Dr Saikat Santra  

I have been a consultant in paediatric inherited metabolic disorders at Birmingham Children’s Hospital since 2011 and am currently the Clinical Lead managing children with IMDs across the wider West Midlands. Since 2022 I have also been an Honorary Consultant at Cardiff and Vale University Health Board, overseeing the MDT management of children with IMDs in mid/south Wales.  I have previously held the Chair of the Royal College of Paediatrics and Child Health’s Speciality Advisory Committee for paediatric inherited metabolic medicine and remain the Quality Advisor. I have been a Committee Member of BIMDG for 2 years. I am a Chief/Principal Investigator in a number of research studies across different areas of IMD including lysosomal storage disorders, familial hypercholesterolaemia and disorders of intermediary metabolism and have a particular interest in paediatric lipidology, neurometabolic disorders, metabolic disorders affecting the liver and the application of new techniques in genomic diagnosis.

On 2nd October 2025 Birmingham Children’s Hospital hosted a BIMDG workshop on ERT with the intention of sharing and harmonising practice across the country in a number of areas that might improve patient experience, efficiency and cost. All paediatric and adult metabolic centres (not just the HSS Lysosomal centres) were invited to send delegates from medical, nursing and/or pharmacy and provided information in advance on current practice.  Sessions were held to discuss:

1. Pre-homecare Management - such as the number of infusions done in hospital before starting home infusions and the use of premedications to prevent infusion-associated reactions
2. Prescription Management – such as the frequency of deliveries and duration of a single prescription
3. Refrigeration and Drug Stability 
4. Patient/Carer independence with infusions
5. Working outside SmPC guidance eg with infusion rates

Feedback from each of these sessions and the associated proposals for changes in practice will be presented and it is hoped that attendees will consider introducing some of these proposals at their units over the next 1-2 years.