No abstract

Lysosomal storage disorders are rare multiorgan, degenerative conditions requiring invasive treatment. Rare disorders pose unique challenges; therefore, exploring their impact is crucial for understanding family needs. This novel review aimed to understand the psychosocial outcomes for parents of children with lysosomal storage disorders. Five electronic databases were systematically searched. Thirty‐eight (23 qualitative, 10 qualitative and 5 mixed methods) studies were included, analysed using a sequential explanatory narrative synthesis and appraised for their methodological quality. Quantitative data revealed the multifaceted impact on parents' psychological and social wellbeing. Qualitative data informed the challenges that these parents faced which were expressed within three main themes: (a) Uncertainty and the unknown, (b) All‐encompassing impact and (c) Finding a way forward. The synthesis demonstrated that factors associated with the condition (symptoms, behaviour and severity) had a substantial negative impact on parental outcomes, upheld by concurrent loss (deterioration and poor prognosis) and uncertainty. This substantive integrated review revealed considerable unmet parental psychosocial needs.

Critical illness or trauma may trigger a rise in levels of cortisol, noradrenaline and glycogen which stimulate glycogen and fat breakdown causing a rise in blood glucose levels. However, the normal mechanisms to increase insulin production may be impaired by critical illness. Additionally, sedation and causes of reduced level of consciousness can mask the neurological symptoms of hypo or hyperglycaemia, making it particularly important to monitor blood glucose in this group of patients. It is important that health professionals understand the physiological affects of hypo and hyperglycaemia and know the safeguards required in order to minimize the potential for erroneous or misleading blood glucose results, and improve care for this group of patients.