Resource stewardship and Choosing Wisely in a children's hospital.

Objectives: Evidence suggests that approximately 30% of the tests and treatments currently prescribed in healthcare are potentially unnecessary, may not add value, and in some cases cause harm. We describe the evolution of our hospital's Choosing Wisely (CW) program over the first 5 years of existence, highlighting the enablers, challenges, and overall lessons learned with the goal of informing other healthcare providers about implementing resource stewardship initiatives in paediatric healthcare settings. Methods: We describe the development of de novo "top 5" CW lists of recommendations using anonymous surveys and Likert scale scoring. Composition and role of the steering committee, measurement of data and outcomes, and implementation strategies are outlined. Results: Many projects have resulted in a successful decrease in inappropriate utilization while simultaneously monitoring for unintended consequences. Examples include respiratory viral testing in the emergency department (ED) decreased by greater than 80%; ankle radiographs for children with ankle injuries decreased from 88% to 54%; and use of IVIG for treatment of typical ITP cases decreased from 88% to 55%. Early involvement focused within General Paediatrics and the ED, but later expanded to include perioperative services and paediatric subspecialties. Conclusions: An internally developed CW program in a children's hospital can reduce targeted areas of potentially unnecessary tests and treatments. Enablers include credible clinician champions, organizational leadership support, reliable measurement strategies, and dedicated resource stewardship education. The lessons learned may be generalizable to other paediatric healthcare settings and providers looking to introduce a similar approach to target unnecessary care in their own organizations.

Emergency management of the paediatric patient with convulsive status epilepticus.

This guideline addresses the emergency management of convulsive status epilepticus (CSE) in children and infants older than 1 month of age. It replaces a previous position statement from 2011, and includes a new treatment algorithm and table of recommended medications based on new evidence and reflecting the evolution of clinical practice over the past several years. This statement emphasizes the importance of timely pharmacological management of CSE, and includes some guidance for diagnostic approach and supportive care.

The parental experience and perceptions of blenderized tube feeding for children with medical complexity.

OBJECTIVES: Parents of children with medical complexity are often expected to implement complicated plans of care, such as enteral tube feeding, to support the health of their child. Enteral feeding can have psychosocial implications for the parent, child, and family. Blenderized tube feeding (BTF) refers to the administration of pureed food and drinks through a feeding tube. Little is known regarding parents' experiences with BTF. Therefore, the purpose of this qualitative study was to understand the lived experience of BTF from the parent's perspective. METHODS: This qualitative study was a grounded theory analysis utilizing semi-structured interviews of parents who provided at least 50% of their child's diet through BTF. Participants were recruited using purposive sampling from the Complex Care Program at a tertiary care paediatric centre. Interviews were conducted until thematic saturation was achieved. Themes were identified using constant comparative analysis of transcribed interviews. RESULTS: Parents (n=10) felt that BTF positively affected the experience of tube feeding and enhanced their child's health and wellbeing. Parents described BTF as a means of self-empowerment and a mechanism to normalize feeding and care for the entire family. Despite reporting BTF as more time consuming than formula feeding, all parents were satisfied with having made the change, and planned on continuing the diet. CONCLUSION: BTFs can improve the experience of tube feeding and positively address some of the negative psychosocial implications of enteral tube feeding, providing a sense of normalcy and control for parents caring for a child with medical complexity.

A Recurrent De Novo Variant in NACC1 Causes a Syndrome Characterized by Infantile Epilepsy, Cataracts, and Profound Developmental Delay.

