Scoping Review of Symptoms in Children with Rare, Progressive, Life-Threatening Disorders Pawliuk, Colleen; Widger, Kimberley; Dewan, Tammie Joanne; Brander, Gina; Brown, Helen; Hermansen, Anne-Mette; Grégoire, Marie-Claude; Steele, Rose; Siden, Harold (Hal)
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 health care 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 focussed on the most common and distressing symptoms, namely alertness, behavioral 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 De Lange syndrome (n=25) and tuberous sclerosis (n=16). Tone and motor problems was the most commonly investigated symptom (n=141), followed by behavioral problems (n=82) and sleep disturbance (n=71). 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 health care providers. More research needs to be done in these conditions to provide high quality evidence for the care of these children.
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