Community-Based Pediatric Palliative Care: How Services Support Children's and Families' Quality of Life
mental health; child; article; controlled study; human; male; palliative therapy; pain; sibling; spiritual care; quality of life; interview; interpersonal communication; cost effectiveness analysis; adolescent; socialization; caregiver; convenience sample; bereavement support; family; clinician; participatory research; self care; psychological well-being; physical well-being; disease management; community; community integration
Background: The Massachusetts Department of Public Health's Pediatric Palliative Care Network (PPCN) provides Community-Based Pediatric Palliative Care (CBPPC) to children with life-limiting conditions and their families. CBPPC services aim to improve children and families' quality of life (QOL). <br/>Objective(s): To identify perceived domains of QOL important for children and families and to understand whether and how CBPPC supports QOL. <br/>Design(s): A community-based participatory research framework was used to develop recruitment and data collection materials for eight focus groups and seven interviews. Collected data were transcribed and analyzed with an inductive approach. Setting/Subjects: A convenience sample of 33 PPCN caregivers, 20 providers, and seven key informants, including policymakers, community organizations, and hospital-based clinicians, were interviewed virtually in the United States. Measurements: Perceived QOL domains for children and families, respectively, and perceived impact of CBPPC services on QOL. <br/>Result(s): Reported QOL domains described as important for children were socialization/community integration and accessibility; expression/play; and physical wellness. Control or autonomy, psycho-emotional wellness, and self-care were identified as important for families. Clinical services were described as "integral to mental health" through offered spiritual support; advocacy in the community; and education. PPCN's integrative services were noted as distractions from pain and helped improve communication and bonding. Sibling support and bereavement care were also mentioned as impactful on QOL. <br/>Conclusion(s): Family-centered CBPPC was described as supportive of children's and families' QOL. Future studies should consider using population-based QOL measures, leveraging the QOL domains identified through this analysis and other outcome measures in a cost-effectiveness analysis.
Wang Y; Ferreira E; Savageau J; Beitman A; Young M; Gabovitch E; Merriam G; Jozan A; Padgett K; Bateman S
Journal of Palliative Medicine
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/10.1089/jpm.2023.0147" target="_blank" rel="noreferrer noopener">10.1089/jpm.2023.0147</a>
Development, behavior, and biomarker characterization of Smith-Lemli-Opitz syndrome: An update
adolescent; Autism; parent; development; cognition; behavior; Developmental delay; problem behavior; priority journal; case study; interpersonal communication; preschool child; observational study; unclassified drug; childhood; human; article; child; female; male; adult; clinical article; young adult; automutilation; 7 dehydrocholesterol/ec [Endogenous Compound]; 8 dehydrocholesterol/ec [Endogenous Compound]; adaptive behavior; aggression; cerebrospinal fluid level; child rearing; cholesterol/ec [Endogenous Compound]; intelligence quotient; Smith Lemli Opitz syndrome; Smith-Lemli-Opitz syndrome; socialization; Stanford-Binet Intelligence Scale; Sterols; walking; behavior; tone and motor problems; trajectory; characteristics; aggression; development; delayed development
Background: Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive inborn error of cholesterol metabolism syndrome with neurocognitive manifestations. SLOS is the result of mutations in the gene encoding the 7-dehydrocholesterol reductase, which results in the elevation of the cholesterol precursor 7-dehydrocholesterol (7-DHC). Previous reports indicate that intellectual disability, behavioral disturbances, and autism symptoms are frequently part of the SLOS behavioral phenotype. In the current study, we characterize the developmental history and current behavior of 33 individuals with SLOS aged 4 to 23 years and report on biomarkers 7-DHC and 8-DHC in relation to cognition and behavior. Methods: This was an observational case series, wherein participants with SLOS underwent extensive behavioral evaluation of cognitive function, adaptive function, autism symptoms, and problem behaviors, in addition to parent report of developmental milestones. Serum and CSF were contemporaneously obtained from the majority of participants. Results: Developmental milestones such as walking, talking, and toileting were uniformly delayed. Overall levels of cognitive and adaptive functioning were low; no participant received adaptive behavior scores in the average range, and the mean level of cognitive functioning in the full sample was in the moderate range of impairment. Aggressive behavior was present in nearly half of participants. Although the majority of participants had elevated scores on the gold standard autism diagnostic instruments, only about half of participants received a clinical diagnosis of autism spectrum disorder. Finally, while CSF cholesterol was not found to correlate with cognitive or adaptive functioning, both serum and CSF 7-DHC and 8-DHC (and their ratios with cholesterol) were moderately and negatively correlated with functioning in this group. Conclusions: A history of developmental delay, followed by intellectual disability, is common in individuals with SLOS. Although autism spectrum disorder appears to be a frequent diagnosis in this population, it is apparent that the low level of functioning observed in SLOS may artificially inflate scores on standard autism assessments. Our findings further support that cholesterol precursors 7-DHC and 8-DHC are important biomarkers of the level of functioning in SLOS, especially regarding cognitive abilities, and thus may be to explore as mediators within the context of treatment trials.
