Initial outcomes in a sickle cell disease transition clinic.

Background: Transition in sickle cell disease (SCD) is associated with an alarming increase in acute care utilization, cost, and risk of early mortality. Effective transition preparation is crucial to address these issues. We established a multidisciplinary transition clinic at our urban SCD center in the fall of 2021.

Two Sides of a Coin: Case Report of Unilateral Synangiosis and Contralateral Stroke Highlighting Consequences of Disease Progression and Efficacy of Revascularization in Sickle Cell Disease-Associated Moyamoya Syndrome.

Moyamoya syndrome increases the risk of stroke in sickle cell disease, but revascularization surgery can modify this risk. Collaborative management between hematology and neurosurgery offers effective strategies to reduce stroke risk in these patients. We describe a challenging case where a patient with sickle cell disease undergoing standard of care management as prescribed by the Stroke Prevention Trial in Sickle Cell Anemia and revascularization with pial synangiosis subsequently developed rapidly progressive disease in other cerebral vessels and suffered ischemic hemispheric stroke.

Engaging Caregivers and Providers of Children With Sickle Cell Anemia in Shared Decision Making for Hydroxyurea: Protocol for a Multicenter Randomized Controlled Trial.

Background: Sickle cell anemia (SCA) is a genetic blood disorder that puts children at a risk of serious medical complications, early morbidity and mortality, and high health care utilization. Until recently, hydroxyurea was the only disease-modifying treatment for this life-threatening disease and has remained the only option for children younger than 5 years. Evidence-based guidelines recommend using a shared decision-making (SDM) approach for offering hydroxyurea to children with SCA (HbSS or HbS/β0 thalassemia) aged as early as 9 months.

Maintenance of a High Influenza Vaccination Rate and Improvement in Health Outcomes in a Pediatric Sickle Cell Disease Clinic.

Background: Children with sickle cell disease (SCD) are at high-risk of complications from influenza and should receive an influenza vaccination seasonally. Despite this recommendation, vaccination rates remain suboptimal. Boston Medical Center (BMC) previously achieved high influenza vaccination rates among its pediatric patients with SCD.

A Mild Phenotype of Severe β+ Thalassemia in a 16-Month-Old Boy.

β thalassemia is characterized by a deficient production of functional β-globin chains and a relative excess of α-globin chains. An extremely diverse clinical spectrum-asymptomatic to transfusion-dependent-is primarily due to homozygosity or compound heterozygosity for the very large number of β-thalassemia-causing mutations, along with interacting mutations that affect the α-globin and γ-globin genes and their expression. We report a case of a 16-month-old boy who was initially diagnosed with iron deficiency anemia until he was later found to be homozygous for a severe β-thalassemia genotype with a mild hematologic phenotype.

Development of quality indicators for transition from pediatric to adult care in sickle cell disease: A modified Delphi survey of adult providers.

Background: Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality indicators for transition in SCD and to determine the optimal timing between the final pediatric visit and the first adult provider visit.

Procedure: We conducted a modified Delphi survey to reach a consensus on which quality indicators are most important for a successful transition.

Young Adult Perspectives on a Successful Transition from Pediatric to Adult Care in Sickle Cell Disease.

Objective: This qualitative study sought to learn from young adults with sickle cell disease (SCD) about their experience leaving pediatric care and perspective on what makes a successful transition.

Methods: Fifteen young adults with SCD who had left pediatric care within the previous five years participated in focus groups led by a trained moderator. Transcripts were analyzed using grounded theory.

Improvement in influenza vaccination rates in a pediatric sickle cell disease clinic.

Background: Children with sickle cell disease (SCD) are at increased risk of complications from influenza. However, despite widespread recommendations that these patients receive an annual influenza immunization, reported vaccination rates remain very low at under 50%.

Procedure: Our aim was to increase the influenza vaccination rate among our pediatric patients with SCD aged 6 months to 21 years over two influenza seasons, 2012-2013 and 2013-2014, to 80%, consistent with the Health People 2020 goal.