Recent Publications from Our Pediatric Palliative Care Research

Background: Health care providers (HCPs) require ongoing training and mentorship to fully appreciate the palliative care needs of children. Project ECHO® (Extension for Community Healthcare Outcomes) is a model for delivering technology-enabled interprofessional education and cultivating a community of practice among HCPs who care for children with life-limiting illness.

Objectives: To develop, implement, and evaluate the Project ECHO model within the pediatric palliative care (PPC) context. Specific objectives were to evaluate (1) participation levels, (2) program acceptability, (3) HCP knowledge changes, (4) HCP self-efficacy changes, and (5) perceived practice changes after six months.

Intervention: An interprofessional PPC curriculum was informed by a needs assessment. The curriculum was delivered through monthly virtual 90-minute TeleECHO sessions (didactic presentation and case-based learning) from January 2018 to December 2019. The program was freely available to all HCPs wishing to participate.

Design: A mixed-methods design with repeat measures was used. Surveys were distributed at baseline and six months to assess outcomes using 7-point Likert scales. Descriptive and inferential statistical analyses were conducted. The study was approved by the Research Ethics Board at the Hospital for Sick Children.

Results: Twenty-four TeleECHO sessions were completed with a mean of 32 ± 12.5 attendees. Acceptability scores (n = 43) ranged from 5.1 ± 1.1 to 6.5 ± 0.6. HCPs reported improvements in knowledge and self-efficacy across most topics (11 out of 12) and skills (8 out of 10) with demonstrated statistical significance (p < 0.05). Most participants reported positive practice impacts, including enhanced ability to provide PPC in their practice.

Conclusion: Project ECHO is a feasible and impactful model for fostering a virtual PPC-focused community of practice among interprofessional HCPs.

Genome-wide sequencing (GWS) is increasingly being used in neonatal intensive care units. While studies have explored its clinical utility, little is known about parental experiences with this testing post-return of results. We conducted a qualitative study, using an interpretive description framework and thematic analysis, to gain further insight into parents’ perceptions of the value and utility of GWS for their infant. We sought to explore whether parents’ perceptions differ if their child received a diagnosis or not, and whether their child is living or deceased. Semi-structured, telephone interviews were conducted with parents of infants who had rapid exome sequencing while in the neonatal intensive care unit at BC Women’s Hospital in Vancouver, Canada. Interviews addressed perceived benefits and harms of GWS and included an evaluation of decisional regret. Parents of 27 probands were approached and 14 (52%; 13 mothers and 1 father) participated in interviews. On average, 26 months had elapsed from the time of results to the interview. Six themes were identified. Firstly, parents had a positive regard for GWS. The results of GWS helped provide context for their child’s admission to the NICU, and all parents experienced relief following receiving the results. A diagnosis by GWS enabled parents to picture the future, form connections with other parents, and coordinate their child’s care. Lastly, some parents experienced discomfort with the concept of a genetic diagnosis, and interestingly felt lack of a genomic diagnosis indicated a reduced severity of their infant’s condition. Decisional regret post-results was found to be low. Our results highlight how parents cope with the results of GWS and suggest that a genetic counselor can have an important role in helping families understand and adjust to these results in the neonatal intensive care unit.

Family carers of children with serious illness contribute many hours of medical care in addition to usual daily care. Assessing the needs and supports of family carers is not routine practice. This study is the first to utilize the interRAI Family Carer Needs Assessment in carers of children, seeking to evaluate and improve its ability to capture their needs. This is a prospective pilot study of family carers of children with serious illness receiving care at a pediatric hospice. Thirty carers completed the self-assessment form. Additional feedback was sought inquiring about the appropriateness of questions and missing information relevant to the pediatric setting. All participants reported the assessment captured important information across multiple domains. Additional questions surrounding extra costs, home and school supports, as well as direct impacts of caregiving activities on pain and relationships were identified as important adaptations. The most common unmet needs in carers and care recipients were episodic relief from caregiving (n=17) and housing adaptation (n=17), respectively. Overall, a comprehensive assessment form is feasible in identifying the diverse needs of family carers of children. Future research should focus on using pediatric specific interRAI tools to guide improvements in policy and practice that can address unmet needs.

Context: For parents, family, or clinicians of children with rare life-threatening conditions, there is little information regarding likely symptoms, illness trajectory, and end-of-life care.

Objectives: This descriptive analysis of a bereaved cohort recruited in the charting the territory study describes patient characteristics, symptoms, use of medications, discussion of resuscitation orders, and care provided preceding and during the end of life.

Methods: Of the 275 children enrolled in the Charting the Territory study, 54 died between 2009 and 2014. Baseline demographic information, symptoms, interventions, and medical information were collected via chart review, interviews, and surveys.

Results: Fifty-one of the 54 children had complete medical records. Of the seven symptoms evaluated, children were found to have an increase in median symptoms from baseline (n = 2) to time of death (n = 3). Opioids were used in the last 48 hours of life in 29 (56.9%) children, whereas only eight (15.7%) were receiving opioids at baseline. Do Not Attempt Resuscitation orders were in place at baseline in 17 (33.3%) children, increasing to 33 (64.7%) at time of death. Death occurred in a hospice setting in 16 (31.4%) children.

Conclusion: Although much emphasis on pediatric palliative care has been on supportive treatment and symptom management, when faced with a lack of sound understanding of a rare illness, the mode of care can often be reactive and based on critical needs. By developing greater knowledge of symptoms and illness trajectory, both management and care can be more responsive and anticipatory, thereby helping ease illness burden and suffering.

