Key Psychosocial Health Outcomes and Association With Resilience Among Patients With Adult Congenital Heart Disease.

Background: Adult congenital heart disease (ACHD) can negatively impact quality of life (QOL). Strengthening resilience may improve this and other psychosocial outcomes important for living a meaningful life.

Objectives: The purpose of this study was to describe resilience and key psychosocial health outcomes in ACHD and evaluate the associations between resilience and these outcomes.

Costs and impact of disease in adults with sickle cell disease: a pilot study.

We assessed the feasibility to estimate illness burden in adults with SCD, investigated factors associated with health-related quality of life (HRQoL), and estimated societal burden. We recruited 32 participants and collected data on fatigue, HRQoL, and work productivity and activity impairment via patient survey. Health care utilization was abstracted for the 12 months before enrollment using medical chart review.

A randomised controlled trial of a nurse facilitator to promote communication for family members of critically ill patients.

Purpose: Suboptimal communication with clinicians, fragmented care and failure to align with patients' preferences are determinants of post intensive care unit (ICU) burden in family members. Our aim was to evaluate the impact of a nurse facilitator on family psychological burden.

Methods: We carried out a randomised controlled trial in five ICUs in France comparing standard communication by ICU clinicians to additional communication and support by nurse facilitators.

Test-retest reliability of a mobile application of the patient reported outcomes burdens and experiences (PROBE) study.

Introduction: The Patient Reported Outcomes, Burdens, and Experiences (PROBE) questionnaire is a patient-reported outcome tool that assesses quality of life and disease burden in people with haemophilia (PWH).

Aim: To assesses the test-retest reliability of PROBE when completed using the mobile phone application.

Methods: We recruited PWH, including carriers, and individuals with no bleeding disorders who attended haemophilia-related workshops or via social media.

Qualitative Analysis of Perspectives on Lung Transplant among People with Cystic Fibrosis.

Lung transplant (LTx) is a potentially lifesaving treatment option for individuals with advanced cystic fibrosis (CF), but more people with CF (PwCF) and advanced lung disease die each year than undergo transplant in the United States. Little is known about these individuals' LTx information needs and factors influencing their decision-making process related to transplant. To examine PwCF's experiences with and preferences for provision of LTx information and to identify transplant information needs that CF clinicians are well positioned to address.

Definitions of resilience and resilience resource use as described by adults with congenital heart disease.

Background: Adult congenital heart disease (ACHD) is a lifelong illness that presents ongoing challenges to quality of life. Fostering personal resilience resources to sustain well-being can enhance patients' psychosocial health.

Objective: We aimed to describe patients' resilience experiences: how they understand, develop, and utilize resilience resources in managing ACHD.

Perspectives About Racism and Patient-Clinician Communication Among Black Adults With Serious Illness.

Importance: Black patients with serious illness experience higher-intensity care at the end of life. Little research has used critical, race-conscious approaches to examine factors associated with these outcomes.

Objective: To investigate the lived experiences of Black patients with serious illness and how various factors may be associated with patient-clinician communication and medical decision-making.

Hospital Culture and Intensity of End-of-Life Care at 3 Academic Medical Centers.

Importance: There is substantial institutional variability in the intensity of end-of-life care that is not explained by patient preferences. Hospital culture and institutional structures (eg, policies, practices, protocols, resources) might contribute to potentially nonbeneficial high-intensity life-sustaining treatments near the end of life.

Objective: To understand the role of hospital culture in the everyday dynamics of high-intensity end-of-life care.

Resilient together-ALS: leveraging the NDD transdiagnostic framework to develop an early dyadic intervention for people with amyotrophic lateral sclerosis and their informal care-partners.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive weakness and eventual death, usually within 3-5 years. An ALS diagnosis is associated with substantial emotional distress for both the affected person and their family care-partners which impairs the ability to engage in important conversations about long term care planning, negatively impacts ALS symptoms for the patient, and quality of life for both patient and care-partner. Here we 1) discuss published works identified by the authors about psychosocial interventions for the ALS population, 2) identify a lack of early, dyadic interventions to support psychosocial needs of people with ALS and care-partners; 3) describe the Neurodegenerative Diseases (NDD) framework for early dyadic intervention development and 4) propose an adaptation of an evidence-based early dyadic psychosocial intervention, Recovering Together, for the unique needs of people with ALS and their care-partners (Resilient Together-ALS; RT-ALS) using the NDD framework.

