Liver-to-lung microembolic NETs promote gasdermin D-dependent inflammatory lung injury in sickle cell disease.

Acute lung injury, referred to as the acute chest syndrome, is a major cause of morbidity and mortality in patients with sickle cell disease (SCD), which often occurs in the setting of a vaso-occlusive painful crisis. P-selectin antibody therapy reduces hospitalization of patients with SCD by ∼50%, suggesting that an unknown P-selectin-independent mechanism promotes remaining vaso-occlusive events. In patients with SCD, intraerythrocytic polymerization of mutant hemoglobin promotes ischemia-reperfusion injury and hemolysis, which leads to the development of sterile inflammation.

Assessing the Feasibility of a Novel mHealth App in Hematopoietic Stem Cell Transplant Patients.

Hematopoietic stem cell transplantation (HCT) is a curative treatment option for patients with hematologic conditions but presents many complications that must be managed as a complex, chronic condition. Mobile health applications (mHealth apps) may permit tracking of symptoms in HCT. In seeking strategies to manage the complexities of HCT, our team collaborated with Sicklesoft, Inc.

User-Centered App Design for Acutely Ill Children and Adolescents.

The high level of acceptance and consistent use of smartphones by children and adolescents present new opportunities to monitor and collect health data. For acutely ill children and adolescents, collecting symptom data via smartphone applications (apps) provides patient-reported data that can be collected daily and offers the potential to provide a more comprehensive picture of the symptom experience. The purpose of this study was to employ user-centered design principles and medical professional input in order to obtain feedback and insight into redesigning our Technology Recordings for better Understanding Blood and Marrow Transplant (TRU-PBMT) app.

Use of Mobile Health Apps and Wearable Technology to Assess Changes and Predict Pain During Treatment of Acute Pain in Sickle Cell Disease: Feasibility Study.

Background: Sickle cell disease (SCD) is an inherited red blood cell disorder affecting millions worldwide, and it results in many potential medical complications throughout the life course. The hallmark of SCD is pain. Many patients experience daily chronic pain as well as intermittent, unpredictable acute vaso-occlusive painful episodes called pain crises.

Customization of the TRU-PBMT App (Technology Recordings to better Understand Pediatric Blood and Marrow Transplant).

Purpose: Our investigative team is integrating mobile health technologies into pediatric blood and marrow transplant (PBMT) care. We aim to evaluate whether patient-generated health data can be used to monitor health status and enhance symptom management. While there are numerous health-related apps, none address the symptoms or care needs specific to PBMT patients.

Abstract Animations for the Communication and Assessment of Pain in Adults: Cross-Sectional Feasibility Study.

Background: Pain is the most common physical symptom requiring medical care, yet the current methods for assessing pain are sorely inadequate. Pain assessment tools can be either too simplistic or take too long to complete to be useful for point-of-care diagnosis and treatment.

Objective: The aim was to develop and test Painimation, a novel tool that uses graphic visualizations and animations instead of words or numeric scales to assess pain quality, intensity, and course.

Mobile health intervention for youth with sickle cell disease: Impact on adherence, disease knowledge, and quality of life.

Background: Adherence to illness self-management among youth with sickle cell disease (SCD) positively impacts health outcomes and decreases overall healthcare costs. Despite this, children with SCD face several barriers to adherence, with adherence rates that remain moderate to low. The current feasibility study examined the Intensive Training Program (ITP), a mobile health (mHealth) intervention for youth with SCD designed to promote disease knowledge, adherence, and patient-provider communication.

Utilizing a Novel Mobile Health "Selfie" Application to Improve Compliance to Iron Chelation in Pediatric Patients Receiving Chronic Transfusions.

Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes.

Attitudes of Primary Care Physicians Toward Sickle Cell Disease Care, Guidelines, and Comanaging Hydroxyurea With a Specialist.

Background: Sickle cell disease (SCD) is a complex chronic disease requiring multidisciplinary care that involves primary care physicians (PCPs) working with a hematologist or SCD specialists. However, PCPs often lack access to SCD specialists and are unaware of SCD guidelines or efficacious treatment.

Methods: We partnered with Community Care of North Carolina (CCNC) to identify assigned PCPs for SCD patients with Medicaid across North Carolina.

Phase 1 Study of a Sulforaphane-Containing Broccoli Sprout Homogenate for Sickle Cell Disease.

Sickle cell disease (SCD) is the most common inherited hemoglobinopathy worldwide. Our previous results indicate that the reduced oxidative stress capacity of sickle erythrocytes may be caused by decreased expression of NRF2 (Nuclear factor (erythroid-derived 2)-like 2), an oxidative stress regulator. We found that activation of NRF2 with sulforaphane (SFN) in erythroid progenitors significantly increased the expression of NRF2 targets HMOX1, NQO1, and HBG1 (subunit of fetal hemoglobin) in a dose-dependent manner.

Usability and Feasibility of an mHealth Intervention for Monitoring and Managing Pain Symptoms in Sickle Cell Disease: The Sickle Cell Disease Mobile Application to Record Symptoms via Technology (SMART).

