Implementation and Evaluation of a Pediatric Palliative Care Curriculum for Adult-Track HPM Fellows.

Introduction: Hospice and Palliative Medicine (HPM) fellowship training provides education on caring for patients from early childhood through adulthood. Yet, there are few guidelines about how to teach these key components, and more specifically the components of Pediatric Palliative Care (PPC), to fellows who have a variety of backgrounds prior to entering HPM fellowship.

Methods: We created a curriculum in PPC that could be used with adult-track HPM fellows.

Improving well-being among women in neonatology.

Objective: To evaluate the factors that improve professional and personal well-being amongst women in neonatology (WiN).

Study Design: A 30-question survey of multiple choice, rank order, and open-ended questions focused on professional and personal factors that affect the well-being of WiN members. Quantitative and qualitative methods were used to determine leading factors and themes.

Long-term quality of life after hematopoietic cell transplant for sickle cell disease in childhood: A STELLAR interim analysis.

We prospectively collected PROMIS©25 and PROMIS©29 surveys in the Sickle Cell Transplant Evaluation of Long Term and Late Effects Registry (STELLAR). Mobility and social participation T-scores were decreased; all other domains were within the norm.

Parental perspective on the risk of infertility and fertility preservation options for children and adolescents with sickle cell disease considering hematopoietic stem cell transplantation.

Background: Allogeneic hematopoietic stem cell transplantation (HCT) performed in children from human leukocyte antigen (HLA)-identical related donors is associated with very high survival rates and disease-free survival. Patients are exposed to gonadotoxic alkylating agents or irradiation in the HCT conditioning regimen. Consequently, infertility is a major long-term complication of HCT for sickle cell disease (SCD).

Multimodal phenotyping and correlates of pain following hematopoietic cell transplant in children with sickle cell disease.

Introduction: There is limited understanding of pain, patient-reported outcomes (PROs) of health-related quality of life (HRQoL), psychological factors, and experimental pain sensitivity before and following hematopoietic cell transplant (HCT) in children with sickle cell disease (SCD).

Methods: Individuals aged 8 years and older, English speaking, and scheduled for a HCT were invited to participate in an observational study where they completed assessments of pain, PROs, psychological factors, and qualitative interviews before and around 3 months, 6 months, 1 year, and 2 years post-HCT. An optional substudy of experimental pain sensitivity before and around 6 month, 1 year, and 2 years post-HCT was also offered.

Decision-making about gene therapy in transfusion dependent thalassemia.

Background: Hematopoietic stem cell transplantation (HSCT) is a treatment option with curative intent for patients with transfusion dependent thalassemia (TDT) but its application is limited by the lack of suitable donors and acceptability due to the related morbidity/mortality. Transplantation of autologous genetically modified hematopoietic cells, gene therapy (GT) is emerging as a promising treatment option for TDT as it eliminates graft versus host disease (GVHD) and need for immunosuppression. Early results of GT suggest that many, but not all patients achieve transfusion independence after the procedure.

You Too? Female Radiologists' Narratives on Discrimination and Harassment.

Background: There is a paucity of examples of gender discrimination and sexual harassment in radiology. This lack of knowledge can limit radiologists' ability to relate to this topic, acknowledge its importance and impact, and take actions toward improvement.

Materials And Methods: We conducted a qualitative analysis using the model of thematic analysis developed by Braun and Clarke of narrative responses to a survey on the topic of #MeToo, which was distributed to a social media group of female radiologists.

A Decision Support Tool for Allogeneic Hematopoietic Stem Cell Transplantation for Children With Sickle Cell Disease: Acceptability and Usability Study.

Background: Individuals living with sickle cell disease (SCD) may benefit from a variety of disease-modifying therapies, including hydroxyurea, voxelotor, crizanlizumab, L-glutamine, and chronic blood transfusions. However, allogeneic hematopoietic stem cell transplantation (HCT) remains the only nonexperimental treatment with curative intent. As HCT outcomes can be influenced by the complex interaction of several risk factors, HCT can be a difficult decision for health care providers to make for their patients with SCD.

A pilot study of the acceptability, feasibility and safety of yoga for chronic pain in sickle cell disease.

Objectives: To determine the acceptability, feasibility and safety of yoga for chronic pain in sickle cell disease.

Design And Setting: In Part A of this two-part study, adolescents with SCD and chronic pain (Group 1) and their parent (Group 2) completed a survey designed to capture pain characteristics, attitudes and practices related to yoga, and potential acceptability of a yoga program. In Part B, the study assessed the feasibility and safety of an instructor-led group yoga program.

Educational needs of patients and caregivers living with sickle cell disease results in development of web-based patient decision aid.

Aims: We performed an assessment to understand perceived decisional needs among those living with sickle cell disease. Additionally, we desired understanding of their preferred methods and quality of learning and sought guidance in development of a web-based patient decision aid.

Design: The purpose of this qualitative study was to determine ways patients and caregivers receive education about sickle cell disease and available therapies.

Primary caregiver decision-making in hematopoietic cell transplantation and gene therapy for sickle cell disease.

