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Scoping review of symptoms in children with rare, progressive, life-threatening disorders

Colleen Pawliuk1, Kim Widger2, Tammie Dewan3

  • 1BC Children's Hospital Research Institute, Vancouver, British Columbia, Canada.

BMJ Supportive & Palliative Care
|December 14, 2019
PubMed

Insights

Symptom management for children with rare Q3 conditions lacks evidence, with research concentrated on a few conditions. More studies are needed to improve care and quality of life for these children.

Area of Science:

  • Pediatric rare diseases
  • Clinical symptom management
  • Evidence-based practice

Background:

  • Q3 conditions are progressive, incurable childhood disorders impacting lifespan and causing significant symptoms.
  • Healthcare professionals face challenges due to limited evidence for managing symptoms in these complex pediatric conditions.
  • Effective symptom management is crucial for enhancing the quality of life for children with complex health needs.

Purpose of the Study:

  • To systematically identify and map existing literature on symptom management for children with Q3 conditions.
  • To focus on common and distressing symptoms including alertness, behavior, continence, breathing, constipation, feeding, sleep, temperature regulation, tone, and motor issues.
  • To provide a comprehensive overview of the current evidence base for pediatric symptom management.

Main Methods:

  • A scoping review methodology was employed.
  • Searches were conducted in major electronic databases: Ovid MEDLINE, Embase, and CINAHL.
  • A comprehensive grey literature search was also performed to capture all relevant studies.

Main Results:

  • The review synthesized data from 292 studies.
  • Rett syndrome (n=69), Cornelia de Lange syndrome (n=25), and tuberous sclerosis (n=16) were the most frequently studied conditions.
  • Tone and motor problems (n=141), behavioral issues (n=82), and sleep disturbances (n=62) were the most investigated symptoms.

Conclusions:

  • Current evidence for symptom management in Q3 conditions is condition-specific and may not generalize.
  • The dispersed and inaccessible nature of the literature complicates healthcare provision.
  • Further research is essential to generate high-quality evidence for the care of children with Q3 conditions.
Abstract

Keywords:
symptoms and symptom management

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