Thurm A; Tierney E; Farmer C; Albert P; Joseph L; Swedo S; Bianconi S; Bukelis I; Wheeler C; Sarphare G; Lanham D; Wassif C A; Porter F D
Journal of Neurodevelopmental Disorders
2016
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/10.1186/s11689-016-9145-x" target="_blank" rel="noreferrer noopener">10.1186/s11689-016-9145-x</a>
Autism traits in children and adolescents with Cornelia de Lange syndrome
Communication; adolescent; Autism; emotion; age; verbal communication; communication disorder; priority journal; interpersonal communication; skill; intellectual impairment; human; article; child; female; male; adult; diagnosis; clinical article; daily life activity; disease severity; autism; Cornelia de Lange syndrome; phenotype; compulsion; language; stereotypy; adaptive behavior; socialization; Aberrant Behavior Checklist; behavior disorder; Behavioral phenotype; checklist; Childhood Autism Rating Scale; de Lange syndrome; hyperactivity; lethargy; limb; maladjustment; psychological rating scale; rating scale; Repetitive behaviors; rigidity; social cognition; Social cognition; Vineland Adaptive Behaviors Scales; behavioral problems; De Lange syndrome; trajectory; characteristics; irritability
Cornelia de Lange syndrome (CdLS) is a cohesinopathy causing delayed growth and limb deficits. Individuals with CdLS have mild to profound intellectual disability and autistic features. This study characterizes the behavioral phenotype of children with CdLS, focusing on autistic features, maladaptive behaviors, and impact of age. Children with CdLS (5-18 years) were administered normed instruments to characterize autism features (Childhood Autism Rating Scale, CARS), maladaptive behaviors (Aberrant Behavior Checklist), and adaptive skills (Vineland Adaptive Behaviors Scales). CdLS features and severity were rated with Diagnostic Criteria for CdLS. Forty-one children with CdLS (23 females, 18 males) were classified as having "no autism" (n=7; 17.1%), "mild autism" (n=17; 41.4%), and "severe autism" (n=17; 41.4%), using CARS scores. Characteristic items were abnormal emotional response, stereotypies, odd object use, rigidity, lack of verbal communication, and low intellectual functioning. Verbal communication deficits and repetitive behaviors were higher compared to sensory, social cognition, and behavior abnormalities (P<0.0001). Maladaptive behaviors associated with autism traits were stereotypies (P=0.003), hyperactivity (P=0.01), and lethargy (P=0.03). Activities of daily living were significantly affected; socialization adaptive skills were a relative strength. However, with advancing age, both socialization (P<0.0001) and communication (P=0.001) domains declined significantly. CdLS is characterized by autistic features, notably excessive repetitive behaviors and expressive language deficits. While other adaptive skills are impacted, socialization adaptive skills are less affected. Advancing age can worsen communication and socialization deficits relative to neurotypical peers. © 2014 Wiley Periodicals, Inc.
