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Abstract Sickle cell disease (SCD) is a group of inherited blood disorders caused by a mutation in the beta subunit of hemoglobin (HbS). SCD will hereafter be referred to as sickle cell anemia (SCA) as this is the term our patients and their families prefer. There are approximately 5000 Canadians living with SCA including children. Pediatric SCA patient education can: improve knowledge, decrease hospitalization, improve medication possession ratio, lead to better SCA‐related functioning, and lower pain impact. Innovative educational materials were developed to improve knowledge and self‐efficacy regarding the illness management of patients and parents/guardians. Patients (n = 5; aged 8–18) with SCA and parents (n = 5) of patients (aged 0–18) were recruited via flyers sent directly to patients and distributed through partner patient organization Sickle Cell Awareness Network of Saskatchewan. Patient and parent focus groups were held separately over Zoom to receive feedback for the video. An additional interview was held for a participant that required a translation of the video. Audio recordings were transcribed using Zoom and Otter.ai. The coding of transcripts was facilitated by NVivo (QSR International Pty Ltd, 2022, release 1.6.2). The thematic analysis centered around SCA management concepts relevant to the research aims. Important themes that emerged included ‘Age Appropriateness’, ‘Empowerment’, ‘Knowledge Gaps’, ‘Linguistic Accessibility’, ‘Medication Adherence’, ‘Strength in Community’, and ‘Transition to Adult Care’. The video was well received, and “brought peace of mind”. Patient feedback was incorporated into the final version of the educational materials.

Abstract Background Sickle cell disease (SCD) is associated with an increased risk of cardiovascular disease that may be due to a variety of possible risk factors, including abnormal blood pressure. Blood pressure (BP) of children and adolescents with SCD has been reported to be lower compared to the BP of the general pediatric population. Methods To confirm this prior observation, we compared reference BP values for children with SCD with reference BP values of the general pediatric population. We hypothesized that children with SCD do not have lower BPs than children without SCD. Results Systolic BP differed for both males and females, over the different age groups between pediatric subjects with and without SCD. Systolic BP was higher in children with SCD, in both obese and non-obese populations. Diastolic BP did not differ between the groups. Conclusions Our analysis demonstrated that systolic BP values are indeed higher in children with SCD than in the general pediatric population. This finding is consistent with the most recent literature showing abnormal BP patterns in the SCD pediatric population utilizing 24-hour BP monitoring devices. This is an important step for recognizing abnormal BP as a risk factor for cardio- and neurovascular events in SCD.

Abstract Background Diffuse intrinsic pontine glioma (DIPG) is one of the most devastating diseases among children with cancer, thus novel strategies are urgently needed. Aims We retrospectively evaluated DIPG patients exposed to the carbohydrate restricted ketogenic diet (KD) with regard of feasibility, safety, and overall survival (OS). Methods and results Searches of MEDLINE and Embase identified five hits meeting the search criteria (diagnosis of DIPG and exposure to KD). One additional case was identified by contact with experts. Individual patient data were extracted from publications or obtained from investigators. The inclusion criteria for analysis of the data were defined as DIPG patients who were exposed to the KD for ≥3 months. Feasibility, as described in the literature, was the number of patients able to follow the KD for 3 months out of all DIPG patients identified. OS was estimated by the Kaplan‐Meier method. Five DIPG patients (males, n = 3; median age 4.4 years; range, 2.5‐15 years) meeting the inclusion criteria were identified. Analysis of the available data suggested that the KD is generally relatively well tolerated. Only mild gastro‐intestinal complaints, one borderline hypoglycemia (2.4 mmol/L) and one hyperketosis (max 7.2 mmol/L) were observed. Five out of six DIPG patients identified adhered for ≥3 months (median KD duration, 6.5 months; range, 0.25‐2 years) to the diet. The median OS was 18.7 months. Conclusion Our study provides evidence that it may be feasible for pediatric DIPG patients to adhere for at least 3 months to KD. In particular cases, diet modifications were done. The clinical outcome and OS appear not to be impacted in a negative way. KD might be proposed as adjuvant therapy when large prospective studies have shown feasibility and safety. Future studies might ideally assess the impact of KD on clinical outcome, quality of life, and efficacy.

Abstract Patients with severe anemia can present with non‐specific symptoms, including shock and respiratory distress. Ensuring a rapid, targeted workup is initiated and providing prompt transfusions as necessary are critical for both diagnostic success and clinical improvement.

