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.
They say a picture is worth a thousand words, but looking at this picture, I could tell you THOUSANDS. These are lancets and test strips from our sharps container at home. These are all from glucose checks and not even half of what we’ve used over the last few months.
To me, these are hundreds of pokes on the sweetest baby toes you’ve ever seen. These are scared moments when his sugar has dropped too low. These are late nights and early mornings awoken by his crying. These are calibrations on his CGM. Testing how new foods affected his sugar. These are from moments when he was throwing up and we didn’t know how long we had before he’d become hypo, when we were checking every 30 minutes (or less), all while rushing in Houston traffic trying to get to the Texas Medical Center. But, mostly, thankfully, these were our reassurance that he was okay. This is what our lives have revolved around for the last nine months.
This is us checking for “Sugar Bugs” – no idea why we started calling it that but at some point, one of us said it and it stuck. How many sugar bugs are we going to find? Let’s catch lots and lots of them! Yay!!! We found 92! Seems kind of silly but we are trying to make it a positive and fun experience. He doesn’t cry anymore when the lancet pierces his skin or wake up when we poke him at night. His big toes are so callused that sometimes getting a small drop of blood is a challenge. I remember when we first started this journey, his poor toes became so beat up and turned red with bruises.
Now, here we are, just days away from the start of our “Cure Fast” at Texas Children’s. I am so anxious. We’ve slowly reduced his Diazoxide intake. He’s gone from 9.54mg/kg to 8.18mg/kg. So far he’s doing really well. He’s had a few numbers near 70 but for the most part, he’s hanging out in the 120 range. I pray that this is a good sign. I’m scared to be disappointed because no matter how many times I tell myself “Don’t get your hopes up”, they’re up. After all, I am a mom with HI Hopes. It’s who I am. I want this so bad for him, for us. The next two weeks, I suspect will be a roller coaster of emotions but I want to know. It’s the not knowing that has me feeling so crazy. If he has to live with HI longer, or even the rest of his life, we’re going to manage, I know that, but I really pray he doesn’t have to.
My hopes are that one day the words that come to mind when I look at that pile of lancets and strips are in past tense. That they become things that we used to have to do. Distant memories as a life free of hyperinsulinsm start making new ones in our lives. How sweet that life could be. I don’t know if that’s what’s in the future for us but I’m holding that dream close to my heart and never letting go.
Our lives aren’t easy on most days. Five hospitalizations in the last few months have left us stressed, scared, and overwhelmed. Yet we are stopping to enjoy the sweetness in our lives. Jackson is about to be one year old!
In the last year we’ve learned so much and met some of the most amazing people.We’ve seen our family and friends rally around us, we’ve learned to advocate, we’ve seen our older child grow and mature in her fight for her brother, and we’ve seen our “Sugar Baby” become a hero right before our eyes. No, it’s not always easy but life can sure be sweet.
Jackson has been mostly stable. A few scares here and there when his sugar drops for no apparent reason and we fight to get it back up, but I’m happy to say that has become the exception rather than the rule.
He’ll be readmitted in the next few weeks for our first Safety Fast since diagnosis. Our goal will be to fast him on Diazoxide alone to see how long he can go without eating. This isn’t a full proof method but this is one of the only tests we can do to gauge if he’s getting any better, worse, needs an increase in medication, and how long we have if he’s unable to eat before things take a dangerous turn for the worse.
So many things can affect your blood sugar; illness, pain, outside temperature, level of energy burned, to name a few. So how he responds in the hospital may not be how he responds in a “real life” scenario but it’ll give us a starting point.
The CGM was finally approved and we love it! We had to go through the State of Texas Independent Review Organization (IRO) for a final appeal but UHC’s decision was over turned!
The reviewing doctor responded with:
This young infant was diagnosed around four months of age with altered mental status and seizures. There was concern that he had been hypoglycemic and possibly experiencing seizures for some time. He has since stabilized on diazoxide. However, despite diligence to do blood glucose monitoring every three hours, frequent feedings, and conscientious medication administration, he continues to have severe hypoglycemia spells. He is unable to communicate symptoms with his parents, increasing likelihood of severe hypoglycemia. This inturn increases his risk of brain damage and intellectual disability. Use of a Dexcom G5 continuous glucose monitoring system would allow for near continuous glucose monitoring, allowing the family to intervene as needed, preventing periods of severe hypoglycemia and reducing potential risk for brain damage. This device is medically necessary and appropriate for this baby boy.
This was a long fight that required standing over fax machines, staying on hold for hours, tons of phone calls, and possibly a few tears on my end. I’ll never understand why I had to fight so hard but in the end, the reviewing doctor understood the severity of the situation and did the right thing.
Right now we have the CGM placed on the top of his thigh. The first insertion was a challenge and he cried a lot but now we’re getting the hang of it. He uses an iPhone to act as a transmitter and as long as he’s on wifi (like at daycare), I can check his sugar through an app on my phone every five minutes. There are alerts for when his sugar is dropping too fast, under 70, and under 55. Definitely worth the fight.
The Dexcom was denied once again after an urgent appeal to United Healthcare.
Ugh. This is frustrating. The letter stated I was out of appeals and my next step would be to get a third party involved. The reason: The FDA has only approved the CGM for Type 1 Diabetes. I’m just one mom, there’s no way I can get the FDA to help us, right?
I know insurance companies are all about the bottom line. I get that. I understand that. I really do but this morning when Jackson’s sugar dropped into the 60s without warning, we would have known he was declining if he had this machine.
To the doctor who gets to make these critical decisions: To you he may just be another “claim”, to me, he is my whole world. Please do the right thing.
Jackson’s endocrinologist requested that he receive a Dexcom G5 Continuous Glucose Monitor. He warned it would likely be denied by the insurance company.
I seriously thought, there’s no way. How could the insurance company deny a device that could literally save his life? I have talked to the insurance company several times since Jackson’s birth and truth be told, they’ve been incredibly pleasant to talk to. No issues whatsoever.
When the insurance company denied the claim, even after prior authorization, I was a little shocked. I know, I had been warned but they had been so easy to work with, I expected more from them. I decided to call and talk to them. Surely, if they understood Hyperinsulinism, there’s no way they would deny it, right? WRONG
Their reason? “BECAUSE PATIENT DOES NOT HAVE TYPE 1 DIABETES, THIS DEVICE IS NOT MEDICALLY NECESSARY”. Ok, deep breaths. So I go into a long drawn out explanation of HI, the risks and the need for continuing to monitor blood glucose levels. I try to stick with simple facts:
- He doesn’t have diabetes, but has a disease that affects his glucose level.
- He is severely hypoglycemic.
- If his blood glucose level (BGL) drops and I don’t catch it, he could suffer brain damage or worse.
- He’s six months old, he can’t tell me if he doesn’t feel good. This machine will communicate for him.
- This machine isn’t just necessary for diabetics, it’s necessary for all people who have to monitor BGL 24-7.
That wasn’t enough justification. So in a desperate plea, I submitted an appeal. Telling them everything I knew about the disease and basically begged that they’d re-consider. Julie with Congenital Hyperinsulinism International also wrote a letter encouraging them to reconsider. Asked that they look at the facts. The truth is, if he suffers brain damage, the medical expenses will far exceed that than the coverage of this device. It’s the right thing for them to do and could save them money in the long run.
They said they’d make a determination on the appeal in 30 days. So… we wait.