Empathy Expression in Interpreted and Noninterpreted Care Conferences of Seriously Ill Children
Humans; Child; Cohort Studies; Female; Male; Communication; Prospective Studies; Emotions; Empathy
BACKGROUND AND OBJECTIVES: Clinician empathy is associated with improved
Olszewski AE; Bogetz J; Mercer A; Bradford MC; Scott M; Fields B; Williams K; Rosenberg AR; Trowbridge A
Pediatrics
2023
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1542/peds.2022-059447" target="_blank" rel="noreferrer noopener">10.1542/peds.2022-059447</a>
Palliative care education in an Australian undergraduate pharmacy curriculum: an exploratory descriptive study
Palliative Care; Education; Adolescence; Adult; Female; Human; Male; Questionnaires; Surveys; Australia; Clinical Reasoning; Communication; Confidence; Curriculum; Descriptive Research; Descriptive Statistics; Education Pharmacy; Empathy; Exploratory Research; Mann-Whitney U Test; Prospective Studies; Qualitative Studies; Reflection; Self Concept; Students Undergraduate; Thematic Analysis
Ong JA; Rego J; Ung T; Kumar S; Ash K; Saini B; Yates P
International Journal of Pharmacy Practice
2023
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="https://www.mdpi.com/2227-9067/10/3/482"><span>10.1093/ijpp/riac093</span></a>
Pediatric Palliative Care at Home: A Prospective Study on Subcutaneous Drug Administration
drug administration; Home Care Services; home care services hospital-based; Palliative Care; Pediatrics; Prospective Studies; subcutaneous infusions; supportive care; symptom management
CONTEXT: The subcutaneous route is a useful alternative for drug administration in palliative care. Although there is scientific evidence on its use in adult patients, the literature in pediatric palliative care is almost nonexistent. OBJECTIVES: To describe the experience of a pediatric palliative care unit (PPCU) with in-home subcutaneous drug administration symptom control. METHODS: Prospective observational study of patients receiving home-based subcutaneous treatment administered as part of a PPCU treatment regimen over 16 months. Analysis includes demographic and clinical variables and treatment received. RESULTS: Fifty-four different subcutaneous lines were inserted in the 15 patients included, mainly in the thigh (85.2%). The median time of needle in situ was 5.5 days (range: 1-36 days). A single drug was administered in 55.7% of treatments. The most frequently used drugs were morphine chloride (82%) and midazolam (55.7%). Continuous subcutaneous infusion was the predominant administration route (96.7%), with infusion rates oscillating between 0.1 mL/h and 1.5 mL/h. A statistically significant relationship was found between the maximum infusion rate and induration onset. Of the 54 lines placed, 29 (53.7%) had an associated complication requiring line removal. The primary cause for removal was insertion-site induration (46.3%). Subcutaneous lines were mainly used to manage pain, dyspnea, and epileptic seizures. CONCLUSION: In the pediatric palliative care patients studied, the subcutaneous route is most frequently used for administering morphine and midazolam in continuous infusion. The main complication was induration, especially with longer dwell times or higher infusion rates. However, further studies are required to optimize management and prevent complications.
García-López I; Chocarro-González L; Martín-Romero I; Vázquez-Sánchez JM; Avilés-Martínez M; Martino-Alba R
Journal of Pain and Symptom Management
2023
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.jpainsymman.2023.05.011" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2023.05.011</a>
Context of a neonatal death affects parental perception of end-of-life care, anxiety and depression in the first year of bereavement
Bereavement; Perinatal Death; Terminal Care; Anxiety; Bereavement; Depression; Female; Humans; Infant; Infant Newborn; Parents/px [Psychology]; Perception; Prospective Studies; Terminal Care; Terminal Care/px [Psychology]
BACKGROUND: Neonatal death is often preceded by end-of-life medical decisions. This study aimed to determine whether the context of death - after a decision of withholding or withdrawing life-sustaining treatment (WWLST) or despite maximum care - was associated with subsequent risk of parental anxiety or depression. The secondary objective was to assess parents' perceptions of end-of-life care according to death context., METHODS: Prospective single center observational study of all neonatal deaths in a neonatal intensive care unit over a 5-year period. Data were collected during hospitalization and from face-to-face interviews with parents 3 months after the infant's death. Anxiety and depression were assessed using Hospital Anxiety and Depression Scale (HADS) questionnaires, completed by parents 5 and 15 months after death., RESULTS: Of 179 deaths, 115 (64%) occurred after the WWLST decision and 64 (36%) despite maximum care. Parental satisfaction with newborn care and received support by professionals and relatives was higher in the first condition. Sixty-one percent of parents (109/179) attended the 3-month interview, with the distribution between groups very close to that of hospitalization. The completion rates of the HADS questionnaires by the parents who attended the 3-month interview were 75% (82/109) at 5 months and 65% (71/109) at 15 months. HADS scores at 5 months were consistent with anxiety in at least one parent in 73% (60/82) of cases and with depression in 50% (41/82). At 15 months, these rates were, respectively, 63% (45/71) and 28% (20/71). Risk of depression at 5 months was lower after a WWLST decision (OR 0.35 [0.14, 0.88], p = 0.02). Explicit parental agreement with the WWLST decision had an equivocal impact on the risk of anxiety at 5 months, being higher when expressed during hospitalization, but not at the 3-month interview., CONCLUSIONS: Context of death has a significant impact on the emotional experience of parents after neonatal loss, which underlines the importance of systematic follow-up conversations with bereaved parents. Copyright © 2023. The Author(s).
Cambonie G; Desage C; Thaller P; Lemaitre A; de Balanda KB; Combes C; Gavotto A
BMC Palliative Care
2023
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<a href="http://doi.org/10.1186/s12904-023-01183-8" target="_blank" rel="noreferrer noopener">10.1186/s12904-023-01183-8</a>
Uncertainty and Perinatal Post?Traumatic Stress Disorder in the Neonatal Intensive Care Unit
Male; Female; Infant; Palliative Care; Parents; Infant Newborn; Questionnaires; Prospective Studies; Intensive Care Units Neonatal; Hospitalization; Mental Disorders; Intensive Care Units; Patient Discharge; Data Analysis Software; Human; Descriptive Statistics; Stress Disorders Post-Traumatic; Perinatal Care; Uncertainty; Scales; Psychosocial Factors; Comparative Studies; Coefficient Alpha; Summated Rating Scaling; After Care; Hypothesis; Parent-Infant Relations; Psychologists; Reliability and Validity
Parents of infants in the neonatal intensive care unit (NICU) are at increased risk of developing perinatal post?traumatic stress disorder (PPTSD), a mental health condition known to interfere with healthy parental and infant attachment. Feelings of uncertainty about illness have been theorized as an antecedent to post?traumatic stress, however the relationship has not been explored in parents of infants requiring care in the NICU. The purpose of this prospective study was to explore parental uncertainty during and after NICU discharge and the relationship between uncertainty and PPTSD. The sample consisted of 319 parents during NICU hospitalization and 245 parents at 3 months postdischarge. Parents who screened positive for PPTSD 3 months after hospital discharge reported more uncertainty both while in the NICU and 3 months after hospital discharge (p < 0.001). In parents with a personal or family history of mental illness, the moderated/mediating structural probit analysis showed no direct or indirect effect of uncertainty during hospitalization or at 3 months after hospital discharge on screening positive for PPTSD. In parents who did not report personal or family history of mental illness, uncertainty at 3 months after hospital discharge had a direct effect (b = 0.678, p < 0.001) and indirect mediating effect (b = 0.276, p < 0.001) on screening positive for PPTSD. The results provide actionable implications for mental health and NICU providers: (1) routine screening for uncertainty and risk factors including previous personal and family history of mental illness, and (2) the development of NICU follow?up support services to mitigate risk for PPTSD.
Malin KJ; Johnson TS; Brown RL; Leuthner J; Malnory M; White?Traut R; Rholl E; Lagatta J
Research in Nursing and Health
2022
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<a href="http://doi.org/10.1002/nur.22261" target="_blank" rel="noreferrer noopener">10.1002/nur.22261</a>
Medicinal cannabis in children and adolescents with autism spectrum disorder: A scoping review
Adolescent; anxiety; autism spectrum disorder; Autism Spectrum Disorder; behaviour; Child; Child, Preschool; communication; Humans; hyperactivity; Infant; Infant, Newborn; Medical Marijuana; Parents; Prospective Studies; Retrospective Studies; Medicinal Cannabis
BACKGROUND: Autism spectrum disorder (ASD) is a neurodevelopmental condition estimated to affect 1 in 66 children in Canada and 1 in 270 individuals worldwide. As effective therapies for the management of ASD core and associated symptoms are limited, parents are increasingly turning to clinicians for advice regarding the use of medicinal cannabis to manage behavioural disturbances. OBJECTIVE: The objective of this scoping review was to identify and map symptoms, outcomes and adverse events related to medicinal cannabis treatment for ASD-related behaviours. METHODS: Ovid MEDLINE, Embase, CINAHL, PsycInfo, Web of Science Core Collection, Google Scholar and grey literature sources were searched up to 5 January 2020 for studies. Included studies met the following criteria: (1) investigate the use of medicinal cannabis, (2) at least 50% participants had ASD, (3) at least 50% of the study population was 0-18 years old and (4) any study design (published or unpublished). RESULTS: We identified eight completed and five ongoing studies meeting the inclusion criteria. All studies reported substantial behaviour and symptom improvement on medicinal cannabis, with 61% to 93% of subjects showing benefit. In the three studies reporting on concomitant psychotropic medication usage and with cannabis use, up to 80% of participants observed a reduction in concurrent medication use. Adverse events related to cannabis use were reported in up to 27% of participants related, and two participants had psychotic events. CONCLUSIONS: Early reports regarding medicinal cannabis in paediatric ASD symptom management are presented as positive; the evidence, however, is limited to very few retrospective cohort and observational studies. Evidence of safety and efficacy from prospective clinical trials is needed.
