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What Causes Congenital Hyperinsulinism?
CHI is a genetic disease that a baby is born with. It is caused by a flaw in one of the genes that controls how the pancreas releases insulin. These faulty genes tell the pancreas to release insulin all the time, even when it is not needed. It is not caused by anything the mother did during pregnancy.
Why Is Low Blood Sugar So Dangerous?
The brain's main food is blood sugar, also called glucose. The brain needs a constant supply of it to work. In CHI, the severe low blood sugar starves the brain of its fuel. If this is not treated, it can cause seizures, permanent brain damage, and in some cases, can be life-threatening.
What Are the Symptoms?
The symptoms are the signs of low blood sugar in a baby. A baby with CHI may be very sleepy, floppy, and hard to wake up. They may have a weak cry and be very irritable. They may also have tremors or jittery movements. In severe cases, the baby may have a seizure.
How Is It Diagnosed?
Doctors will suspect CHI if a newborn has low blood sugar that does not get better. The diagnosis is confirmed with blood tests. A "critical sample" of blood is taken when the baby's sugar is low. The test will show that the insulin level is high, even though it should be zero. Genetic testing can also be done to find the faulty gene.
How Is Congenital Hyperinsulinism Treated?
Treatment must be started right away to protect the brain. The first step is to give the baby sugar, often through an IV, to raise their blood sugar. The main treatment is a medicine that tells the pancreas to stop making so much insulin. If medicine does not work, the baby may need surgery to remove part of their pancreas.
FAQs on Congenital Hyperinsulinism
Is CHI the same as diabetes?
No. In fact, it is the exact opposite. In type 1 diabetes, the pancreas does not make *enough* insulin. In CHI, the pancreas makes *too much* insulin.
Is this a common condition?
No, it is a very rare disorder. It is estimated to affect about 1 in every 25,000 to 50,000 births.
Can my child outgrow CHI?
Some forms of CHI are temporary and the baby will outgrow them. Other forms are permanent and will require lifelong management with medicine or diet.
When to See Your Doctor
This condition is almost always found in the hospital right after a baby is born. You must get medical help immediately if you have a newborn at home who is very sleepy and you cannot wake them up, or if they are having seizures or jittery, strange movements. This is a medical emergency.