On June 20, 2000 weighing just over eight pounds, my son Evan was born. Shortly after his birth doctors realized that Evan was special. He was born with severe hypoglycemia, and was put in the NICU unit at our local hospital. There he was put on IV glucose, and hydrocortisone (steroid). The neonatal doctors did everything they possibly could to try and keep Evans blood sugar levels stable. He continued to have dangerously low levels. Along with the blood sugar problems we noticed that his blood pressure was running higher than normal. After a couple of days and no success at keeping his levels up, the neonatal doctor called in a local endocrinologist. After seeing Evan she contacted several doctors at the Medical University of South Carolina. She then proceeded to start Evan on a drug called diazoxide, which is used to kelp keep blood sugar levels up. After 24-48 hours and no signs of improvement, Evan was then started on Octreotide injections. The neonatal doctor told me that he had never seen a case like Evans before and this problem was more serious that what they have ever seen. He said that in most cases, out of a hundred babies ninety-eight of them would be treated with IV glucose and the other two would be treated with a steroid. And that within twenty-four hours everything is normal but with Evan that didn’t happen. We were informed that the extremely low blood sugar levels could cause brain damage among other things. After two days passed and Evan’s sugar levels continued to drop dangerously low.The endocrinologist felt that in the best interest of Evan’s safety he should be sent to the Medical University of South Carolina. So at just ten days old little Evan took his first trip, two and a half, hours away from home. Evan was transported by ambulance and we had to drive. The, drive felt more like six or seven hours and two and a half. Once Evan reached MUSC he was taken off the diazoxide, because it really wasn’t doing him any good. Though still on IV glucose and octreotide injections Evan’s sugar levels still plummeted. After an ultrasound was done the endocrinologist told us that Evan was born with hyperinsulinemia his pancreas is producing more insulin than his cells could absorb, and this causes him to have severe hypoglycemia- hyperinsulinism. Evan had to have a special IV put in to run higher doses of glucose to try and keep his sugar levels stable. ( this line is called a pic line, and is threaded into one of the main veins leading to his heart.) After trying for two days and three hair shavings later they managed to get the line placed. It was in the left side of his neck. Evan was put on amlodipine for his high blood pressure. A echocardiagram was done on his heart, which showed that the linen around his heart was thicker than normal. And because of his sugar problems his heart was enlarged. Evan was also placed on a another blood pressure medicine called hydrochlorothiazide. The side effect to this medicine was to make the heart go back down to normal size and help keep his sugar levels up. After a couple of days he was taken off the hydrochlorothiazide. We were also told that Evan had a heart murmor, which was probably normal because most infants are born with one. It usually goes away after a couple of months. The endocrinologist talked with us about the possibility of surgery to remove part of the pancreas. With blood sugar checks every thirty minutes and feeds every two hours, his sugar levels still continued to go up and then drop dangerously low. The poor little fellow’s heels were black and blue from being stuck so much. Evan was taken out of NICU and put in a regular room. With several days passing and his levels still dropping too low too fast, there was more talk about surgery. The endocrinologist told me that they were getting down to the last things they can do medicine wise. Evan was still on feeds every two hours during the day, but was placed on continuous feeds at night , through a NG tube, with thoughts that this might help with the lows at night. After coming off the continuous feeds at 8am he received a bolus of formula. When his lows were less than 40 he received a 10cc bolus of D10 glucose. With none of this helping and still having lows the endocrine doctor put Evans safety first. And made arrangements for surgery. The pediatric surgeons came around to talk with us. According to Dr. Hebra (pediatric surgeon) that 10 years ago Evan would have had 70% of his pancreas removed within the first few days of birth. Latest studies indicated that removing 95-98% of the pancreas is successful and often increases the baby’s chances of having a normal life. With the surgeons explaining what they planned to do, they used drawings and diagrams. They explained the advantages of surgery which were, coming out of surgery and requiring no medications or coming out and requiring a little medications for a short period of time. And outgrowing the low sugar levels as he got older. We were also told about the dangers of surgery, which included getting to the pancreas without hitting the splean, or