Fletcher S; Pawliuk C; Ip A; Huh L; Rassekh SR; Oberlander TF; Siden H
Child: Care, Health and Development
2022
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<a href="http://doi.org/10.1111/cch.12909" target="_blank" rel="noreferrer noopener">10.1111/cch.12909</a>
A Pilot Study of the Effects of COMPLETE: A Communication Plan Early Through End of Life, on End-of-Life Outcomes in Children With Cancer
child; hospice; Child; Humans; cancer; communication; palliative care; Communication; Terminal Care; Prospective Studies; hospice; Death; Pilot Projects; Hospice Care; goals-of-care; Neoplasms/therapy; cancer; palliative care; communication; Child; goals-of-care
Context: Most children with cancer die in hospital settings, without hospice, and many suffer from high-intensity medical interventions and pain at end of life (EOL). Objective(s): To examine the effects of COMPLETE: a communication plan early through EOL to increase hospice enrollment in children with cancer at EOL. Method(s): This is a two-phase, single-arm, two-center, and prospective pilot study of hospice enrollment in children with cancer whose parents received COMPLETE. COMPLETE is a series of medical doctor (MD)/registered nurse (RN)-guided discussions of goals of care using visual aids that begin at diagnosis. COMPLETE training for MD/RNs in Phase II was revised to increase their use of empathy. Preintervention/postintervention measurements for child include: time of hospice enrollment, pain, high-intensity medical interventions at EOL, and location of death; and for parent the following: uncertainty and hope. Result(s): Twenty-one parents of 18 children enrolled in the study, and 13 children were followed through EOL. At EOL, 11 (84.6%) died on home hospice or inpatient hospice, and only two (15%) received high-intensity medical interventions. Similar to published findings in the initial 13 parents enrolled in Phase I, parents in Phase II (n = 7) had improvement in hope and uncertainty, and child pain was decreased. Revised training resulted in significant improvement in MD/RN (N = 6) use of empathy (11% in Phase I vs. 100% in Phase II; P = 0.001). Conclusion(s): COMPLETE resulted in increased hospice enrollment in children with cancer at EOL compared with historical controls. In preanalysis/postanalysis, COMPLETE decreased child pain while supporting hope and reducing uncertainty in their parents. Copyright © 2020 American Academy of Hospice and Palliative Medicine
Moody KM; Hendricks-Ferguson VL; Baker R; Perkins S; Haase JE
Journal of Pain and Symptom Management
2020
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<a href="http://doi.org/10.1016/j.jpainsymman.2020.03.033" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2020.03.033</a>
Vulnerability and Agency across Treatment-Seeking Journeys for Acutely Ill Children: How Family Members Navigate Complex Healthcare Before, During and After Hospitalisation in A Rural Kenyan Setting
Adult; Child Preschool; Cohort Studies; Female; Humans; Infant; Male; Qualitative Research; Prospective Studies; Family; Continuity of Patient Care; Hospitalization; Child Mortality; Social Support; Acute Disease; Caregivers; Health Facilities; Family Characteristics; Vulnerable Populations; Rural Population; Kenya/epidemiology; Agency; Child Health; Childhood acute illness; Delivery of Health Care/standards; Treatment-seeking; Vulnerability
BACKGROUND: Child mortality rates during hospitalisation for acute illness and after discharge are unacceptably high in many under-resourced settings. Childhood vulnerability to recurrent illness, and death, is linked to their families' situations and ability to make choices and act (their agency). We examined vulnerability and agency across treatment-seeking journeys for acutely ill children and considered the implications for policy and practice. METHOD: A qualitative sub-study was embedded within the prospective CHAIN Network cohort study, which is investigating mechanisms of inpatient and post-hospital discharge mortality among acutely ill young children across a spectrum of nutritional status. Primary data were collected from household members of 20 purposively selected cohort children over 18 months through formal interviews (total n = 74), complemented by informal discussions and observations. Data were analysed using narrative and thematic approaches. RESULTS: Treatment-seeking pathways were often long and complex, particularly for children diagnosed as severely malnourished. Family members' stories reveal that children's carers, usually mothers, navigate diverse challenges related to intersecting vulnerabilities at individual, household and facility levels. Specific challenges include the costs of treatment-seeking, confusing and conflicting messaging on appropriate care and nutrition, and poor continuity of care. Strong power inequities were observed between family members and health staff, with many mothers feeling blamed for their child's condition. Caregivers' agency, as demonstrated in decision-making and actions, often drew on the social support of others but was significantly constrained by their situation and broader structural drivers. CONCLUSION: To support children's care and recovery, health systems must be more responsive to the needs of families facing multiple and interacting vulnerabilities. Reducing incurred treatment costs, improving interpersonal quality of care, and strengthening continuity of care across facilities is essential. Promising interventions need to be co-designed with community representatives and health providers and carefully tested for unintended negative consequences and potential for sustainable scale-up.
Zakayo SM; Njeru RW; Sanga G; Kimani MN; Charo A; Muraya K; Sarma H; Uddin MF; Berkley JA; Walson JL; Kelley M; Marsh V; Molyneux S
International Journal for Equity in Health
2020
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<a href="http://doi.org/10.1186/s12939-020-01252-x" target="_blank" rel="noreferrer noopener">10.1186/s12939-020-01252-x</a>
Shortening and validation of the Patient Engagement In Research Scale (PEIRS) for measuring meaningful patient and family caregiver engagement
OBJECTIVE: To shorten the Patient Engagement In Research Scale (PEIRS) to its most essential items and evaluate its measurement properties for assessing the degree of patients' and family caregivers' meaningful engagement as partners in research projects. METHODS: A prospective cross-sectional web-based survey in Canada and the USA, and also paper-based in Canada. Participants were patients or family caregivers who had engaged in research projects within the last 3 years, were ≥17 years old, and communicated in English. Extensive psychometric analyses were conducted. RESULTS: 119 participants: 99 from Canada, 74 female, 51 aged 17-35 years and 50 aged 36-65 years, 60 had post-secondary education, and 74 were Caucasian/white. The original 37-item PEIRS was shortened to 22 items (PEIRS-22), mainly because of low inter-item correlations. PEIRS-22 had a single dominant construct that accounted for 55% of explained variance. Analysis of PEIRS-22 scores revealed the following: (1) acceptable floor and ceiling effects (<15%), (2) internal consistency (ordinal alpha = 0.96), (3) structural validity by fit to a Rasch measurement model, (4) construct validity by moderate correlations with the Public and Patient Engagement Evaluation Tool, (5) good test-retest reliability (ICC(2,1) = 0.86) and (6) interpretability demonstrated by significant differences among PEIRS-22 scores across three levels of global meaningful engagement in research. CONCLUSIONS: The shortened PEIRS is valid and reliable for assessing the degree of meaningful patient and family caregiver engagement in research. It enables standardized assessment of engagement in research across various contexts. PATIENT OR PUBLIC CONTRIBUTION: A researcher-initiated collaboration, patient partners contributed from study conception to manuscript write-up.
Hamilton CB; Hoens AM; McKinnon AM; McQuitty S; English K; Hawke LD; Li LC
Health Expectations
2021
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<a href="http://doi.org/10.1111/hex.13227" target="_blank" rel="noreferrer noopener">10.1111/hex.13227</a>
Medication Order Errors at Hospital Admission Among Children With Medical Complexity
Medication Errors; Medication Reconciliation; Child; Hospitalization; Hospitals Pediatric; Humans; Patient Admission; Prospective Studies
OBJECTIVES: We sought to characterize the nature and prevalence of medication order errors (MOEs) occurring at hospital admission for children with medical complexity (CMC), as well as identify the demographic and clinical risk factors for CMC experiencing MOEs. METHODS: Prospective cohort study of 1233 hospitalizations for CMC from November 1, 2015, to October 31, 2016, at 2 children's hospitals. Medication order errors at admission were identified prospectively by nurse practitioners and a pharmacist through direct patient care. The primary outcome was presence of at least one MOE at hospital admission. Statistical methods used included χ2 test, Fisher exact tests, and generalized linear mixed models. RESULTS: Overall, 6.1% (n = 75) of hospitalizations had ≥1 MOE occurring at admission, representing 112 total identified MOEs. The most common MOEs were incorrect dose (41.1%) and omitted medication (34.8%). Baclofen and clobazam were the medications most commonly associated with MOEs. In bivariable analyses, MOEs at admission varied significantly by age, assistance with medical technology, and numbers of complex chronic conditions and medications (P < 0.05). In multivariable analysis, patients receiving baclofen had the highest adjusted odds of MOEs at admission (odds ratio, 2.2 [95% confidence interval, 1.2-3.8]). CONCLUSIONS: Results from this study suggest that MOEs are common for CMC at hospital admission. Children receiving baclofen are at significant risk of experiencing MOEs, even when orders for baclofen are correct. Several limitations of this study suggest possible undercounting of MOEs during the study period. Further investigation of medication reconciliation processes for CMC receiving multiple chronic, home medications is needed to develop effective strategies for reducing MOEs in this vulnerable population.
Blaine K; Wright J; Pinkham A; O'Neill M; Wilkerson S; Rogers J; McBride S; Crofton C; Grodsky S; Hall D; Mauskar S; Akula V; Khan A; Mercer A; Berry JG
Journal of Patient Safety
2022
<a href="http://doi.org/10.1097/pts.0000000000000719" target="_blank" rel="noreferrer noopener">10.1097/pts.0000000000000719</a>
Admission factors associated with nutritional status deterioration and prolonged pediatric intensive care unit stay in critically ill children: PICU-ScREEN multicenter study
Child; Child Preschool; chronic disease; Critical Illness; Humans; Infant; Intensive Care Units Pediatric; length of stay; Length of Stay; malnutrition; Malnutrition; nutritional status; Nutritional Status; outcomes; pediatric intensive care unit; Prospective Studies; Retrospective Studies
BACKGROUND: Early identification of patients in the pediatric intensive care unit (PICU) at risk of nutritional status (NS) deterioration and poor outcomes is desirable. We aimed to identify factors associated with NS deterioration and prolonged PICU stay. METHODS: Prospective cohort study in eight Brazilian PICUs with children <18 years with a PICU stay >72h. We used multivariable logistic regression to identify the clinical, laboratory, and nutrition variables at admission that were associated with outcomes. NS deterioration was defined as the reduction in weight-for-age, body mass index-for-age or mid-upper arm circumference-for-age z-score ≥1 during PICU stay. Prolonged PICU stay was defined as ≥13 days. RESULTS: We enrolled 363 eligible patients, median age 11.3 months (interquartile range:3.1-45.6) and 46% had at least one complex chronic condition (CCC). NS deterioration was observed in 23% of participants and was associated with CCC (odds ratio [OR]:2.71; 95% confidence interval [CI]:1.44-5.09), after adjusting for severity risk score, leukocyte count, obesity, and PICU site. Prolonged PICU stay was associated with age <2 years (OR:1.95; 95%CI:1.03-3.66), fluid overload (>10%) over the first 72h (OR:2.66; 95%CI:1.50-4.73), and hypoalbuminemia (<3.0 g/dL) (OR:2.05; 95%CI:1.12-3.76), after adjusting for CCC, severity risk score, undernutrition, early nutrition therapy, and PICU site. CONCLUSIONS: CCC at admission was associated with NS deterioration. Age <2 years, fluid overload, and hypoalbuminemia at PICU admission were associated with prolonged PICU stay. These factors must be further evaluated as part of an admission nutrition screening tool for critically ill children.
