Priority 4 from the Liver Glycogen Storage Disease (International) PSP

UNCERTAINTY: How should sickness and emergency situations be managed for patients with liver Glycogen Storage Disease? (JLA PSP Priority 4)
Overall ranking 4
JLA question ID 0090/4
Explanatory note

There are several manners in which sickness and emergency situations can be managed, for example with an emergency protocol or hospitalization. This question aims to find appropriate management options for different situations for patients with liver GSD.

Evidence

For details of the evidence checked, please see the spreadsheet held on the JLA website.
Health Research Classification System category Metabolic and endocrine
Extra information provided by this PSP
Original uncertainty examples Is there a specific protocol to follow when a GSD patient gets sick? ~ What can be done about the emergency department of any hospital, with the intention for them to have the enough knowledge with the protocol on a GSD? ~ How to treat the fever? Which medicine is the most indicated? ~ I worry to know that in hospitals where patients with hepatic GSD are being treated, do not have an emergency protocol established. We are always parents who have to give these guidelines.
Submitted by Patient, carer and healthcare professionals
PSP information
PSP unique ID 0090
PSP name International Glycogen Storage Disease PSP
Total number of uncertainties identified by this PSP 72 (To see a full list of all uncertainties identified, please see the detailed spreadsheet held on the JLA website)
Date of priority setting workshop 24 May 2019