My nine year old daughter has nesidioblastosis and has been on diazoxide [a prescription medication] since she was six months old. She also has chronic fatigue syndrome, dyslexia and a really low confidence which is not helped by the side effects of diazoxide. Her doctor never seems to bother with her. No tests have been carried out for seven years. Is there an alternative to diazoxide? Would it be better to have her pancreas removed? We have had many years of heartache over this, and we need it to stop.

There have been some important recent advances in the understanding and treatment of PHHI (Persistant Hyperinsulinemic Hypoglycemia of Infancy), a name that has come to replace that of nesidioblastosis. I rather doubt that chronic fatigue syndrome and the dyslexia, due to the diazoxide, but they may be secondary results of poor control of hypoglycemia. If this is the case, it would be worth considering a calcium channel blocker like niphedipine as an alternative. This was at one time much used in the treatment of hypertension, but as long ago as 1996, its use in HPPI was demonstrated (Lindlay. KJ.Archives Disease in Childhood, vol 74, page 369,1996). There have been similar reports too that have been more recent. Octreotide which is a somatostatin analogue has been also used successfully, but it has a major disadvantage in that it has to be given by injection.

If you can make friends with the librarian in one of your local hospitals, he/she could help you also to get two other reports: the first by Pascale de Lonlay Debeney and others in The New England Journal of Medicine (vol 340 on April 15, 1999), and the second by Rahier.J.in The Archives of Disease in Childhood: Fetal Neonatal Edition (vol 82 page 108, 2000). Together these show that about half the cases of HPPI are due to a focal islet cell hyperplasia, and the rest are due to a diffuse abnormality of the beta cells. If the first group can be defined by a combination of pancreatic catheterisation and intra-operative histology, the results of a limited resection of the focal lesion have been excellent, whereas with surgery in the diffuse group, it was not easy to strike a balance between subsequent continuing hypoglycemia on the one hand and diabetes on the other.

If the surgeons and radiologists at the Childrens Hospitals have the technical support for this procedure, surgery might be an option sooner rather than later.

I am afraid all this is going to require rather a lot of work on your part, both assembling all the information and persuading the paediatric endocrinology consultant to help.

DOB