Name: Your Name Here
Next Of Kin: _________________________________ Relationship: ______________________
Contact Details: ___________________________________________________________
Home Address: ___________________________________________________________________
NHS No: _____________________
Conditions:
1 ____________________________________
2 ____________________________________
3 ____________________________________
Medication currently being taken:
1
2
3
Medical Professionals
Consultants: Dr _________________
Contact Details: Dr ___________________
Consultants: Dr _________________
Contact Details: Dr ___________________
Consultants: Dr _________________
Contact Details: Dr ___________________
Consultants: Dr _________________
Contact Details: Dr ___________________
Urgent Considerations:
1 Bipap
2 Wheelchair User
3 Anything else etc
Allergies
1 _________________________________
I have a rare metabolic condition known as Pompe Disease
Oxygen Caution
Respiratory Care Information
Normal Partial Pressure of Carbon Dioxide (PC02) and
End Tidal Carbon Dioxide (CO2): 35-45 mmHg
Normal Oxygen Saturation (Sa02): 95 – 100%
Important: High Flow Oxygen may shut down my breathing
Do not give me oxygen before checking end tidal or blood C02 levels. A low oxyhaemoglobin saturation may indicate C02 retention and need positive pressure ventilation.
If supplemental oxygen is given continuously monitor C02 levels. Non invasive bi-level ventilation may be required.
Mechanical cough assist may be needed to clear mucus.
I will need help:
- Rising from the floor or a seat
- Standing unaided
- Walking
The symptoms of Late Onset Pompe Disease (LOPD) are listed below.
People with LOPD may have some or all of the symptoms below.
Head and Shoulders
- Muscle weakness in eyes
- Dropped eye lids
- Morning headaches
- Sleep apnoea
- Uses a BiPAP to breathe
- Difficulty swallowing
- Difficulty clearing airway after eating
- Weak cough
- Difficulty clearing throat
- Speaking voice changed, volume, texture
- Impaired alertness
Arms and hands
- Weak grip
- Struggling to hold items
- Arms feeling heavy
- Difficulty lifting arms above shoulders
Body/Torso
- Protruding stomach
- Bowel issues
- Bladder frequency/leakage
- Respiratory insufficiency/failure
- Muscle weakness in mid section
- Difficulty bending and straightening
- Continence Issues
Hips and Legs
- Difficulty getting up from a seated position
- Difficulty bending and straightening Difficulty climbing stairs
- Weaker front thigh muscles Unsteady when standing for longer periods
- Waddling gait
- Unable to stand alone
- Muscle cramps
- Struggling to walk
- Thicker calf muscles
- Tightness in calf muscles
- Pain when standing for short/long periods
- Unsteadiness when turning round
- Difficulty lifting foot
- Dropped foot, sometimes feels heavy/sags
Some users are reliant on a wheelchair or walking aid
General Well Being
- Feeling tired
- Temperature regulation – feeling hot/cold
- Tremors – feeling shaky
- Limbs feeling heavy
- Feeling totally exhausted
- Mental health – depression/anxiety
Test Results (may show anomalies in the following)
- Low oxygen levels
- Raised CO2 levels
- Raised Creatine kinase (CK) levels
Please be aware that the person may be on Enzyme Replacement Therapy (ERT).
Addition Support and Therapies
This person will also have a metabolic consultant that should be consulted prior to treatment and/or therapies.
Pompe Disease 