Ventura JC; Oliveira LDA; Silveira TT; Hauschild DB; Mehta NM; Moreno YMF; PICU-ScREEN Study Group
Journal of Parenteral and Enteral Nutrition
2022
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<a href="http://doi.org/10.1002/jpen.2116" target="_blank" rel="noreferrer noopener">10.1002/jpen.2116</a>
Healthcare Satisfaction and Unmet Needs Among Bereaved Parents in the NICU
Neonatal intensive care; Newborn Infant; Bereavement; Delivery of Health Care; Infant; Needs Assessment; Palliative Care; Parents; Personal Satisfaction; Prospective Studies; Terminal Care
BACKGROUND: Learning directly from bereaved parents about their experiences in the neonatal intensive care unit (NICU) can improve services at end-of-life (EOL) care. Parents who perceive that their infant suffered may report less satisfaction with care and may be at greater risk for distress after the death. Despite calls to improve EOL care for children, limited research has examined the EOL experiences of families in the NICU. PURPOSE: We examined parent perceptions of their infant's EOL experience (eg, symptom burden and suffering) and satisfaction with care in the NICU. METHODS/SEARCH STRATEGY: Forty-two mothers and 27 fathers (representing 42 infants) participated in a mixed-methods study between 3 months and 5 years after their infant's death (mean = 39.45 months, SD = 17.19). Parents reported on healthcare satisfaction, unmet needs, and infant symptoms and suffering in the final week of life. FINDINGS/RESULTS: Parents reported high levels of healthcare satisfaction, with relative strengths in providers' technical skills and inclusion of the family. Greater perceived infant suffering was associated with lower healthcare satisfaction and fewer well-met needs at EOL. Parents' understanding of their infant's condition, emotional support, communication, symptom management, and bereavement care were identified as areas for improvement. IMPLICATIONS FOR PRACTICE: Parents value comprehensive, family-centered care in the NICU. Additionally, monitoring and alleviating infant symptoms contribute to greater parental satisfaction with care. Improving staff knowledge about EOL care and developing structured bereavement follow-up programs may enhance healthcare satisfaction and family outcomes. IMPLICATIONS FOR RESEARCH: Prospective studies are needed to better understand parental perceptions of EOL care and the influence on later parental adjustment.
Baughcum AE; Fortney CA; Winning AM; Dunnells ZDO; Humphrey LM; Gerhardt CA
Advances in Neonatal Care
2020
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<a href="http://doi.org/10.1097/ANC.0000000000000677" target="_blank" rel="noreferrer noopener">10.1097/ANC.0000000000000677</a>
Prospective, phenotype-driven selection of critically ill neonates for rapid exome sequencing is associated with high diagnostic yield
Aged; Critical Illness; exome sequencing; Exome/genetics; Genetic Testing; Humans; Infant; Infant Newborn; intensive care unit; neonates; Phenotype; Prospective Studies; Whole Exome Sequencing
PURPOSE: To investigate the impact of rapid-turnaround exome sequencing in critically ill neonates using phenotype-based subject selection criteria. METHODS: Intensive care unit babies aged <6 months with hypotonia, seizures, a complex metabolic phenotype, and/or multiple congenital malformations were prospectively enrolled for rapid (<7 day) trio-based exome sequencing. Genomic variants relevant to the presenting phenotype were returned to the medical team. RESULTS: A genetic diagnosis was attained in 29 of 50 (58%) sequenced cases. Twenty-seven (54%) patients received a molecular diagnosis involving known disease genes; two additional cases (4%) were solved with pathogenic variants found in novel disease genes. In 24 of the solved cases, diagnosis had impact on patient management and/or family members. Management changes included shift to palliative care, medication changes, involvement of additional specialties, and the consideration of new experimental therapies. CONCLUSION: Phenotype-based patient selection is effective at identifying critically ill neonates with a high likelihood of receiving a molecular diagnosis via rapid-turnaround exome sequencing, leading to faster and more accurate diagnoses, reducing unnecessary testing and procedures, and informing medical care.
Gubbels CS; VanNoy GE; Madden JA; Copenheaver D; Yang S; Wojcik MH; Gold NB; Genetti CA; Stoler J; Parad RB; Roumiantsev S; Bodamer O; Beggs AH; Juusola J; Agrawal PB; Yu TW
Genetics in Medicine
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1038/s41436-019-0708-6" target="_blank" rel="noreferrer noopener">10.1038/s41436-019-0708-6</a>
Healthcare utilization and costs of pediatric home mechanical ventilation in Canada
Adult; Child; Female; Humans; Male; Child Preschool; Middle Aged; Quality of Life; Longitudinal Studies; Canada; Prospective Studies; pediatrics; Health Care Costs; Health Expenditures; Patient Acceptance of Health Care; Tracheostomy; healthcare costs; healthcare utilization; home mechanical ventilation; long-term mechanical ventilation; ventilator assisted individual; Ambulatory Care/economics; Caregivers/economics; Home Care Services/economics; Respiration Artificial/economics
BACKGROUND: Children using home mechanical ventilation (HMV) live at home with better quality of life, despite financial burden for their family. Previous studies of healthcare utilization and costs have not considered public and private expenditures, including family caregiver time. Our objective was to examine public and private healthcare utilization and costs for children using HMV, and variables associated with highest costs. METHODS: Longitudinal, prospective, observational cost analysis study (2012-2014) collecting data on public and private (out-of-pocket, third-party insurance, and caregiving) costs every 2 weeks for 6 months using the Ambulatory Home Care Record. Functional Independence Measure (FIM), WeeFIM, and Caregiving Impact Scale (CIS) were measured at baseline and study completion. Regression modeling examined a priori selected variables associated with monthly costs using Andersen and Newman's framework for healthcare utilization, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($1CAD = $0.78USD). RESULTS: Forty two children and their caregivers were enrolled. Overall median (interquartile range) monthly healthcare cost was $12 131 ($8159-$15 958) comprising $9929 (89%) family caregiving hours, $996 (9%) publicly funded, and $252 (2%) out-of-pocket (<1% third-party insurance) costs. With higher FIM score (lower dependency), median costs were reduced by 4.5% (95% confidence interval: 8.3%-0.5%), adjusted for age, sex, tracheostomy, and daily ventilation duration. Note: since the three cost categories did not sum to the total statistically derived median cost, the percentage of each category used the sum of median public + caregiver lost time + private out-of-pocket + third-party insurance as the denominator. CONCLUSIONS: For HMV children, most healthcare costs were due to family caregiving costs. More dependent children incur highest costs. The financial burden to family caregivers is substantial and needs to considered in future policy decisions related to pediatric HMV.
Nonoyama ML; Katz SL; Amin R; McKim DA; Guerriere D; Coyte PC; Wasilewski M; Zagorski B; Rose L
Pediatric Pulmonology
2020
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<a href="http://doi.org/10.1002/ppul.24923" target="_blank" rel="noreferrer noopener">10.1002/ppul.24923</a>
Genome Sequencing as a Diagnostic Test in Children With Unexplained Medical Complexity
Child; Female; Humans; Male; Reproducibility of Results; Child Preschool; Canada; Prospective Studies; Predictive Value of Tests; Genetic Testing/statistics & numerical data; Somatoform Disorders/diagnosis; Whole Genome Sequencing/statistics & numerical data
IMPORTANCE: Children with medical complexity (CMC) represent a growing population in the pediatric health care system, with high resource use and associated health care costs. A genetic diagnosis can inform prognosis, anticipatory care, management, and reproductive planning. Conventional genetic testing strategies for CMC are often costly, time consuming, and ultimately unsuccessful. OBJECTIVE: To evaluate the analytical and clinical validity of genome sequencing as a comprehensive diagnostic genetic test for CMC. DESIGN, SETTING, AND PARTICIPANTS: In this cohort study of the prospective use of genome sequencing and comparison with standard-of-care genetic testing, CMC were recruited from May 1, 2017, to November 30, 2018, from a structured complex care program based at a tertiary care pediatric hospital in Toronto, Canada. Recruited CMC had at least 1 chronic condition, technology dependence (child is dependent at least part of each day on mechanical ventilators, and/or child requires prolonged intravenous administration of nutritional substances or drugs, and/or child is expected to have prolonged dependence on other device-based support), multiple subspecialist involvement, and substantial health care use. Review of the care plans for 545 CMC identified 143 suspected of having an undiagnosed genetic condition. Fifty-four families met inclusion criteria and were interested in participating, and 49 completed the study. Probands, similarly affected siblings, and biological parents were eligible for genome sequencing. EXPOSURES: Genome sequencing was performed using blood-derived DNA from probands and family members using established methods and a bioinformatics pipeline for clinical genome annotation. MAIN OUTCOMES AND MEASURES: The primary study outcome was the diagnostic yield of genome sequencing (proportion of CMC for whom the test result yielded a new diagnosis). RESULTS: Genome sequencing was performed for 138 individuals from 49 families of CMC (29 male and 20 female probands; mean [SD] age, 7.0 [4.5] years). Genome sequencing detected all genomic variation previously identified by conventional genetic testing. A total of 15 probands (30.6%; 95% CI 19.5%-44.6%) received a new primary molecular genetic diagnosis after genome sequencing. Three individuals had novel diseases and an additional 9 had either ultrarare genetic conditions or rare genetic conditions with atypical features. At least 11 families received diagnostic information that had clinical management implications beyond genetic and reproductive counseling. CONCLUSIONS AND RELEVANCE: This study suggests that genome sequencing has high analytical and clinical validity and can result in new diagnoses in CMC even in the setting of extensive prior investigations. This clinical population may be enriched for ultrarare and novel genetic disorders. Genome sequencing is a potentially first-tier genetic test for CMC.
