IUGR and hyperinsulinism (HI), O's story (long!)

amother

Jade

Wed, Jul 20 2016, 12:08 pm

I'm writing our baby, O's story on here solely because when I was googling those scary words in the title (HI), I got next to nothing. HI is apparently very rare and BH our outcome, so far, is good, but I really needed an encouraging story to get me through an incredibly scary period of our lives. I hope this will one day help someone else and give them some encouragement and hope.

When I went for my routine anatomy ultrasound at 20 weeks pregnant with my 3rd baby, the tech noticed some 'stuff' she needed to call the dr to see. It turned out baby had 3 soft markers for T21 (ds). The ones relevant to my story were short femurs and humeruses. After seeing genetics, many follow up ultrasounds etc, it became clear that my baby was IUGR - intra-uterine growth restricted, which basically means he wasn't growing properly in utero. Again, seeing as my placenta and cord blood flow seemed fine, the most likely scenario pointed to a chromosomal defect, until an amniocentesis ruled that out.

Although I was going for twice weekly NSTs with dopplers, which he always passed, his growth was starting to fall off the charts and I was warned to keep an eye on movement, as IUGR is one of the leading causes of stillbirth. BH, I took the warning seriously and went in to be monitored after feeling little movement one morning. O arrived via c section due to extreme fetal distress at 34+6 weighing 1440 grams.

At first, our NICU stay seemed to be going smoothly. O needed very minimal oxygen support and started packing on the grams quickly. He also latched on and nursed as soon as I was able to go and see him and was a pro with the bottle too. I anticipated a 3 week, 4, max, stay.

The trouble started when they tried to wean him off the tpn. O's blood sugar kept dropping and they had to keep upping his dose. This at first, was stressful only because his veins kept failing and they had to reinsert the IV multiple times a day, which eventually resulted in an infection. The staff at first thought the infection might actually have caused the low glucose, but once it was clear, his blood sugar once again plummeted. At this point they put him on the most common drug for hyperinsulinism (testing showed that the low glucose was caused by the pancreas producing too much insulin which is common in babies born at a low birthweight, but only for a few days at most), dizoxide, but after a while, that stopped working.

After a week or so, they had a pediatric endocrinologist come and see him and she put him on hydrocortisone, as at that point they saw his cortisol levels were low and recommended he be transferred to the children's hospital NICU for extensive testing to determine what could be causing this. She told me it was most likely a disease linked to his adrenal or pituitary glands. The best we could hope for was something curable or at least simple enough to treat.

By the time we were transferred, I was so desperate to take my baby home I was just praying they would find something!

After more days and weeks of testing, during which O reached a weight healthy enough for discharge, and finding nothing, one dr had a theory that the low cortisol levels had been caused by his system being thrown out of whack by his traumatic birth and proposed we try weaning and see what happens.

Weaning O off the hydrocortisone went really well, but when they tried to extend his feeds to more than 3 hours (he was on feeds every 2 hours to maintain bs levels and ended up with an NG tube as he was often too tired to eat that often), his blood sugar dropped. Again. At this point the drs were telling us they had no clue what was wrong with him or when he could be discharged.

All his teams (neonatal, GI, endo, genetics and who knows who else) ended up meeting to decide what to do and (probably in large part due to my tantrums) decided to discharge him provided I learn to take his blood sugar, insert and use the NG tube and him passing a 4 hour fasting challenge. At this point, he was back on diazoxide as well.

He did great with the 4 hour fast, but prior to coming home also needed surgery on bilateral hernias in his scrotum and an umbilical hernia. When they went to intubate him, something went wrong and he coded and ended up back in the NICU while they tried to stabilize him for a few days. He was sedated, had a picc line put in and ended up on a jet ventilator for a week or so. The silver lining is that once he was (again) ready for discharge, they did a 6 hour fasting challenge which he passed and gave us leeway to be able to safely go 4 hours between feeds.

Once home, O did really well, transitioning to mostly nursing and being able to have the feeding tube taken out. We had a lot of follow ups, especially with endocrinology and I asked the dr what she thought the reason for his HI could be. I was shocked to hear that the theory now was his small size due to IUGR was the most likely cause and that his HI was hopefully transient. At the hospital, I'd been constantly told he was too old/big to still have this kind of HI. (Truthfully he didn't exactly fit the profile as he'd been resistant to the diazoxide at one point. Asso, as mentiones, this type of HI rarely extends beyond the first few days of life. Still, it would have been nice to know that this was a possibility!)

To make a very very long story shorter Smile

, around a month ago, at 6 months (actual) of age, O was successfully weaned off the diazoxide, discharged by genetics and is doing really well developmentally, BH! I am still testing his blood sugar 2-3 times a week or after any change (extending feeds etc) or illness, but he has a 10 hour fasting challenge next month and if he passes, PG, will likely be discharged from endo, ending all the heel pricks and anxiety! Smile

When trying to research HI, I found a lot of disturbing prognoses and not much in the way of good outcomes. O is my little miracle, but I don't want it to be this hard for other parents to find hope.

Feel free to ask any questions or to be pmed for any additional information wanted/needed Smile