Meet our warrior angel, Gabriel Paul!! We expected a big baby, but he shocked us when he came into the world at 12 pounds and 7 ounces, apneic, and with very low blood sugar… I thank God my OB was the doc on-call; I trust him with my life, and he saved both of ours. Gabriel’s birthday is the day we started to learn about a new rare condition in my family of zebras: Hyperinsulinism.
Even having worked a year in as a social worker in a Diabetes clinic, I never knew that the opposite of Diabetes was an equally serious condition, nor one that would one day become part of my daily life. People’s experiences with Hyperinsulinism vary widely: some do not respond well to medications available and may need up to 98% of their pancreas removed, which may then lead to Type I Diabetes; some have a small portion of their pancreas affected and are cured by having that one part removed; some are symptomatic for a while and “grow out of” needing treatment; some have virtually no symptoms at any point; and many fall somewhere in the middle.
Gabriel grew exponentially in the three weeks between his growth ultrasound and birth due to the amount of insulin he was producing, none of his data at the time told us that this thing that “never happens” was happening to him. We had also already tested negative on the standard genetic testing panel, which we now know does not screen for the type of Hyperinsulinism that Gabriel has (which is rare among the already rare). I was only able to hold him for one hour during his first week of life due to his needing to have a central line placed through his umbilical cord to administer high concentration of glucose. Once this line was replaced with a PICC, we were able to enjoy snuggles until his breathing became too labored, resulting in more time apart until a machine called NAVA helped him start to regulate better and allowed him to avoid intubation. In addition to weeks of continuous glucose, Gabriel also needed continuous feeding through an NG tube with formula added to make it very high calorie (his breathing was too fast to safely eat by mouth). After some time, they were able to wean the concentration of glucose as well as work towards bolus feedings (higher volume at one time instead of continuous feeds, like a regular meal; still through an NG). This was a long, very difficult process for me. When he was five weeks and change, he was finally able to start trying to eat by mouth; I had an amazing Speech Pathologist support me in my efforts to help him learn to eat by mouth as soon as he was able. When he was 6 weeks and two days old, he was able to come home to his big brother - without an NG tube (yay!!) and with various medications. Due to his Hyperinsulinism-induced size, Gabriel also hurt his shoulder being born, so started Physical and Occupational Therapies while in the NICU; he continues to work with wonderful PTs and OTs today, and he is SUCH a hard worker!! I can never thank enough all of the doctors, nurses, OT/PT/Speech therapists, social workers, respiratory therapists, Child Life Specialists, pharmacists, house keeping, chaplains, and Ronald McDonald Charities for providing us support, keeping us safe, and getting Gabriel home.
Gabriel turned me from a person who can’t stand needles to a mommy who still can’t stand needles but checks his blood several times a day to make sure that he is ok. Gabriel’s numbers like to bounce a lot (I mean A LOT!!) and keep everyone on their toes. We have had one hospitalization since and have been fortunate to be able to stay ahead of other hospitalizations through success with medication adjustments when he has gotten sick. We know that we won’t always luck out in this department, and every sniffle he gets makes me wonder if this is the time we will end up in the hospital again, but I am grateful each of the numerous times we have been able to treat his lows from home. For every family that has to cope with this, it is, at times, scary, stressful, and exhausting. I cannot count the number of times we have watched the glucometer with bated breath, praying for a comforting number. I have many fears related to his health, his medications and their side-effects, and how he (and our other family members) will be socially and emotionally impacted by this as he grows; and I have faith in our health care providers and researchers. There are many people more easily controlled and many more difficult to control – I am a huge believer that optimism makes a difference and am just glad each day that we have this beautiful, joyful, incredibly tolerant little soul in our lives, and for all of the absolutely INCREDIBLE support that I have from my family, friends, and providers.
Please join us in supporting Raring to Go for CHI (Congenital Hyperinsulinism International) in the Million Dollar Bike Ride for Orphan Disease Research!! The money raised will go towards a research grant that will help advance treatments for Hyperinsulinism in order to #StopTheLows to give these kids success and a better quality of life. This event will be held in Philadelphia on May 20th, and University of Pennsylvania will match every dollar raised up to $50,000!!