Congenital Hyperinsulinism (CHI) was the last thing on my mind when it came to having a baby...

Many people have not heard of CHI so I've written a quick overview using simple words, hoping this helps?! haha....

Congenital Hyperinsulinism (CHI) is a disorder where insulin is excessively secreted in the body, not being regulated by glucose appropriately - this causes hyperinsulinaemic hypoglycaemia (hypo) episodes where insulin levels are high and ketone bodies aren't being made - this is extremely dangerous for the brain and the protection of brain damage; as it means it isn't getting the fuel (glucose) or ketone bodies (which generate different fuels) it needs. A safe blood sugar level for CHI is 3.5 and above - a hypo is anything below 3.5. There are two types - focal, where a certain area of the pancreas (a lesion) is producing too much insulin, or Diffuse, where the entire pancreas is affected.

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It's a very brief summary, but here's my daughters story so far..

On Saturday 13th April @ 17.25pm; Orlaith Rae was born. ♡
I could go on and on about the feeling you come across after just having a baby but it really is the cliche feeling of feeling complete and unconditional love. All of sudden, nothing else in the world mattered except her existence.  I never thought I'd be a mother at 23, I had so much more I wanted to do in life but there I was, holding a beautiful baby girl and nothing will ever come close to it.

Orlaith was an extremely shaky and jittery baby. I assumed these were traits of hers, but it continued into her first night at the hospital, only to get worse. Orlaith would go through episodes of being floppy and in a daze to being vocal and screaming the place down, reluctant to feed. In the morning, she had routine OBs and it become clear Orlaith wasn't going home. Doctors kept coming in and observing her before telling us she needed to go to SCBU (special care baby unit) - she was admitted on a 0.9 blood sugar level - it should be above 2.6 for normal newborns. This didn't mean anything at the time but we were made aware the feeding and jittering was being caused by low blood sugar levels and she needed medical assistance - at first I was just confused, I couldn't understand a word the Doctor was saying, all I heard was him say 'antibiotics' over and over again.

Orlaith was taken to SCBU and was instantly put onto a 10% dextrose IV drip - this didn't do much and she went upto 12.5% then 15% .. She still lingered in the 0.'s, even dropping to as low as 0.2.
Due to Orlaith's veins being fragile - as with any new born - she had a IV line put into her belly button, as the dextrose percentage was too high for regular veins. Numerous canulars failed but Orlaith luckily remained stable. A few days after birth, one of the staff nurses told us they will be taking blood to check for "Hyperinsulinism" and briefly described what is was, but we didn't know the severity then. The results came back and she was on the fence, neither positive and negative. Our hospital stayed in daily contact with Great Ormond Street hospital, one of the only TWO centres in the UK which deal with CHI, to indicate what steps need to be made. Orlaith also developed severe reflux and would struggle to hold a feed down - resulting in the NG tube you see in her nose, this has been used since birth to help feed her. She's more than capable to take a bottle but with the reflux was extremely difficult (majority of CHI babies suffer with reflux and aren't keen on feeding, exactly like the day she was born).

As the weeks went on, SCBU tried numerous medicines and kept her on the IV drip constantly, as she'd only hypo without it. All together, it was THREE times we titrated the IV fluids with feed and she was able to come off the drip. However, Orlaith would only last a couple of days and she'd be back on the drip and meds were being increased. How Orlaith didn't slip into seizures, comas, or developed brain damage is beyond us. Drs indicated to us we were awaiting a transferral to Gt. Ormond Street but it could be some time until the bed was available. It got to the point where Orlaith kept dipping dangerously low and she needed a higher dextrose percentage, but she would need surgery to fit a central line as the glucose is too strong for veins and would damage them.

 Within a space of a day, I wasn't only told about surgery to fit the line- I was told she may have brain damage and a tumour on the pancreas. The next day we were transferred to Southampton hospital for surgery to fit a central line for the weekend, thankfully all was a success. We arrived back to our local hospital all fine - Orlaith was stable and the plan was to keep it that way until we could be transferred to GOSH to see the specialist team and top notch consultant. . Early hours the next morning after returning back from Southampton, Orlaith started having cardiac episodes and stopped breathing - I am forever grateful and thankful to the nurses who were on shift that night, without them we wouldn't have our daughter today. During all what was happening, bloods were taken which indicated low levels (of what, I'm not too sure!) and Orlaith had a blood transfusion which helped her remain stable. Orlaith had also had a brain scan which showed there was NO damage on the brain, overjoyed is an understatement.

By this point, Oralith was nearly 7 weeks old and we were finally being transferred to GOSH as a bed had become available. We've been here nearly 4 weeks and the team are simply amazing - coming from somewhere which had never heard of the condition, here it is like second nature. Orlaith remains on a 30% dextrose and occasionally hypo's, but here we have the ability to control it. After routine blood, her hyperinsulinism was confirmed and it indicated the CHI may be focal... This could be the best news, as hopefully it would mean surgery, recovery then home. Diffuse is more difficult to control and get rid of, but there is still a possibility of it so we're open to the outcome - Orlaith will need a PET scan to confirm the current findings. Fingers crossed it's focal and HI may well be cured!

I'm so proud of my daughter, at ten weeks old she has endured so much; she's so brave and words can't describe how much I love her. ♡♡♡