Simon Gurney
DOB: 1965 January 15th
NHS No. 4821386739
POMPE Disease / Muscular Dystrophy
Do not lie flat or recline !
Weakened diaphragm. Will cause respiratory failure if supine.
Do Not Sedate
No Hi flow Oxygen If supplementary oxygen is required check my C02 levels carefully
Sit me up and help me onto a chair Assist with my mobility
Ventilator Settings: Mode= ST IPAP:18.0 EPAP:5.0 Timax/min: 2.0/1.3 RT 450
Medication: Perindopril 8mg Daily
POMPE DISEASE DIAGNOSIS AND MANAGEMENT GUIDELINE
See page 281 for SURGERY/ANESTHESIA details
Malignant hyperthermia precautions should be taken
http://help.gurney.co.uk/pompe_disease.pdf
CONSENSUS TREATMENT RECOMMENDATIONS FOR LATE-ONSET POMPE DISEASE
http://help.gurney.co.uk/Pompe-Position-Statement.pdf
Emergency Contact Information:
Mr. Benjie Cabbell Manners
Cromer Hall
Cromer
Norfolk
NR27 9JG
United Kingdom
Tel: +44 1263 511575
Mob: +44 7771 971933
cabman@clara.net
Emergency Number
+44 203 456 7890
On Call Metabolic Consultant
Dr. Robin Lachmann
Charles Dent Metabolic Unit
Box 92
The National Hospital for Neurology and Neurosurgery
33 Queen Square
London WC1N 3BG
Tel 1: 020 3448 3213
Tel 2: 020 3448 8778
Fax: 020 7209 2146
robin.lachman@uclh.nhs.uk
Travel Medical Insurance:
NFU Mutual
Policy No. 098X6979964/N06
Tel: +33 1 45 16 64 65
Ana Amado Fondo, Kharen Cuarentas and Katrina Palmitos
Metabolic Clinical Nurse Specialists (CNS)
Phone: 0203 4483272 / 3606/ 3404
Mobile: 07970749106
Email address: uclh.metabolic.unit.nursing.team@nhs.net
IMPORTANT INFORMATION REGARDING YOUR VENTILATOR
Patient Name: Simon Gurney
Patient Hospital Number: 13/21962
Contact telephone numbers in event of a breakdown/ventilator problem:
***24 hour hotline - 0207 351 8020***Breakdowns***
**We do ask if you could - when possible - call between 9.30am and 4.30pm.