Advance Directives for Adolescents and Young Adults Living With Neuromuscular Disease: An Integrative Review of the Literature.

The use of advance directives is an important component in helping individuals living with chronic and/or life-threatening illnesses establish goals of care and make decisions regarding care at the end of life. Advance care planning may help achieve enhanced health outcomes, yet it is not routinely offered to adolescents/young adults living with neuromuscular disease. An integrative review of the literature was conducted to examine the evidence related to the use of advance directives with adolescents/young adults living with neuromuscular disease and to identify reasons why they are not being used and how this can be improved. Three-hundred-seven studies were retrieved from PubMed, CINAHL, and EMBASE. Five studies met the final inclusion search criteria and were included in the analysis. Four themes emerged from the literature: conversations about advance directives with adolescents/young adults with neuromuscular disease are not being conducted, only a small number of patients have documented advance directives, patients want to have conversations about goals of care and want to have them sooner, and there is a lack of evidence in this area. These findings may influence neuromuscular clinicians' practice surrounding the use of advance directives and increase their knowledge regarding the need for discussions regarding goals of care.

Implementation of a Follow-Up System for Pediatric Sepsis Survivors in a Large Academic Pediatric Intensive Care Unit.

Background: Survivors of pediatric sepsis often develop new morbidities and deterioration in quality of life after sepsis, leading to a need for improved follow-up for children who survive sepsis. Objective: To implement a follow-up system for pediatric sepsis survivors in a pediatric health system. Methods: We performed a retrospective case series of patients treated for sepsis from October 2018 through October 2019 in a pediatric intensive care unit in a quaternary children's hospital, and describe implementation of a follow-up system for sepsis survivors. Program planning started in 2017 with multidisciplinary meetings including physical, occupational, and speech therapists, teachers, neuropsychologists, and coordinators from other survivorship programs (neonatology, stroke, and oncology). In 2018, a workshop was held to consult with local and national experts. The Pediatric Sepsis Survivorship Program launched in October 2018 led by a nurse coordinator who met with families to educate about sepsis and offer post-discharge follow-up. Patients with high pre-existing medical complexity or established subspecialty care were referred for follow-up through existing care coordination or subspecialty services plus guidance to monitor for post-sepsis morbidity. For patients with low-moderate medical complexity, the nurse coordinator administered a telephone-based health-assessment 2-3 months after discharge to screen for new physical or psychosocial morbidity. Patients flagged with concerns were referred to their primary physician and/or to expedited neuropsychological evaluation to utilize existing medical services. Results: Of 80 sepsis patients, 10 died, 20 were referred to care coordination by the program, and 13 had subspecialty follow-up. Five patients were followed in different health systems, four were adults not appropriate for existing follow-up programs, four remained hospitalized, and four were missed due to short stay or unavailable caregivers. The remaining 20 patients were scheduled for follow-up with the Pediatric Sepsis Program. Nine patients completed the telephone assessment. Four patients were receiving new physical or occupational therapy, and one patient was referred for neuropsychology evaluation due to new difficulties with attention, behavior, and completion of school tasks. Conclusions: Implementation of an efficient, low-cost pediatric sepsis survivorship program was successful by utilizing existing systems of care, when available, and filling a follow-up gap in screening for select patients.

Thromboprophylaxis in a pediatric hospital: a patient-safety and quality-improvement initiative.

OBJECTIVES: Venous thromboembolism (VTE) prophylaxis for patients at risk is often overlooked in pediatric health care institutions, which provides an opportunity to improve patient care. The objectives of this study were to review our current hospital practice, identify a population at high risk, and formulate institutional guidelines for thromboprophylaxis. METHODS: This was a prospective patient-safety and quality-improvement project performed at a large pediatric tertiary care hospital. We developed criteria for assessing risk and selecting prophylactic intervention through analysis of the age distribution and underlying medical conditions of patients with VTE at our center, literature review of adult recommendations for thromboprophylaxis, and consensus opinion of multiple specialists at our institution. A patient-care policy was developed to assess VTE risk and prescribe the appropriate thromboprophylaxis regimen. The primary outcome measure was compliance with thromboprophylaxis guidelines in patients at risk for VTE. RESULTS: Over the 4-year study period, the observed rate of VTE prophylaxis in patients at risk increased from a baseline of 22% to an average rate of 82%, and there were intermittent improvements up to 100%. CONCLUSIONS: Although some of the details may vary from center to center, many of the discussed principles and practices involved in instituting a VTE-prevention program are applicable to other pediatric institutions. Despite the fact that the risk of VTE in hospitalized children is much lower than that in adults, there are patients in pediatric hospitals who deserve systematic screening and thoughtful application of preventative measures.

Endocrine problems in critically ill children: an overview.

The endocrine system maintains a delicate balance of physiologic processes including growth and sexual maturation, energy production and utilization, fluid and electrolyte balance, and circulatory function. Although endocrine regulation of growth and sexual maturation is a significant issue in general pediatrics, disorders of energy production and utilization, fluid and electrolyte balance, and circulatory function are the endocrine causes of critical illness in children. Care of the child with critical endocrine disease requires an understanding of endocrine pathophysiology, keen history taking and assessment skills, and knowledge of the pharmacology of synthetic hormone treatment. This article will provide an overview of common endocrine problems encountered in critically ill children with attention to endocrine problems that are unique to pediatrics and that may pose diagnostic and treatment dilemmas for healthcare providers without experience or education in pediatric critical care.

Obstructive sleep apnea syndrome in children.

Obstructive sleep apnea syndrome is characterized by recurrent episodes of partial or complete obstruction of the upper airway during sleep. This results in the disruption of normal ventilation and sleep patterns. The symptoms, polysomnographic findings, pathophysiology, and treatment of obstructive sleep apnea syndrome are significantly different in children from those seen in adults.

Challenges of advanced practice nursing in pediatric acute and critical care: education to practice.

The APN role of the future is dependent on our ability to document through research that NPs, CNSs, and the consolidated role of the NP/CNS plays a critical role in the delivery of high quality cost-effective care. Further information is needed regarding how the APN contributes to and enhances the care delivered by the healthcare team. Cost effectiveness and quality outcome studies are needed including those that describe morbidity and mortality rates, patient satisfaction, and cost effectiveness of models of care that includes APNs. Brooten and Naylor suggest the inclusion of sensitive nursing outcomes, including functional status, mental status, stress level, satisfaction with care, caregiver burden, cost of care. Defining and clarifying the APN functions and qualities of scope of practice is imperative. Perhaps there are populations best served by APNs. Contributions such as continuity, consistency of care, attention to issues such as immobility, skin integrity, and health promotion may have a value added effect. Time motion studies and process logs may add to the information about APNs in pediatric acute and critical care. Professional certification validating competence is essential for the practice of APNs caring for sick children and their families. A disparity exists between the primary care examination now available and the practice of NPs in pediatric acute and critical care. A certification examination is needed with content consistent with the practice of pediatric acute care NP. APNs must possess sufficient knowledge and skill to meet the needs of patients and families in the changing healthcare environment. According to Strodtbeck and colleagues, flexibility, ability to be a self directed learner, critical thinking, relationship skills, and leadership skills including interpersonal insight, interpersonal competence, and ability to stimulate group discussion will serve APNs well as they move into the century. Transitioning brings exciting opportunities along with challenges. Using a blend of abilities, the pediatric acute care APN can provide optimal care to sick children and families.