Costain G; Walker S; Marano M; Veenma D; Snell M; Curtis M; Luca S; Buera J; Arje D; Reuter MS; Thiruvahindrapuram B; Trost B; Sung WWL; Yuen RKC; Chitayat D; Mendoza-Londono R; Stavropoulos DJ; Scherer SW; Marshall CR; Cohn RD; Cohen E; Orkin J; Meyn MS; Hayeems RZ
JAMA Network Open
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1001/jamanetworkopen.2020.18109" target="_blank" rel="noreferrer noopener">10.1001/jamanetworkopen.2020.18109</a>
Medical complications in a telemedicine home care programme for paediatric ventilated patients
Child; Female; Humans; Male; Child Preschool; Quality of Life; Prospective Studies; Monitoring Physiologic; Hospitalization/statistics & numerical data; Telemedicine; chronic respiratory failure; numerical data; Home Care Services/organization & administration/statistics & numerical data; Intensive Care Units Pediatric/organization & administration/statistics &; invasive mechanical ventilation; life-threatening event; paediatric home care; Respiration Artificial/adverse effects/methods; Telemedicine/organization & administration/statistics & numerical data; Tracheotomy/rehabilitation; ventilator-dependent children
INTRODUCTION: Advances in paediatric medicine have increased survival rates for patients with severe chronic illnesses, of which the most complex are ventilator-dependent children (VDCs). Although home care improves their quality of life, morbidity and mortality rates are high. Our aim was to study the medical complications (events) that occur at home and assess the usefulness of telemedicine in their detection and treatment. METHODS: A prospective clinical study (2007-2017) was performed for tracheotomised VDCs. We used a high-density data telemedicine monitoring system from our Paediatric Intensive Care Unit and analysed events during the first two years of home care to study how different variables inter-correlated with the four most common ones: hospital admissions, admissions avoided, event durations and life-threatening events (LTEs); the significance level was set at an alpha of 0.05 in all cases. RESULTS: All our VDCs were included (n = 12); there were 141 events, and these were homogeneously distributed over the study period. The incidence was higher in children who were ventilator dependent for more than 12 h a day (70.9%, p < 0.001) and the main cause was respiratory (69.5%, p < 0.001). Telemedicine was the main initial care and monitoring approach (86.5% and 90.1%, respectively, p < 0.001); 13 events were LTEs, nine were resolved telemedically, four required medicalised transfer to hospital and three resulted in a hospital admission. DISCUSSION: Clinical complications are frequent in VDCs receiving home care, and respiratory decompensation is the most frequent cause. Telemedicine facilitated diagnosis and early treatment, and was useful in managing LTEs.
Muñoz-Bonet JI; López-Prats JL; Flor-Macián EM; Cantavella T; Domínguez A; Vidal Y; Brines J
Journal of Telemedicine and Telecare
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1177/1357633x19843761" target="_blank" rel="noreferrer noopener">10.1177/1357633x19843761</a>
Safety and pharmacokinetics of medical cannabis preparation in a monocentric series of young patients with drug resistant epilepsy
Adolescent; Adult; Child; Female; Humans; Male; Child Preschool; Dose-Response Relationship Drug; Young Adult; Prospective Studies; Cannabidiol (CBD); Childhood; Delta-9-tetrahydrocannabinol (THC); Drug-resistant epilepsy; Medical cannabis galenic preparation; Drug Resistant Epilepsy/drug therapy; Medical Marijuana/administration & dosage/adverse effects/pharmacokinetics
OBJECTIVES: To evaluate safety and pharmacokinetic parameters (PK) of medical cannabis in add-on for children and young adults with drug-resistant epilepsy. DESIGN, SETTING: Ten patients (4 females, 6 males, age 2.5-23.2 years) were enrolled in a prospective open trial with a galenic preparation (decoction) of Italian cannabis (FM2, ratio THC:CBD = 3:5, range THC 5.2-7.2 %; CBD 8.2-11.1 %). Patients received the first dose in Hospital, progressively augmented by CBD dose titration (from 1 to 4 mg/kg/day). OUTCOME MEASURES: In order to assess safety, blood parameters, heart rates and electrocardiograms (ECGs) were evaluated before the enrollment and during the follow up. The PK study was performed measuring THC and CBD concentrations by UHPLC-MS/MS in plasma samples collected during the first administration and at each follow-up visit. RESULTS: Two out of ten patients stopped the treatment for adverse events (detected in 6/10: gastroenteric, sleep or behavioral disorders) and difficulties in drug supply. We observed minor ECG alterations in two patients and asymptomatic transient reductions of fibrinogen after 6 months of therapy. The PK study during follow-up revealed statistically significant correlations between THC-CBD blood concentrations and: volumes of decoction, FM2 and THC-CBD daily dosages. CONCLUSIONS: The present study, although with some limitations, shows a good safety profile of medical cannabis in children and young patients with drug-resistant epilepsy and encourages the possibility of further studies with oral cannabis-based drugs. The correlations between THC-CBD plasma concentrations and their administered dosages underline the need of a therapeutic drug monitoring for cannabinoids therapy.
Gherzi M; Milano G; Fucile C; Calevo MG; Mancardi MM; Nobili L; Astuni P; Marini V; Barco S; Cangemi G; Manfredini L; Mattioli F; De Grandis E
Complementary Therapies in Medicine
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.ctim.2020.102402" target="_blank" rel="noreferrer noopener">10.1016/j.ctim.2020.102402</a>
Undernutrition at PICU Admission Is Predictor of 60-Day Mortality and PICU Length of Stay in Critically Ill Children
Adolescent; Anthropometry; Body Mass Index; Brazil/epidemiology; Child; Child Nutrition Disorders/*mortality; Critical Illness/*mortality; Female; Humans; Infant; Intensive Care Units; Length of stay; Length of Stay/*statistics & numerical data; Male; malnutrition; Mortality; Nutritional status; Patient Admission/*statistics & numerical data; Patient Discharge/statistics & numerical data; Pediatric intensive care unit; Pediatric/*statistics & numerical data; Predictive Value of Tests; Preschool; Proportional Hazards Models; Prospective Studies; Regression Analysis; Time Factors
BACKGROUND: There are few studies that assess the role of different nutritional assessment variables at pediatric intensive care unit (PICU) admission in predicting clinical outcomes. OBJECTIVE: To identify nutritional variables in the first 4 days of PICU stay that predict 60-day mortality and time to discharge alive from the PICU. DESIGN: Single-center prospective study in Southern Brazil, conducted between July 2013 and February 2016. At PICU admission, children with z scores <-2 for body mass index (BMI)-for-age, mid-upper arm circumference (MUAC)-for-age, and triceps skinfold thickness (TSF)-for-age were considered as undernourished. PARTICIPANTS/SETTING: There were 199 patients, aged <15 years, with PICU stay >48 hours. MAIN OUTCOME MEASURES: Sixty-day mortality and time to discharge alive from the PICU. STATISTICAL ANALYSIS PERFORMED: Cox regression model was applied to determine predictors of 60-day mortality and time to discharge alive from the PICU. RESULTS: Median age was 23.1 months (interquartile range=3.9 to 89.1), and 63% were male, with 18% prevalence of undernutrition at admission by BMI-for-age. Median PICU stay was 7 days (interquartile range=4 to 12), and 60-day mortality was 12%. After adjusting for sex, age, Pediatric Index of Mortality 2, and presence of complex chronic conditions, undernutrition based on BMI-for-age (hazard ratio [HR]=3.75; 95% CI=1.41 to 9.95; P=0.008), MUAC-for-age (HR=7.62; 95% CI=2.42 to 23.97; P=0.001), and TSF-for-age (HR=4.01; 95% CI=1.14 to 14.15; P=0.031) was associated with higher risk of 60-day mortality. Based on MUAC-for-age with the same adjustment model, undernourished children had longer time to discharge alive from the PICU (HR=0.45; 95% CI=0.21 to 0.98; P=0.045). CONCLUSIONS: Undernutrition at PICU admission based on different anthropometric variables was predictive of 60-day mortality and longer time to discharge alive from the PICU.
Ventura J C; Hauschild D B; Barbosa E; Bresolin N L; Kawai K; Mehta N M; Moreno Y M F
Journal of the Academy of Nutrition and Dietetics
2020
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<a href="http://doi.org/10.1016/j.jand.2019.06.250" target="_blank" rel="noreferrer noopener">10.1016/j.jand.2019.06.250</a>
State Variation in Posthospital Home Nursing for Commercially Insured Medically Complex Children
After Care; Child; Descriptive Statistics; Geographic Factors; Health – Economics; Health Care Costs; Health Services Accessibility; Home Nursing; Human; Insurance; Medically Fragile; Patient Discharge; Probability; Prospective Studies; Retrospective Design
BACKGROUND AND OBJECTIVES: Home nursing is essential for children with medical complexity (CMC), but provision varies substantially across states. Our objectives were to quantify state-to-state variability in distribution of posthospitalization home nursing to commercially insured CMC and to rank-order states. METHODS: Retrospective cohort study of hospitalized commercially insured children with ≥1 complex chronic condition from birth to 18 years of age in the Truven MarketScan database. Cohort eligibility criteria were hospital discharge between January 2013 and November 2016 and at least 30 days of follow-up after discharge. Two primary outcome measures were used: receipt of any home nursing within 30 days of hospital discharge (yes or no) and number of days of posthospitalization home nursing (1-30 days). A composite metric encompassing both receipt and quantity was created by evaluating the 95th percentile of days of home nursing (0-30 days). RESULTS: Overall, 9.9% of the sample received home nursing. After we adjusted for patient characteristics, the probability of receiving home nursing varied across states, ranging from 3.4% to 19.2%. Among home nursing recipients, the adjusted median home nursing days across states ranged from 6.6 to 24.5 days. The adjusted 95th percentile of days of home nursing (across the entire of sample, including recipients and nonrecipients of home nursing) ranged from 6.8 to 22.6 days. CONCLUSIONS: We observed striking state-to-state variability in receipt of home nursing and mean number of days of posthospitalization home nursing among commercially insured CMC after adjustment for demographic and clinical differences. This suggests opportunities for state-level improvement.
Rasooly I R; Shults J; Guevara J P; Feudtner C
Pediatrics
2020
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<a href="http://doi.org/10.1542/peds.2019-2465" target="_blank" rel="noreferrer noopener">10.1542/peds.2019-2465</a>
Characteristics and Outcomes of Critical Illness in Children With Feeding and Respiratory Technology Dependence
Adolescent; Artificial/adverse effects/*statistics & numerical data; Biomedical Technology; Case-Control Studies; Child; Critical Care/*methods; Critical Illness/therapy; Health Care/*statistics & numerical data; Humans; Infant; Intensive Care Units; Newborn; Outcome Assessment; Parenteral Nutrition; Pediatric/statistics & numerical data; Preschool; Prospective Studies; Respiration; Severity of Illness Index; Total/adverse effects/*statistics & numerical data
OBJECTIVES: Children with dependence on respiratory or feeding technologies are frequently admitted to the PICU, but little is known about their characteristics or outcomes. We hypothesized that they are at increased risk of critical illness-related morbidity and mortality compared with children without technology dependence. DESIGN: Secondary analysis of prospective, probability-sampled cohort study of children from birth to 18 years old. Demographic and clinical characteristics were assessed. Outcomes included death, survival with new morbidity, intact survival, and survival with functional status improvement. SETTING: General and cardiovascular PICUs at seven participating children's hospitals as part of the Trichotomous Outcome Prediction in Critical Care study. SUBJECTS: Children from birth to 18 years of age as part of the Trichotomous Outcome Prediction in Critical Care study. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Children with technology dependence composed 19.7% (1,989/10,078) of PICU admissions. Compared with those without these forms of technology dependence, these children were younger, received more ICU-specific therapeutics, and were more frequently readmitted to the ICU. Death occurred in 3.7% of technology-dependent patients (n = 74), and new morbidities developed in 4.5% (n = 89). Technology-dependent children who developed new morbidities had higher Pediatric Risk of Mortality scores and received more ICU therapies than those who did not. A total of 3.0% of technology-dependent survivors (n = 57) showed improved functional status at hospital discharge. CONCLUSIONS: Children with feeding and respiratory technology dependence composed approximately 20% of PICU admissions. Their new morbidity rates are similar to those without technology dependence, which contradicts our hypothesis that children with technology dependence would demonstrate worse outcomes. These comparable outcomes, however, were achieved with additional resources, including the use of more ICU therapies and longer lengths of stay. Improvement in functional status was seen in some technology-dependent survivors of critical illness.
