Scoping review of symptoms in children with rare, progressive, life-threatening disorders
symptoms and symptom management
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.
Pawliuk C; Widger K; Dewan T; Brander G; Brown H L; Hermansen A M; Grégoire M C; Steele R; Siden H
BMJ Supportive & Palliative Care
2019
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1136/bmjspcare-2019-001943" target="_blank" rel="noreferrer noopener">10.1136/bmjspcare-2019-001943</a>
Charting the Territory: End-of-Life Trajectories for Children with Complex Neurological, Metabolic and Chromosomal Conditions
DNAR orders; pediatrics; terminal care
OBJECTIVES: 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. This descriptive analysis of a bereaved cohort recruited in the Charting the Territory (CTT) study describes patient characteristics, symptoms, use of medications, discussion of resuscitation orders and care provided preceding and during the end-of-life. METHOD(S): Of the 275 children enrolled in the CTT study, 54 died between 2009 and 2014. Baseline demographic information, symptoms, interventions and medical information were collected via chart review, interviews and surveys. RESULT(S): 51 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(S): While 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. Copyright © 2020 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.
Bao D; Feichtinger L; Andrews G; Pawliuk C; Steele R; Siden H H
Journal of pain and symptom management
2020
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
<a href="http://doi.org/10.1016/j.jpainsymman.2020.08.033" target="_blank" rel="noreferrer noopener">10.1016/j.jpainsymman.2020.08.033</a>