Normalization of Symptoms in Advanced Child Cancer: The PediQUEST-Response Case Study.

CONTEXT: Children, adolescents and young adults with cancer continue to experience significant symptom suffering throughout their illness. OBJECTIVES: To identify barriers to effective symptom management in pediatric advanced cancer. METHODS: Using a qualitative multiple case study we refined the Pediatric Quality of Life and Evaluation of Symptoms Technology Response to the Pediatric Oncology Symptom Experience (PediQUEST Response), a pediatric palliative care (PPC) intervention. Twenty-three children aged ≥2 years old with advanced cancer, their parents and primary and PPC clinicians were enrolled. Children and parents reported symptoms weekly over 4-months using the Memorial Symptom Assessment Scale (MSAS) administered by an electronic system (PediQUEST). When symptom distress episodes (SDEs) were reported (MSAS symptom score ≥33) we studied symptom management processes using interviews of family members/clinicians, and chart abstractions. Data were coded and analyzed using grounded theory and NVivo software. RESULTS: Children reported 308 SDEs within 193 surveys and parents 529 SDEs in 165 surveys administered. We conducted 85 and 88 interviews with families and clinicians respectively. While we confirmed the presence of known barriers, we identified a prominent theme, that symptoms were "normalized." Patients, parents, and all clinicians, including PPC specialists, got accustomed to high symptom burden and lacked expectations that distress could be alleviated. We defined "normalization of symptoms," as the process by which symptom related suffering is appraised as unavoidable. CONCLUSION: Normalization of symptoms is a pervasive barrier enacted by all involved in caring for children with advanced cancer. Strategies to overcome normalization are critical to ease child distress.

Spanish Adaptation of the Pediatric Memorial Symptom Assessment Scale for Children, Teens, and Caregivers.

CONTEXT: There are no validated Spanish tools to assess symptom burden in pediatric cancer. The Pediatric Memorial Symptom Assessment Scale (Pediatric-MSAS) is an English valid multidimensional and comprehensive instrument. OBJECTIVES: To validate Pediatric-MSAS-Spanish (MSAS-Child, MSAS-Teen, and MSAS-Caregiver versions) in patients with cancer treated in two public hospitals in Buenos Aires, Argentina. METHODS: Cross-sectional study, classical psychometric theory. We recruited a convenience sample of 148 caregivers of children ≥ two years, 51 young children (seven to 12 years), and 48 adolescents (≥13 years). We assessed feasibility, comprehensibility, internal consistency, and convergent and known-groups validity. RESULTS: Pediatric-MSAS-Spanish was feasible, acceptable, and comprehensible. Reliability of MSAS-total and subscale scores was satisfactory (Cronbach alpha: 0.90, 0.89, 0.71, respectively, for caregiver, teen, and child MSAS-total score). MSAS-total caregiver, teen, and child scores met a priori criteria for convergent validity correlating with Pediatric Quality of Life Inventory total scores (Spearman correlation (rs) = -0.59, -0.66, and -0.32, respectively) and visual -analogue well-being scores (rs = -0.63, -0.46, and -0.4, respectively). Caregiver-teen correlation was strong for total (rs = 0.78) and physical (rs = 0.85) scores, and moderate for global distress index (rs = 0.64) and psychological (rs = 0.45) scores. MSAS-total caregiver-child correlation was moderate (rs = 0.30) and Kappa analysis showed poor agreement. All MSAS-Caregiver scores and MSAS-Teen total and physical scores differentiated inpatients/outpatients and patients on/off-treatment, while MSAS-Teen psychological and global distress index subscales or MSAS-Child scores did not. CONCLUSION: Pediatric-MSAS-Spanish is feasible and reliable for assessing symptom burden in children with cancer. Validity of MSAS-Caregiver and MSAS-Teen was largely supported. Further work on MSAS-Child is warranted.

Development of a Stakeholder Driven Serious Illness Communication Program for Advance Care Planning in Children, Adolescents, and Young Adults with Serious Illness.

OBJECTIVES: To develop a generalizable advance care planning (ACP) intervention for children, adolescents, and young adults with serious illness using a multistage, stakeholder-driven approach. STUDY DESIGN: We first convened an expert panel of multidisciplinary health care providers (HCPs), researchers, and parents to delineate key ACP intervention elements. We then adapted an existing adult guide for use in pediatrics and conducted focus groups and interviews with HCPs, parents, and seriously ill adolescents and young adults to contextualize perspectives on ACP communication and our Pediatric Serious Illness Communication Program (PediSICP). Using thematic analysis, we identified guide adaptations, preferred content, and barriers for Pedi-SICP implementation. Expert panelists then reviewed, amended and finalized intervention components. RESULTS: Stakeholders (34 HCPs, 9 parents, and 7 seriously ill adolescents and young adults) participated in focus groups and interviews. Stakeholders validated and refined the guide and PediSICP intervention and identified barriers to PediSICP implementation, including the need for HCP training, competing demands, uncertainty regarding timing, and documentation of ACP discussions. CONCLUSIONS: The finalized PediSICP intervention includes a structured HCP and family ACP communication occasion supported by a 3-part communication tool and bolstered by focused HCP training. We also identified strategies to ameliorate implementation barriers. Future research will determine the feasibility of the PediSICP and whether it improves care alignment with patient and family goals.

