Lysosomal engorgement in tissue biopsies is evident, as are vacuolated lymphocytes on peripheral blood smear. Affected patients with MPS VI require ongoing medical care from a number of subspecialists. In addition, patients should receive routine pediatric care, including immunizations. Perform ongoing evaluations for the development of valvular cardiac disease. Such evaluations include annual echocardiograms.
Many patients have evidence for obstructive airway disease.
Conduct ongoing assessments for the development of clinically significant hypoventilation. Patients typically have contractures and hand deformities. A program of physical therapy may be beneficial in maintaining optimal function. Patients who develop clinically significant valvular heart disease may require valve replacement. Those with obstructive airway disease sometimes benefit from tonsillectomy and adenoidectomy.