My 15 month old daughter, diagnosed with nesidioblastosis (PPHI) after birth, is on octreotide and nifedipine. (Diazoxide had no effect.) She also has seizures and takes Valproate. Do you know of another drug to treat PPHI? Do you know any foods or a way of eating to prevent hypoglycemia? How do epilepsy medications affect PPHI?
Despite all the difficulties the doctors seem to have in managing PPHI (Persistent Hyperinsulinemic Hypoglycemia of Infancy) in your small daughter, they do seem to know what they are doing. Octreotide, nifedipine and diazoxide are the standard drugs, and I know of nothing new, although Lanreotide (an octreotide-like drug) is available in a long-acting form called Somatolin Actagel which need only be given once a month by subcutaneous injection. This might be a convenience, but I doubt if it would be more successful than the drugs already used and I have found no reports of its use in PPHI.
In fact, I think that it is probably time to talk to the doctors about surgery. The risk of surgery is of producing diabetes by taking away too much of the pancreas and the need to re-operate if too little is removed. Recently some important advances have been made in the ability to distinguish PPHI that it due to diffuse islet cell hyperplasia and a strictly localised. lesion. This has improved the outcome, but does require specialised facilities. See a previous question which discusses this. Another recent and very helpful reference which can be found on the Internet is A Aynsley-Green, K Hussain, J Hall, J M Saudubray, C Nihoul-Fékété, P De Lonlay-Debeney, F Brunelle, T Otonkoski, P Thornton, K J Lindley Practical management of hyperinsulinism in infancy in Arch Dis Child Fetal Neonatal Ed 2000;82:F98-F107 (March).
With hypoglycemia still a problem, you might try mixing unhydrolysed cornstarch into the diet to see if this will help. It would be most easily given in milk after mixing it first into a thin paste and then diluting with more milk. If this is not available to you, it is possible that a corresponding rice product would help to maintain blood sugars.
I am not qualified to suggest any changes in anticonvulsant therapy. That is a matter for pediatric neurologist, but I do think that it is important to know whether the seizures are still related to hypoglycemia or whether there might be a separate cause.
Original posting 23 May 2002
Posted to Nesidioblastosis
Last Updated: Tuesday April 06, 2010 15:09:33
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