|
|
Mucopolysaccharide & Related DiseasesSociety Aust. Ltd |
|
|
|
| ||||
|
Medical problems in Children affected by MPS III
Of all the MPS diseases, Sanfilippo syndrome produces the mildest physical abnormalities. It is important, however, that simple and treatable conditions such as ear infections are not overlooked simply because behaviour problems make examination difficult.
A few physical problems need special attention. Please click on any of the headings below or scroll down to read all sections.
Conductive deafness
It is important that the ears are checked regularly, as ear problems will affect the speech and responsiveness of those affected. Sometimes pain is experienced and can lead to aggressive and distressed behaviour. "Glue ear" can be treated with the insertion of grommets under general anaesthetic.
Bowel problemsMany Sanfilippo children have bouts of severe diarrhoea, the cause of which is not fully understood. It is thought that there may be a defect in the autonomic nervous system which controls those bodily functions which are usually beyond voluntary control. The problem may disappear as the child gets older but it can be made worse by antibiotics prescribed for other reasons. Some children may be helped with drugs which slow down the bowel frequency (eg imodium) and others by a change to a diet low in roughage. Constipation may become a problem as the child gets older and less active and as the muscles weaken. If an increase in roughage in the diet and in fluids does not help or is not possible, the doctor may prescribe laxatives or a disposable enema. EpilepsyAt a later stage in the disorder, a number of children with Sanfilippo syndrome will start to have frequent minor seizures when they momentarily alter their level of consciousness (petit mal). Some may have generalised seizures (grand mal), involving either loss of consciousness or physical jerking. Both forms of epilepsy can be controlled by medication and there is a range of medications available which your doctor can prescribe. Challenging behaviourUnfortunately there is no "magic cure" for many of the behavioural problems experienced by individuals affected by MPS III. The changes in behaviour result from the changes in the brain and are difficult to treat with medication. There are many different drugs that can be tried in the treatment challenging behaviour, the most common of which is melleril. Melleril has a long history of use in this situation. Consult your doctor about this. Sleep disturbanceSleep disturbance is common in individuals with MPS III. There have been promising results using a naturally occurring chemical called melatonin to treat sleep disturbance. Melatonin is synthesised from serotonin in pineal gland in our brain and is secreted in response to the light/dark cycle important in the initiation of sleep. When it becomes dark our pineal gland starts to secrete serotonin and this activates that part of our brain which sends us to sleep. Melatonin is now the drug of first choice for treating sleep disturbance in neurologically impaired children in the UK and can be highly effective in about ¾ children.There are some important things about Melatonin which you need to know if you are going to use it, or it is unlikely to assist:
Back to MPS III | Characteristics of MPS III | Medical Problems of those affected by MPS III | Current Research into MPS III Table of Diseases | Aust MPS Statistics | MPS I | MPS II | MPS III | MPS IV | MPS VI | ML II and ML III |
|
