Supportive care and treating ongoing complications should always continue.
Patients with MPS VII can have respiratory and cardiovascular complications, skeletal manifestations, loss of hearing and vision, and gastrointestinal symptoms.3
Symptomatic management may include1,3
- Oxygen for respiratory insufficiency
- Continuous positive airway pressure (CPAP) machines
- Tracheotomy for severe airway obstruction
- Physical therapy for joint stiffness
- Cardiac valve replacement therapy
Anesthesia, surgical interventions, and emergencies
Patients with MPS VII may have cervical cord compression, cervical instability, and compressed airways. Giving an anesthetic to such patients requires skill and should always be undertaken by an experienced anesthetist.1-3
If the patient must go to a different hospital in the event of an emergency, be sure to inform the anesthetist that there may be problems with intubation. Also work with the MPS VII patient and their family to help inform their school, work, and any other caregivers of these concerns.1-3
Joint stiffness and limitation of motion are common features of MPS VII and can cause significant loss of function.
Range-of-motion exercises (passive stretching and bending of the limbs) may offer some benefits in preserving joint function, and should be started early.