Failed Cure Fast and New Challenges

Jackson didn’t pass the safety fast. He became unstable pretty quick. I cried quite a bit. So desperate for relief. The good thing is, he isn’t worse. We are still managing the disease as we always have. Hyperinsulinism continues to be our reality.

Due to some issues on the scheduling side, we had a late start. We officially began the study on Saturday afternoon and within 24 hours, he became unstable. He started requiring feeds every 2 hours just to maintain above 70 blood sugar levels. About 20 hours off of the diazoxide, we were headed for the 40s. This was a clear sign that he is still severely hypoglycemic. We administered double the glucagon we had in the past to bring his sugar back up. We also ran critical labs once again. This time, the results were again consistent with congenital hyperinsulinism. Ketones were at .10 and insulin levels were high. We started him back on his meds and stopped the study. We spent the next few days getting the medication built back up in his system and getting him stable once again.

This visit we were blessed to be followed by Dr. Nadia Merchant (who consulted with Dr. Paul along the way). Dr. Merchant is an incredible doctor who went all the way to her fellowship in genetics before changing her course and going to endocrinology. Two of the most complicated fields of medicine. She is one of the most intelligent women I’ve ever met. Dr. Merchant is also a little person. Standing at 3’6” tall, she will tell you, she has had to overcome so much in life. People always tried put her in a box. Tried to limit her. But, not her parents. They pushed her. She wasn’t allowed to use her genetic condition as a crutch. She sat with us for nearly an hour. She knows how disappointed we are but reassured us that there are so many things to be thankful for. We agree.

Jackson has medication that works, very well on him. We live in the United States with access to some of the best doctors in the world. We live in Houston with access to the Texas Medical Center being treated by a hospital that is very comfortable managing this disease. We have doctors that care about us on a personal level. There are plenty of things to be grateful for. We have a plan going forward, more tests to run, more programs to enter. Even though she’s not our main doctor, she made a commitment to us to see us through. She agreed it won’t always be easy, but with such a great support system, Jackson’s potential is limitless. Even though it isn’t the answer we wanted, we’re committed to making the best of our situation.

Unfortunately HI isn’t all we’ve had to worry about. Jackson’s lungs continued to deteriorate. Nine hospitalizations requiring high flow oxygen to aid him in getting over various viruses. We had an original diagnosis of Reactive Airway Disease, then Asthma, then a ton of possibilities. Inhaled steroids provided no improvement and he never, ever, was able to breath “normally”. After tons of appointments, a triple endoscopy, and swallow study, and suffering permanent lung damage, we found the he has silent aspiration. Poor kid. He’s been aspirating his whole life, and we never knew. The cause if a laryngeal cleft and vocal cords that were slightly too short, preventing them from closing all the way. He had two procedures last week and we’re praying for improvement.

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