Professional Standards and the Role of the Advanced Practice Registered Nurse in Hospice and Palliative Care.

Palliative care aims to optimize quality of life and reduce physical, psychological, social, and spiritual suffering for people living with serious and life-threatening illness throughout the life span. There are different educational pathways to becoming an advanced practice registered nurse (APRN) and a range of areas in which APRNs may specialize, including hospice and palliative care. National guidelines and professional standards have been developed to guide the delivery of high-quality palliative care and to demonstrate the need for all nurses to be competent in providing primary palliative care. Advanced practice registered nurses are well poised to integrate palliative care standards into their practice in a variety of settings and in myriad ways including clinical care, program development, leadership, education, and advocacy. This article is the first in a series that will highlight the different roles of the hospice and palliative care APRN, including the doctor of nursing practice, clinical nurse specialist, pediatric APRN, adult/gerontology APRN, and community-based APRN in a variety of settings throughout the country.

Outcomes of a National Training Curriculum to Advance Generalist Level Palliative Care.

Background: The adoption of palliative care as an integral component of health care has led to the need for generalist level providers, especially important in serious illnesses such as cancer. Objectives: The goals of this National Cancer Institute-funded training program were to (1) identify the eight domains of quality palliative care applied to oncology practice, (2) demonstrate skills for oncology advanced practice registered nurses (APRNs) in the domains of palliative care, and (3) develop goals for implementing the skills training in practice through process improvement, staff education, and clinical care. Design: The training program led by the End of Life Nursing Education Consortium (ELNEC) project included oncology APRNs in a three-day training course with one-year follow-up for ongoing support and to assess impact. Settings: Five training courses included 430 APRNs from 46 U.S states including both pediatric and adult oncology settings. The project included 25% minority participants. Measurement: Measures included participant goal implementation, course evaluations, and surveys to assess implementation and palliative care practices (precourse, 6 and 12 months postcourse). Results: The ELNEC oncology APRN training course resulted in changes in practice across domains, improved perceived effectiveness in clinical practice, and valuable insight regarding the challenges in generalist level palliative care implementation. Conclusion: The ELNEC oncology APRN course serves as a model for the palliative care field to advance generalist level practice. Future training efforts can build on this project to reach more oncology professionals and those in other areas of serious illness care.

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.

Using the Nurse Coaching Process to Support Bereaved Staff During the COVID-19 Crisis.

Nurses are confronting a number of negative mental health consequences owing to high burdens of grief during COVID-19. Despite increased vaccination efforts and lower hospitalization and mortality rates, the long-term effects of mass bereavement are certain to impact nurses for years to come. The nurse coaching process is an evidence-based strategy that nurse leaders can use to assist staff in mitigating negative mental health outcomes associated with bereavement. The End-of-Life Nursing Education Consortium brought together a team of palliative nursing experts early in the pandemic to create resources to support nurses across settings and promote nurse well-being. This article shares a timely resource for health systems and nursing administration that leverages the nurse coaching process to support bereaved staff in a safe and therapeutic environment.

Primary Palliative Care Clinical Implications: Oncology Nursing During the COVID-19 Pandemic.

The COVID-19 pandemic continues to affect the health and well-being of individuals and communities worldwide. Patients with cancer are particularly vulnerable to experiencing serious health-related suffering from COVID-19. This requires oncology nurses in inpatient and clinic settings to ensure the delivery of primary palliative care while considering the far-reaching implications of this public health crisis. With palliative care skills fully integrated into oncology nursing practice, health organizations and cancer centers will be better equipped to meet the holistic needs of patients with cancer and their families receiving care for serious illness, including improved attention to physical, psychosocial, cultural, spiritual, and ethical considerations.

Clinical Experience With Gene Therapy in Older Patients With Spinal Muscular Atrophy.