Introduction: The use of medicinal cannabis in the paediatric age group is increasing despite the lack of evidence for its efficacy or safety.

Objective: To map the available evidence on the efficacy and safety of medicinal cannabis in children and adolescents.

Methods: We conducted a scoping review and searched six electronic databases and grey literature. A study was eligible for inclusion when it investigated the efficacy or safety of medicinal cannabis for any condition, more than half of the participants were 0 to 18 years old, and had any study design except single case reports.

Results: We included 36 studies in our final analysis, 32 of which investigated the efficacy or safety of cannabis in treatment-resistant epilepsy. The remaining 4 studies examined patients with cancer, dysautonomia, Epidermolysis Bullosa, and motor disorders.

Conclusions: There is a lack of evidence on the efficacy and safety of medicinal cannabis in most paediatric conditions.

BACKGROUND AND OBJECTIVES: Children with medical complexity (CMC) often have multiple life-limiting conditions with no unifying diagnosis and an unclear prognosis and are at high risk for morbidity and mortality. Advance care planning (ACP) conversations need to be uniquely tailored to this population. Our primary objective for this study was to develop an in-depth understanding of the ACP experiences from the perspectives of both parents and health care providers (HCPs) of CMC.

METHODS: We conducted 25 semistructured interviews with parents of CMC and HCPs of various disciplines from a tertiary pediatric hospital. Interview guide questions were focused on ACP, including understanding of the definition, positive and negative experiences, and suggestions for improvement. Interviews were conducted until thematic saturation was reached. Interviews were audio recorded, transcribed verbatim, coded, and analyzed using content analysis.

RESULTS: Fourteen mothers and 11 HCPs participated in individual interviews. Interviews revealed 4 major themes and several associated subthemes (in parentheses): (1) holistic mind-set, (2) discussion content (beliefs and values, hopes and goals, and quality of life), (3) communication enhancers (partnerships in shared decision-making, supportive setting, early and ongoing conversations, consistent language and practice, family readiness, provider expertise in ACP discussions, and provider comfort in ACP discussions), and (4) the ACP definition.

CONCLUSIONS: Family and HCP perspectives revealed a need for family-centered ACP for CMC and their families. Our results aided the development of a family-centered framework to enhance the delivery of ACP through a holistic mind-set, thoughtful discussion content, and promoting of conversation enhancers.

Background: Patients receiving palliative care often interact with a variety of health care providers across various settings. While patients may experience good care from these services, the connection between these can be disjointed as care providers may work siloed from each other. This is particularly true in out-of-hospital and hospital emergency settings, where providers have no prior knowledge of the patient, particularly their advanced directives (ADs) and goals of care. In the Emergency Department or when paramedics respond to the home, ADs are further challenged by issues of clarity of content, contextual relevance, and accessibility.

Objectives: (1) What content should be in AD for medical emergencies, and (2) what would ensure the AD is accessible in times of crisis?

Design: Phase 1 involved a review of existing AD and published literature to generate a list of candidate elements. Phase 2 presented these in an online survey using modified Delphi method to paramedics, emergency nurses, and physicians. During phase 3, a focus group with palliative and emergency care providers and information technology experts was held regarding current accessibility of AD and a vision for improvement. The detailed focus group notes were coded using inductive analysis.

Results: Fifty-five candidate elements were provided for the Delphi. After three rounds, 36 panelists achieved consensus on 46 elements. Participation was greater than 80% in all rounds. From the focus group on access, six themes emerged; (1) imprecise language, (2) mismatch of protocols, (3) lack of understanding by patients/families, (4) lack of AD, (5) difficulty accessing AD, and (6) opportunities: database, education.

Conclusion: This project makes recommendations to improve palliative care in emergency or crisis situations and facilitate care consistent with patient’s goals: (1) a consensus-based template for AD content; and (2) development of a centralized database. These findings served as the foundation for the “Paramedics Providing Palliative Care at Home” program.

Background Q3 conditions are progressive, metabolic, neurological or chromosomal childhood conditions without a cure. Children with these conditions face an unknown lifespan as well as unstable and uncomfortable symptoms. Clinicians and other healthcare professionals are challenged by a lack of evidence for symptom management for these conditions.

Aims In this scoping review, we systematically identified and mapped the existing literature on symptom management for children with Q3 conditions. We focused on the most common and distressing symptoms, namely alertness, behavioural problems, bowel incontinence, breathing difficulties, constipation, feeding difficulties, sleep disturbance, temperature regulation, tone and motor problems and urinary incontinence. For children with complex health conditions, good symptom management is pertinent to ensure the highest possible quality of life.

Methods Scoping review. Electronic database searches in Ovid MEDLINE, Embase and CINAHL and a comprehensive grey literature search.

Results We included 292 studies in our final synthesis. The most commonly reported conditions in the studies were Rett syndrome (n=69), followed by Cornelia de Lange syndrome (n=25) and tuberous sclerosis (n=16). Tone and motor problems were the most commonly investigated symptom (n=141), followed by behavioural problems (n=82) and sleep disturbance (n=62).

Conclusion The evidence for symptom management in Q3 conditions is concentrated around a few conditions, and these studies may not be applicable to other conditions. The evidence is dispersed in the literature and difficult to access, which further challenges healthcare providers. More research needs to be done in these conditions to provide high-quality evidence for the care of these children.