Heneghan J A; Reeder R W; Dean J M; Meert K L; Berg R A; Carcillo J; Newth C J L; Dalton H; Tamburro R; Pollack M M
Pediatric Critical Care Medicine
2019
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<a href="http://doi.org/10.1097/pcc.0000000000001868" target="_blank" rel="noreferrer noopener">10.1097/pcc.0000000000001868</a>
Changes in Siblings Over Time After the Death of a Brother or Sister From Cancer
Adolescence; Adult; Bereavement; Child; Content Analysis; Fathers -- Psychosocial Factors; Female; Human; Male; McNemar's Test; Middle Age; Mothers -- Psychosocial Factors; Multimethod Studies; Neoplasms -- Mortality; Prospective Studies; Semi-Structured Interview; Siblings -- Psychosocial Factors
Background: Limited research has examined the impact of a child's death from cancer on siblings. Even less is known about how these siblings change over time. Objective: This study compared changes in siblings 1 (T1) and 2 (T2) years after the death of a brother or sister from cancer based on bereaved parent and sibling interviews. Methods: Participants across 3 institutions represented 27 families and included bereaved mothers (n = 21), fathers (n = 15), and siblings (n = 26) ranging from 8 to 17 years old. Participants completed semistructured interviews. Content analysis identified emerging themes and included frequency counts of participant responses. McNemar tests examined differences in the frequency of responses between T1 and T2 data. Results: Participants reported similar types of changes in bereaved siblings at both time points, including changes in sibling relationships, life perspectives, their personal lives, and school performance. A new theme of "openness" emerged at T2. Frequencies of responses differed according to mother, father, or sibling informant. Overall, participants less frequently reported changes at T2 versus T1. Compared with findings in the first year, participants reported greater sibling maturity at follow-up. Conclusion: Overall changes in bereaved siblings continued over 2 years with less frequency over time, with the exception of increases in maturity and openness. Implications for Practice: Providers can educate parents regarding the impact of death of a brother or sister over time. Nurses can foster open communication in surviving grieving siblings and parents as potential protective factors in families going through their grief.
Akard T F; Skeens M A; Fortney C A; Dietrich M S; Gilmer M J; Vannatta K; Barrera M; Davies B; Wray S; Gerhardt C A
Cancer Nursing
2019
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<a href="http://doi.org/10.1097/NCC.0000000000000573" target="_blank" rel="noreferrer noopener">10.1097/NCC.0000000000000573</a>
Responsiveness of the motor function measure in neuromuscular diseases
Middle Aged; Male; Aged; Mobility Limitation; Young Adult; Child; Humans; Adult; Prospective Studies; Adolescent; Perception; Female; Disability Evaluation; Motor Skills; Physical Therapy Modalities; Muscular Dystrophy Duchenne/physiopathology/rehabilitation; Neuromuscular Diseases/physiopathology/rehabilitation; tone and motor problems; SMA1; Q3 conditions; tool development; scale development; motor function measure; MFM
OBJECTIVES: To study the responsiveness (sensitivity to change) of the Motor Function Measure (MFM) in detecting change in neuromuscular disease patients with the intent of using this measure in future clinical trials. DESIGN: Prospective cohort observational study. SETTING: Inpatient and outpatient facilities for follow-up and treatment of neuromuscular diseases. PARTICIPANTS: Patients (N=152) with various neuromuscular diseases aged 6 to 60 years. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE(S): We used the MFM total score and its 3 subscores on 2 measurements grossly 1 year apart. The physicians and the patients (or proxy) were asked to provide their perceived change in functional status since the first MFM. These changes were expressed in 3 outcomes: deterioration, stability, or improvement. RESULTS: The overall 12-month-standardized mean change of the total score mean +/- SD annual total score change was -2.4+/-5.5 points (P<.001), with patients with Duchenne muscular dystrophy (DMD) presenting the most significant change (-5.8+/-6.3, P<.001). The change in patients reporting deterioration (34%) was significantly larger than that of those reporting stability (47%) or improvement (10%) (-4.4+/-6.4 vs -2.0+/-5.6 and +0.9+/-4.4 points, respectively, P<.01). The 12-month-standardized total score changes were significantly greater in physician-rated deteriorated (49%) versus stable patients (51%), with mean differences in scores being -5.3+/-7.6 and -1.2+/-5.3, respectively (P<.001). CONCLUSIONS: The MFM showed a good responsiveness, especially in patients with DMD and agreements with patients' and physicians' perceived change. Confirming this responsiveness requires larger age groups of patients with DMD and other neuromuscular diseases as well as disease-specific interexamination delays.
Vuillerot C; Payan C; Girardot F; Fermanian J; Iwaz J; Berard C; Ecochard R
Archives of Physical Medicine and Rehabilitation
2012
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<a href="http://doi.org/10.1016/j.apmr.2012.05.025" target="_blank" rel="noreferrer noopener">10.1016/j.apmr.2012.05.025</a>
Movement disorder in ataxia-telangiectasia: treatment with amantadine sulfate
Adolescent; Amantadine/tu [Therapeutic Use]; Antiparkinson Agents/Antiparkinson [Therapeutic Use]; Ataxia Telangiectasia/co [Complications]; Child; Preschool; Disability; Evaluation; Humans; Movement Disorders/dt [Drug Therapy]; Movement Disorders/et [Etiology]; Neurologic Examination; Prospective Studies; Severity of Illness Index; 0 (Antiparkinson Agents); BF4C9Z1J53 (Amantadine); tone and motor problems; ataxia telangiectasia; pharmacologic intervention; amantadine sulfate
Ataxia-telangiectasia is a cerebellar neurodegenerative disorder presenting with ataxia, chorea, myoclonus, and bradykinesia. Literature on treatment of movement disorders is scarce. We treated 17 children (aged 11.2 +/- 3.9 years) for 8 weeks with the dopaminergic and anti-N-methyl-d-aspartate (NMDA) agent amantadine sulfate 6.3 +/- 0.87 mg/kg/d. Ataxia was assessed by using the International Cooperative Ataxia Scale, parkinsonism by the Unified Parkinson Disease Rating Scale, and chorea/myoclonus by the Abnormal Involuntary Movement Scale. Responders were considered those patients who had at least 20% improvement in the summation of the 3 scales. Overall, 76.5% of patients were responders, with a mean 29.3% improvement. Ataxia, involuntary movements, and parkinsonism improved significantly (25.3%, 32.5%, and 29.5%, respectively); (P < .001, t test). Side effects were mild and transient, and they did not lead to drug discontinuation. Amantadine is a well-tolerated and effective treatment for motor symptoms in ataxia telangiectasia. Assessment of long-term effects and a double-blind study should follow.
Nissenkorn A; Hassin-Baer S; Lerman S F; Levi Y B; Tzadok M; Ben-Zeev B
Journal of Child Neurology
2013
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<a href="http://doi.org/10.1177/0883073812441999" target="_blank" rel="noreferrer noopener">10.1177/0883073812441999</a>
Naltrexone therapy of apnea in children with elevated cerebrospinal fluid beta-endorphin
beta-Endorphin/cerebrospinal fluid; Male; Sleep Apnea Syndromes/cerebrospinal fluid/drug therapy; Infant Newborn; Leigh Disease/cerebrospinal fluid; Child; Humans; Prospective Studies; Naltrexone/therapeutic use; Female; Child Preschool; Infant; Biomarkers of Pain; child; female; male; beta-Endorphin/cerebrospinal fluid; Naltrexone/therapeutic use; Sleep Apnea Syndromes/cerebrospinal fluid/drug therapy; breathing difficulties; Leigh syndrome; pharmacologic intervention; oral naltrexone; naltrexone; sleep apnea
Previous studies have indicated increased immunoreactivity of the endogenous opioid peptide beta-endorphin in the cerebrospinal fluid (CSF) of infants under 2 years of age with apnea. To assess the role of endogenous opioids in the pathogenesis of apnea in children, the effect of oral treatment with the opioid antagonist naltrexone was studied in apneic infants, as well as in older apneic children, with demonstrated increases in CSF immunoreactive beta-endorphin (i-BE). In the 8 apneic infants with elevated i-BE in lumbar CSF (range, 55-155 pg/ml; normal, 17-52 pg/ml), no further apnea occurred during naltrexone therapy (1 mg/kg/day, by mouth). Five children (2-8 years old) with apnea of unknown cause had elevated CSF i-BE (range, 74-276 pg/ml) compared to 6 age-matched nonapneic children (range, 15-48 pg/ml). No apneic events occurred during naltrexone therapy, except in 1 child during stressful events, but apnea recurred in some patients after attempts to discontinue naltrexone treatment. Adverse effects of naltrexone included complaints of headaches in 2 children and symptoms of a narcotic withdrawal syndrome during the first 3 days of treatment in 1 child. Three children with Leigh's syndrome had elevated CSF i-BE (range, 104-291 pg/ml) and their apnea also responded to naltrexone. We conclude that elevated endogenous opioids contribute to the pathogenesis of apnea in children and may even result in physical dependence.