BACKGROUND: Onasemnogene abeparvovec was recently approved for the treatment of spinal muscular atrophy (SMA) in children younger than two years; however, clinical trials were primarily completed in children younger than seven months, so practical experience dosing older children began in summer 2019. Here, we look at the safety and efficacy of onasemnogene in seven infants older than seven months who were treated at our center. METHODS: Seven patients were included. RESULTS: Acute viral symptoms with emesis and/or fever were seen in six of seven patients two to three days after the infusion. Thrombocytopenia occurred in four of seven patients, and six of seven patients had prolonged steroid courses due to persistently elevated liver enzymes, one of whom required escalation to intravenous steroids. All patients demonstrated motor improvements, which were apparent by three months, although with continued progress in those patients followed for longer periods of time. CONCLUSIONS: Overall, onasemnogene appears to be efficacious in children older than seven months and well tolerated. Side effects were similar to those previously reported, although more common and in some cases more severe and more prolonged than seen in the original trials. The impact of age, weight, and other confounding factors on development of side effects still needs to be elucidated.

Palliative Nursing Summit: Nurses Leading Change and Transforming Primary Palliative Care: Nursing's Role in Providing Pain and Symptom Management.

Effective pain and symptom management is a crucial part of the care of those with life-threatening illnesses and their family members. Nurses in all settings have a vital role in providing this essential care. A recent summit held on May 12, 2017, "Nurses Leading Change and Transforming Palliative Care," convened by the Hospice and Palliative Nurses Association and attended by leaders of 26 nursing organizations strongly endorsed the importance of nursing's role in pain and symptom management and developed an agenda to advance these efforts. The agreed-upon goals of pain and symptom treatment include care that is effective (as defined by the patient), efficient (delivered in a timely manner), and safe (reduced risk of respiratory depression, prevention and early management of adverse effects, as well as attention to methods to prevent diversion such as safe storage and disposal). Through an iterative process, desired patient outcomes and nursing actions were outlined to support effective pain and symptom management in primary palliative care. These recommendations were categorized as education, clinical care, research, and regulatory concerns. This article reports on the outcomes of this summit related to pain and symptom management.

Nurses' Experiences of Spiritual Communication with Seriously III Children.

OBJECTIVE: The goal of this study was to explore nurse experiences in communication with children about spiritual topics in order to develop training in this area. BACKGROUND: Although spiritual care is essential in pediatric palliative care, few providers receive training about communication with ill children about spirituality. METHODS: Researchers developed a brief survey to prompt nurses to reflect on pediatric palliative care experiences that included spiritual discussions. Nurses attending training courses voluntarily submitted stories. Qualitative data were thematically analyzed by members of the research team, consisting of two researchers with expertise in palliative care, spirituality, and communication and two expert pediatric palliative care clinicians. RESULTS: Nurses' spiritual conversations with children revealed that children question God and the reason for their illness, have a desire to talk about the afterlife as a way of understanding their limited lifespan, and to share descriptions of an afterlife, in these cases described as heaven. Nurses conveyed the importance of being present and engaging in spiritual communication with children. DISCUSSION: Communication training is needed and should prepare providers to respond to a child's spiritual questioning, assist parents when the child initiates discussion about the afterlife, and help parent and child understand the spiritual meaning of their illness. Chaplains serve as spiritual care experts and can help train nurses to screen for spiritual distress, have greater competence in spiritual communication, and to collaborate with chaplains in care. Quality palliative care is incomplete without attention to spiritual care.

Exploring the spiritual needs of families with seriously ill children.

Although we know that families of seriously ill children experience spiritual distress, especially at the end of the child's life, there is little information on the specific spiritual needs of families. In order to develop further training for nurses in paediatrics and help nurses develop skills for communicating about spirituality, this research examined the spiritual needs of families based on nurses' experiences with families of seriously ill children. Nurses' experiences revealed that families' anger with God, blame/regret, forgiveness, and ritual and cultural traditions are salient spiritual needs requiring effective nurse communication skills to support families of ill children.

Contributions of palliative care to pediatric patient care.

OBJECTIVES: To provide an overview of pediatric palliative care (PPC) as it relates to children and families living with oncologic disease. DATA SOURCES: Journal articles, clinical research reports, clinical guidelines, and national statistics. CONCLUSION: As new treatment protocols become available, the need for simultaneous supportive PPC, including adequate pain and symptom management, is evident. Further research and PPC program development is necessary for adherence to the current recommendation that PPC should be initiated at the time of diagnosis and continue throughout the course of a child's disease. IMPLICATIONS FOR NURSING PRACTICE: Palliative care nursing holds a specific role in the pediatric oncology setting. Registered nurses and advanced practice nurses should be adequately trained in PPC because they are in an optimal role to contribute to interdisciplinary PPC for pediatric oncology patients and their families.