Patterns of Care at the End of Life for Children and Young Adults with Life-Threatening Complex Chronic Conditions.

OBJECTIVE: To characterize patterns of care at the end of life for children and young adults with life-threatening complex chronic conditions (LT-CCCs) and to compare them by LT-CCC type. STUDY DESIGN: Cross-sectional survey of bereaved parents (n = 114; response rate of 54%) of children with noncancer, noncardiac LT-CCCs who received care at a quaternary care children's hospital and medical record abstraction. RESULTS: The majority of children with LT-CCCs died in the hospital (62.7%) with more than one-half (53.3%) dying in the intensive care unit. Those with static encephalopathy (AOR, 0.19; 95% CI, 0.04-0.98), congenital and chromosomal disorders (AOR, 0.28; 95% CI, 0.09-0.91), and pulmonary disorders (AOR, 0.08; 95% CI, 0.01-0.77) were significantly less likely to die at home compared with those with progressive central nervous system (CNS) disorders. Almost 50% of patients died after withdrawal or withholding of life-sustaining therapies, 17.5% died during active resuscitation, and 36% died while receiving comfort care only. The mode of death varied widely across LT-CCCs, with no patients with pulmonary disorders dying receiving comfort care only compared with 66.7% of those with CNS progressive disorders. A majority of patients had palliative care involvement (79.3%); however, in multivariable analyses, there was distinct variation in receipt of palliative care across LT-CCCs, with patients having CNS static encephalopathy (AOR, 0.07; 95% CI, 0.01-0.68) and pulmonary disorders (AOR, 0.07; 95% CI, 0.01-.09) significantly less likely to have palliative care involvement than those with CNS progressive disorders. CONCLUSIONS: Significant differences in patterns of care at the end of life exist depending on LT-CCC type. Attention to these patterns is important to ensure equal access to palliative care and targeted improvements in end-of-life care for these populations.

Research Priorities in Pediatric Palliative Care.

OBJECTIVE: To synthesize the perspectives of a broad range of pediatric palliative care (PPC) clinicians and parents, to formulate a consensus on prioritization of the PPC research agenda. STUDY DESIGN: A 4-round modified Delphi online survey was administered to PPC experts and to parents of children who had received PPC. In round 1, research priorities were generated spontaneously. Rounds 2 and 3 then served as convergence rounds to synthesize priorities. In round 4, participants were asked to rank the research priorities that had reached at least 80% consensus. RESULTS: A total of 3093 concepts were spontaneously generated by 170 experts and 72 parents in round 1 (65.8% response rate [RR]). These concepts were thematically organized into 78 priorities and recirculated for round 2 ratings (n = 130; 53.7% RR). Round 3 achieved response stability, with 31 consensus priorities oscillating within 10% of the mode (n = 98; 75.4% RR). Round 4 resulted in consensus recognition of 20 research priorities, which were thematically grouped as decision making, care coordination, symptom management, quality improvement, and education. CONCLUSIONS: This modified Delphi survey used professional and parental consensus to identify preeminent PPC research priorities. Attentiveness to these priorities may help direct resources and efforts toward building a formative evidence base. Investigating PPC implementation approaches and outcomes can help improve the quality of care services for children and families.

Feasibility of Conducting a Palliative Care Randomized Controlled Trial in Children With Advanced Cancer: Assessment of the PediQUEST Study.

CONTEXT: Pediatric palliative care randomized controlled trials (PPC-RCTs) are uncommon. OBJECTIVES: To evaluate the feasibility of conducting a PPC-RCT in pediatric cancer patients. METHODS: This was a cohort study embedded in the Pediatric Quality of Life and Evaluation of Symptoms Technology Study (NCT01838564). This multicenter PPC-RCT evaluated an electronic patient-reported outcomes system. Children aged two years and older, with advanced cancer, and potentially eligible for the study were included. Outcomes included: pre-inclusion attrition (patients not approached, refusals); post-inclusion attrition (drop-out, elimination, death, and intermittent attrition (IA; missing surveys) over nine months of follow-up); child/teenager self-report rates; and, reasons to enroll/participate. RESULTS: Over five years, of the 339 identified patients, 231 were eligible (in 22, we could not verify eligibility); 84 eligible patients were not approached and 43 declined participation. Patients not approached were more likely to die or have brain tumors. We enrolled 104 patients. Average enrollment rate was one patient per site per month; shortening follow-up from nine to three months (with optional re-enrollment) increased recruitment by 20%. A total of 87 patients completed the study (24 died) and 17 dropped out. Median IA was 41% in the first 20 weeks of follow-up and more than 60% in the eight weeks preceding death. Child/teenager self-report was 94%. Helping others, low burden procedures, incentives, and staff attitude were frequent reasons to enroll/participate. CONCLUSION: A PPC-RCT in children with advanced cancer was feasible, post-inclusion retention adequate; many families participated for altruistic reasons. Strategies that may further PPC-RCT feasibility include: increasing target population through large multicenter studies, approaching sicker patients, preventing exclusion of certain patient groups, and improving data collection at end of life.