transient hyperinsulinism

It’s more than a news headline – This could change everything.

/ Renee J / Leave a comment

I know this is a long post, but if you have a few minutes, I hope you’ll read it and continue supporting my family and others like ours. The more you know!

The Orphan Drug Act (ODA) was passed in the U.S. in 1983 to encourage drug companies to research and develop therapies for rare diseases. Why is it called “orphan”? Because rare disease therapies generally lack sponsors to develop them. The market is small, research is expensive, and there is very little return on investment.

There are over 30 million Americans living with more than 7,000 rare conditions—yet only 5% of these diseases have an approved treatment. This means that the majority of those affected have no treatment options at all. For many families, taking a loved one home and being told to “make the best of the time they have left” is a devastating reality.

To incentivize research, the Orphan Drug Designation grants manufacturers and sponsors a 50% tax credit on qualified clinical testing, along with access to several grants to help offset development costs. According to the FDA, in the decade before the ODA was passed, only 10 treatments were developed for rare diseases. Since then, this incentive has helped bring nearly 500 new medications to patients—a proven track record of success.

Today, the House passed the Tax Cuts and Jobs Act, and the Senate will vote after Thanksgiving. This bill includes a proposal to repeal or weaken the Orphan Drug Tax Credit. This is one of the few tax credits that has directly saved lives. I’m asking you to stand with the #raredisease community to ensure it is not weakened or repealed as part of tax reform.

How Can You Help?

Follow the link below, enter your address and zip code, and a pre-written letter to your senators and representatives will be generated for you. All you need to do is enter your name, home address, email, and hit submit. It’s that easy.

https://salsa3.salsalabs.com/…/p/dia/action4/common/public/…

As always, thank you so much for your support. xoxo

#TheMoreYouKnow #WeHaveHIHopes #Hyperinsulinism #OrphanDrugs #TaxReform

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Safety Fast for Hyperinsulinism

/ Renee J / Leave a comment

wehavehihopes cgm low.png

Safety Fast for Hyperinsulinism

We arrived at Texas Children’s Hospital on Sunday at 7 PM for Jackson’s first Safety Fast since his discharge in September. We knew it would be tough and prepared as best we could. We didn’t think he would last longer than eight hours, but we needed to know.

Some parents of HI kids choose to skip the fast, and I understand why—withholding food to force a hypoglycemic state sounds barbaric. But we felt it was necessary to ensure his diazoxide dose was high enough to sustain him and to check for any changes in his lab results.

The fast began at 8:30 PM. He stayed on schedule with his diazoxide and was allowed water. His starting blood sugar was 81. We checked every three hours until he dropped below 70, then every hour until 55. Our goal was to see how low he would go before showing symptoms, then administer glucagon, complete a glucagon challenge, and run labs.

The Results: A Shock to Everyone

Jackson’s blood sugar remained above 70 for 10 hours—far longer than expected. But when it dropped, it plummeted—going from 70 to below 50 in just two hours. He hovered between 44–32 for hours.

He never showed typical signs of hypoglycemia—no sweating, clamminess, or shaking. Since he can’t communicate, we don’t know what he felt, but he was cranky, asking for milk, and visibly miserable. His CGM alarm kept going off, and we learned that when blood sugar drops below 40, the app stops showing numbers and just reads LOW.

After three hours, his face lost color, and we stopped the fast, took labs, and administered glucagon. Despite everything, he remained alert, playing and even laughing between crying. At that point, he had fasted for 15 hours.

Unexpected Findings

In past glucagon challenges, Jackson’s blood sugar always rose as expected. Not this time.

  • Starting glucose: 34
  • 15 minutes: 44
  • 30 minutes: 38
  • 60 minutes: 32

Instead of rising, his glucose dropped. He was immediately given D10 (dextrose) through his IV and allowed to eat. His sugar quickly spiked to 210, and after stopping the dextrose, he stabilized between 85–120.

Then came the biggest shock—when Dr. Paul reviewed the results, he said something we never expected to hear:

Jackson was making ketones.

For the first time.

HI kids don’t make ketones—that’s part of the disease. But Jackson did.

Even more surprising, the glucagon response showed that his liver was distributing glycogen appropriately—meaning the glucagon “failure” wasn’t due to a liver issue.

These results suggest that Jackson may have Transient Hyperinsulinism—something we never thought possible at this point.

