I know how hard it is to live with and manage congenital hyperinsulinism (HI). That is why I am joining Team CHIbra to raise funds to address the critical need for research for diagnostic tests that would increase timely diagnosis of HI, tools for better management, new treatments, and one day, a cure.HI is a life-threatening disorder that causes dangerously low blood sugar levels. Prolonged or severe low blood sugar can cause seizures, brain damage, and even death. The consequences of HI are preventable; however, HI is often overlooked, misdiagnosed, or even when detected, mistreated.

Liam was born via section on 7-21-14, 10lbs 14oz, and amazing. We knew something was going on at our 20 week scan when the tech said this baby was measuring well over the 100th percentile. When he was born, his breathing was labored and he was rushed to the NICU at Morristown Hospital. Thankfully one of the nurses checked his blood sugar and it was so low it didn’t register. He was immediately given an umbilical picc line that administered D10 directly to him to raise his blood sugar. Since I was in recovery, I could only FaceTime my lil man to check in for the first 24 hours. Luckily Dad was able to be with him and get all of the updates.

His picc line was eventually moved to the side of his head, first Mohawk! We met with some amazing doctors who ran a number of tests to try and determine what exactly was causing such low continuous numbers. Liam was eventually given an NG tube for eating, and genetic tests were ordered. He had inherited two recessive mutated ABCC8 genes resulting in his diagnosis of diffuse congenital hyperinsulinism. This meant Liam had lesions all over his pancreas that secreted insulin at about 7x the normal rate. 

After 9 days in Morristown’s NICU we were transferred to CHOP’s NICU to meet with their hyperinsulinism team. On August 26th when Liam was 5 weeks old he had 98% of his pancreas removed and a permanent g-tube placed. After a lot of monitoring and patience he was able to come home on September 15th with no medical support, his blood sugar was controlled on diet alone. This only occurs with about 15% of patients. 

From that moment on everything we thought to expect about having a baby was thrown out the window. Getting up every 3 hours to feed happened, but it came along with a sugar check and the feed was via pump. When Liam got a little older and started to pull his feeding tube out, we learned how to put it back in. Again, with a lot of patience, Liam learned to eat on his own and we pulled his gtube a month after his first birthday.

Liam will be 9 years old this July. His blood sugar is still diet controlled, he wears his freestyle libre daily, and is incredibly aware of how his body reacts to different foods and activities. He gets frustrated, but we are all in absolute awe of how incredible he has handled his condition. This does not define him or make him who he is, it’s just his thing. Eventually it’s expected that small portion of pancreas that is left will stop working and Liam will be considered an acquired type 1 diabetic. There is no timeline or way we will know this other than when his numbers start to trend differently. Until then, he will just keep doing what he’s been doing, and staying awesome.

A friend said to me when we first found out Liam’s diagnosis, that one day this will just be a small memory in a slew of really great ones. I remember thinking that was insane. I had a baby in the hospital, scheduled for surgery, with a condition a knew very little about. But now, almost 9 years later, she couldn’t have been more right. Everyday he hears “dude what’s your sugar” probably more times than he can count. We are still learning foods that work and don’t. When he’s sick we have to adjust between medicine and meals. More importantly, this is a kid who is so much more than CHI. He loves video games and will debate with us for hours which marvel movie is the best. He will watch every new show on Disney+  and then every single video that breaks down each episode. Liam is an amazing big brother and every single day he makes us all laugh. Every year, even every month, it does become a smaller and smaller memory. Living in a hotel room. The uncertainty of it all. The biggest memory was the first day home and the relief that it finally was here. I hope that memory never becomes small.