Prospective cohort study of spinal muscular atrophy types 2 and 3.

OBJECTIVE: To characterize the natural history of spinal muscular atrophy type 2 and type 3 (SMA 2/3) beyond 1 year and to report data on clinical and biological outcomes for use in trial planning. METHODS: We conducted a prospective observational cohort study of 79 children and young adults with SMA 2/3 who participated in evaluations for up to 48 months. Clinically, we evaluated motor and pulmonary function, quality of life, and muscle strength. We also measured SMN2 copy number, hematologic and biochemical profiles, muscle mass by dual x-ray absorptiometry (DXA), and the compound motor action potential (CMAP) in a hand muscle. Data were analyzed for associations between clinical and biological/laboratory characteristics cross-sectionally, and for change over time in outcomes using all available data. RESULTS: In cross-sectional analyses, certain biological measures (specifically, CMAP, DXA fat-free mass index, and SMN2 copy number) and muscle strength measures were associated with motor function. Motor and pulmonary function declined over time, particularly at time points beyond 12 months of follow-up. CONCLUSION: The intermediate and mild phenotypes of SMA show slow functional declines when observation periods exceed 1 year. Whole body muscle mass, hand muscle compound motor action potentials, and muscle strength are associated with clinical measures of motor function. The data from this study will be useful for clinical trial planning and suggest that CMAP and DXA warrant further evaluation as potential biomarkers.

Muscle volume estimation by magnetic resonance imaging in spinal muscular atrophy.

Thigh muscle volume was assessed using magnetic resonance imaging in 16 subjects with spinal muscular atrophy. Scans were successful for 14 of 16 subjects (1 type 1, 6 type 2, and 7 type 3) as young as 5.7 years. Muscle volume with normal and abnormal signal was measured using blinded, semiautomated analysis of reconstructed data. Results were compared with segmental lean mass estimated by dual-energy X-ray absorptiometry and correlated with clinical and electrophysiological measures of disease severity. Muscle volume was reduced with abnormal signal quality. Test-retest reliability (r = .99) and correlation with dual-energy X-ray absorptiometry (r = .91) were excellent. Type 2 subjects had lower volume (3.5 ± 1.6 vs 6.3 ± 2.8 mL/cm height; P = .06) and higher percentage of muscle with abnormal signal (68% ± 20% vs 47% ± 27%; P = .14) than type 3. Reproducibility, tolerability, and strong correlation with clinical measures make magnetic resonance imaging a candidate biomarker for clinical research.

Consensus statement on standard of care for congenital muscular dystrophies.

Congenital muscular dystrophies are a group of rare neuromuscular disorders with a wide spectrum of clinical phenotypes. Recent advances in understanding the molecular pathogenesis of congenital muscular dystrophy have enabled better diagnosis. However, medical care for patients with congenital muscular dystrophy remains very diverse. Advances in many areas of medical technology have not been adopted in clinical practice. The International Standard of Care Committee for Congenital Muscular Dystrophy was established to identify current care issues, review literature for evidence-based practice, and achieve consensus on care recommendations in 7 areas: diagnosis, neurology, pulmonology, orthopedics/rehabilitation, gastroenterology/ nutrition/speech/oral care, cardiology, and palliative care. To achieve consensus on the care recommendations, 2 separate online surveys were conducted to poll opinions from experts in the field and from congenital muscular dystrophy families. The final consensus was achieved in a 3-day workshop conducted in Brussels, Belgium, in November 2009. This consensus statement describes the care recommendations from this committee.

Autonomic symptoms in carriers of the m.3243A>G mitochondrial DNA mutation.

BACKGROUND: The m.3243A>G mutation can cause multisystem medical problems and can affect the autonomic nervous system. OBJECTIVE: To study the frequency and spectrum of autonomic symptoms associated with the m.3243A>G mitochondrial DNA point mutation. Design, Setting, and Patients We studied a cohort of 88 matrilineal relatives from 40 families, including 35 fully symptomatic patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), 53 carrier relatives, and 16 controls using a questionnaire based on existing standard instruments for the evaluation of autonomic dysfunction. We compared the questionnaire with an expert evaluation. We compared data among the 3 groups using the Mantel-Haenszel chi(2) test to determine the statistical significance of differences between groups. RESULTS: Mutation carriers frequently had symptoms of autonomic dysfunction, specifically gastrointestinal and orthostatic intolerance. CONCLUSIONS: Carriers of the m.3243A>G mutation have frequent autonomic symptoms. The m.3243A>G mutation should be considered as an etiological factor in patients with autonomic dysfunction and a medical or family history suggestive of mitochondrial disease. Because some autonomic symptoms are treatable, early detection and proactive management may mitigate the burden of morbidity.

