Of all the MPS disorders, Hurler has the most severe and complex symptoms and the earliest death rate. In Hurler syndrome every body system is affected. The typical cause of death is the cardiovascular system or pulmonary system shutting down.
Children with MPS I-H die before their tenth birthday. Mental retardation, dwarfism, and cardiovascular issues are severe. The nasal bridge is very small, sometimes almost nonexistent. Children with Hurlers tend to have a distinct look due to the coarse facies, much like Down syndrome children look alike (Banikazemi et al., 2009).
As shown in Figure 2, coarse facies, small nose bridge, eyes are spread further apart, small blunt teeth, and wider round face are all evidences of MPS I-H. This picture (Figure 2) was taken approximately 5 years post-transplant.
Hurler syndrome is the only MPS disorder that bone marrow transplantation has shown to be effective. With that said, it is not a cure, it does not reverse bone abnormalities, and it does not reverse damage already existing within the cardiovascular system (J. Clarke, personal communication, January 1995).
Disclaimer This was written as a college paper. Any and all comments on these web pages are my personal opinion. I am not a medical practitioner, nor do I claim to be one. Use this site at your discretion and consult a healthcare professional if you feel someone you know has the symptoms listed.