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November 7, 2000


Question from :

From, USA: My 11 year old nephew has DIDMOAD [Wolfram Syndrome]. We would like to know answers to some of these questions: Does optic atrophy happen to diabetes patients only or it can happen to non-diabetes persons also? Can my nephew live a normal life span with the help of insulin or not? Generally, what is the life span of this kind of patient? Can optic atrophy lead to total blindness and deafness in these cases? Is any research going on for treatment of this kind of disease and where? Can transplantation of the pancreas of a healthy human remove the insulin dependent diabetes? What care should parents of the child take to avoid or delay future complications of these diseases?


To try to answer your questions:

Optic atrophy has many causes and can occur with and without either diabetes or DIDMOAD.
DIDMOAD is a very rare condition so that life tables do not exist; but since none of the individual components are life threatening if given good care, it would be quite possible to live into the adult years.
The DIDMOAD Syndrome can vary in severity; but it is certainly possible for the severest cases to completely lose sight and hearing.
Perhaps your nephew’s doctor can try and find more in PubMed by searching under ‘Wolfram’s Syndrome’,’DIDMOAD’ or ‘Diabetes and Deafness’. There is also avery good report which his doctor should be able to find in the library in The Lancet, section 2, vol 346, page 1458 in December 1995.
At the moment I do not think that any transplant center would accept a person with DIDMOAD for transplantation in part because it seems possible that the autosomal recessive change might still affect mitochondrial function in the transplanted islet cells.
You will need to work with your own doctor about the details of both the medical care for the diabetes mellitus and the diabetes insipidus as well as for advice on custodial care.

You might also find help in a new family oriented web site for these children at www.wolframsyndrome.org.