damaging the liver or the pancreas ducts. And with all surgeries there is the possibility of loosing too much blood. Their main goal was to go in and see if there were and tumors on his the pancreas that could be causing the production of too much insulin. If there were no tumors they could remove, then they would proceed with a 95% sub-total pancreatectomy. Well a date was set for surgery. On August 1, 2000 at just 6 weeks old Evan went to surgery with the possibility of having 95% of his pancreas removed. The night before surgery Evan was giving higher doses of steroids (hydrocortisone) and boluses of glucose by IV. He was taken back to surgery about 9:30am and spent approximately 8 hours in surgery. During the surgery we received phone calls in the waiting room with updates of the surgery, and how he was doing. The first call we received was to tell us they had placed the broviac line. The next saying they found no tumors and was proceeding to remove 95% of his pancreas. The last call we received was to let us know that they were finishing up surgery and Evan was doing just fine. Evan was on a ventilator until the morning after surgery. While in PICU we were given another diagnosis this time it was Nesidioblastosis Idopathic Hyperinsulinism. They only pain medicine he had was demerol and that was the night of surgery. Other than that all he had was tylenol. With a scar going almost completely across his little tummy, he was doing really good.he was taken off the octreotide after surgery and it was only given as needed. He still remained on the IV glucose and the steroid was changed to prednisone. They Slowly began to wean him off the glucose. On Saturday August 5, 2000 Evan was eventually taken completely off the glucose. He was taken out of PICU and put back in a regular room. By 7pm that same night his blood sugar level dropped to 27 and he was put back on IV glucose and octreotide every 4 hours. After a couple of days he was changed to every 6 hours of octreotide. Slowly they began to wean him off the glucose again. He stayed on 1.5cc’s an hour for a few days. With blood sugar levels over 60 he was weaned completely off the glucose once again. Still on octreotide every 6 hours, prednisone every 4 hours, and amlodipine every 12 hours. He was tried on every 3 hours, which he done quiet well. Within the last 2 weeks he was in the hospital he had two lows under 40 which were29 one night and 32 one morning. My mom and I were taught how to give him octreotide injections, check his blood sugar, and what to do if they were under 40. We also had to learn how to put a feeding tube in his nose and check the placement, and how to care for his broviac line which is in his chest and change the dressing on it. I have two other children at home , ages 6 and 4 who have none of these problems. I often thought to myself "out of my 3 children one of them came with their own instruction book". On August 29, 2000, just over 2 months old, we finally got to bring our little Evan home, weighing a little over 14lbs. Not even home for a full 24 hours we had a low in the 30’s, for which his endocrine Dr. increased his octreotide injections. After being home a total of nine days we had to take Evan to the local ER for a fever of 102.8. Once we reached the ER his fever had gone up to 103.8. We learned that his skin had gotten infected oround the exit site of the broviac line. With thought that this could cause infection in the blood stream, he was heli-lifted back to MUSC, where he was treated with IV antibiotics and released 3 days later.Now having to change the dressing on the broviac line everyday. On September 20 he had his first clinic appointment with three different Drs. At the pediatric cardiology clinic, the Dr. said they did not hear the murmor and more, at that time. At the pediatric surgery clinic, the surgeon said he was doing great and his broviac looked really good. At the last clinic appointment we, seen pediatric endocrine Dr. She adjusted some things with his schedule. He is now on every 4 hours feeds and blood sugar checks before feeds. And checking blood sugar levels when he shows signs of hypoglycemia. On October 18 we had our second endocrine clinic visit, his Dr said we may be cutting back on some of his meds at our next appointment and his broviac may be able to come out. The only reason he didn’t take it our then was because he had too many lows under 50 for the past month than what they would like to see. The disease that Evan has is commonly known as Presistant Hyperinsulinemic-Hypoglycemia of Infantcy. (PHHI) PHHI is a rare genetic disease caused by, the presence of extremely high levels of insulin in parallel with low and very low glucose levels. God blessed me with a special little boy who needs very special care and attention. There many other families that have a child that suffers from the same thing that Evan does. We have found out that because of this being a rare disease that there are not too many doctors that study this. And because the funds are not available there is limited research done on this disease.