Management of Sickle Cell Disease Complications Beyond Acute Chest Syndrome.

Sickle cell disease results in numerous complications that can lead to significant morbidity and mortality. Amongst them, acute chest syndrome is the leading cause of mortality. As a result, most providers are in tune with this complication and well versed with management.

Association between hospital admissions and healthcare provider communication for individuals with sickle cell disease.

To test the hypothesis that caregivers' or adult participants' low ratings of provider communication are associated with more hospital admissions among adults and children with sickle cell disease (SCD), respectively. Secondarily, we determined whether there was an association between the caregivers' or participants' health literacy and rating of providers' communication. Primary data were collected from participants through surveys between 2014 and 2016, across six sickle cell centers throughout the U.

Risk factors for hospitalizations and readmissions among individuals with sickle cell disease: results of a U.S. survey study.

Objective: Hospital admissions are significant events in the care of individuals with sickle cell disease (SCD) due to associated costs and potential for quality of life compromise.

Methods: This cross-sectional cohort study evaluated risk factors for admissions and readmissions between October 2014 and March 2016 in adults with SCD (n = 201) and caregivers of children with SCD (n = 330) at six centres across the U.S.

Modifying factors of the health belief model associated with missed clinic appointments among individuals with sickle cell disease.

Objectives: Outpatient care is critical in the management of chronic diseases, including sickle cell disease (SCD). Risk factors for poor adherence with clinic appointments in SCD are poorly defined. This exploratory study evaluated associations between modifying variables from the Health Belief Model and missed appointments.

Recent treatment guidelines for managing adult patients with sickle cell disease: challenges in access to care, social issues, and adherence.

Introduction: Advances in research, medical care, and public health practice have led to individuals with sickle cell disease (SCD) living into adulthood. However, premature mortality persists in youth and young adults with SCD, and adults with SCD are subjected to increased disease burden, organ damage, pain, and disruptions in family and work life.

Areas Covered: These issues have led to inappropriate utilization of hospital resources, significantly increasing costs related to prolonged inpatient stays, high readmission rates, and increased emergency room visits.

Adherence to iron chelation therapy and associated healthcare resource utilization and costs in Medicaid patients with sickle cell disease and thalassemia.

Background: Sub-optimal patient adherence to iron chelation therapy (ICT) may impact patient outcomes and increase cost of care. This study evaluated the economic burden of ICT non-adherence in patients with sickle cell disease (SCD) or thalassemia.

Methods: Patients with SCD or thalassemia were identified from six state Medicaid programs (1997-2013).

Multicenter COMPACT study of COMplications in patients with sickle cell disease and utilization of iron chelation therapy.

Background: Over the past few decades, lifespans of sickle cell disease (SCD) patients have increased; hence, they encounter multiple complications. Early detection, appropriate comprehensive care, and treatment may prevent or delay onset of complications.

Objective: We collected longitudinal data on sickle cell disease (SCD) complication rates and associated resource utilization relative to blood transfusion patterns and iron chelation therapy (ICT) use in patients aged ≥16 years to address a gap in the literature.

Prevalence and progression of chronic kidney disease in adult patients with sickle cell disease.

Aim: We evaluated the prevalence and progression of chronic kidney disease (CKD) during the 5-year period in a cohort of patients with sickle cell disease (SCD) aged 18 years and older.

Methods: We studied 98 patients with SCD. Chronic kidney disease stages I through V were defined based on estimated glomerular filtration rate (eGFR), and albuminuria grades were defined based on spot urine protein-to-creatinine ratio according to the 2012 Kidney Disease Improving Global Outcomes recommendations.

Leg ulcers in sickle cell disease: current patterns and practices.

Leg ulcers are a debilitating complication of patients with sickle cell disease, and their frequency in North America was reported to be 2.5% by the Cooperative Study of Sickle Cell Disease more than 20 years ago. We sought to determine if the frequency of leg ulcers in sickle cell patients in the United States had declined and to assess which treatments providers use most commonly.