Most cases of transient HI resolve within the first 30 days of life—some within the first year. Jackson was diagnosed at four months old and just celebrated his first birthday, so the odds were not in our favor.

But now?

Dr. Paul believes there’s reason to have HI HOPES.

Testing the Theory: Three Options

  1. Let him outgrow his dose until he’s below 4mg/kg
  2. Slowly wean him down
  3. Admit him for a Cure Fast

We’ve chosen to admit him. It’s not that we can’t do this at home, but we want the added security of a team who knows this disease inside and out. If something happens, we want an army behind us.

So in two weeks, we’re going back.

A Mother’s Reality Check

Yesterday, Dr. Paul looked at me and said, “You know, if he outgrows this, you’re going to go through withdrawals.”

He might be right.

Since August, my brain has been running 100 mph—researching, advocating, managing appointments, fighting insurance companies, running a Facebook page, a website, planning a fundraiser, constantly checking my phone for his CGM readings.

If he no longer has HI, we’ll suddenly be thrown into “normal” life—something I’ve never considered a possibility.

But I’d much rather have a calm life with a healthy kid than a hectic one with a sick kid.

None of this is guaranteed. But there’s hope.

This morning, I let Jackson sleep in before daycare. Yesterday was rough on him, and he needed the rest. When I walked into his room, he jumped up to greet me—and I was overcome with emotion.

No matter what the Cure Fast shows next week, I will never stop fighting for him.

This kid is amazing.
He is strong.
He is a fighter.

He is sunshine and rainbows in every storm we go through.

And he deserves a life free of illness.

I want this for him so badly.

#wehaveHIhopes

I See the Grace

/ Renee J / Leave a comment

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Two years ago, I posted a picture on my Facebook page—an excerpt from Matthew 17:20:

“If you have faith as small as a mustard seed, nothing will be impossible for you.”

I posted it while clinging to hope during an impending miscarriage, believing I could will a miracle. Until the miscarriage was over, I held onto hope.

Now, here I am again—at His mercy—trying to will a cure for Jackson.

A friend introduced me to “Thy Will” by Hillary Scott, a song written after she suffered a pregnancy loss. It resonates deeply.

As a Christian, I wish I had unwavering faith all the time, but I don’t. I’m flawed, human, and I struggle.

I get angry with God. Sometimes I give Him the silent treatment.
I feel resentful, unheard, ignored.
I want to be in control.

And sometimes, I’m just plain mad.

I know, I know—you aren’t supposed to be mad at God, and if you are, you shouldn’t admit it. But He knows me. He hears me. He sees my broken heart.

He knows I’m scared for Jackson.
For the impact on my daughter.
For my marriage.
For losing our home.

He knows how fiercely I love my children.

So why them?

I may never know the answer, but when I start feeling lost, those words replay in my mind:

“Thy will be done.”

And I remind myself—He is God. I am not.

I don’t get to call the shots in my story, in Jackson’s, or in anyone else’s.

I can pout, stomp, and shout.

But in the end, it doesn’t change anything.

And when my tantrum is over, that truth is still there:

Thy will be done.

Faith in the Hardest Moments

Some days, I can’t see past the struggles.

I suspect all parents of chronically ill children have those days.

I have my share of moments when I dwell.

When people ask, “How are you?”—I rarely tell the truth.

You put on the brave face, smile, and move forward. Because letting people see how broken you feel is harder than pretending you’re okay.

I think the mustard seed verse speaks to those moments.

Not the moments when life is going perfectly, and faith is as big as a football field.

It’s the low days, when faith feels shaken and small, when self-pity creeps in, when unanswered prayers feel overwhelming.

That’s when I need to remember:

As long as I don’t give up, nothing is impossible.

I See the Grace

There are nights when I’m so tired.

The CGM alarm goes off.
I jump out of bed like a machine, navigating to Jackson’s crib by memory.
I try to check his sugar in the dark, half-asleep.
I fumble with the test strip, get an error, start over.
All while trying to be a stealth ninja, not waking him up.

This is my normal.

But in the morning, when I drag my tired feet into his room, God’s grace smacks me across the face.

And I see it.

I see the grace in:

  • His eyes, full of joy and resilience.
  • His hugs, wrapping around me like a lifeline.
  • His laughter, echoing through the house.
  • The way he smiles at a nurse, even after she’s held him down for another IV.
  • The way he makes strangers smile, just by looking at them.