Myer E C; Morris D L; Brase D A; Dewey W L; Zimmerman A W
Annals of Neurology
1990
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<a href="http://doi.org/10.1002/ana.410270112" target="_blank" rel="noreferrer noopener">10.1002/ana.410270112</a>
Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease)
Cross-Sectional Studies; Disease Progression; Young Adult; Child; Humans; Adult; Prospective Studies; Adolescent; Child Preschool; Regression Analysis; Reproducibility of Results; Analysis of Variance; Neuropsychological Tests; Mutation; Disabled Persons; Genotype; Homozygote; Membrane Glycoproteins; Molecular Chaperones; Neuronal Ceroid-Lipofuscinoses; tone and motor problems; NCL3; tool development; scale development; UBDRS
OBJECTIVE: To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the UBDRS in subjects with JNCL since 2002; during that time, the scale has been refined to improve reliability and validity. Now that therapies are being proposed to prevent, slow, or reverse the course of JNCL, the UBDRS will play an important role in quantitatively assessing clinical outcomes in research trials. METHODS: We administered the UBDRS to 82 subjects with JNCL genetically confirmed by CLN3 mutational analysis. Forty-four subjects were seen for more than one annual visit. From these data, the rate of physical impairment over time was quantified using multivariate linear regression and repeated-measures analysis. RESULTS: The UBDRS Physical Impairment subscale shows worsening over time that proceeds at a quantifiable linear rate in the years following initial onset of clinical symptoms. This deterioration correlates with functional capability and is not influenced by CLN3 genotype. CONCLUSION: The UBDRS is a reliable and valid instrument that measures clinical progression in JNCL. Our data support the use of the UBDRS to quantify the rate of progression of physical impairment in subjects with JNCL in clinical trials.
Kwon J M; Adams H; Rothberg P G; Augustine E F; Marshall F J; Deblieck E A; Vierhile A; Beck C A; Newhouse N J; Cialone J; Levy E; Ramirez-Montealegre D; Dure L S; Rose K R; Mink J W
Neurology
2011
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<a href="http://doi.org/10.1212/WNL.0b013e318237f649" target="_blank" rel="noreferrer noopener">10.1212/WNL.0b013e318237f649</a>
Prospective study of spinal muscular atrophy before age 6 years. DCN/SMA Group
Male; Child; Humans; Prospective Studies; Female; Child Preschool; Infant; Neurologic Examination; Motor Skills/physiology; Muscle Contraction/physiology; Range of Motion Articular/physiology; Reflex Stretch/physiology; Spinal Muscular Atrophies of Childhood/diagnosis/physiopathology; Sucking Behavior/physiology; tone and motor problems; SMA1; trajectory; characteristics; tongue fasciculation; facial weakness
Spinal muscular atrophy (SMA) is a common neuromuscular disorder of childhood, associated with a high mortality rate during the first 2 years of life. Most practitioners expect patients with SMA to follow a progressive course with loss of muscle strength and function over 2-10 years. Counselling sessions with parents frequently emphasize the high mortality rate and risk for respiratory failure. The progressive nature of SMA has been attributed to the loss of motor neurons. Fifty-eight children, ages 6 years and younger, were examined between January, 1987, and April, 1992, as part of a large, multicenter collaborative study of SMA. Muscle function was evaluated at regular intervals using a standardized protocol that was demonstrated to be reliable. We determined a prevalence of 56% for tongue fasciculations, a prevalence of 22% for facial weakness, and persistent deep tendon reflexes in one patient. Improved motor function and acquired milestones during the study were documented. This work should contribute toward a better understanding of the natural history of SMA.
Iannaccone S T; Browne R H; Samaha F J; Buncher C R
Pediatric Neurology
1993
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<a href="http://doi.org/10.1016/0887-8994(93)90082-n" target="_blank" rel="noreferrer noopener">10.1016/0887-8994(93)90082-n</a>
Cognitive and motor skills in achondroplastic infants: neurologic and respiratory correlates
Brain/pathology; Male; Electroencephalography; Humans; Prospective Studies; Female; Child Preschool; Infant; Neuropsychological Tests; Psychomotor Performance; Intelligence; Atrophy; Achondroplasia/complications/physiopathology/psychology; Evoked Potentials Somatosensory; Intellectual Disability/etiology; Respiration Disorders/etiology/physiopathology; Sleep Apnea Syndromes/etiology/physiopathology; sleep disturbance; tone and motor problems; achondroplasia; trajectory; characteristics; respiratory dysfunction
Thirteen infants with achondroplasia underwent psychometric testing as part of a comprehensive neurologic assessment. As a group, mental development was average and motor development was delayed, although a wide range of scores was obtained. Foramen magnum measurements were correlated with respiratory dysfunction, abnormal somatosensory evoked potentials, and delayed motor development. Abnormal polysomnogram outcome was associated with reduced mental capacity. In light of the reported increased frequency of respiratory dysfunction in achondroplasia, these findings warrant careful attention and further study.
Hecht J T; Thompson N M; Weir T; Patchell L; Horton W A
American Journal of Medical Genetics
1991
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1002/ajmg.1320410215" target="_blank" rel="noreferrer noopener">10.1002/ajmg.1320410215</a>
Parental assessment of comfort in newborns affected by life-limiting conditions treated by a standardized neonatal comfort care program
Palliative Care/mt [Methods]; Parents/px [Psychology]; Patient Comfort/st [Standards]; Adult; Female; Humans; Infant; Intensive Care; Male; Neonatal/og [Organization & Administration]; New York; Newborn; Patient Care Team/og [Organization & Administration]; Practice Guidelines as Topic; Prospective Studies; Qualitative Research; Surveys and Questionnaires; Young Adult
OBJECTIVE: To assess the perception of parents concerning the state of comfort maintained in their infants born with life-limiting conditions and treated by a standardized neonatal comfort care program. STUDY DESIGN: Participants were parents (n=35 families) who elected comfort care for their newborns diagnosed with life-limiting conditions. Standardized comfort measures including family/infant bonding, warmth, nutrition/hydration and pain/discomfort management were provided to all infants. Parents consented to receive a questionnaire with quantitative response options and open-ended questions. RESULTS: Forty-two questionnaires (26 from mothers and 16 from fathers) were collected and analyzed. Most parents reported that their child was treated with respect, in a caring, peaceful and non-invasive environment. To the question 'Do you think that overall your baby received comfort' mode response was 'always'. CONCLUSION: Parents of infants with life-limiting conditions perceive that their babies experience comfort as a result of the care provided by the standardized Neonatal Comfort Care Program.
Parravicini E; Daho M; Foe G; Steinwurtzel R; Byrne M
Journal of Perinatology
2018
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<a href="http://doi.org/10.1038/jp.2017.160" target="_blank" rel="noreferrer noopener">10.1038/jp.2017.160</a>
Children Enrolled in Hospice Care Under Commercial Insurance: A Comparison of Different Age Groups
Data Analysis Software; Age Factors; Male; Human; Sex Factors; Length of Stay; Child; Prospective Studies; Female; Child Preschool; Analysis of Variance; Infant; Adolescence; Health Care Costs; Insurance Health; Pediatric Care; Pennsylvania; Descriptive Statistics; Retrospective Design; Funding Source; Chi Square Test; Cross Sectional Studies; Health Resource Utilization; Academic Medical Centers -- Pennsylvania; Chronic Disease -- Classification -- In Infancy and Childhood; Comparative Studies; Geographic Factors; Hospice Care -- Economics -- In Infancy and Childhood; Hospice Patients -- Psychosocial Factors; Pearson's Correlation Coefficient
Background: Although most children at end of life have commercial insurance, little is known about their demographic and clinical characteristics, what care they are receiving, and how much it costs. Objectives: To describe commercially insured children who enrolled in hospice care during their last year of life and to examine differences across age-groups. Methods: A retrospective cohort study was conducted using 2005 to 2014 data from the MarketScan Commercial Claims and Encounters database from Truven Health Analytics. Variables were created for demographics, health, utilization, and spending. Analyses included χ2 and analysis of variance tests of differences. Results: Among the 17 062 children who utilized hospice, 49% had a preferred provider organization (PPO). Hospice length of stay averaged less than 5 days. Over 80% of children visited their primary care physician. Eight percent had hospital readmissions, and 38% had emergency department (ED) visits. Average expenditures were US$3686 per month or US$44 232 annually. The most common condition for children less than 1 year was cardiovascular (21.96%). Neuromuscular conditions were the most frequent (7.89%) in children aged 1 to 5 years, while malignancies (10.53% and 11.32%, respectively) were prevalent in ages 6 to 14 and 15 to 17. Children less than 1 year had the highest frequency of hospital readmissions (16.25%) with the lowest ED visits (28.67%) while incurring the highest expenses (US$11 211/month). Conclusions: The findings suggest that commercially insured children, who enroll in hospice, have flexible coverage with a PPO. Hospital readmissions and ED visits were relatively low for a population who was seriously ill. There were significant age-group differences.
Lindley Lisa C; Cohrs A C; Keim-Malpass J; Leslie D L
American Journal of Hospice & Palliative Medicine
2019
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<a href="http://doi.org/10.1177/1049909118789868" target="_blank" rel="noreferrer noopener"> 10.1177/1049909118789868</a>
Children's outcomes at 2-year follow-up after 4 years of structured multi-professional medical-ethical decision-making in a neonatal intensive care unit
OBJECTIVE: We reviewed our decisions about continuation/withdrawal of life-sustaining treatments in a group of critically ill newborns who were discussed in structured medical ethical decision-making meetings, and provide the surviving children's outcomes at 2-year follow-up. STUDY DESIGN: In an explorative observational study, 61 cases were evaluated. The children involved had been discussed in such a structured way from 2009 to 2012 in a level III-D neonatal intensive care unit. RESULTS: Decisions made were: full treatment (n=6), earlier restriction cancelled (n=3), treatment restriction (n=30) and palliative care (n=22). Parents of six children disagreed with the decision proposed. Thirteen (54%) of the 24 children who survived (39%) had moderate to severe neurological problems; 8 (33%) had additional sequelae; only one 2-year-old child was healthy. CONCLUSIONS: Decisions made varied to a large extent. The poor outcomes should be disseminated among decision makers. Future studies must explore new ways to improve outcome prediction, extend follow-up periods and consider what living with severe handicaps really means for both child and family.
de Boer JC; Gennissen L; Williams M; van Dijk M; Tibboel D; Reiss I; Naghib S; Sol J
Journal of Perinatology
2017
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<a href="http://doi.org/10.1038/jp.2017.30" target="_blank" rel="noreferrer noopener">10.1038/jp.2017.30</a>
Use of a clinical pathway to improve the acute management of vaso-occlusive crisis pain in pediatric sickle cell disease
adolescent; Child; Female; Humans; infant; Male; Young Adult; Pain; Pain Management; Adult; Analgesics; Prognosis; Follow-Up Studies; Prospective Studies; Critical Pathways; Anemia; Preschool; Arterial Occlusive Diseases; Sickle Cell; Tertiary Care Centers
BACKGROUND: The most common, debilitating morbidity of sickle cell disease (SCD) is vaso-occlusive crisis (VOC) pain. Although guidelines exist for its management, they are generally not well-followed, and research in other pediatric diseases has shown that clinical pathways improve care. The purpose of our study was to determine whether a clinical pathway improves the acute management of sickle cell vaso-occlusive crisis (VOC) pain in the pediatric emergency department (PED). PROCEDURE: Pain management practices were prospectively investigated before and after the initiation of a clinical pathway in the PED of an urban, tertiary care center with 50,000 ED visits per year and approximately 200 active sickle cell patients. The pathway included instructions for triage, monitoring, medication administration, and timing of assessments and interventions. Data were eligible from 35 pre-pathway and 33 post-pathway visits. Primary outcome was time interval to administration of first analgesic medication. Statistical analysis was by Student's t-test, using natural-log-transformed data for outcomes with skewed distribution curves. RESULTS: Time interval to first analgesic improved from 74 to 42 minutes (P = 0.012) and to first opioid from 94 to 46 minutes (P = 0.013). The percentage of patients who received ketorolac increased from 57% to 82% (P = 0.03). Decrease in time interval to subsequent pain score assessment was not statistically significant (110 to 72 minutes (P = 0.07)), and change in pain score was not different (P = 0.25). CONCLUSIONS: The use of a clinical pathway for sickle cell VOC in the PED can improve important aspects of pain management and merits further investigation and implementation.