Intervention to Promote Communication About Goals of Care for Hospitalized Patients With Serious Illness: A Randomized Clinical Trial.

Importance: Discussions about goals of care are important for high-quality palliative care yet are often lacking for hospitalized older patients with serious illness.

Objective: To evaluate a communication-priming intervention to promote goals-of-care discussions between clinicians and hospitalized older patients with serious illness.

Design, Setting, And Participants: A pragmatic, randomized clinical trial of a clinician-facing communication-priming intervention vs usual care was conducted at 3 US hospitals within 1 health care system, including a university, county, and community hospital.

Identification of Palliative Care Needs and Mental Health Outcomes Among Family Members of Patients With Severe Acute Brain Injury.

Importance: Family members of patients with severe acute brain injury (SABI) are at risk for poor psychological outcomes.

Objective: To explore the utility of the early use of a palliative care needs checklist in identifying care needs of patients with SABI and family members who are at risk of poor psychological outcomes.

Design, Setting, And Participants: This prospective cohort study included patients with SABI in an intensive care unit (ICU) for 2 days or more and a Glasgow Coma Scale score of 12 or lower and their family members.

Race-conscious serious illness communication: An interpersonal tool to dismantle racism in practice and research.

Background: Racism significantly contributes to inequitable care quality and outcomes for people of color with serious illness, their families, and their communities. Clinicians use serious illness communication (SIC) to foster trust, elicit patients' needs and values, and deliver goal-concordant services. Current SIC tools do not actively guide users to incorporate patients' experiences with racism into care.

Do They Have a Choice? Surrogate Decision-Making After Severe Acute Brain Injury.

Objectives: In the early phase of severe acute brain injury (SABI), surrogate decision-makers must make treatment decisions in the face of prognostic uncertainty. Evidence-based strategies to communicate uncertainty and support decision-making are lacking. Our objective was to better understand surrogate experiences and needs during the period of active decision-making in SABI, to inform interventions to support SABI patients and families and improve clinician-surrogate communication.

Value Placed on Comfort vs Life Prolongation Among Patients Treated With Maintenance Dialysis.

Importance: Patients receiving maintenance dialysis experience intensive patterns of end-of-life care that might not be consistent with their values.

Objective: To evaluate the association of patients' health care values with engagement in advance care planning and end-of-life care.

Design, Setting, And Participants: Survey study of patients who received maintenance dialysis between 2015 and 2018 at dialysis centers in the greater metropolitan areas of Seattle, Washington, and Nashville, Tennessee, with longitudinal follow-up of decedents.

Building the foundation for a community-generated national research blueprint for inherited bleeding disorders: research priorities in health services; diversity, equity, and inclusion; and implementation science.

Background: The National Hemophilia Foundation (NHF) conducted extensive all-stakeholder inherited bleeding disorder (BD) community consultations to inform a blueprint for future research. Sustaining and expanding the specialized and comprehensive Hemophilia Treatment Center care model, to better serve all people with inherited BDs (PWIBD), and increasing equitable access to optimal health emerged as top priorities.

Research Design And Methods: NHF, with the American Thrombosis and Hemostasis Network (ATHN), convened multidisciplinary expert working groups (WG) to distill priority research initiatives from consultation findings.

Building the foundation for a community-generated national research blueprint for inherited bleeding disorders: facilitating research through infrastructure, workforce, resources and funding.

Background: The National Hemophilia Foundation (NHF) conducted extensive, inclusive community consultations to guide prioritization of research in coming decades in alignment with its mission to find cures and address and prevent complications enabling people and families with blood disorders to thrive.

Research Design And Methods: With the American Thrombosis and Hemostasis Network, NHF recruited multidisciplinary expert working groups (WG) to distill the community-identified priorities into concrete research questions and score their feasibility, impact, and risk. WG6 was charged with identifying the infrastructure, workforce development, and funding and resources to facilitate the prioritized research.

