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

 

 

I See the Grace

11141172_10153165086870502_3955407104832353909_nTwo years ago, I posted this picture on my Facebook page. It’s an excerpt from Matthew 17:20. “If you have faith as small as a mustard seed, nothing will be impossible for you”. I posted this as I clung to hope for an impending miscarriage. The whole verse is: “Because you have so little faith. Truly I tell you, if you have faith as small as a mustard seed, you can say to this mountain, ‘Move from here to there,’ and it will move. Nothing will be impossible for you.” In that moment of my life, I thought I could will a miracle and until the miscarriage was over, I held on to hope.

Now, here I am, at His mercy trying to will a cure for Jackson. My friend, Rebecca Smith, introduced me to a song by Hillary Scott – Thy Will Be Done. It’s a beautiful song that was written when Mrs. Scott suffered the loss of her pregnancy.

As a Christian, I wish that I had unwavering faith ALL of the time but I don’t. I’m flawed and human, and I probably challenge God way more than I should. I get angry with Him, sometimes I even give Him the silent treatment, I feel resentful, unheard, ignored, and I want to be in control.  There are moments when quite frankly, I’m pissed. I know, I know, you aren’t supposed to be mad at God and if you are, you probably shouldn’t say it out loud or admit to it. I know He hears me. He knows me, better than anyone. He knows my broken heart. He knows I’m scared for Jackson, for the impact of things on my daughter, for my marriage, to lose the roof over our heads. He knows how fiercely and deeply I love my children, so why them?  I may never know the answers but when I’m starting to feel lost again, I hear those words replay in my head – THY WILL BE DONE. Then I have to remind myself – He is God and I am not. It’s one of those things that, of course, I know but it’s hard to accept that I’m not in control. I don’t get to call the shots in my story, in Jackson’s, or for anyone else. I’ve been given this life and I have to try my best to be a good steward of it. I can pout, stomp, and shout all I want. It’s doesn’t change anything. When I’m done with my tantrum, it’s still there – Thy Will Be Done.

There are some days, here and there, that I can’t see past the struggles. I suspect all parents of chronically ill children have those days. I have my fair share of moments in which I dwell. When people ask “How are you?” – you rarely tell them the truth. You put on the brave face and smile so they don’t see how broken you feel.  I think the mustard seed text speaks to those moments. Not the moments when life is going perfectly and my faith is the size of a football field. It’s my low days, when my faith is shaken and diminished, overtaken by self-pity, that it may truly be as small as a mustard seed, that I need to be reminded as long as I don’t give up, nothing is impossible. It’s easy to dwell when everything seems so unfair and you’re surrounded by unanswered prayers. Those days and moments are typically the exceptions rather than the rule but they are a real part of this journey. Not every day is a walk in the park, smelling roses, and chasing butterflies.

There are nights when I am so tired. I hear Jackson cry or his CGM alarm goes off. I jump out of bed like a well oiled machine. I have one eye open, navigating by memory to his crib. Trying to check his sugar in the dark. Half of the time not getting enough blood on the test strip, getting an error on the glucometer, then having to start all over again, all while trying to be a stealth ninja and not wake him. Those nights are my “normal”  But, when it’s time to rise and shine and start our day, I drag my tired feet, walking into his room to pick him up, and out of no where, like a lightening flash sometimes, God’s grace SMACKS me across the face and I see it. I see the grace in my son’s eyes, in his hugs, his resilience, the precious moments spent laughing and tickling him,  the excitement he gets when he catches my attention from across the room, when he smiles at a nurse after she’s been holding him down to put in another IV line, when he makes a stranger smile just by looking at them. I see the grace.

Jackson has taught me so much about life. His struggles teach lessons to everyone around him. He may not change the entire world, but little by little, this kid changes the world around him. I can almost promise you that my disagreements with God are probably not over. We have a long road ahead of us. The only thing that is a certainty, is God is in control and is driving this ship. On my worse days, when my faith is so little it can hardly be seen, I’ll continue to believe that all things are possible and I will remind myself of the beautiful grace He’s given me, which I fall short of deserving so often.

“Not Medically Necessary”

I really have a love/heat relationship with our insurance provider. We have private insurance through my husband’s employer and trust me, without it, we’d be well over $750K in debt. So in many ways, I am so thankful for it but it’s not free. With premiums, deductibles, and maximum out of pocket, we’re drowning. So when we get the dreaded package in the mail, informing us that “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. Denied

Last month, Jackson needed tubes put in his ears. This is a very basic and common procedure that literally takes 15 minutes. Not a big deal, right? Well for someone who has Congenital Hyperinsulinism, it’s not that simple. The procedure requires general anesthesia (being put to sleep), which requires the patient to fast (not eat) starting at midnight before the procedure. There lies the problem. Individuals with HI, typically, can not fast for long periods of time. The nature of their disease requires carbs because they are constantly hypoglycemic. Jackson needs to eat every 2.5 – 3 hours, even over night.

