Self-management Needs of Adults With Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease (CHD) are an emerging adult heart disease subset, now outnumbering the pediatric population with CHD. OBJECTIVE: We aimed to gain understanding and knowledge of what adults with CHD perceive as important for self-management and describe these needs across demographic factors, developmental characteristics, lesion severity, and quality of life. METHODS: We used a descriptive mixed-methods online survey merging 4 instruments: Adult CHD Self-management Experience Questionnaire; Adult CHD Demographic Questionnaire; Adaptive Behavior Assessment System, Third Edition; and Stanford Quality of Life Visual Numeric. Participants with CHD 18 to 30 years of age with initial defect repair before 12 months of age were recruited through support from the Adult Congenital Heart Association, clinic adult CHD support groups, and newspaper advertising. Thematic analysis for short-answer questions, descriptive analysis for demographic data and the visual numeric, and intrument-specific scoring assistant software for the Adaptive Behavior Assessment System were used. RESULTS: We received 22 responses from 13 women and 9 men. These individuals represented 15 different heart defect diagnoses, mostly of moderate or complex lesion severity. Most had postsecondary education and were employed. Four prominent themes emerged related to self-management: desire for connectivity-psychological support; a plan for the future-education about health and life expectations; coping needs-skills for mental stress; and access to care-navigation of healthcare systems. CONCLUSIONS: Future longitudinal research and replication studies with larger samples are needed. Educational materials and targeted interventions that promote self-management benefit the aging adult with CHD population.

Societal value of newborn screening for severe combined immune deficiency in Arkansas: An economic analysis.

Newborn screening (NBS) is a public health program that detects genetic conditions in neonates enabling treatment before clinical symptoms manifest. Severe combined immune deficiency (SCID) is a primary immune deficiency found in the absence of functioning T and B lymphocytes. Hematopoietic cell transplantation is a potentially curative treatment if received within the first 42 months of life; without treatment, this condition is fatal in the first 2 years of life due to severe opportunistic infections. SCID was added to the recommended uniform panel of conditions for inclusion in state NBS programs in 2010. This manuscript examines the societal costs and benefits of NBS for SCID in Arkansas and implications to health services and social welfare. Total cost per year of all NBS for SCID and resulting early treatment for one patient with SCID in Arkansas is estimated at $1,078,714. Cost of late treatment of one patient with SCID is estimated at $1.43 million. Based on an expected diagnosis of one patient per year in Arkansas, this results in an estimated net cost savings for NBS for SCID in Arkansas of $351,286 per year. Based on cost-effectiveness analysis, NBS for SCID in Arkansas is cost-effective, with higher societal benefit than cost.

Infective endocarditis: call for education of adults with CHD: review of the evidence.

Advanced surgical repair procedures have resulted in the increased survival rate to adulthood of patients with CHD. The resulting new chronic conditions population is greater than one million in the United States of America and >1.2 million in Europe. This review describes the risks and effects of infective endocarditis - a systemic infectious process with high morbidity and mortality - on this population and examines the evidence to determine whether greater patient education on recognition of symptoms and preventative measures is warranted. The literature search included the terms "infective endocarditis" and "adult congenital heart disease". Search refinement, the addition of articles cited by included articles, as well as addition of supporting articles, resulted in utilisation of 24 articles. Infective endocarditis, defined by the modified Duke Criteria, occurs at a significantly higher rate in the CHD population due to congenitally or surgically altered cardiac anatomies and placement of prosthetic valves. This literature review returned no studies in the past five years assessing knowledge of the definition, recognition of symptoms, and preventative measures of infective endocarditis in the adult CHD population. Existing data are more than 15 years old and show significant knowledge deficits. Studies have consistently shown the need for improved CHD patient knowledge with regard to infective endocarditis, and there is no recent evidence that these knowledge deficits have decreased. It is important to address and decrease knowledge deficits in order to improve patient outcomes and decrease healthcare utilisation and costs.

Leadership, health systems and policy: Doctoral education and integrated clinical application.

Scientifically rigorous and readily transferable nursing research as a foundation for development of sound local and national policy can be further delivered into the policy arena by PhD prepared nurse leaders. Core curricular elements in the preparation of nurse scientists to advance the profession must therefore include competencies in leadership, health care systems, health economics, and policy. We present a curriculum model from a university in the southern United States that includes both a theoretical and application approach to incorporate a Leadership in Health Care Systems Course and Field Experience. Implementation of this approach is thought to result in a nurse scientist to have better preparation to engage in and with the broader health care system, policy, and interprofessional collaboration more quickly. We then present a student exemplar of a doctoral student's leadership field experience in a national advocacy and legislative process and discuss educational and professional gains from this lived experience.