Hyperinsulinism – Edison's story

Just a few days after he was born, Edison became limp and unresponsive. His mum and dad feared the worse but a diagnosis of hyperinsulinism (HI) led to life saving treatment at Great Ormond Street Hospital (GOSH). Mum, Lucy, tells their story.

“On a Saturday in July 2016 our son Edison was born. His dad, Peter, and I were over the moon. By Monday morning, however, we knew something was seriously wrong.

“He deteriorated very quickly, becoming limp and unresponsive. We had an emergency visit from the community midwife, and she rang for an ambulance.

“Both myself and Peter were in shock as the ambulance staff tended to him. We were rushed to Southampton's neonatal unit (NNU), and the doctors and nurses stabilised him. His condition was critical – he looked so small and still, and had so many wires and leads surrounding him.

“We were taken away into a separate room, and told to prepare for the worst. However, a part of us held on to hope and by the Tuesday afternoon we had an answer.”

“Edison had hyperinsulinism (HI). We instantly started reading up on it and turned to the leaflet on the GOSH website.

“The simple way to describe HI is as the complete opposite to diabetes. With HI, the insulin acts erratically. This can mean that the brain isn't being fed with enough sugars, allowing it to enter a hypoglycaemic state (hypo) and cause fits.

“When we arrived at Southampton’s NNU, Edison had a blood sugar level of 0.2 – a world apart from the usual levels of 4–7. He was fighting very hard to stay with us, and despite Southampton NNU’s excellent efforts, after three weeks they knew it was time for us to go to GOSH.”

“The team on Rainforest ward were amazing. What had been a dramatic process of checking blood sugars at Southampton became instant and simple, and soon we were checking his blood sugars and doing his injections four times a day.

“Over the next five weeks Edison was stabilised, feeding from a bottle and put on the drug octreotide to manage his condition. Our next HI milestone was the PET scan, which shows whether the HI is focal or diffuse and where it is in the pancreas. A diagnosis of focal HI would have the potential to be cured with surgery.

“To have the scan, we had to wait until Edison weighed 5kg, and we were allowed to go home for three weeks before his scan date. This was the longest time we’d spent at home since he was born. We were so excited!

“The first few days were hard. As soon as we got home Edison had a hypo (a sugar of 3.5 or below) and over the next few days he had several more. Fortunately, none of them were seriously low and all came up after a feed or with Glucogel.

“It was stressful and there were definitely times that we thought of jumping on a train back to GOSH, but we kept the team updated with his weight and blood sugar levels, giving them all the information they needed to adjust Edison’s medicine dosages. Soon, we were ready to go back for the scan.”

“Kate, one of the specialist nurses, spent the whole day with us when we came back to GOSH for Edison’s scan. We took full advantage of having her around and picked her brain on any and every question that came into our minds. It was so useful and gave us such a sense of relief!

“We couldn't believe it when we heard that Edison's HI was focal, and its location in the tip of the pancreas made him ideal for a surgical route of treatment. Within days we had spoken to the surgeon. He said would try to get Edison back for the operation within the next two months, so we went home to wait, excited but nervous all at the same time.”

“Just four weeks later, we were back – surgery day was here. Three and a half hours after Edison went to sleep, a smiling surgeon popped up to the room to tell us all had gone really well and Edison was in recovery. We were so relieved!

“We still couldn't believe there was an actual chance that Edison could be cured – we had never known him without HI so it was hard to imagine a life without injections and checking blood sugars.

“Over the following days, Edison was weaned off the sugars and we checked his blood regularly. His blood sugars were holding, and we couldn't believe it. He did a six-hour fast, and he didn't even get close to a hypo.

“To triple check he was stable, we stayed in for a couple of extra days. We did an eight hour and he was absolutely fine – our son had been cured!”

“We have now been home for one week, and today is the last day of us checking Edison’s blood sugars. We are still getting our heads around processing the roller coaster of the last four months but there have been several amazing days thanks to Edison, our families, and most of all every single member of the GOSH team. To them we will be grateful and in awe for the rest of our lives.”