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Medical problems of those affected by MPS VIMedical problems include: Skeletal DifficultiesThe neck is short and sometimes restricted in movement. Occasionally the spinal cord may be compressed by its surrounding membranes which are greatly thickened, partly due to storage of mucopolysaccharides. More rarely compression may be caused by a structural defect in the upper vertebrae of the spine. Both these problems can be treated surgically if necessary. Joint StiffnessJoint stiffness and the movement of many joints, especially the arms, may become severely limited due to malformation of the bones and ligaments. Clothing may need to have fastenings down the front to allow for ease of dressing. Heart ConditionsMost of those affected will have some form of heart disease but this may not cause problems till later in life. Storage of mucopolysccharides in the cells may lead to narrowing (stenosis) or weakening of certain valves in the heart. As the blood is pumped from one part of the heart to another the valves open and close to stop blood flowing back in the wrong direction. If the valves are narrowed the doctor may hear a harsh murmur as the blood is forced through. Weakened valves allow blood to leak back. Valvular heart disease may not lead to any trouble for many years but if it is causing great strain on the heart, an operation may be possible to replace the damaged value. Such surgery can be most successful, enabling the patient to return to a more normal life.Corneal CloudingCorneal clouding is also present in individuals with Maroteaux-Lamy syndrome. The outer layer at the front of the eye (cornea) becomes cloudy due to the storage of mucopolysaccharides. If corneal clouding becomes so marked that vision is severely restricted a corneal graft is possible. Occasionally vision deteriorates due to damage to the retina or the optic nerve.AnaesthesiaAnaesthesia should not be undertaken lightly in MPS VI. There can be difficulties anaesthetising someone suffering from the disease and an experienced anaesthetist should always be involved. Adult sufferers should ideally wear some sort of identification bracelet with a contact medical person in case of emergency. With children, parents should inform their child's school and any person who looks after the child in case they cannot be contacted in an emergency. If you have to go to a different hospital in an emergency you should tell the anaesthetist that there might be problems with intubation. The MPS Society has more information on this important subject.Dental ProblemsTeeth are poorly formed and the enamel may not be very strong. It is important to clean the teeth regularly. Sometimes the gums may grow down more than usual. You must tell your dentist if your child has a heart problem and you will probably be advised that he or she should be given antibiotics before and after any dental treatment. This is because bacteria in the mouth might get into the bloodstream during treatment and cause an infection which could damage the heart valve further. Back to MPS VI | Characteristics of MPS VI | Medical Problems of those affected by MPS VI | Current Research into MPS VI Table of Diseases | Aust MPS Statistics | MPS I | MPS II | MPS III | MPS IV | MPS VI | ML II and ML III |
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