Patients with sickle cell disease frequently experience severe pain events that lead to unplanned healthcare utilization. Mobile health tools (mHealth) may help prevent these events by providing remote monitoring and self-management support. This article describes the feasibility of the Sickle cell disease Mobile Application to Record symptoms via Technology (SMART), an mHealth app developed to help sickle cell disease patients monitor and manage their day-to-day symptoms.

Patients welcome the Sickle Cell Disease Mobile Application to Record Symptoms via Technology (SMART).

The widespread use of mobile phones among patients provides a unique opportunity for the development of mobile health intervention designed specifically for sickle cell disease, which will improve self-management as well as health care delivery. Our objective was to determine the receptiveness of patients with sickle cell disease to technology and a mobile application (app) designed for sickle cell disease. Phase I included 100 patients who completed a survey inquiring about receptiveness to technology and use of mobile devices to self-manage and communicate with providers.

Factors associated with survival in a contemporary adult sickle cell disease cohort.

In this study, the relationship of clinical differences among patients with sickle cell disease (SCD) was examined to understand the major contributors to early mortality in a contemporary cohort. Survival data were obtained for 542 adult subjects who were enrolled since 2002 at three university hospitals in the southeast United States. Subjects were followed up for a median of 9.

Effect of propranolol as antiadhesive therapy in sickle cell disease.

Sickle red blood cells (SSRBCs) adhere to both endothelial cells (ECs) and the extracellular matrix. Epinephrine elevates cyclic adenosine monophosphate in SSRBCs and increases adhesion of SSRBCs to ECs in a β-adrenergic receptor and protein kinase A-dependent manner. Studies in vitro as well as in vivo have suggested that adrenergic stimuli like epinephrine may contribute to vaso-occlusion associated with physiologic stress.

MYH9 and APOL1 are both associated with sickle cell disease nephropathy.

Renal failure occurs in 5-18% of sickle cell disease (SCD) patients and is associated with early mortality. At-risk SCD patients cannot be identified prior to the appearance of proteinuria and the pathobiology is not well understood. The myosin, heavy chain 9, non-muscle (MYH9) and apolipoprotein L1 (APOL1) genes have been associated with risk for focal segmental glomerulosclerosis and end-stage renal disease in African Americans.

Living with sickle cell disease: traversing 'race' and identity.

Objectives: Sickle cell disease (SCD) has a distinctive social history that continues to influence research and clinical practice related to the disease. Despite the historical link between SCD and concepts of 'race', there is limited empirical information on the relationships among SCD patients' 'race'/ancestry/ethnicity/nationality, their beliefs and attitudes associated with these identities, and their SCD experiences and outcomes. We conducted a preliminary study to explore some of these relationships.

Clinical and sociodemographic factors predict coping styles among adults with sickle cell disease.

Unlabelled: Sickle cell disease (SCD) presents a significant physical and psychological burden for persons with this chronic disease; however, little is known about how individuals with SCD--adult patients in particular--cope with disease-related stressors. The aim of this study was to identify and characterize factors that may influence the styles of coping among adults with SCD.

Methods: The study sample included 46 adult patients with SCD-20 men and 26 women--with an average age of 32.

Cardiopulmonary complications leading to premature deaths in adult patients with sickle cell disease.

Sickle cell disease (SCD) is associated with early mortality. We sought to determine the incidence, cause, and risk factors for death in an adult population of patients with SCD. All patients aged >/=18 years seen at the Adult Sickle Cell Center at Duke University Medical Center between January 2000 and April 2005 were enrolled.

Surgical and obstetric outcomes in adults with sickle cell disease.

Background: Sickle cell disease patients are more likely than the general population to undergo surgery and usually do so at a younger age. Female sickle cell disease patients also have special gynecological and obstetric issues related to their disease.

Methods: We collected data through standardized clinical report forms, patient interviews, and medical records from 509 adult sickle cell disease patients.

beta(2)-Adrenergic receptor and adenylate cyclase gene polymorphisms affect sickle red cell adhesion.

Sickle red cell (SS RBC) adhesion is thought to contribute to sickle cell disease (SCD) pathophysiology. SS RBC adhesion to laminin increases in response to adrenaline stimulation of beta(2)-adrenergic receptors (beta(2)ARs) and adenylate cyclase (ADCY6), and previous evidence suggests such activation occurs in vivo. We explored whether polymorphisms of the beta(2)AR and ADCY6 genes (ADRB2 and ADCY6, respectively) affect RBC adhesion to laminin.

Identification of genetic polymorphisms associated with risk for pulmonary hypertension in sickle cell disease.

Up to 30% of adult patients with sickle cell disease (SCD) will develop pulmonary hypertension (pHTN), a complication associated with significant morbidity and mortality. To identify genetic factors that contribute to risk for pHTN in SCD, we performed association analysis with 297 single nucleotide polymorphisms (SNPs) in 49 candidate genes in patients with sickle cell anemia (Hb SS) who had been screened for pHTN by echocardiography (n = 111). Evidence of association was primarily identified for genes in the TGFbeta superfamily, including activin A receptor, type II-like 1 (ACVRL1), bone morphogenetic protein receptor 2 (BMPR2), and bone morphogenetic protein 6 (BMP6).