Whole-exome sequencing (WES) has increasingly enabled new pathogenic gene variant identification for undiagnosed neurodevelopmental disorders and provided insights into both gene function and disease biology. Here, we describe seven children with a neurodevelopmental disorder characterized by microcephaly, profound developmental delays and/or intellectual disability, cataracts, severe epilepsy including infantile spasms, irritability, failure to thrive, and stereotypic hand movements. Brain imaging in these individuals reveals delay in myelination and cerebral atrophy. We observe an identical recurrent de novo heterozygous c.892C>T (p.Arg298Trp) variant in the nucleus accumbens associated 1 (NACC1) gene in seven affected individuals. One of the seven individuals is mosaic for this variant. NACC1 encodes a transcriptional repressor implicated in gene expression and has not previously been associated with germline disorders. The probability of finding the same missense NACC1 variant by chance in 7 out of 17,228 individuals who underwent WES for diagnoses of neurodevelopmental phenotypes is extremely small and achieves genome-wide significance (p = 1.25 × 10-14). Selective constraint against missense variants in NACC1 makes this excess of an identical missense variant in all seven individuals more remarkable. Our findings are consistent with a germline recurrent mutational hotspot associated with an allele-specific neurodevelopmental phenotype in NACC1.

Choice of maintenance intravenous fluids among paediatric residents in Canada.

BACKGROUND: Recent literature and guidelines support routine use of isotonic intravenous (IV) fluids for maintenance therapy in hospitalized infants and children. Current prescribing practices are unknown. OBJECTIVE: To elicit paediatric residents' choice of maintenance IV fluids, particularly with regard to tonicity, in a variety of clinical scenarios and patient ages. We hypothesized that residents would choose isotonic fluids in most cases, but there would be substantially more variability in fluid choice in the neonatal age group. METHODS: An Internet-based survey was e-mailed to trainees in the 17 paediatric residency programs across Canada, via the Canadian Paediatric Program Directors. The survey instrument included questions related to training, followed by a series of questions eliciting choice of IV fluid in a variety of clinical situations. RESULTS: A total of 147 survey responses were submitted (22% response rate). Isotonic solutions were selected by >75% across all clinical scenarios involving infants and children. Very hypotonic fluids were seldom chosen. There was more variability in fluid choice in neonates, with evidence of significant differences in fluid tonicity based on senior versus junior resident status and geographical location. CONCLUSIONS: Results imply a predominance of isotonic fluid use in infants and children, suggesting that clinical practice has changed in response to risk of hyponatremia with hypotonic IV fluids. As hypothesized, there was more variability with respect to choice of maintenance fluids in neonates. This likely reflects a paucity of guidance in an age group with unique physiologic factors affecting fluid and electrolyte status.

Diagnosis and management of typical, newly diagnosed primary immune thrombocytopenia (ITP) of childhood.

This practice point applies to children aged 90 days through 17 years who have typical, newly diagnosed primary immune thrombocytopenia (ITP). Current recommendations on management and information from recent studies are summarized with the goal of decreasing variable practice among providers and improving patient-centred care. Options for initially managing young patients with ITP who experience bruising, petechiae, or occasional mild epistaxis not interfering with daily living include observation without pharmacotherapy as a first-line option. When active therapy is pursued, choices include the use of corticosteroids and intravenous immunoglobulin. Children with moderate or severe bleeding continue to require hospitalization and treatment. Shared decision-making can enhance patient-centred care and ensure that the families have a full understanding of the management options available.

Making it easier to 'choose wisely'.

The Choosing Wisely campaign has stimulated many clinicians to think about the appropriateness of various tests and treatments. Most of the recommendations published thus far are adult-focused. In this commentary, we discuss the development and early implementation of a Choosing Wisely 'top 5' list specifically aimed at children being cared for at our tertiary care children's hospital. We hope that this will encourage others involved in the health care of infants and children to engage in further thought and discussion about the appropriateness of current tests and therapies. Despite often focusing on the deficiencies, we are privileged to have a highly developed and well-resourced health care system in Canada which allows us tremendous freedom to order tests and treatments. It is incumbent on us as health care providers to exercise that privilege with the utmost responsibility and strive to choose wisely and thoughtfully when selecting tests and therapies for our patients.

General paediatric inpatient deaths over a 15-year period.