Trends in prescribing practices for management of haemophilia: 1999-2021.

Introduction: People with haemophilia rely on specialists for their care, yet the specific dosing regimens of treatments prescribed by these specialists have not been widely studied.

Aim: The objective of this study is to describe trends in clinician prescribing practices for the management of haemophilia in the United States (US).

Methods: We administered surveys to members of the Hemostasis and Thrombosis Research Society via paper surveys at its in-person annual symposia in 1999 and 2015, and an online survey in 2021.

Assessment of Natural Language Processing of Electronic Health Records to Measure Goals-of-Care Discussions as a Clinical Trial Outcome.

Importance: Many clinical trial outcomes are documented in free-text electronic health records (EHRs), making manual data collection costly and infeasible at scale. Natural language processing (NLP) is a promising approach for measuring such outcomes efficiently, but ignoring NLP-related misclassification may lead to underpowered studies.

Objective: To evaluate the performance, feasibility, and power implications of using NLP to measure the primary outcome of EHR-documented goals-of-care discussions in a pragmatic randomized clinical trial of a communication intervention.

A Patient Decision Aid About Conservative Kidney Management in Advanced Kidney Disease: A Randomized Pilot Trial.

Rationale & Objective: Available decision aids for patients about treatment of advanced chronic kidney disease (CKD) often lack information on conservative kidney management (CKM). We assessed the feasibility and acceptability of a decision aid on CKM among patients with advanced CKD and their family members.

Study Design: Randomized pilot trial.

Humanistic burden of problem joints for children and adults with haemophilia.

Introduction: The "problem joint" (PJ) concept was developed to address patient-centric needs for a more holistic assessment of joint morbidity for people with haemophilia (PwH).

Aim: To quantify the humanistic burden of PJs in PwH to further support validation of the PJ outcome measure.

Methods: Multivariable regression models evaluated the relationship between PJs and health-related quality of life (HRQoL, EQ-5D-5L) and overall work productivity loss (WPL) using data from the 'Cost of HaEmophilia: a Socioeconomic Survey' population studies (adults: CHESS II, CHESS US+; children/adolescents: CHESS-Paeds).

A World of Maximalist Medicine: Physician Perspectives on Palliative Care and End-of-life for Patients With Pulmonary Arterial Hypertension.

Context: Physicians who specialize in pulmonary arterial hypertension (PAH) care for patients facing a serious, life-limiting illness. Palliative care is underutilized in patients with PAH, and little is known about how best to provide palliative care to this patient population.

Objectives: Using a qualitative approach, assess physicians' perspectives on barriers and facilitators to the use of palliative care in PAH.

Depression and anxiety in persons with Von Willebrand disease.

Objective: We assessed sociodemographic and clinical characteristics associated with depression and anxiety in individuals with Von Willebrand disease (VWD) aged ≥12 years.

Methods: The study collected data on patients' sociodemographic, joint problems and health-related quality of life (HRQoL) using EQ-5D-3L, 8-item patient health questionnaire for depression and 7-item Generalized Anxiety Disorder Questionnaire from participants in seven geographically diverse US haemophilia treatment centres.

Results: Analyses included 77 participants.

One Year Later: Family Members of Patients with COVID-19 Experience Persistent Symptoms of Posttraumatic Stress Disorder.

Family members of critically ill patients with coronavirus disease (COVID-19) have described increased symptoms of posttraumatic stress disorder (PTSD). Little is known about how these symptoms may change over time. We studied changes in PTSD symptoms in family members of critically ill patients with COVID-19 over 12 months.

Predictors of Documented Goals-of-Care Discussion for Hospitalized Patients With Chronic Illness.

Context: Goals-of-care discussions are important for patient-centered care among hospitalized patients with serious illness. However, there are little data on the occurrence, predictors, and timing of these discussions.

Objectives: To examine the occurrence, predictors, and timing of electronic health record (EHR)-documented goals-of-care discussions for hospitalized patients.