17492832_10155042694115502_762686812060261926_oWorking with the Texas Children’s Hospital ENT and Endo departments – Jackson was admitted to TCH the night before the procedure. He ate a nice healthy dinner and then was put on a dextrose (sugar) drip. His blood sugar remained stable and for the first time in his life, he slept for nearly eight hours straight! He was stable enough for surgery and everything went off without a hitch.  We were discharged within 45 minutes of the surgery. The stay and surgery were a success!

This week, the dreaded thick envelope arrived from the insurance company and we now know that means it’s an EOB (explanation of benefits) along with an appeals package. Each time that thick envelope arrives, we know something else has been denied. I have appealed before and it’s not that I “can’t” do it. It’s that I shouldn’t have to fight with them when they have access to all of his medical records. It’s exhausting sending the same information, time and time again but I will, and the new fight begins and the fear of assuming $4,000 of additional debt hangs over our heads.

Welcome to Holland

In the last several months, I keep coming across an essay titled Welcome to Holland. Some people love it, others not so much, but I think it’s an easy way to put something so Welcomecomplex into an analogy everyone can understand.

Welcome to Holland

By Emily Pearl Kingsley

I am often asked to describe the experience of raising a child with a disability to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It’s like this:

When you’re going to have a baby, it’s like planning a fabulous vacation trip to Italy. You buy a bunch of guidebooks and make your wonderful plans… the Coliseum, Michelangelo’s David, the gondolas of Venice. You may learn some handy phrases in Italian. It’s all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, “Welcome to Holland.”

“Holland?!”, you say. “what do you mean Holland? I signed up for Italy! I’m supposed to be in Italy. All my life, I’ve dreamed of going to Italy!”

The stewardess replies, “There’s been a change in the flight plan. We’ve landed in Holland and it is here you must stay.”

The important thing is that they haven’t taken you to a horrible, disgusting, filthy place full of pestilence, famine and disease. It is just a different place. So, you must go and buy new guidebooks. You must learn a whole new language. You will meet a whole new group of people you would never had met. It is just a different place. It is slower-paced than Italy, less flashy than Italy, but after you have been there while and you catch your breath, you look around and you begin to notice that Holland has windmills, Holland has tulips, Holland even has Rembrants. But everyone you know is busy coming and going from Italy and they’re all bragging about what a wonderful time they had there. And for the rest of your life you will say, “Yes, that’s where I was supposed to go. That is what I had planned.”

The pain of that will never, ever, ever go away because the loss of that dream is a very significant loss. But if you spend your life mourning the fact that you didn’t go to Italy, you may never be free to enjoy the very special, the very lovely things about Holland.

Sometimes I feel like I’m in both places at once and other times I feel like I’m in Greenland so far removed from everyone. The point of the essay is to say life doesn’t always turn out like you expect. I have been a parent for nearly 15 years and it hasn’t always gone as planned but even still, I never expected this journey.

When you’re pregnant everyone asks “What do you want? A boy or a girl?” – I gave the standard answer that most parents give “I don’t care, as long as the baby is healthy”. I said that so many times and of course, I meant it. With genetic testing before pregnancy, high risk appointments every two weeks, more ultrasounds than one could ever hope for, I really thought we would land in “Italy” safe and sound. I never even considered a detour.

I have probably spent too much time mourning the loss of a “typical” childhood for Jackson. I fear his childhood memories will consist of hospital rooms, being hooked up to machines, constant pokes, be woken up every night and forced to eat, and stressed out parents worried about him, about money, and everything that comes with this package – I hate that for him. But, I can’t change where our lives have taken us. I can, however, stop and enjoy all of the beauty that our “Holland” has to offer. I’ve been entrusted with two beautiful children, who love me, and make me a better (albeit exhausted) person. This journey is hard but I promise you, it’s worth it. I just hope we can stay in one spot for awhile, I’m not ready to be a “world traveler”.

Life Can Sure be Sweet

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!

hihopes picture.png

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.

Why we have “HI” hopes

Jackson was diagnosed with Hyperinsulinism (HI) September 2016, when he was four months old. After spending near three weeks at Texas Children’s Hospital in Houston, TX, we are now home and learning to manage this disease. We continue to have “HI” hopes for this disease. For a cure. For answers. For doctors to be able to identify this disease faster. For a long, fulfilling, future for our son and all of those affected by this disease.


https://ghr.nlm.nih.gov/condition/congenital-hyperinsulinism describes HI as: Congenital hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin, which is a hormone that helps control blood sugar levels. People with this condition have frequent episodes of low blood sugar (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, or difficulty feeding. Repeated episodes of low blood sugar increase the risk for serious complications such as breathing difficulties, seizures, intellectual disability, vision loss, brain damage, and coma.

The severity of congenital hyperinsulinism varies widely among affected individuals, even among members of the same family. About 60 percent of infants with this condition experience a hypoglycemic episode within the first month of life. Other affected children develop hypoglycemia by early childhood. Unlike typical episodes of hypoglycemia, which occur most often after periods without food (fasting) or after exercising, episodes of hypoglycemia in people with congenital hyperinsulinism can also occur after eating.

#wehaveHIhopes