OBJECTIVE: To retrospectively review trends of general paediatric inpatient deaths at a tertiary care children's hospital over a 15-year period. METHODS: Data were collected for all patients who died on the general paediatric wards or paediatric intensive care unit (PICU) during 1998, 2005 or 2012 and had a 'general paediatric condition'-an underlying condition or diagnosis that would normally result in admission to a general paediatric ward. Data were related to: demographics, health services utilization, information about provision and orders related to cardiopulmonary resuscitation (CPR) at time of death and involvement of palliative care services. RESULTS: Eighty-five inpatients met inclusion criteria: 35 in 1998, 27 in 2005 and 23 in 2012. Nearly 95% of general paediatric patients who died in 1998 did so in the PICU, 59.3% in 2005 and 69.6% in 2012. The median age of death decreased from 3 years in 1998 to 2 years in 2012. The proportion of patients with 'no CPR' orders at time of death increased from 31.4% in 1998 to 87.0% in 2012. Similarly, the proportion of patients with palliative care team involvement prior to death increased from less than 10% in 1998 to 73.9% in 2012. CONCLUSIONS: The absolute number of inpatient general paediatric deaths has decreased from 1998 to 2012 at this hospital. A larger proportion of these deaths are occurring on the general paediatric wards rather than in the PICU over time. 'No CPR' orders and palliative care consultations are becoming more prevalent in these patients prior to death.

Patient disclosure of medical errors in paediatrics: A systematic literature review.

Medical errors are common within paediatrics; however, little research has examined the process of disclosing medical errors in paediatric settings. The present systematic review of current research and policy initiatives examined evidence regarding the disclosure of medical errors involving paediatric patients. Peer-reviewed research from a range of scientific journals from the past 10 years is presented, and an overview of Canadian and international policies regarding disclosure in paediatric settings are provided. The purpose of the present review was to scope the existing literature and policy, and to synthesize findings into an integrated and accessible report. Future research priorities and policy implications are then identified.

Les erreurs médicales sont courantes en pédiatrie. Cependant, peu de recherches abordent leur processus de divulgation en milieu pédiatrique. La présente analyse systématique des projets de recherche et de politique les plus récents a porté sur les données probantes relatives à la divulgation des erreurs médicales auprès de patients d'âge pédiatrique. Les recherches révisées par les pairs tirées d'une série de revues scientifiques des dix dernières années sont présentées, de même qu'un aperçu des politiques canadiennes et internationales sur la divulgation en milieu pédiatrique. La présente analyse visait à évaluer les publications et les politiques existantes et à synthétiser les résultats dans un rapport intégré et accessible. Les prochaines priorités de la recherche et leurs conséquences sur les politiques sont ensuite exposées.

Predictors of bacteremia among children with sickle cell disease presenting with fever.

INTRODUCTION: Bacterial sepsis is more common and potentially life threatening in children with sickle cell disease (SCD). Identification of variables that predict bacteremia may aid clinicians in recognizing patients with SCD at higher risk for sepsis. OBJECTIVE: To determine whether absolute neutrophil count (ANC) >20×10/L is an independent risk factor for bacteremia in children with SCD and to identify other predictors of bacteremia in this population. METHODS: A case-control study was conducted. Subjects were 0 to 18 years of age admitted to a tertiary care pediatric hospital over a 17-year period with SCD and fever at presentation. Cases had bacteremia, whereas controls had negative blood cultures. RESULTS: Data were analyzed for 40 cases and 120 controls. ANC>20×10/L was significantly more prevalent among cases (odds ratio [OR], 7.0; 95% confidence interval [CI], 2.6-18.9). Cases were more likely to have emesis (OR, 2.9; 95% CI, 1.0-8.4) and a higher proportion of band cells (OR, 1.3; 95% CI, 1.1-1.4) at presentation. CONCLUSIONS: In a febrile child with SCD, an ANC>20×10/L, a higher proportion of band cells, and the presence of vomiting were associated with an increased likelihood of bacteremia.

Bronchiolitis: Recommendations for diagnosis, monitoring and management of children one to 24 months of age.

