1. An Assessment of Goals of Care for Children with Complex, Life-Limiting Conditions Gary D. Ceneviva MD, Nicole C. Hahnlen RN, Michele L. Shaffer PhD, Paul Felker MSW, Jennifer L. Ruth MD, Harry P. Bramley DO, Robert F. Tamburro MD Pennsylvania State University, Departments of Pediatrics and Public Health Sciences Penn State Hershey Children’s Hospital, Hershey, PA Author Name, Author Name, Affiliation, Affiliation, Affiliation, Affiliation, Affiliation The Hummingbird Program at Penn State Hershey Children’s Hospital was established thanks to the generous gift of Ed and Jeanne Arnold Abstract Introduction: Palliative care seeks to relieve physical, emotional, social, and spiritual distress, assist in decision-making, and enhance quality of life (QOL). Although goals of care should be elicited to assist families in decision-making, little has been published addressing this topic in children with life-limiting conditions. Hypothesis: Goals of care for children with life-limiting conditions are individualized, yet share commonality. Methods: Goals of care obtained during initial consultation from 1/07-6/08 were reviewed. Data abstracted included demographics, diagnoses, time from diagnosis until consult, and level of support. The WHO QOL-BREF assessment was utilized to categorize goals of care into 1 of 4 domains: 1)physical/ independence 2) psychological/spiritual 3) social and 4) environment. McNemar's test and logistic regression analysis were performed. Results : 104 goals of care were identified among 33 patients (1-7 goals/patient). Ages ranged from newborn to 19 years (median 3 years). Diagnosis to consult time ranged from < 1 month to 19 years (median 14 months). Diagnostic categories included neuromuscular (n=14), genetic (n=6), cardiovascular (n=4), respiratory (n=3), malignancy (n=2), and others (n=4). 13 children had support limited. Physical/independence was the most common QOL domain reported (63% of goals). All patients identified at least one goal pertaining to a physical domain; more than any other domain (p < 0.001). Comfort, independence, health maintenance, and being home were the most common goals expressed. Age, ethnicity, spirituality, diagnosis, diagnosis to consult time, and level of support had no statistical association with the goals of care. Conclusions: Goals of care for children with life-limiting conditions are individualized and commonly include physical and independence QOL domains. Understanding these goals may assist in complex decision-making and use of program resources. Assessing data over larger patient populations and monitoring goals over time is recommended. Introduction  Children with complex, life-limiting conditions are a highly diverse population with a variety of illness trajectories that are often characterized by prognostic uncertainty.  Families of these children frequently face many challenging decisions.  Palliative care seeks to relieve the physical, emotional, social, and spiritual distress produced by these life-limiting conditions, assist in complex decision making, and enhance quality of life.  To assist families in making these complex decisions, it is recommended that goals of care be elicited.  Very little has been published regarding the parental goals of care for these children. Objective The objective of this study is to describe goals of care for children with complex life-limiting conditions and to assess variables that may influence the goals chosen by families for these children. Methods  Goals of care were elicited from the parents and children with complex life- limiting conditions during initial consult from January 2007 through June 2008.  A complex medical condition was defined as any serious illness or injury that is either life-limiting or life- threatening and requires specialty pediatric care and hospitalization in a tertiary care center.  Data abstracted included: diagnoses, demographics, time from diagnosis until consult, and resuscitative status.  Primary diagnoses were categorized into one of the following categories: neuromuscular, cardiovascular, respiratory, renal, gastrointestinal, hematologic or immunologic, metabolic, genetic, and malignancy.  The World Health Organization’s QOL- BREF assessment was utilized to categorize goals of care into 1 of 4 domains 1) physical/independence, 2) psychological/spiritual, 3) social, and 4) environment.  Comparisons between domains in the percentages of patients identifying at least one goal were made using McNemar’s tests. Logistic regression was used to identify associations between domains and other variables. Results  104 goals of care were identified among 33 patients (1-7 goals/patient)  Ages ranged from newborn to 19 years with a median of 3 years  Diagnosis to consult time ranged from less than a month to 19 years (median 14 months)  Table 1 denotes primary diagnoses  Goals relating to the physical health and independence QOL domain were the most commonly reported (Table 2)  All patients identified at least one goal pertaining to a physical health domain ; more than any other domain (p < 0.001).  13 children had support limited  Age, ethnicity, spirituality, diagnosis, diagnosis to consult time, and level of support had no statistical association with the goals of care Results Conclusions & Implications  Goals of care for children with complex life- limiting conditions are individualized and commonly include physical and independence QOL domains.  Understanding these goals may provide guidance during complex decision-making and utilization of program resources.  Assessing these data over larger patient populations and monitoring goals over time is recommended Table 1. Primary Diagnoses Neuromuscular (N = 15) Genetic (N = 6) Holoprosencephaly 1q21.1 deletion Grade IV IVH/ Hydrocephalus Trisomy 10p Traumatic brain injury Epidermolysis bullosa Lissencephaly Triploidy 69 XXXY Hypoxic ischemic encephalopathy Mucopolysaccharidosis type 3 Hydranencephaly Jacobsen / Paris-Trousseau Spinal muscular atrophy type 1 Hematologic / Immunologic (N = 2) Cerebral palsy Klippel-Trenaunay syndrome Near-drowning Wegener’s granulomatosis Meningitis Malignancy (N = 2) Respiratory (N = 3) ALL s/p HSCT ; chronic GVHD Cystic Fibrosis Metastatic osteosarcoma Pulmonary alveolar proteinosis Renal (N=1) Cardiovascular (N = 4) Renal agenesis Congenital cyanotic heart disease Table 2. Goals of Care (N=104) Physical Health (N=65,63%) To be comfortable or without suffering 19 To restore/ maintain health, including growth 11 To improve quality of life 8 To promote independence 8 To achieve age appropriate milestones/ activities 7 To reduce physical symptoms 5 To increase mobility 3 To prolong life 2 To reduce dependence on treatments/ technology 2 Environment (N= 17,16%) To be home 11 To improve health care/ coordination of care 3 To return to school 2 To have privacy for family visitation 1 Psychological / Spiritual (N=15,14%) To enhance positive feelings/self esteem 8 To receive sacraments/ return to church 3 To maintain appearance 2 To reduce anxiety 1 To minimize cognitive deficits 1 Social Relationships (N=7, 7%) To be accepted by friends/ family 3 To remain part of family 2 To communicate with others 1 To create family memories 1 ID # 420