Sugar Bugs

Lancets and Test StripsThey 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.

wehavehihopes toesThis 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.

Safety Fast for Hyperinsulinism

We arrived at Texas Children’s Hospital on Sunday at 7pm to start Jackson’s first Safety Fast since being released from the hospital in September. We knew it was going to be tough and we were as prepared as we could be. We didn’t think Jackson would last longer than eight hours but we really wanted to know.

I’ve read that several parents of HI kids prefer not to do the fast and skip the process. Withholding food and forcing a child into a hypoglycemic state does sound barbaric but we felt it was necessary to make sure his dosage of diazoxide was high enough to sustain him for a considerable amount of time, and to see if there were any changes in his lab results.

We started the fast at 8:30pm. He stayed on schedule with his dosage of diazoxide and was allowed water. He started with a blood glucose level of 81. From the start of the fast until his blood sugar dropped below 70, we checked his sugar every three hours. 70 – 55, every hour. Our goal was to see how low he would go before becoming symptomatic.  Then at that point, administer glucagon, do the glucagon challenge (sugar checks every 15 minutes expecting to see a rise of at least 30 points in an hour), then run labs.

wehavehihopes cgm low.pngJackson’s blood sugar stayed above 70 for 10 hours. We were shocked because that hasn’t been typical for him. He tends to drop below 70 over night but never below 65. We later found that our glucometer is about 15 – 25 points off from the hospitals. Theirs being much more accurate and confirmed by lab results. Jackson went from 70 to below 50 in about 2 hours, then hovered between 44-32 for HOURS.  He never really showed, outwardly, typical symptoms of hypoglycemia. No sweating, clamminess, or shaking. Since he can’t communicate we don’t know what he was feeling on the inside but he was cranky, asking for milk, and overall a bit miserable. His CGM alarm was relentlessly going off. We learned that when your blood sugar drops below 40, you no longer see numbers, the app just shows LOW. After about 3 hours or so, he did lose the color in his face and we immediately stopped the fast, took labs, and administered glucagon. He was still active, playing, laughing between crying, very alert, but there wasn’t a point in pushing him much more. At that point, he had been fasting for 15 hours.

When Jackson has taken the glucagon challenge in the past, his blood sugar always rose as expected – Not this time. It was 34 when the injection was given, @ 15 minutes it was 44, @ 30 minutes it was 38, @ 60 minutes it was 32. I wasn’t expecting this and honestly, didn’t know what it meant. He was immediately given D10 (dextrose) through his IV and was allowed to eat. His blood sugar quickly came back up, all the way to 210! We turned off the dextrose and he stayed between 85-120.

When Dr. Paul returned to go over the the lab results and discuss the glucagon challenge we heard words that we never expected to hear:

  1. Jackson was making ketones! This is something that he hadn’t done in the past which is typical of those with HI.
  2. The glucagon reaction suggested that his liver was distributing glycogen appropriately which is why it didn’t “work”.

These two results plus more things that are over my head, suggested that it is possible that Jackson has TRANSIENT HYPERINSULINISM! Something we did not think was possible at this point. From what I understand, I’m obviously no expert, is that most babies with transient hyperinsulinism are identified within days of birth and it typically resolves within the first 30 days of life but some later in the first year. Seeing as Jackson just celebrated his birthday and wasn’t diagnosed until he was four months old, the odds were not in our favor so we really never considered it. But, now, could it be? Dr. Paul thinks there is plenty of reasons to have HI HOPES.

There are three ways to test this theory:

  1. Let him outgrow his dose of diazoxide to get below 4mg/kg.
  2. Slowly start weening him down.
  3. Admit him back into the hospital for a Cure Fast.

We’ve decided on having him admitted. It’s not that we don’t think we’re capable of doing this at home, it’s that there is an added comfort of being surrounded by a team that is more well versed in this disease than we are. In an emergency, there is an army behind us. We think it’s the best decision to make for Jackson. So in two weeks, we’re going back.

Dr. Paul looked at me yesterday and said “You know, if he has outgrown this, you’re going to go through withdrawals.” He might be right. I have been running 100mph since August. My brain is constantly on 10 things at once, running from doctor to doctor, researching, advocating, running a Facebook page, a website, planning a fundraiser, up all night, constantly checking my phone to see what his CGM is reading, fighting with insurance companies, scared for him, afraid of the unknowns of the disease, that if he no longer has it, we will suddenly be moved into a “normal” life. I don’t even know what that looks like, since it has never been considered a possibility. That said, I’d much rather have a calm life with a healthy kid, than a hectic one with a sick kid.