Adiposity is increased among high-functioning, non-ambulatory patients with spinal muscular atrophy.

The relationship between body composition and function in spinal muscular atrophy (SMA) is poorly understood. 53 subjects with SMA were stratified by type and Hammersmith functional motor scale, expanded score into three cohorts: low-functioning non-ambulatory (type 2 with Hammersmith score < 12, n=19), high-functioning non-ambulatory (type 2 with Hammersmith score > or = 12 or non-ambulatory type 3, n=17), and Ambulatory (n=17). Lean and fat mass was estimated using dual-energy X-ray absorptiometry. Anthropometric data was incorporated to measure fat-free (lean mass in kg/stature in m(2)) and fat (fat mass in kg/stature in m(2)) mass indices, the latter compared to published age and sex norms. Feeding dysfunction among type 2 subjects was assessed by questionnaire. Fat mass index was increased in the high-functioning non-ambulatory cohort (10.4+/-4.5) compared with both the ambulatory (7.2+/-2.1, P=0.013) and low-functioning non-ambulatory (7.6+/-3.1, P=0.040) cohorts. 12 of 17 subjects (71%) in the high-functioning non-ambulatory cohort had fat mass index > 85th percentile for age and gender (connoting "at risk of overweight") versus 9 of 19 subjects (47%) in the low-functioning non-ambulatory cohort and 8 of 17 ambulatory subjects (47%). Despite differences in clinical function, a similar proportion of low functioning (7/18, 39%) and high functioning (2/7, 29%) type 2 subjects reported swallowing or feeding dysfunction. Non-ambulatory patients with relatively high clinical function may be at particular risk of excess adiposity, perhaps reflecting access to excess calories despite relative immobility, emphasizing the importance of individualized nutritional management in SMA.

Bioelectrical impedance analysis can be a useful screen for excess adiposity in spinal muscular atrophy.

Accurate, noninvasive measures of body composition are needed for management of patients with spinal muscular atrophy. Fat mass index (fat mass/height(2) in kg/m(2)) was measured in 16 subjects with spinal muscular atrophy using 5 bioelectrical impedance analysis equations and compared with a reference method, dual-energy x-ray absorptiometry. The machine default equation, validated by Cordain, was the primary analysis. Fat mass index calculated by impedance measures differed by between -2.5 kg/m(2) and 1.7 kg/m(2) from the reference mean (8.3 ± 5.0 kg/m(2)). The Cordain equation provided the smallest difference (-0.4 ± 2.0 kg/m(2)), with correlation coefficient of 0.92. The Cordain equation showed high sensitivity (85.7%) and specificity (100%) for prediction of ''at risk for overweight'' (fat mass index > 85th percentile for age and gender). Although insufficiently accurate for use as a research tool, bioelectrical impedance can have application as a well-tolerated, noninvasive, easily used screening tool for excess adiposity in patients with spinal muscular atrophy.

Reliability of telephone administration of the PedsQL Generic Quality of Life Inventory and Neuromuscular Module in spinal muscular atrophy (SMA).

Clinical research visits are challenging for people with SMA because of limited mobility and intercurrent illnesses. Missing data threaten the validity of research results. Obtaining outcomes remotely would represent a solution. To evaluate reliability of telephone administration of the PedsQL Pediatric Generic Core Quality of Life Inventory 4.0 (Generic) and Neuromuscular Module 3.0 (NM) in SMA, we recruited 21 participants of a Natural History Study for telephone administration of both modules no more than 7 days before or after an in-person study visit. We found excellent reliability between telephone and in-person administration of both modules with the NM slightly better than the Generic. Reliability of the child and parent forms was similar. We concluded that both modules can be administered reliably over the telephone to SMA patients and caregivers, expanding the utility of these tools in clinical trials. Notably, telephone administration is reliable in children as young as 8 years.