The article presents data on the study of emotional state characteristics and coping strategies of adolescents in the stressful situation of oncohematological diseases treatment. Two equalized by socio-demographic and clinical parameters groups of patients 11-18 were examined. The experimental group consisted of 28 adolescents undergoing treatment with hemopoietic stem cell transplantation (HSCT) (M = 14.25, SD = 2.20, M: 57.1%, F: 42.9%). The control group consisted of 30 adolescents undergoing chemotherapy (M = 13.71, SD = 2.12, M: 53.3%, F: 46.7%). The psychological state of adolescents in the course of treatment by НSCТ, in comparison with the state of adolescents of the control group, which is characterized by a significantly higher level of distress and anxiety. Significant differences in the use of coping strategies with stress in patients of the two groups were obtained. Adolescents of the experimental group use a less extensive repertoire of productive coping strategies, often use such an ineffective strategy as "discharge" These differences may be associated with less availability, as well as an increase in depletion of various coping resources under special treatment conditions by the НSCТ. The results of the study confirmed that НSCТ is objectively and subjectively more stressful, which indicates the need for additional monitoring of both the psychological state and the need for psychological care for patients in this group, the development of specialized programs for psychological support for transplantation.

The results of the study of individual factors of psychological adaptation to the stressful situation of treatment by hematopoietic stem cells transplantation in adolescents and their mothers are presented. Measures were obtained from 28 adolescents aged 11—18 (M=14,25, 16m/12f) and their mothers aged 31—53 (M=41,1). The results show a significant positive correlation between levels of adjustment of adolescents and their mothers (r=0,643, p=0,001), between levels of adjustment to treatment and emotional distress of adolescents (r=0,544; p=0,007). The original hypothesis of significant associations between the levels of emotional distress, adjustment and individual factors such as appraisal of stressful event was confirmed: the lower level of adolescent’s adjustment was associated with their mothers’ appraisal of HSCT as a Threat (r=0,463, p=0,030) or Loss (r=0,450, p=0,035); high level of adolescent’s distress was associated with his/hers subjective appraisal of HSCT as a Threat (r=0,695, p=0,000) and Loss (r=0,659, p=0,000). Significant associations were also found between psychological adjustment of adolescents and coping behavior (of ad- olescents and parents), which confirms the importance of further research on both individual and family factors of psychological adjustment to HSCT.

Abstract Introduction Learners have repeatedly expressed a desire for more structured training in communicating with families, especially when sharing life-altering information and breaking bad news. Concurrently, parents have indicated that pediatricians could conduct difficult conversations with greater skill. Based on local needs assessments and available pediatric literature, this guide presents didactic materials and a workshop-style, case-based, longitudinal approach for teaching communication skills to learners in pediatrics. Methods The customizable guide can be implemented as a 1-hour didactic presentation, a 1- to 3-hour workshop, or an integrated longitudinal curriculum. Unlike other available resources for breaking bad news, this guide is specifically designed for pediatrics and uses evidence-based communication guidelines developed for pediatric settings. The guidelines are modified from the adult-centered SPIKES (setting, perception, involvement, knowledge, emotions, summary) approach. The material was created by clinicians, educators, and parents of pediatric patients. In addition to video-based didactic materials and pediatric case scenarios, the guide includes materials for assessment, evaluation, and personal reflection. Results The modified SPIKES approach and didactic portion of this resource were validated as an initial training tool, yielding significant improvements in self-efficacy of pediatric providers and learners. Evaluations of the role-playing components provided by pediatric residents and fellows have been positive for the format and value of the learning experience. Participants reported a particular benefit from the inclusion of parent perspectives. Discussion Without a formal communication curriculum, learners must rely on chance observation of life-altering conversations during clinical rotations. This guide provides pediatric educators with structured, evidence-based materials to teach advanced communication skills.

Abstract Background To identify the predictive factors for malignancies using basic clinical and laboratory information in children presenting with musculoskeletal pain and eventually diagnosed with juvenile idiopathic arthritis (JIA) or malignancy. Methods A retrospective case–control chart review research examining laboratory data from patients referred for musculoskeletal pain in 2001–2010 and diagnosed with malignancy or JIA was performed. The validity of each test for the diagnosis of neoplasia was assessed by calculating the sensitivity, specificity, positive predictive values (PPV), negative predictive values (NPV) and likelihood ratios. Results A total of 134 patients were enrolled. Statistically significant differences were found in neutrophil count, Hb, LDH, IgA and C4 values, ANA, anti-EA EBV IgG and anti-CMV IgG titres. High LDH value and anti-CMV IgG were the most predictive factors for neoplasia. High specificity factors for neoplasia were abnormal values of neutrophil count, Hb, IgA and C4, and the presence of anti-EA EBV and anti-CMV IgG. High PPV were recorded for abnormal neutrophil count, Hb value and anti-CMV titre. A low NPV was found only for anti-EA EBV and anti-CMV titres. Conclusions In this setting of patients, minimum changes in neutrophil count, particularly if associated with low Hb and high LDH levels, are to be thoroughly considered, because they appear as the most predictive factors for the diagnosis of tumour.