cell disease"

Original Article

Maternal and Infant Outcomes in a Subset of Patients with Sickle Cell Disease in South Carolina

Objectives: Sickle cell disease (SCD), which disproportionately affects minorities, increases complications during pregnancy. Severe maternal mortality is increased in women with SCD, including morbidity related to the disease and other nondisease-related complications. It also can have devastating complications for fetuses, with increases in premature birth and low birth weight. This…

Posted in: maternal mortality 2 sickle cell disease 28

Original Article

Rate of Food Insecurity Among Households with Children with Sickle Cell Disease is Above the National Average

Objectives: Despite studies demonstrating the negative impact of food insecurity on health in children, limited research has been done to assess the prevalence and sequelae of food insecurity in sickle cell disease (SCD). We tested the hypothesis that food insecurity is common in children with SCD and is associated with…

Posted in: food insecurity 4 public health 15 sickle cell disease 28

Original Article

Sickle Cell Disease, More Than Just Pain: The Mediating Role of Psychological Symptoms

Objectives: Perceived stress is associated with sickle cell disease (SCD) pain; however, little is known about psychological mechanisms that may clarify this link among adult patients. This study explored whether anxiety and depression symptoms explained the relation between perceived stress and SCD pain episode frequency among 70 African-American adults (51.4%…

Posted in: anxiety 8 Depression 22 pain 18 sickle cell disease 28 stress 8

Original Article

Contraceptive Methods and the Impact of Menstruation on Daily Functioning in Women with Sickle Cell Disease

Objectives: Women with sickle cell disease (SCD) are living longer as a result of advances in the care of their underlying disease. With the population growing of women living with SCD, reproductive health issues in this population have become an emphasized area of medical care. We sought to describe current…

Posted in: contraception 14 quality of life 11 sickle cell disease 28

Original Article

Lessons Learned from Building a Pediatric-to-Adult Sickle Cell Transition Program

Objective: More effective transitions and transfers of young people with sickle cell disease (SCD) into the adult healthcare setting is a focus of both primary care and specialty care medical organizations. Effective transition and transfer requires six core elements: establishing a policy, tracking progress, administering transition readiness assessments, planning for…

Posted in: care coordination 2 medical home 2 sickle cell disease 28 transition of care 4

Original Article

Maternal/Perinatal Outcome in Women with Sickle Cell Disease: A Comparison of Two Time Periods

Objective: To compare pregnancy outcomes in women with sickle cell disease from recent deliveries with a similar group delivered earlier. Methods: During a 12-year period (2005–2016), data from pregnant women with hemoglobin SS or SC were collected from three university medical centers and compared with earlier studies (1979–2003) involving similar…

Posted in: sickle cell disease 28

Review Article

CME Article: Management of Sickle Cell Disease in Children

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…

Posted in: children 22 primary care 64 sickle cell anemia 6 sickle cell disease 28

Review Article

CME Article: Sickle Cell Disease in Pregnancy

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…

Posted in: pregnancy 48 sickle cell disease 28

Original Article

CME Article: Effect of Free Dental Services on Individuals with Sickle Cell Disease

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…

Posted in: sickle cell disease 28

Multidisciplinary Clinical Case Study

CME Article: A Case-Based Approach to Transition of Care for Patients With Sickle Cell Disease

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…

Posted in: chronic disease 5 healthcare transition 2 sickle cell disease 28

Original Article

CME Article: High Percentage of Evanescent Red Cell Antibodies in Patients with Sickle Cell Disease Highlights Need for a National Antibody Database

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…

Posted in: sickle cell disease 28

Review Article

CME Article: Perioperative Management in Sickle Cell Disease

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…

Posted in: sickle cell disease 28 surgery 23 thromboembolism 6 transfusion 5

Review Article

CME Article: Osteonecrosis in Sickle Cell Disease

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…

Posted in: avascular necrosis 2 osteonecrosis 4 sickle cell disease 28

Original Article

CME Article: Examination of the Patient and Hospitalization Characteristics of 30-Day SCD Readmissions

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…

Posted in: healthcare utilization 6 readmissions 4 sickle cell disease 28

Original Article

CME Article: Utility of the Montreal Cognitive Assessment as a Screening Test for Neurocognitive Dysfunction in Adults with Sickle Cell Disease

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…

Posted in: cognitive impairment 2 screening 33 sickle cell disease 28

Review Article

CME Article: Fat Embolism Syndrome Secondary to Bone Marrow Necrosis in Patients with Hemoglobinopathies

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…

Posted in: sickle cell disease 28

Original Article

CME Article: Symptomatic Avascular Necrosis: An Understudied Risk Factor for Acute Care Utilization by Patients with SCD

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…

Posted in: avascular necrosis 2 hospitalization 13 osteonecrosis 4 sickle cell disease 28 utilization 2

Original Article

CME Article: Dural Venous Sinus Diameters in Children with Sickle Cell Disease: Correlation with History of Stroke in a Case–Control Study

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,…

Posted in: magnetic resonance imaging 13 sickle cell disease 28 Stroke 16

Original Article

Primary Care Providers’ Comfort Levels in Caring for Patients with Sickle Cell Disease

Objective: The purpose of this study was to determine the comfort levels of primary care providers in caring for individuals with sickle cell disease (SCD) and determine factors that improved or lessened provider comfort. Methods: We surveyed providers at the annual Johns Hopkins Community Physicians retreat. The survey consisted of…

Posted in: hemoglobinopathies 3 primary care 64 sickle cell disease 28

Original Article

Edmonton Symptom Assessment System for Outpatient Symptom Monitoring of Sickle Cell Disease

Objectives: Although the extension of palliative care methodology to sickle cell disease (SCD) care has been proposed, there is no current standard for symptom assessment. Our goal was to assess the feasibility of integrating the Edmonton Symptom Assessment System (ESAS) into the outpatient management of SCD. Methods: Seventy-five adult patients…

Posted in: palliative care 3 sickle cell disease 28
SMA Menu