My son, who has nesidioblastosis and had the 95% pancreatectomy, is also on diazoxide, nifedipine, and Viocase, but it has been hard to keep his sugars up, and they are usually around 45-60 mg/dl [2.5-3.3 mmol/L], even with all of his medications. What are the chances of someone like my son outgrowing this? What are the chances he will develop diabetes? If he does develop diabetes, will he have vision, kidney, and limb problems? Do you know of any nesidioblastosis support groups?

In view of the fact that your son’s hypoglycemia still seems to be uncontrolled despite a 95% pancreatectomy together with the continuing use of diazoxide and nifedipine it would appear that he is still hyperinsulinemic. Whether this is because he had the diffuse form of PHHI and insufficient pancreas was removed or whether he had the focal form with the focus lodged in the remaining head of the pancreas or just possibly in an ectopic site are possibilities that I am sure have been considered. However, I imagine that even though there are additional approaches with medical treatment such as octreotide and subcutaneous glucagon that your son’s doctors feel that natural resolution is unlikely and that the risks of treatment complications, particularly of hypoglycemic brain damage justify further surgery even though this will in all probability result in insulin dependent diabetes. If this does happen then the chances of long term diabetes complications will depend primarily on how successfully you are able to control his blood sugar.

Certainly with ultrafine needles, almost painless means of sampling blood sugars, and the use of new insulins like Lantus (insulin glargine) and Humalog have made this much easier to achieve. The vulnerability of this group has not yet been evaluated over the long term however. As to support groups, I think you should ask the Medical Social Worker with whom you have been affiliated whether she knows of any local support group for PHHI which might be of help initially; otherwise, I think that you would be best to depend on this site or perhaps (for nutritional problems) on the nearest cystic fibrosis group.

DOB

[Editor’s comment: See Alternate Site Testing and Children with PHHI.

SS]