Congenital Hyperinsulinism Center

Treatment

The goal of treatment of HI is to avoid low blood sugar and prevent the risk of brain damage. Medical treatment is usually attempted before surgical treatment.

Medical Treatment

Includes feeding, as well as drugs that block insulin secretion.

Feeding

Oral: babies may need to be fed a specific amount of sugar or other carbohydrates on a certain schedule to avoid low blood sugar. These feedings may include breast milk or infant formula, glucose water (also called dextrose water), and in older infants, cereals or bread and other starches. Feedings alone are rarely sufficient treatment.

Tube: involves an infusion of dextrose water or formula into the stomach. The tube is passed down the nose and into the stomach. For long term treatment, a gastrostomy tube can be placed directly in the stomach. Tube feedings may be used when the baby can not take in enough at feeding times. In this situation, formula can be infused over a short period of time through the tube. Babies can easily become dependent on the tube feeding and have difficulty eating by mouth. Careful attention must be paid to avoid this dependence. Some babies who take enough formula by mouth still need a continuous infusion of nasogastric dextrose to keep the blood sugar normal. This type of tube treatment does not typically interfere with the baby's feeding ability.

Drugs

Diazoxide (Proglycem): oral medication given 1-3 times a day. Works on the KATP channel to block the release of insulin. In some types of HI, diazoxide works well , in others it is ineffective. Common side effects of Diazoxide are:

Octreotide (Sandostatin): given by injection 2-4 times a day. It works by decreasing the secretion of insulin, however, it does not work directly on the SUR. In place of shots, octreotide may be given as a continuous infusion using an insulin pump. Common side effects of octreotide are:

Glucagon: an injectable drug that briefly raises the blood sugar by releasing sugar from the liver into the bloodstream. Used as an emergency drug to quickly raise the blood sugar for 1 hour when the baby has low blood sugar but cannot feed. Can also be infused slowly into the vein while the baby is in the hospital for several days while tests are performed and treatment decisions are made. Chronic treatment of HI with glucagon at home is not currently feasible.

A common side effect of Glucagon is a brief period of nausea and vomiting.

Surgical Treatment

Removal of part of the pancreas (partial or subtotal pancreatectomy). Surgery is performed when medical treatment fails to prevent low blood sugar. The type of surgery depends upon the type of HI. With Diffuse KATP HI, a 95 % pancreatectomy is commonly performed. With Focal KATP HI, removal of only the diseased focal area of the pancreas is attempted.

Surgical treatment can result in:

For the first few days after surgery almost all babies have high blood sugar and may require insulin. The high sugar immediately after surgery is a result of the stress of surgery and anesthesia. It is not an indication of the baby's ultimate need for insulin or any other treatment. The outcome of surgery can be tested after the infant has recovered from the operation and is eating normally again.

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