Srivastava S; Landy-Schmitt C; Clark B; Kline A D; Specht M; Grados M A
American Journal of Medical Genetics Part A
2014
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.a.36573" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.36573</a>
Enhancing resilience in hospitalized children and adolescents
hospitalized child; Anxiety; biofeedback; California; Child; clinical psychology; Comorbidity; complication; distress syndrome; Female; Hospitalization; Human; Male; Mental Health; music; pain; Palliative therapy; pet therapy; PostTraumatic Stress Disorder; psychiatrist; psychosocial care; Socialization; wellbeing
Objectives: This presentation will describe a collaborative intervention that can enhance resilience in hospitalized patients when they are struggling with the emotional sequelae of their medical illness. The presentation will discuss the specialty services at the University of California, San Francisco, Benioff Children's Hospital (UCSF BCH) that work cooperatively to support salutogenesis (the origin of health), in contrast to pathogenesis. The efficacy of our multidisciplinary approach will be demonstrated through descriptions of changes in patients' mental status and subjective reports of distress from patients and parents. Methods: This seminar will present our multidisciplinary approach to enhancing well-being and supporting resilience. The presentation will describe the collaboration of several complementary specialties. Primary source material and anecdotal reports from clinical cases will be presented from these medical, psychological, and psychosocial disciplines. Results: At UCSF BCH, specialists play complementary roles in the effort to support the emotional well-being of hospitalized patients. The child and adolescent consultation-liaison psychiatrist provides insight into psychiatric comorbidities that may be contributing to distress and interfere with coping. Pharmacologic interventions target the symptoms of these comorbid illnesses. Pediatric palliative care physicians address the physiologic symptoms endured by children with serious medical illnesses. Clinical psychologists help to identify the feedback loop between anxiety and pain and enhance a sense of safety through empirically validated interventions for anxiety and traumatic stress. The Child Life Specialist's role includes preparation for procedures; creating and implementing a coping plan for individual procedures and overall hospitalization through art and music; and providing psychosocial support through socialization, medical play, pet therapy, and biofeedback. Conclusions: Diverse disciplines can work collaboratively to support the overall well-being of the hospitalized child and his/her family members by effectively promoting coping. This multidisciplinary approach to enhancing resilience enables children to thrive even if their illnesses are life-limiting.
2017
Ihle EC
Journal Of The American Academy Of Child And Adolescent Psychiatry
2017
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.jaac.2017.07.064" target="_blank" rel="noreferrer">10.1016/j.jaac.2017.07.064</a>
Sexual health and chronic illness in childhood
Child; Humans; Patient-Centered Care; Nurse's Role; Child Psychology; Child Development; Body Image; adolescent; Adolescent Transitions; Chronic Disease/nursing/psychology; Pediatric Nursing/organization & administration; Mass Media; Socialization; Reproductive Medicine; Sex Education/organization & administration; Sexuality/physiology/psychology
2004
Finnegan A
Paediatric Nursing
2004
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).
Journal Article
<a href="http://doi.org/10.7748/paed2004.09.16.7.32.c939" target="_blank" rel="noreferrer">10.7748/paed2004.09.16.7.32.c939</a>
Gender differences in parenting a child with cancer
Child; Female; Humans; Male; Adult; Attitude to Health; Parent-Child Relations; Questionnaires; adolescent; Preschool; Adaptation; Psychological; Models; Parents/psychology; Parent caregivers; Gender Identity; Neoplasms/psychology; Parenting/psychology; Socialization
This study of 124 parents of children diagnosed with cancer investigates parents' perceptions of their role in the illness situation. The study found that mothers and fathers differ in their experience of and response to parenting a child with cancer. These differences appear to reflect traditional parenting roles characterized by a gender-based division of labor. Sex-role socialization theory is discussed as an explanatory model of the parenting experience. Practice recommendations are offered to medical social workers and other health care professionals concerned about the long term psychosocial adjustment of parents with chronically ill children.
1996
Brown KA; Barbarin OA
Social Work In Health Care
1996
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).
Journal Article
<a href="http://doi.org/10.1300/j010v22n04_04" target="_blank" rel="noreferrer">10.1300/j010v22n04_04</a>
Society and the adolescent self-image
Adolescent Psychology; Self Concept; adolescent; Socialization
Rosenberg M
1965
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Book/Book Section