Bronchiolitis is the most common reason for admission to hospital in the first year of life. There is tremendous variation in the clinical management of this condition across Canada and around the world, including significant use of unnecessary tests and ineffective therapies. This statement pertains to generally healthy children ≤2 years of age with bronchiolitis. The diagnosis of bronchiolitis is based primarily on the history of illness and physical examination findings. Laboratory investigations are generally unhelpful. Bronchiolitis is a self-limiting disease, usually managed with supportive care at home. Groups at high risk for severe disease are described and guidelines for admission to hospital are presented. Evidence for the efficacy of various therapies is discussed and recommendations are made for management. Monitoring requirements and discharge readiness from hospital are also discussed.

La bronchiolite est la principale cause d'hospitalisation avant l'âge de un an. La prise en charge clinique de cette maladie varie considérablement selon les régions du Canada et du monde, y compris une grande utilisation de tests inutiles et de thérapies inefficaces. Le présent document de principes porte sur des enfants en santé de deux ans ou moins qui sont atteints d'une bronchiolite. Le diagnostic de bronchiolite repose d'abord sur l'anamnèse de la maladie et sur les résultats de l'examen physique. En général, les examens de laboratoire sont inutiles. La bronchiolite est une maladie spontanément résolutive, qui est généralement prise en charge par des soins de soutien à domicile. Par ailleurs, les groupes très vulnérables à une bronchiolite grave sont décrits, et les indications d'admission à l'hôpital sont présentées. Les données probantes sur l'efficacité des diverses thérapies et les recommandations de prise en charge sont exposées. La surveillance requise et le moment du congé de l'hôpital sont également abordés.

Constitutive hypercoagulability in pediatric sickle cell disease patients with hemoglobin SS genotype.

Background: Constitutive inflammation and hemostatic activation have been identified as key contributors to the pathophysiology of sickle cell disease (SCD), leading to clinical consequences such as vaso-occlusive crises and stroke. Patients with hemoglobin SS (HbSS) and hemoglobin SC (HbSC) genotypes are reported to have different symptoms, as do patients in steady-state and crisis situations. Differences among these groups remain unclear in pediatric patients. Objectives: To compare hemostatic activity in HbSS and HbSC pediatric patients during steady state, in crisis, and in clinical follow-up and compare HbSS and HbSC patients with normal healthy children. Methods: Whole-blood coagulation assay thromboelastography (TEG) was used to assess hemostatic activity. In parallel, flow cytometry was used to assess procoagulant surface expression of platelets and red blood cells. Results: TEG results indicated no significant differences in clotting onset (R time), clot maximum amplitude, or maximum rate of thrombus generation among steady-state, crisis, and follow-up subgroups of HbSS and HbSC patients. TEG parameters did not differ significantly between HbSC patients and healthy children, while HbSS patients showed significantly shorter R time and greater maximum amplitude and maximum rate of thrombus generation, all indicative of a constitutive hypercoagulable state. Flow cytometry results did not detect increased platelet integrin αIIbß3 activation or red blood cell procoagulant surface expression in SCD patients compared with unaffected children. Conclusion: Our results indicate that pediatric SCD patients with the HbSS genotype have constitutively activated hemostasis relative to HbSC patients and healthy children. It remains to be determined how treatments that improve clinical outcomes in SCD patients affect this constitutively hypercoagulable state.

A child exposed to primidone not prescribed for her.

A 7.5-year-old girl who was treated with phenobarbital (PHB) for epilepsy was admitted with decreased levels of consciousness. She had been known to have high PHB levels of unknown cause, without symptoms. Her PHB levels were very high, as expected, but primidone levels were also detected although she and her parents denied history of primidone administration. We wished to rule out intentional unprescribed use of primidone. Our retrospective review showed 3 other children with high PHB concentrations where primidone was also detected when PHB levels were over 130 µmol/L. Complementary studies confirmed that high-dose PHB can convert to its prodrug primidone, which has not been reported previously.

Exploring the usefulness of comprehensive care plans for children with medical complexity (CMC): a qualitative study.