Bone mineral density patterns in vitamin D deficient African American men with sickle cell disease.

Objective: To describe bone mineral density (BMD) patterns by densitometry in adult African American (AA) men with sickle cell disease (SCD) who are vitamin D deficient (Vit DD).

Inclusion/exclusion Criteria: All SCD phenotypes were eligible. Those with chronic renal failure or hyperparathyroidism were excluded.

Hemodynamic predictors of mortality in adults with sickle cell disease.

Background: Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH.

Objectives: To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization.

Methods: Nine-year follow-up data (median, 4.

Sickle cell pain: a critical reappraisal.

Sickle cell pain includes 3 types: acute recurrent painful crises, chronic pain syndromes, and neuropathic pain. The acute painful crisis is the hallmark of the disease and the most common cause of hospitalization and treatment in the emergency department. It evolves through 4 phases: prodromal, initial, established, and resolving.

A transition pilot program for adolescents with sickle cell disease.

Introduction: Transition from pediatric to adult care is challenging for adolescents with chronic illnesses, including those with sickle cell disease (SCD). We describe a pilot program created to facilitate transition from pediatric to adult care by helping adolescents with SCD identify an adult medical home.

Methods: We investigated the feasibility of this program by evaluation of overall participation, satisfaction, and acceptance.

Prevalence of 25-hydroxyvitamin D deficiency in an urban general internal medicine academic practice.

Vitamin D deficiency has received increased academic interest because of its association with many common disease processes. The goal of our study was to document the prevalence of vitamin D deficiency. A retrospective chart review of 25-hydroxyvitamin D (ng/mL) levels at the University of Tennessee Health Science Center was conducted on general internal medicine patients over an 18-month period.

A randomized phase II trial of Arginine Butyrate with standard local therapy in refractory sickle cell leg ulcers.

Sickle cell leg ulcers are often debilitating, refractory to healing, and prone to recurrence. Healing of leg ulcers was incidentally observed during dose-ranging trials of Arginine Butyrate in beta haemoglobinopathies. Here, a controlled Phase II trial was performed in sickle cell patients who had lower extremity ulcers refractory to standard care for at least 6 months.

Prevalence of 25-hydroxyvitamin D deficiency in an urban general internal medicine academic practice.

Vitamin D deficiency has received increased academic interest because of its association with many common disease processes. The goal of our study was to document the prevalence of vitamin D deficiency. A retrospective chart review of 25-hydroxyvitamin D (ng/ml) levels at the University of Tennessee Health Science Center was conducted on general internal medicine patients over an 18-month period.

Sickle cell hospital unit: a disease-specific model.

American urban hospitals often serve large populations of sickle cell disease (SCD) patients. Those hospitals that choose to implement an adult SCD-specific inpatient unit have the opportunity to acquire multiple operational benefits. Such units may ultimately reduce patient morbidity and mortality; improve timely access to quality medical care in a cost-effective manner; reduce overcrowding in the emergency department; and increase patient, family, physician, and payer satisfaction.

Development and validation of SIMS: an instrument for measuring quality of life of adults with sickle cell disease.

In recent years, there has been a growing interest in the assessment of quality of life (QOL) issues, particularly in chronic debilitating conditions. Several instruments have been developed, tested, and validated in the general population and in other chronic diseases; however, few studies have examined QOL issues in adults with sickle cell disease (SCD). We developed Sickle Cell Impact Measurement Scale (SIMS), an instrument for measuring the QOL of adults with SCD.

Identification of hemochromatosis gene polymorphisms in chronically transfused patients with sickle cell disease.

Three polymorphic gene mutations in the human hemochromatosis (HFE) gene (C282Y, H63D, S65C) are associated with non-transfusion-related iron overload in Caucasians. More recently, these mutations have also been identified in African-Americans. However, the prevalence of HFE gene mutations in African-Americans with sickle cell disease (SCD) has not been described.