Background: Improved outcomes and the availability of clinical trials of hematopoietic cell transplantation (HCT) from alternate donors and genetically modified autologous hematopoietic progenitor cells have expanded the applicability of HCT for sickle cell disease (SCD). To understand the perspective of primary caregivers exploring HCT in the current milieu, we asked the research question "What motivates primary caregivers to decide to consider HCT and to seek, and to attend, an HCT consultation?"

Procedures: We conducted qualitative interviews with primary caregivers within one week after a consultation for HCT for SCD. Data were analyzed using open and axial coding stages of grounded theory methodology.

Assessment of Patient and Caregiver Attitudes and Approaches to Decision-Making Regarding Bone Marrow Transplant for Sickle Cell Disease: A Qualitative Study.

Importance: Bone marrow transplant (BMT) is a potentially curative treatment for sickle cell disease (SCD). Patient and caregiver attitudes toward BMT for SCD and the willingness to accept risks of BMT vary, but these attitudes are not well understood.

Objective: To understand patient and caregiver perceptions of and attitudes toward BMT for SCD and decision-making about BMT.

Patient and family experience with chronic transfusion therapy for sickle cell disease: A qualitative study.

Background: There is a limited understanding of the patient and family experience of Chronic Transfusion Therapy (CTT) for prevention of complications of Sickle Cell Disease (SCD). We sought to understand patient and family experience with CTT using qualitative methods.

Methods: Fifteen parents of children < 18 years old and nine children 12-18 years old with SCD who were receiving CTT for > 1 year were interviewed using a semi-structured interview format, and interviews were analyzed using open coding methods.

Comparative Effectiveness of a Web-Based Patient Decision Aid for Therapeutic Options for Sickle Cell Disease: Randomized Controlled Trial.

Background: Hydroxyurea, chronic blood transfusions, and bone marrow transplantation are efficacious, disease-modifying therapies for sickle cell disease but involve complex risk-benefit trade-offs and decisional dilemma compounded by the lack of comparative studies. A patient decision aid can inform patients about their treatment options, the associated risks and benefits, help them clarify their values, and allow them to participate in medical decision making.

Objective: The objective of this study was to develop a literacy-sensitive Web-based patient decision aid based on the Ottawa decision support framework, and through a randomized clinical trial estimate the effectiveness of the patient decision aid in improving patient knowledge and their involvement in decision making.

Management of Chronic Pain in Adults Living With Sickle Cell Disease in the Era of the Opioid Epidemic: A Qualitative Study.

Importance: The hallmark of sickle cell disease (SCD) is vaso-occlusive pain that may be acute and episodic or may progress to chronic, persistent pain with unpredictable and disabling exacerbations. Patients with SCD rely on opioids almost exclusively for acute and chronic pain management.

Objective: To understand how the current opioid epidemic and subsequent guidelines from the Centers for Disease Control and Prevention are associated with the management of acute and chronic pain for patients with SCD.

A Qualitative Study of Chronic Pain and Self-Management in Adults with Sickle Cell Disease.

Acute, intermittent vaso-occlusive pain is the hallmark of sickle cell disease (SCD) and is associated with substantial morbidity and impaired quality of life (QOL). The subgroup of adults with SCD who transition from recurrent, acute pain to chronic, persistent pain have even greater QOL impairment and higher rates of healthcare utilization. Self-management is central to SCD management; however, its role in chronic pain management is not established.

From trust to skepticism: An in-depth analysis across age groups of adults with sickle cell disease on their perspectives regarding hydroxyurea.

Despite its efficacy, the uptake of HU in adults with sickle cell disease (SCD) is poor likely due to a combination of system, provider, and patient-related factors. We investigated attitudes of adult patients towards HU by conducting qualitative interviews with 95 adult SCD patients (age 18 to 67 years old, 71 were female). While 53% of all participants reported that they were currently taking HU, patients ranging in age 18-30 years (Group 1) were more likely to report current HU use as compared to those (Group 2) ranging in age 31-67 years (65% vs.

Experiences and Decision Making in Hematopoietic Stem Cell Transplant in Sickle Cell Disease: Patients' and Caregivers' Perspectives.

Sickle cell disease (SCD) is one of the most commonly inherited hemoglobin disorders that has a significant impact on quality of life, increased childhood morbidity, and premature mortality. Currently, hematopoietic stem cell transplant (HSCT) is the only treatment with a curative intent. The objective of this study was to determine patients' and caregivers' knowledge of HSCT, the factors influencing the decision to pursue HSCT, their experiences, and the impact of a successful HSCT on their daily living.

Proponent or collaborative: Physician perspectives and approaches to disease modifying therapies in sickle cell disease.

Sickle cell disease (SCD) is an inherited blood disorder that primarily affects African-American and other ethnic minority populations. There are three available disease-modifying therapies for sickle cell disease: hydroxyurea (HU), bone marrow transplantation (BMT), and chronic blood transfusion (CBT). Since these treatments vary in their therapeutic intent, efficacy in preventing progression of the disease, short and long-term adverse effects, costs and patient burden, the decision-making process regarding these therapies is complicated for both the patient and healthcare provider.