BACKGROUND: The Medical Home model recommends that Children with Special Health Care Needs (CSHCN) receive a medical care plan, outlining the child's major medical issues and care needs to assist with care coordination. While care plans are a primary component of effective care coordination, the creation and maintenance of care plans is time, labor, and cost intensive, and the desired content of the care plan has not been studied. The purpose of this qualitative study was to understand the usefulness and desired content of comprehensive care plans by exploring the perceptions of parents and health care providers (HCPs) of children with medical complexity (CMC). METHODS: This qualitative study utilized in-depth semi-structured interviews and focus groups. HCPs (n = 15) and parents (n = 15) of CMC who had all used a comprehensive care plan were recruited from a tertiary pediatric academic health sciences center. Themes were identified through grounded theory analysis of interview and focus group data. RESULTS: A multi-dimensional model of perceived care plan usefulness emerged. The model highlights three integral aspects of the care plan: care plan characteristics, activating factors and perceived outcomes of using a care plan. Care plans were perceived as a useful tool that centralized and focused the care of the child. Care plans were reported to flatten the hierarchical relationship between HCPs and parents, resulting in enhanced reciprocal information exchange and strengthened relationships. Participants expressed that a standardized template that is family-centered and includes content relevant to both the medical and social needs of the child is beneficial when integrated into overall care planning and delivery for CMC. CONCLUSIONS: Care plans are perceived to be a useful tool to both health care providers and parents of CMC. These findings inform the utility and development of a comprehensive care plan template as well as a model of how and when to best utilize care plans within family-centered models of care.

Risk of acute hyponatremia in hospitalized children and youth receiving maintenance intravenous fluids.

Hospital-acquired acute hyponatremia is increasingly recognized as a cause of morbidity and mortality in children. It has been attributed primarily to the use of hypotonic intravenous (IV) fluids to maintain fluid and electrolyte requirements. This practice point outlines current understanding of the problem and summarizes recent research dealing with this issue. Detailed recommendations are made for the prescription of IV maintenance fluids in children between one month and 18 years of age. The use of isotonic fluid (D5W.0.9% NaCl) is recommended in most circumstances. Hypotonic IV fluids containing less than 0.45% NaCl should not be used to provide routine IV fluid maintenance requirements.

L'hyponatrémie aiguë d'origine nosocomiale est de plus en plus reconnue comme une cause de morbidité et de mortalité chez les enfants. Elle est surtout attribuée à l'utilisation de solutés intraveineux (IV) hypotoniques pour respecter les besoins liquidiens et électrolytiques. Le présent point de pratique expose les connaissances actuelles du problème et contient un résumé des recherches récentes sur le sujet. Il contient également des recommandations détaillées sur la prescription de soluté IV d'entretien chez les enfants de un mois à 18 ans. On recommande d'utiliser des liquides isotoniques (D5W et NaCl à 0,9 %) dans la plupart des cas. Il faut éviter les solutés IV hypotoniques qui contiennent moins de 0,45 % de NaCl pour administrer les solutés IV d'entretien habituels.

A Comparison of Short Versus Long Course Intravenous Antibiotics When Treating Urinary Tract Infection in Infants <60 Days of Age.

Urinary tract infections (UTIs) are a common reason for hospitalization in infants younger than 60 days, and the optimal approach to intravenous (IV) antibiotic therapy upon UTI diagnosis in this cohort is unknown. We determined whether there was an association between IV antibiotic therapy duration (long [>3 days] vs short [≤3 days]) and treatment failure via a retrospective review of infants with confirmed UTIs receiving IV antibiotics at a tertiary referral center. A total of 403 infants were included; 39% were treated with ampicillin and cefotaxime, and 34% with ampicillin and gentamycin or tobramycin. The median IV antibiotic duration was 5 (interquartile range: 3-10) days, and 5% of patients experienced treatment failure. The treatment failure rate was similar in both short- and long-course IV antibiotic groups (P > .05), and there was no significant association between treatment duration and failure. We conclude that treatment failure for infants hospitalized with UTI is uncommon and not associated with IV antibiotic duration.