Table 1 Clinical areas of focus and recommended speciality focus

Table 2: General principles for the management of patients with MPS VI

all

Table 3: Recommended routine monitoring and assessments in patients with MPS VI

all

Table 4: Guidance statement for galsulfase

geneticist, metabolic physician, paediatrician, nurse, physiotherapist

Table 5: Guidance statements for HSCT

anaesthetist, bone marrow transplant expert/hematopoietic stem cell transplant expert, geneticist, paediatrician, nurse

Table 6: Guidance statements for continuous positive airway pressure (CPAP), non-invasive positive pressure ventilation (NIPPV), oxygen supplementation and hypercapnia monitoring

anaesthetist, ear-nose-throat specialist, geneticist, paediatrician, respiratory physician/pulmonologist, nurse

Table 7: Guidance statements for anaesthesia

all

Table 8: Guidance statements for hip reconstruction, hip replacement and growth modulation surgeries

anaesthetist, geneticist, orthopaedic surgeon, neurosurgeon, paediatrician, physiotherapist

Table 9: Guidance statements for decompression of the spinal cord, spinal stabilisation and thoracolumbar kyphoscoliosis

anaesthetist, geneticist, orthopaedic surgeon, neurosurgeon, paediatrician, physiotherapist

Table 10: Guidance statement for corneal transplantation

anaesthetist, geneticist, ophthalmologist, paediatrician

Table 11. Guidance statements for decompression of the median nerve, tenosynovectomy and pulley release

anaesthetist, geneticist, hand surgeon, orthopaedic surgeon, neurosurgeon, paediatrician

Table 12: Guidance statement for cardiac valve replacement and left ventricular apical aneurysms

anaesthetist, cardiologist, geneticist, paediatrician

Table 13: Guidance statements for tonsillectomy and/or adenoidectomy, tracheostomy and insertion of ventilation tubes

anaesthetist, geneticist, ear-nose-throat specialist, paediatrician, respiratory physician/pulmonologist