General Management


A balanced diet is important for health and well-being. There is no scientific evidence that a particular diet has any helpful effect in MPS I and problems such as diarrhoea tend to come and go naturally. A change in diet may ease problems such as production of excessive mucus or diarrhoea. Reducing intake of milk, dairy products and sugar, as well as avoiding foods with too many additives and colouring have sometimes helped. It is advisable to consult your doctor or a dietician if major dietary changes are planned to ensure that essential nutrients are not left out. If the problems are eased, foods can be reintroduced one at a time to test whether any particular item seems to increase symptoms.

 It is important to note that no diet can prevent the storage of mucopolysaccharides because they are made by the body. Reducing sugar intake or other dietary components does not reduce this storage.


The airway may be narrower than usual and the tongue is often enlarged, making it difficult to open the mouth widely.

 Because of the narrow airway insertion of a very small breathing tube may be required for surgery. Placing the tube (intubation) may prove difficult. In addition, the neck may be somewhat lax and repositioning it during anaesthesia or intubation could injure the spinal cord. In some cases, it may be difficult to remove the breathing tube after surgery due to swelling that may have occurred and it may need to be left in place.

 In view of the anaesthetic risks, it is recommended that all surgery (including elective surgery) is performed at a specialist medical centre rather than a local hospital, with anaesthetists who are experienced in managing difficult airways.

 It is highly recommended that teachers and caregivers are informed of the anaesthetic risks in case of emergency.

 There is a more detailed explanation of this subject in the specialist anaesthetic booklet published by the MPS Society.

 Physical therapy

Individuals should be as active as possible to improve their general health and a physiotherapist may be able to suggest ways of achieving this. Limitation of motion and joint stiffness can cause significant loss of function. Range of motion exercises (passive stretching and bending of the limbs) may help preserve joint function and should be started early. Physiotherapy and hydrotherapy can be useful to help individuals achieve specific and realistic goals in daily life or to drain mucus from the chest. Exercises that cause pain should be avoided. Once significant limitation has occurred, increased range of motion may not be achieved, although further limitation may be minimised. For children, the best forms of physiotherapy are exercises that are introduced through play and which do not involve stretching or rotation of the joints.