Crosby was born with CHI

10 years with CHI: What has changed?

Born with congenital hyperinsulinism (CHI), Crosby had to have 98% of his pancreas removed and a gastric tube (“G-tube”) inserted to stabilize his blood sugar within one week of his birth.

CHI is a rare disease that causes the body to make too much insulin, leading to potentially severe hypoglycemia (low blood sugar). If not properly treated, CHI can cause both cognitive and physical disabilities.

Luckily, Crosby’s parents learned about his rare disease prenatally through unrelated genetic testing. Still, their early diagnosis would not take away the fact that their child would be born with CHI nor that multiple treatment options would not work for him. 

After having the near-total pancreatectomy, Crosby still made too much insulin and was dangerously hypoglycemic. Given his genetics, it was known that the standard first-line treatment options would be ineffective for him. Therefore, for the first six months of his life, Crosby received a constant infusion of dextrose through his G-tube to stabilize his blood sugar, which was inconvenient, challenging, and imperfect. 

After about six months, he began taking a medication requiring 3 injections per day. This allowed him to reduce the time he spent connected to the continuous dextrose infusion. Eventually, however, that medication stopped working for him. and the family temporarily returned to continuous (24 hours per day / 7 days per week) dextrose via Crosby’s G-tube.

Finally, they found, and switched to, a once monthly treatment administered by injection. But these injections were extremely painful for Crosby and involved an approximately 4-inch-long needle, which was terrifying for Crosby to see (and anticipate) month after month.  

While the injections helped, they were still a traumatic experience for Crosby. What is the right timing to tell your child he has to go get a painful shot to minimize his fear and anxiety – the week before, the day before, a minute before?”

A medical and social challenge

Congenital hyperinsulinism is a medical condition. But it is also a social barrier and challenge for children growing up with it. 

From early on, kids with rare diseases become aware that they are different. Other children and even parents, would sometimes ask about or touch Crosby’s G-tube, not realizing they are being intrusive or potentially causing damage.

To ensure Crosby’s health and safety, the family has a nurse attend school with him every day to help immediately treat any blood sugar lows. For Crosby, and other children with CHI, having an extra caregiver around can help ease anxiety around the chance of a serious medical problem.

This comfort, however, can make it hard to socialize and be a kid without worrying what might happen without parents or a nurse right around the corner. Because of this, Crosby’s family had to be the ones to host playdates and sleepovers. When they didn’t host, Crosby’s parents would often stay or even sit in the driveway when Crosby would attend a birthday party or spend time at a friend’s house.

A first sleepover can be intimidating for any kid, so imagine what is it like to be at your first sleepover and constantly think about your rare medical condition or about whether the birthday cake is going to make your blood sugar spike and then plummet?” 

But today, with the help of other parents and his Dexcom, Crosby has gained a new level of independence. He can now more comfortably hang out at his friends’ houses while his parents are reassured that he is looked after.

What’s next for Crosby?

Crosby is now 10 years old and in somewhat of a “honeymoon” period with his hyperinsulinism. When he was 8 years old, he was weaned off the monthly injection and is not currently on any medication. However, he still suffers from hypoglycemia, but the incidents are decreased. His family monitors his blood sugar with a Dexcom and regular finger prick checks, and Crosby is getting more adept at monitoring and responding to his own blood sugar. For now, this combination, along with snacks, helps maintain his blood sugar levels. But this won’t last forever.

Because Crosby had to have a near-total pancreatectomy at birth, he has slowly been on his way to diabetes. Most likely around puberty, what is left of his pancreas will stop functioning and he will develop insulin-requiring diabetes. By now, he has experienced episodes of hyperglycemia, and it has been difficult for him emotionally to process the changes in his condition.  

Even though having CHI is something that Crosby has always known, it still is scary and challenging. Whether it is his monthly injections, inserting the new Dexcom device every 10 days, needing to prick his finger, treating (and attempting to limit) incidents of both hypoglycemia and hyperglycemia, or the prospect of diabetes, this is all part of living with CHI. But Crosby is resilient. For the last 10 years, he has overcome many hurdles. Nevertheless, his rare condition will require him to face obstacles for the rest of his life.

Supporting each other

While CHI is a rare disease, there is a strong support system through other families, nurses, hospitals, and more. Congenital Hyperinsulinism International is a group that links everyone together through advocacy, information, resources, and support. 

Note: always consult with a physician for further information about treating a health condition.