2014-04
Ender KL; Krajewski JA; Babineau J; Tresgallo M; Schechter W; Saroyan JM; Kharbanda A
Pediatric Blood & Cancer
2014
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Journal Article
<a href="http://doi.org/10.1002/pbc.24864" target="_blank" rel="noreferrer">10.1002/pbc.24864</a>
Patients with malignant hematological disorders treated on a palliative care unit: prognostic impact of clinical factors
adolescent; Female; Humans; Male; retrospective studies; Survival Rate; Adult; Analgesics; Prospective Studies; Aged; Middle Aged; Risk Factors; Time Factors; Serum Albumin; Parenteral Nutrition; Palliative Care; Opioid; Databases; Factual; Disease-Free Survival; Blood Transfusion; Hematologic Neoplasms; Hemoglobins; Idiopathic; Platelet Count; Purpura; Thrombocytopenic
A reliable estimation of prognosis in patients receiving palliative care is desirable in order to facilitate clinical decision finding. For patients with advanced hematological malignancies, only few data are available to estimate prognosis of the individual's remaining life span. Here, we sought to investigate potential clinical prognostic parameters in patients with hematological malignancies admitted to a palliative care unit. Using a prospectively collected database, we analyzed clinical and laboratory parameters regarding their prognostic impact in 290 patients with malignant hematological diseases. The parameters included patient-related factors such as Eastern Cooperative Oncology Group (ECOG) performance status, need for transfusions, parenteral nutrition or analgetics, and laboratory values (hemoglobin, platelet count, lactic dehydrogenase (LDH), albumin, total protein, calcium, and C-reactive protein (CRP)) as well as referral symptoms (including anemia, infection, fever, fatigue, and dyspnea). In univariate analyses, LDH (>248 U/l), albumin corrected calcium (>2.55 mmol/l), CRP (>50 mg/l), albumin (<30 g/l), platelet count (<90 × 10(9)/l), total protein (≤60 g/l), hemoglobin (<10 g/dl), opioid treatment, performance status (ECOG >2), and need for parenteral nutrition or blood transfusion significantly correlated with impaired survival. Multivariate analysis showed that low performance status, low platelet count, opioid based pain therapy, high LDH, and low albumin were associated with poor prognosis. Using these five parameters, patients were divided into three "risk groups": low risk (presence of zero to one factor), intermediate risk (two to three factors), and high risk. Median survival for the poor risk patients was 10 days, and the intermediate and low risk patients survived a median of 63 and 440 days, respectively (p < 0.0001). Several clinical and laboratory parameters were associated with a poor prognosis of patients with hematological malignancies treated on a palliative care unit. These parameters might help clinicians to estimate prognosis of remaining life span and individualize treatment and/or end-of-life care options for patients.
2014-02
Kripp M; Willer A; Schmidt C; Pilz LR; Gencer D; Buchheidt D; Hochhaus A; Hofmann W-K; Hofheinz R-D
Annals Of Hematology
2014
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Journal Article
<a href="http://doi.org/10.1007/s00277-013-1861-7" target="_blank" rel="noreferrer">10.1007/s00277-013-1861-7</a>
Developmental trajectories of mobility and self-care capabilities in young children with cerebral palsy
Child; Female; Humans; infant; Male; Prospective Studies; Cerebral Palsy; Child Development; Self Care; Preschool; PEDI Study
OBJECTIVE: To describe development of mobility and self-care capabilities in young children (aged 1-4 years) with cerebral palsy, and to examine whether the development of mobility and self-care capabilities differs by cerebral palsy severity in terms of 5 distinct Gross Motor Function Classification System (GMFCS) levels. STUDY DESIGN: This prospective longitudinal cohort study included 100 children with cerebral palsy (aged 1.5 or 2.5 years at baseline) and their parents. Mobility and self-care capabilities were assessed by the Pediatric Evaluation of Disability Inventory during yearly assessments from inclusion up to age 4.5 years. Longitudinal data for 92 children were available for analysis. Repeated-measures analyses with random coefficient analysis were performed using linear mixed models. RESULTS: Despite large variations among individuals in the development of mobility and self-care capabilities in young children with cerebral palsy, distinct developmental trajectories were found for children in different GMFCS levels. The estimated change per month differed significantly by GMFCS level for both outcomes. CONCLUSIONS: This longitudinal study provides an evidence base for prognosis in daily mobility and self-care skills in young children with cerebral palsy. The developmental trajectories for GMFCS levels can be helpful in communication between professionals and also in discussions of expectations and goal setting with families regarding mobility and self-care in the daily life of young children with cerebral palsy in neonatal follow-up and pediatric practice.
2014-04
Ketelaar M; Gorter JW; Westers P; Hanna S; Verhoef M
The Journal Of Pediatrics
2014
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Journal Article
<a href="http://doi.org/10.1016/j.jpeds.2013.11.070" target="_blank" rel="noreferrer">10.1016/j.jpeds.2013.11.070</a>
Pediatric advance directives: parents' knowledge, experience, and preferences
Child; Cross-Sectional Studies; Female; Humans; Male; Palliative Care; Adult; Attitude to Health; Logistic Models; Prospective Studies; Health Services Needs and Demand; Socioeconomic Factors; end of life; adolescent; Preschool; infant; advance care planning; DNAR; Parents; Parents/px [Psychology]; Advance Directives; Hispanic Americans/sn [Statistics & Numerical Data]; Advance Directives/px [Psychology]; decision making; Attitude; Chronic disease; special-needs children
OBJECTIVES: To explore parents' and caregivers' experience, knowledge, and preferences regarding advance directives (ADs) for children who have chronic illness. METHODS: We conducted a prospective, cross-sectional survey of parents and caregivers of children who have chronic illness. During ambulatory medical visits, participants were asked about previous AD experience and knowledge, future preferences regarding AD discussions, their child's past and current health status, and family demographics. RESULTS: Among 307 participants surveyed, previous AD experience was low, with 117 (38.1%) having heard of an AD, 54 (17.6%) having discussed one, and 77 (25.1%) having known someone who had an AD. Furthermore, 27 (8.8%) participants had an AD or living will of their own, and 8 (2.6%) reported that their chronically ill child had an AD. Previous AD knowledge was significantly more likely among parents and caregivers who had a college degree than those who did not have a high school diploma, yet significantly less likely among primarily Spanish-speaking parents and caregivers than those primarily English-speaking. Interest in creating an AD for the child was reported by 151 (49.2%) participants, and was significantly more likely among families who had more frequent emergency department visits over the previous year. CONCLUSIONS: The limited AD experience and knowledge of parents and caregivers of children who have chronic illness and their interest in creating an AD suggest an unmet need among families of children who have chronic illness, and an opportunity to enhance communication between families and medical teams regarding ADs and end-of-life care.
2014-08
Liberman DB; Pham PK; Nager AL
Pediatrics
2014
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Journal Article
<a href="http://doi.org/10.1542/peds.2013-3124" target="_blank" rel="noreferrer">10.1542/peds.2013-3124</a>
Videoconferencing to reduce stress among hospitalized children
Child; Female; Hospitalization; Humans; Male; Parents; Prospective Studies; Videoconferencing; Stress; Psychological; Hospitalized; Propensity Score
OBJECTIVES: Family-Link is a videoconferencing program that allows hospitalized children and their parents to virtually visit family members and friends using laptops, webcams, and a secure Wi-Fi connection. We evaluated the association of Family-Link use on the reduction in stress experienced by children during hospitalization. METHODS: We offered Family-Link to pediatric patients who had an expected length of hospitalization equal to or greater than 4 days. We measured the stress levels of hospitalized children at admission and discharge using the previously published Parental Stress Survey. We used propensity score matching and multivariable linear regression methods to evaluate the relationship between the use of Family-Link and stress experienced by children during hospitalization. RESULTS: We included a total of 367 children in the study: 232 Family-Link users and 135 non-Family-Link users. Using the propensity score matching method, we found that the use of Family-Link was significantly associated with a greater reduction in overall mean stress compared with non-Family-Link users among the cohort of patients who lived closer to the hospital and had shorter lengths of hospitalization (β = 0.23; 95% confidence interval, 0.03 to 0.43; P < .05). In this cohort, the reduction in overall mean stress was 37% greater among Family-Link users than non-Family-Link users. CONCLUSIONS: The use of videoconferencing by some hospitalized children and families to conduct virtual visits with family and friends outside of the hospital was associated with a greater reduction in stress during hospitalization than those who did not use videoconferencing.