None of this is a guarantee but there’s hope. I let Jackson sleep in this morning before rushing him off to daycare. Yesterday was rough on him and I knew he needed the rest. When I walked into his room, he jumped up to greet me, and I was overcome with emotions. No matter what the Cure Fast shows next week, I’ll never give up on him and wanting him to have the best life possible. This kid is amazing. He is strong. He is a fighter. He’s is sunshine and rainbows in every single storm we go through. He deserves a life free illness and I want this for him so bad. #wehaveHIhopes

 

 

Dr. Jason Goldberg, UTMB

Dr. Goldberg is a “HI Achiever” that was thrown into the HI world by chance – A perfect storm that brought he and Jackson together. Perhaps some sort of divine intervention.

As you know, Texas Children’s Hospital has been instrumental in the diagnosis and treatment of Jackson’s Hyperinsulinism but our journey started before we landed on their doorstep. With me sitting in Dr. Goldberg’s office, scared, and desperate for help. Not knowing that the new doctor we had to see by default, would become the one to help save his life.

When Jackson had his first seizure and starting acting abnormal, we went to our local emergency room. There, the attending doctor dismissed us.  I now know this happens all of the time with these kids. No one looks at a baby and thinks “I wonder if they are hypoglycemic?”. This doctor made me question my own judgement and made me feel like I was overreacting.  He told me “babies do this all of the time when they’re teething, it’s completely normal”. He even laughed off our concerns. We had such a large age gap between kids it was understandable that we’d be quick to jump the gun. I regret taking his words to heart because Jackson continued to suffer. I became too unsure of myself to get an immediate second opinion. Each day, nothing changed. His temperature was cold, he would zone out, I couldn’t get his attention, he had no energy, and he stopped crying for food. After three more days of this, enough was enough. I needed another opinion.

I called to make a same day appointment with our pediatrician. She was out of the office so we took the first available with Dr. Jason Goldberg, he’s with University of Texas Medical Branch (UTMB). I’ll be honest, I was a little disappointed to have to go to someone we’d never been to before but in that moment, I had to take what we could get. I trusted the pediatrician’s office, I just wasn’t in the mood to deal with someone new. As I was headed to the appointment, I knew I wouldn’t be able to leave without answers. I couldn’t have another doctor dismiss me. I needed someone to listen. We needed help. I had never met Dr. Goldberg before but I went in feeling like I was ready for a battle. Hopefully, I didn’t scare him. In our talks since then, I know I made an impression. He’ll tell you, I was in momma bear mode.

Dr. Goldberg walked in our exam room and introduced himself. Without missing a beat and not giving him time to get another word out, I pounced. Looked him dead in his eyes, more desperate than I have ever been, and said “Something is wrong with my son. Don’t tell me I’m overreacting, I have common sense and I am not going to be dismissed. My son needs help”. As straight forward and slightly rude as I was, he said “Ok. You know him better than anyone. You tell me something’s wrong, then something must be wrong. Start from the beginning”. So I did. I could see it in his eyes, he too knew something wasn’t right. This wasn’t normal. He and I both suspected something neurological may be happening. He told me to get in my car and head straight to Texas Children’s. He said “If they won’t see him, call me, if I have to keep this office open late and get a specialist here, I will do it. Whatever we have to do, we’ll do it. We’ll figure this out”. I took Jackson and we headed straight to TCH, and you know how that story goes. We arrived with his blood sugar at 21, likely moments away from brain damage or possibly death. We arrived just in time.

Dr. Goldberg continued to follow Jackson through a shared system UTMB and Texas Children’s have. He even called us a few times during our three week stay, just to see how we were, if there was anything that he could do to help. See, he’s not just a good doctor, he’s a good person.

Once we were ready for our discharge at TCH, we were told to make thing simpler, we should switch Jackson’s primary care to a TCH doctor. Keep all of his healthcare within one system. That may have made things more transparent but Dr. Goldberg helped save his life. We weren’t going anywhere. We were staying with UTMB, we were staying with Dr. Goldberg. He is the best pediatrician to treat Jackson.