Jackson has taught me so much about life.

His struggles teach lessons to everyone around him.

He may not change the entire world, but he changes the world around him.

Faith, Even When It’s Small

I know my disagreements with God aren’t over.

This journey is long.

The only certainty is that God is in control.

And on my worst days, when my faith is so small it can hardly be seen, I will keep believing that all things are possible.

And I will remind myself of the beautiful grace He has given me.

Grace I fall short of deserving—but still receive, every single day.

“Not Medically Necessary”

/ Renee J / Leave a comment

Denied

I have a love/hate relationship with our insurance provider.

We have private insurance through my husband’s employer, and without it, we’d be well over $750K in debt. So in many ways, I am incredibly grateful for it.

But it’s not free.

Between premiums, deductibles, and out-of-pocket maxes, we are drowning.

So when the dreaded package arrives in the mail, informing us:

“This claim is denied. After our review, it has been determined this procedure was not medically necessary.”

It takes the wind out of our sails.

17492832_10155042694115502_762686812060261926_oA Simple Procedure, A Complex Condition

Last month, Jackson needed ear tubes—a routine, 15-minute procedure.

Not a big deal, right?

For most kids, no.

For a child with Congenital Hyperinsulinism, it’s not that simple.

The procedure required general anesthesia, which meant he had to fast from midnight before surgery.

And that’s the problem.

HI kids can’t fast for long periods. Their bodies require constant carbs to prevent dangerous hypoglycemia. Jackson typically needs to eat every 2.5–3 hours—even overnight.

Working with Texas Children’s Hospital ENT and Endocrinology, we made a plan:

  1. He was admitted the night before the procedure.
  2. He had a full dinner, then was placed on a dextrose (sugar) drip.
  3. For the first time in his life, he slept for nearly eight hours straight!

His blood sugar remained stable, and surgery went off without a hitch.

We were discharged 45 minutes later.

The stay and surgery? A complete success.

The Dreaded Envelope

Then, this week—it arrived.

The thick envelope from the insurance company.

By now, we know what it means:

  • An Explanation of Benefits (EOB)
  • An appeals package
  • Another denial

This time? The hospital stay was denied.

“Not Medically Necessary.”

I’ve appealed before. It’s not that I can’t do it.

It’s that I shouldn’t have to.

They have all of his medical records. They know his condition. They know fasting is life-threatening for him.

Yet, every time, I have to send the same information, over and over again.

It’s exhausting.

But I’ll do it.

Because now, a new fight begins—and the fear of assuming $4,000 more in medical debt hangs over our heads.

Insurance Denies – CGM

/ Renee J / Leave a comment

Jackson’s endocrinologist requested a Dexcom G5 Continuous Glucose Monitor, warning us that insurance would likely deny it.

I seriously thought, there’s no way. How could they deny a device that could literally save his life?

Since Jackson’s birth, I’ve spoken to our insurance company many times—and honestly, they’ve always been pleasant to deal with. No issues at all.

So when they denied the claim—even after prior authorization—I was shocked.

Yes, I had been warned, but they had been so easy to work with before. I expected more from them.

The Call

I decided to call and talk to them.

Surely, if they understood Hyperinsulinism, they wouldn’t deny it, right?

Wrong.

Their reason?

“BECAUSE PATIENT DOES NOT HAVE TYPE 1 DIABETES, THIS DEVICE IS NOT MEDICALLY NECESSARY.”

Deep breaths.

So I calmly explained:
✔ He doesn’t have diabetes but has a disease that affects his glucose levels.
✔ He is severely hypoglycemic.
✔ If his blood sugar 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 feels unwell. This machine would communicate for him.
✔ This machine isn’t just for diabetics—it’s for anyone who needs 24/7 blood glucose monitoring.

Still not enough justification.

The Appeal

In desperation, I submitted an appeal, explaining everything I knew about HI and begging them to reconsider.

Julie from Congenital Hyperinsulinism International also wrote a letter, urging them to look at the facts.

The truth is, if Jackson suffers brain damage, the long-term medical costs will far exceed the cost of this device.

It’s the right thing to do—and it would save them money in the long run.

They said they’d make a determination in 30 days.

So… we wait.