The HIlife can be a rollercoaster

Update on our little superhero:
We’ve recently had to increase his diazoxide dose to the highest amount since diagnosis, 12.3mg/kg. For almost two weeks he has become more unstable than he’s been since he was discharged in September. Oddly, only in the mornings/early afternoons. Some days in the 40s, but mostly low 50s/60s. 60s aren’t really too bad and not such a concern. But 40s and low 50s are a sign that something has clearly changed, especially when it happens within 45 minutes of eating. We don’t know why the sudden change. Once he reaches 15mg/kg of medication, we will have no choice but to seek other forms of treatment which include; injectable medication, feeding tube, or after we exhaust all other options, a near total pancreatectomy. Hopefully it doesn’t get that far, that would be the last thing that we’d want, but if he continues his pattern, we may have no choice. Before any of that, Dr. Paul wants him admitted back to Texas Children’s for closer observation, labs, and controlled diet, testing various meal types. If things don’t improve over the weekend, I suspect he’ll be readmitted next week. Despite all that, this kid is a trooper. You’d never know by looking at him that he’s so sick. Which is scary for those watching him, but I’m grateful that at such a young age, he enjoys life so much. ❤

On Wednesday, we had our genetics appointment with Dr. Craigen. We have one more test to run called Global MAPS. This test will look for biochemical abnormalities in his plasma. If that is inconclusive, our final step in the genetic discovery will be applying to be in the Undiagnosed Disease Network (UDN). There is no guarantee that we’ll be accepted but we have a passionate endocrinologist with a strong genetics background championing Jackson’s case to the board. Dr. Nadia Merchant will present his case and will work to forward not only his case, but all of those with undiagnosed genetics suffering from Hyperinsulinism. I think she wants this as much as we do. One bonus is that Baylor College of Medicine is one of two sequencing cores, meaning there are no travel expenses for us to participate in the program. Please pray that Jackson’s case gets approved. It may not change his prognosis but it could lead to great advancements in the Hyperinsulinism world. We really want this.

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.

The Power of a Tribe

From a fellow blogger and friend, Dani.

Be Well: Mind-Body-Spirit

I love quotes. There are so many really good, meaningful quotes about the importance of community. “It takes a village to raise a child.” “We rise by lifting others.””Alone we can do so little; together we can do so much.” “Your vibe attracts your tribe.” Community is not just important, but valuable to the human experience. If I believe one thing only about people, it is that we are powerful when we come together for a common cause. You can see it in volunteerism, in disaster recovery… or in my case, in making life a little easier for a mama with her baby in the hospital.

I’m a part of this amazing community. Most of us have never met each other. We have every spectrum of income, education, politics, and religion. Just about every style of parenting, relationship, career and lifestyle can be found in our group. We are all…

View original post 904 more words

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.

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.