RT Journal Article SR Electronic T1 Scoping review of symptoms in children with rare, progressive, life-threatening disorders JF BMJ Supportive & Palliative Care JO BMJ Support Palliat Care FD British Medical Journal Publishing Group SP 91 OP 104 DO 10.1136/bmjspcare-2019-001943 VO 10 IS 1 A1 Colleen Pawliuk A1 Kim Widger A1 Tammie Dewan A1 Gina Brander A1 Helen L Brown A1 Anne-Mette Hermansen A1 Marie-Claude Grégoire A1 Rose Steele A1 Harold (Hal) Siden YR 2020 UL http://spcare.bmj.com/content/10/1/91.abstract AB 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.