Meet Sophie

Sophie was born four weeks early in January, 2004 and weighed 8 lbs 9 ozs. Her blood sugar level dropped to 8 just a few hours after birth. In the NICU as a newborn baby, we had no idea Hyperinsulinism existed or was possible. We all, doctors included, thought she was going through a transition period also known as "Transit HI". At that time knew nothing about Diffuse or Focal Hyperinsulinism or what we really faced.

Sophie had a PICC line placed in the NICU because a regular IV could not carry the high levels of dextrose needed to keep sugars up - a PICC acts a "line" in the vein up the arm and makes the vein stronger to carry large amounts of fluid.

Sophie came home at 2 weeks old having to take Diazoxide every 8 hours to maintain glucose levels. We did the fasting studies here Brenner Children's Hospital under the wonderful supervision of Dr. Robert Schwartz who was in close contact with Dr. Stanley at Children’s Hospital of Philadelphia. She did well until 8 months of age.

She required IV dextrose at the first sign of a cold or illness because her sugar levels would drop. She began Octreotide injections with the Diazoxide every 8 hours to maintain glucose. At this point we knew we needed to go to CHOP for the PET scan. At this time the PET was still research and a Diffuse case had not been scanned. No one knew what a Diffuse case looked like on film.

We were admitted to CHOP in March, 2005 and Sophie had a PICC line placed with two looms - two lines in one - as she would need so many lines for her surgery and recovery. She then began IV dextrose because she had to stop all meds and be off of them 5 days before surgery. She underwent every possible research study for HI.

In Mid-March, 2005 Sophie had a 95% pancreatectomy and had a G-tube placed. It was confirmed she had Diffuse disease and her entire pancreas was over producing insulin. Sophie was in NICU for her recovery from surgery with the double loom PICC and IVs all for different things.

Sophie's recovery was tough! One week after surgery we thought she was doing great and all of her lines were removed. But then she became very ill, she began to vomit violently and after an ultrasound and upper GI they found that a pocket of pancreatic fluid had formed where the pancreas was removed and closed off her stomach.

The next day a drain tube was placed through her liver into the pocket of fluid so it could drain. Sophie was very sick at this point. They could not get another PICC line in place as she had too much scar tissue from the others. So we were left with only IVs. Her veins were so used and scarred, an IV did not last very long so they would have to do another and another. She had 17 IVs while at CHOP. It took the surgery team, headed by Dr. Adzick, a long time to get her to sleep just to get the drain tube in place. Sophie suffered and I was at my limit. Then after the tube was in place, she suffered even more with pain. Finally after a few days she began to heal and the fluid drained off. This allowed the leak to close off and heal as well. This complication cost us another 3-4 weeks at CHOP. Her complication only occurs about 1 every 3 or 4 years. I was jokingly told, “We do rare very well here”.

Sophie is now insulin dependant on Lantus 7 units every morning and novolog insulin if needed. She is on creon digestive enzymes with every meal and vitamins. Sophie had the g-tube removed and the Gasterostomy fistula corrected. She is now in Kindergarten. She has a machine and low blood sugar supplies as well as Glucagon at school. The staff is well trained. Sometimes I have to go to the school and give her insulin if she needs extra during her school day. She checks her sugar three to four times at school - more if she feels low. It has only been two weeks but she has had only one low so far.

I carry Glucagon in my bag everywhere; if we travel we always have a medical bag for her. She wears a medical bracelet that reads: Insulin dependant, my name and numbers, and the name of our local hospital should she need emergency care. I find most people want to label her as now being a diabetic. She is not diabetic! She is insulin dependant secondary to surgery for Hyperinsulinsim. We do not count carbs for her diet. There is no rhyme or reason to her sugar levels. I know what she can eat and what she can not. Some days her sugar is well controlled, and then there are days where it does not matter what she eats, they are high all day with lots of insulin. She is much better controlled now after surgery than before.

Life is much more manageable now and controlling her blood sugar levels is easier since her surgery. Fighting low sugars was a constant battle and worry. Her battle now is constant but easier to manage. As she gets older it gets easier. She has to understand her limits and checks her sugar and there are always new challenges. Sophie is so strong, she is my hero!!!

I guess I have learned to deal with the "chronic sorrow" that comes with this disease. I have good days but I have my days were I cry too. All I can say now is God gave her to ME! He gave her to me because He knew I would fight for her and teach her to take care of herself. He gave me such a special blessing and trusted this precious angel to my care. I am HONORED!!!