2014-07
Yang NH; Dharmar M; Hojman NM; Sadorra CK; Sundberg D; Wold GL; Parsapour K; Marcin JP
Pediatrics
2014
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Journal Article
<a href="http://doi.org/10.1542/peds.2013-3912" target="_blank" rel="noreferrer">10.1542/peds.2013-3912</a>
Bereaved parents' intentions and suggestions about research autopsies in children with lethal brain tumors
Child; Humans; bereavement; Parents; Prospective Studies; Attitude; Autopsy; Biomedical Research; Intention; Brain neoplasms
OBJECTIVE: To determine bereaved parents' perceptions about participating in autopsy-related research and to elucidate their suggestions about how to improve the process. STUDY DESIGN: A prospective multicenter study was conducted to collect tumor tissue by autopsy of children with diffuse intrinsic pontine glioma. In the study, parents completed a questionnaire after their child's death to describe the purpose for, hopes (ie, desired outcomes of), and regrets about their participation in autopsy-related research. Parents also suggested ways to improve autopsy-related discussions. A semantic content analytic method was used to analyze responses and identify themes within and across parent responses. RESULTS: Responses from 33 parents indicated that the main reasons for participating in this study were to advance medical knowledge or find a cure, a desire to help others, and choosing as their child would want. Parents hoped that participation would help others or help find a cure as well as provide closure. Providing education/anticipatory guidance and having a trusted professional sensitively broach the topic of autopsy were suggestions to improve autopsy discussions. All parents felt that study participation was the right decision, and none regretted it; 91% agreed that they would make the choice again. CONCLUSION: Because autopsy can help advance scientific understanding of the disease itself and because parents reported having no regret and even cited benefits, researchers should be encouraged to continue autopsy-related research. Parental perceptions about such studies should be evaluated in other types of pediatric diseases.
2013-08
Baker JN; Windham JA; Hinds PS; Gattuso JS; Mandrell BN; Gajjar P; West NK; Hammarback T; Broniscer A
The Journal Of Pediatrics
2013
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Journal Article
<a href="http://doi.org/10.1016/j.jpeds.2013.01.015" target="_blank" rel="noreferrer">10.1016/j.jpeds.2013.01.015</a>
Characteristics of family conferences at the bedside versus the conference room in pediatric critical care
Child; Female; Humans; Male; Intensive Care Units; patient care team; decision making; Cohort Studies; Health Care Surveys; Parents; Attitude of Health Personnel; Questionnaires; Prospective Studies; Professional-Family Relations; Physicians; Patient Participation; Intensive Care; Pediatric
OBJECTIVE: To compare characteristics of family conferences at the bedside vs. the conference room in the PICU. DESIGN: Single-site, cohort survey study. SETTING: Thirty-three bed academic PICU in an urban setting. PARTICIPANTS: Ten PICU physicians (90.9%) providing care to 29 patients whose families participated in 58 family conferences. MEASUREMENTS AND MAIN RESULTS: Family conferences, defined as a meeting involving the parent(s) of a PICU patient and the critical care attending physician to discuss a treatment decision, redirection of care from curative to palliative, or deliver bad news, occurred most commonly among families of the sickest patients. Conferences were conducted at the bedside 20 times out of 58 (33%). Although physicians stated a general preference to discuss withdrawal or withholding care in the conference room, there was no difference in location during actual conferences. Physicians preferred the bedside when they wanted the patient to participate (p = 0.01) or because it was perceived to be easier (p < 0.0005) or faster (p = 0.016) to conduct, while the conference room was preferred when additional space was needed (p < 0.0005). Family conferences at the bedside were less likely to include a social worker (p < 0.0005), consultant physicians (p = 0.043), or father of the patient (p = 0.006) as compared with conferences in a conference room. Family conferences convened to discuss a treatment were followed by a decision within 24 hours (42% of the time) and a change in code status (32% of the time). In 32 of 58 family conferences (55%), the attending physician did not have a prior relationship with the family. CONCLUSION: Family conferences in the PICU are common both at the bedside and in conference rooms in a subpopulation of the most critically ill children and frequently result in a treatment decision or change in code status.
2013-03
October TW; Watson Anne C; Hinds PS
Pediatric Critical Care Medicine
2013
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Journal Article
<a href="http://doi.org/10.1097/PCC.0b013e318272048d" target="_blank" rel="noreferrer">10.1097/PCC.0b013e318272048d</a>
Parental explicit heuristics in decision-making for children with life-threatening illnesses
Child; Female; Humans; Male; Young Adult; Cohort Studies; Prospective Studies; Professional-Family Relations; Communication; Choice Behavior; Problem Solving; Hospitals; Pediatric; adolescent; Preschool; Psychological; decision making; infant; Parents/psychology; Palliative Care/psychology; Philadelphia; Parent caregivers; Chronic Disease/psychology/therapy; Interview; Judgment; Aphorisms and Proverbs as Topic; Critical Illness/psychology/therapy
OBJECTIVE: To identify and illustrate common explicit heuristics (decision-making aids or shortcuts expressed verbally as terse rules of thumb, aphorisms, maxims, or mantras and intended to convey a compelling truth or guiding principle) used by parents of children with life-threatening illnesses when confronting and making medical decisions. METHODS: Prospective cross-sectional observational study of 69 parents of 46 children who participated in the Decision-making in Pediatric Palliative Care Study between 2006 and 2008 at the Children's Hospital of Philadelphia. Parents were guided individually through a semistructured in-depth interview about their experiences and thoughts regarding making medical decisions on behalf of their ill children, and the transcribed interviews were qualitatively analyzed. RESULTS: All parents in our study employed explicit heuristics in interviews about decision-making for their children, with the number of identified explicit heuristics used by an individual parent ranging from tens to hundreds. The heuristics served 5 general functions: (1) to depict or facilitate understanding of a complex situation; (2) to clarify, organize, and focus pertinent information and values; (3) to serve as a decision-making compass; (4) to communicate with others about a complex topic; and (5) to justify a choice. CONCLUSIONS: Explicit heuristics played an important role in decision-making and communication about decision-making in our population of parents. Recognizing explicit heuristics in parent interactions and understanding their content and functions can aid clinicians in their efforts to partner with parents in the decision-making process.
Renjilian CB; Womer JW; Carroll KW; Kang T; Feudtner C
Pediatrics
2013
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Journal Article
<a href="http://doi.org/10.1542/peds.2012-1957" target="_blank" rel="noreferrer">10.1542/peds.2012-1957</a>
Talking with parents about end-of-life decisions for their children
adolescent; Child; Female; Humans; infant; Male; Intensive Care Units; Palliative Care; Advance Directives; decision making; Pediatrics; Withholding Treatment; Prospective Studies; Professional-Family Relations; Communication; Life Support Care; Netherlands; Qualitative Research; Pediatric; adolescent; Preschool; decision making; infant; Newborn; DNAR; Professional-Family Relations; Pediatrics; Advance Directives; Communication
BACKGROUND AND OBJECTIVE: Retrospective studies show that most parents prefer to share in decisions to forgo life-sustaining treatment (LST) from their children. We do not yet know how physicians and parents communicate about these decisions and to what extent parents share in the decision-making process. METHODS: We conducted a prospective exploratory study in 2 Dutch University Medical Centers. RESULTS: Overall, 27 physicians participated, along with 37 parents of 19 children for whom a decision to withhold or withdraw LST was being considered. Forty-seven conversations were audio recorded, ranging from 1 to 8 meetings per patient. By means of a coding instrument we quantitatively and qualitatively analyzed physicians' and parents' communicative behaviors. On average, physicians spoke 67% of the time, parents 30%, and nurses 3%. All physicians focused primarily on providing medical information, explaining their preferred course of action, and informing parents about the decision being reached by the team. Only in 2 cases were parents asked to share in the decision-making. Despite their intense emotions, most parents made great effort to actively participate in the conversation. They did this by asking for clarifications, offering their preferences, and reacting to the decision being proposed (mostly by expressing their assent). In the few cases where parents strongly preferred LST to be continued, the physicians either gave parents more time or revised the decision. CONCLUSIONS: We conclude that parents are able to handle a more active role than they are currently being given. Parents' greatest concern is that their child might suffer.
2015-02
de Vos MA; Bos AP; Plötz FB; van Heerde M; de Graaff Bert M; Tates K; Truog RD; Willems DL
Pediatrics
2015
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Journal Article
<a href="http://doi.org/10.1542/peds.2014-1903" target="_blank" rel="noreferrer">10.1542/peds.2014-1903</a>
No change in serum melatonin, or plasma beta-endorphin levels after sevoflurane anesthesia
Female; Humans; Cohort Studies; Adult; Prospective Studies; Middle Aged; Time Factors; Reference Values; Anesthetics; Anesthesia; beta-Endorphin/blood/drug effects; Blood Pressure/drug effects; Dilatation and Curettage/methods; Electroencephalography/methods; General/methods; Heart Rate/drug effects; Inhalation/administration & dosage/pharmacology; Melatonin/blood; Methyl Ethers/administration & dosage/pharmacology; Oximetry/methods; Oxygen/blood; Postoperative Period; Sleep/drug effects
STUDY OBJECTIVE: To investigate the effect of sevoflurane as single anesthetic on melatonin and beta-endorphin plasma levels during the first 24 hours postoperatively. DESIGN: Prospective, open-cohort study. SETTING: University hospital. PATIENTS: 13 ASA physical status I and II, adults, scheduled for dilatation and curettage of the uterus, and 13 healthy volunteers. INTERVENTIONS: Patients received general anesthesia with sevoflurane. MEASUREMENTS: Melatonin and beta-endorphin plasma levels were determined before anesthesia, immediately after, and two, 4, 8, and 24 hours after the end of anesthesia. Melatonin and beta-endorphin were also measured in 13 healthy subjects (controls) not undergoing anesthesia at similar times during the day. Systolic and diastolic blood pressure, heart rate, bispectral index, and oxygen saturation via pulse oximeter (SpO(2)) were recorded before and after anesthesia. Quality of sleep postoperatively was also assessed. MAIN RESULTS: Melatonin levels (pg/mL) in the patients and controls were 8.2 +/- 7.9 versus 15.2 +/- 15.0 before anesthesia and 7.7 +/- 7.9 versus 11.1 +/- 7.0, 6.5 +/- 6.1 versus 15.6 +/- 16.3, and 19.5 +/- 17.9 versus 23.7 +/- 23.3 at the end of anesthesia and 4 and 24 hours after the end of anesthesia, respectively (P = 0.057). At the same time points, beta-endorphin plasma levels (pmol/L) in patients and controls were 5.2 +/- 2.0 versus 4.0 +/- 2.3, 5.4 +/- 3.3 versus 3.9 +/- 2.5, 4.9 +/- 1.2 versus 4.4 +/- 1.7, and 3.7 +/- 2.6 versus 4.2 +/- 1.8, respectively (P= 0.285). The quality of sleep assessed clinically was not altered. CONCLUSION: Sevoflurane as a single anesthetic for minor gynecological procedures did not influence significantly melatonin or beta-endorphin plasma levels. Sleep quality assessed clinically was not influenced.
2007
Fassoulaki A; Kostopanagiotou G; Meletiou P; Chasiakos D; Markantonis S
Journal Of Clinical Anesthiology
2007
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Journal Article
<a href="http://doi.org/10.1016/j.jclinane.2006.07.003" target="_blank" rel="noreferrer">10.1016/j.jclinane.2006.07.003</a>