Dr. Goldberg has gone on to research hyperinsulinism, diazoxide, and be as prepared as possible to help keep Jackson healthy and to make educated decisions. He’s done everything he can to make the transition between both systems seamless and no matter how busy he is, he never rushes us. He always looks at the whole picture. He researches. He asks questions. He cares about my son. He cares about my family. I really couldn’t ask for anything more.

There aren’t many doctors like him and we could never repay him for all he’s done to keep Jackson safe and healthy or the sanity he’s given me on my rough days, when I show up in his office stressed, scared, tired, overwhelmed, or even in tears. Dr. Goldberg is who you’d want in charge of your child’s health. My rare kid, has a rare doctor. We’re pretty lucky.

Dr. David Paul, endocrinologist, TCH

Dr. David Paul is my next HI Achiever.Hyperinsulinism Dr Paul TCH

Dr. Paul is Jackson’s endocrinologist at Texas Children’s Hospital (TCH). We met him on day three of our hospital stay. I remember when he came into the room for the first time. He took the lead in the conversation and I stopped him. What is your name and what is your role?  Everything was a whirlwind. Doctors and nurses were in and out like a revolving door. Everything was running together and I couldn’t make sense of all of the possibilities. So when he walked in for rounds that morning, I was ready. I had a pen and paper in my hands and I wrote down everything he said. I wanted to know it all. So many things were new. So many things were unknown. I was overwhelmed and I was scared. My son was sick and I didn’t know why. I refused to Google anything. Told family and friends if they did internet searches to please keep us out of the loop. I couldn’t go down a rabbit hole. I needed facts. Dr. Paul was my main source of information. He was going to figure this out, I trusted that.

The second morning, when Dr. Paul did rounds, this is what he walked into (picture below). I realized the wall could act as a whiteboard. Our PCU nurse, Renee White, gave me a marker, and I was on a mission. You can’t see the other 5+ feet of wall to the left. I wanted to figure this out. I needed to see everything drawn out.

Hyperinsulinism Dr Paul TCH 2

Some doctors would walk in and see my board and chuckle and say it looked like I was cramming for an exam,  some would be impressed, and some didn’t notice. Dr. Paul walked in the room, saw my notes, and went through each line item, one by one, every day. He never rushed me. There were times he’d stop by after his rounds were done and sit with us another hour or more. Answer questions. Discussing research papers / case studies that I read. A doctor that doesn’t rush you? A doctor that cares what you think? A doctor who is willing to engage a mother’s Google doctorate?

I liked Dr. Paul right off the bat. You can see the wheels turning when he’s faced with complex issues. There’s no mistaking his candor or his intelligence. I tried finding information about him online and in the Hyperinsulinism world, but my search came up empty. Not that I need a doctor to have a shelf full of awards in order to be “experienced” enough to treat my kid but I was surprised that he wasn’t more highly decorated online for his achievements. Dr. Paul has been treating HI since the 90’s. He has seen and treated more kids with HI than most endocrinologist in their entire careers. So while you may not see his name in big fancy lights, he’s an unsung hero in the HI world. Certainly a hero in our world.

In the months following our initial hospital stay, Dr. Paul has continued to be a great doctor. He’s visited Jackson during other hospitalizations which weren’t endo related, humored me in answering far too many questions and emails on random thoughts that I have, introduced us to other wonderful doctors, did everything he could and made himself available when we were ambulanced to a hospital that was scared to treat Jackson (they had never had an HI kid), answered text messages when I’ve been scared over the weekend, far more than any doctor in any other capacity would have ever done.

I really can’t imagine having to go through this journey with another doctor. Trust me, we’ve meet a bunch of them. Even other experts. They were all great, fantastic even, but they’re not him. Not only has he been great to Jackson, he pushes me to be a critical thinker and honestly makes me wish that I would have pursued a career in the medical field.

Since August 2016, our lives have been unpredictable. It often feels like we’re all caught up in a tornado, twisting and turning, at an unpredictable pace and the one thing anchoring us down is him. He keeps my anxiety and fears in check, because I know my son is in the best hands possible. I can ask anything that I want, no matter how silly it may seem to me, and he’s right there to answer. Dr. Paul will do whatever he has to do to keep Jackson safe and healthy. I trust him with Jackson’s life. No better feeling in the world than that.