I have been writing this post in my head for a while now. I still don’t know how to best put it, so I’ll paste the e-mail I sent to family, with explanations added as needed.
Wednesday, July 11, two days after my “alls well!” post (yes, I’ll probably never post an “alls well!” post again!), Littlest woke up at 2230 with a hypoglycemic seizure. We transported to the hospital, and his blood sugar was at 21 mg/dL. To put this in context, most people manifest fairly severe hypoglycemic symptoms at 50 mg/dL.
Throughout a 24 hour period blood plasma glucose levels are generally maintained between 4-8 mmol/L (72 and 144 mg/dL). Although 3.3 or 3.9 mmol/L (60 or 70 mg/dL) is commonly cited as the lower limit of normal glucose, symptoms of hypoglycemia usually do not occur until 2.8 to 3.0 mmol/L (50 to 54 mg/dL).
Children run lower, where an adult is 7o mg/dL, a child could be 60 mg/dL for that same time period (eg: waking number after overnight fasting).
Thus began a long week of testing. Here’s the e-mail I sent to family, Italics are added notes or explanation:
At 8am Thursday, the Dr. group comes in that is assigned to Littlest: two hospitalists, three residents and a couple of med students. Littlest’s glucose has been wildly unstable, and they try different concentrations of dextrose drips (ending up at 30% dextrose at 30ml/hour), and then they lower the drip and try to get him to eat at 1pm. His blood sugar tanks down to 6 (death level, he shouldn’t have survived), and he has another subclinical seizure, where he’s “blind” (can hear, turn to see what is making noise, and can’t focus/see, so he panics. It was the hardest moments of my life as his mommy to date; knowing he was petrified and there was nothing I could do for him) and scared out of his mind. They gave him an injection of 50% dextrose to bring his numbers up, and he stays in the 20s-30s. This is the levels where most of the population would be groggy/sleepy/etc.
Thursday is a blur of multiple Dr’s coming in and questioning me, being sleep deprived, and trying to explain WHAT exactly had been showing up before the hospital stay. I kept taking ketone strips, and he kept having really high, high ketosis with glucose in his urine, even while having really really low glucose readings on his blood work. Mystery? Rather.
The CT we had on Thursday early AM showed slight cerebral edema, and Dr. Woodward, our wonderful Neurosurgeon, thought that the proximal arm of his ventricular shunt could use a shift in position (it’s apparently pressing up to the right frontal lobe a little bit)… so she scheduled a shunt revision with exploratory surgery for Friday… then we canceled it late Thursday night/Early Friday morning because his seizures were definitely blood sugar related and we figured we didn’t need to do any extra brain surgery. (Plus, he couldn’t tolerate any fasting period.) This cerebral edema is now thought to maybe be caused by persistent hypoglycemia, since he had low glucose readings in March with his cardio-pulmonary failure and resuscitation.
Friday, much of the same, trying to get him to take some feeds by mouth, or at least Gtube without his sugar tanking, trying to wean him off the dextrose drip… I had slept at home on Thursday night, and while Joel was there, he ok’d them putting Littlest on a continuous feed to the gtube at night because he just could.not. handle the bolus (larger feeds at a time) without crashing. The continuous feeds were the only thing that kept Littlest alive once we were able to get the sugar drip off. The sugar was causing some blood sugar spikes, but that was less concerning than the “death level” glucose readings he was giving.
On Friday, some of the tests we had run on draws during his REALLY low crash from Thursday came back, and they were pretty sure it was either/or/and Type I diabetes (DM1=diabetes mellitus 1) or Hyperinsulinemia. The next “low” he had, we gave him a hormone called Glucagon, which tells the kidneys to dump all their glucose into the bloodstream. It worked, and brought his blood levels up to normal. That gave the clarity they needed to diagnose him as Hyperinsulinemia: his body can’t control the levels of insulin in his blood, his pancreas can’t regulate production.
The second Endocrinologist we saw tested him for hypopituitarism. That test came back negative (thankfully!), so it seems to be congenital Hyperinsulinemia, and will probably result down the road in a diabetes diagnosis; once his body has worn out its insulin producing ability. We have him on a medication which suppresses the pancreatic production of insulin, and it works, but he is also still on a 24/7 continuous feed of formula; otherwise he has the crazy high/lows. We aren’t out of the woods, we’re still waiting on some metabolic tests we drew during some lows to come back, and we have a *lot* of consultations with various specialties in the next month.
The weekend, and Mon, Tue, Wed, of the next week were spent trying to get him to a balanced level of food+medication (and observing for side effects of the medication). Thursday, we were discharged, on continuous feed, with medication for him that has to be taken every 8 hours, and we are taking his glucose levels every three hours,
‘round the clock. We have an 8 hour break from 10pm-6am. He was also re-admitted two weeks later to get him off continuous feeds while we also had outpatient surgery to remove his Broviac and put in a Mediport. He is now on four feeds, one every three hours, and continuous feeds at night.
Hyperinsulinemia is his body producing too much insulin (as relating to the glucose in his blood) so the insulin depletes the blood of glucose, causing hypoglycemic conditions. Hypoglycemia is a result of his condition, which is Hyperinsulinemia.
The BIG things about this diagnosis:
- Hypoglycemia is very, very dangerous. It affects the brain directly (whereas hyperglycemia (diabetes) affects the heart/organs more).
- Littlest is an “unaware” hypoglycemic. He doesn’t start to show symptoms of not feeling well until he’s WAY below the level where most people are groggy/losing lucidity or passing out. His lowest level (6) is death-level (a Dr. friend told me that there were bodies in the morgue who had higher levels than that; you just do.not.come.back from that level); and he was “just” seizing/having blindness manifest. (His eyes wouldn’t focus on anything, his brain was shutting unnecessary functions off.) This makes it imperative that ANYONE who watches him is well versed in taking his blood glucose, and knows how to give him his emergency hormone to dump all the glucose in his kidneys into his bloodstream if he’s low enough to warrant it. We give it below 60, if he’s below 70, we call his Dr.; his readings have OFTEN been in the 20s… and that’s coma time. I’ve only had one 70s reading while we’ve been home; and it was corrected through sugar to the mouth.
- Hyperinsulinemia has a higher morbidity rate when it’s congenital than diabetes now does. I’m not sure why, but they drilled this into me. I thought it bore repeating. We are pretty sure he’s had it from birth, it just didn’t manifest until his body had been under enough stress to have it show up.
- We are looking into possible genetic (gene-level mutation, not hereditary) causes for the hyperinsulinemia, and his Spina Bifida, since his neurological involvement/function is more severe than his original lesion/etc. would suggest.
- This is a long-term diagnosis, with the probability of it developing into DM1 or DM2 down the road. DM1 and DM2 variances: DM1, your body doesn’t produce insulin. DM2, your cells can’t process insulin. He is most likely going to be a DM1, since his body is overproducing insulin right now, he may pendulum swing to not producing it at all. Esp. if we have to treat it with the pancreatictomy that is used to treat severe hyperinsulinemia.
While all of the above was going on, Joel was fired from his previous firm. It was a very stressful two weeks with Joel having gotten fired the same day we got an “official diagnosis” of paraplegia for Littlest, then Littlest having this life-threatening condition, and another hospital stay! God is good, as always, and we were fairly quickly able to see where the firing was a GOOD thing… God had better things for Joel, and with people who are more like him than he had at the previous firm.
Thank you all for your love, support and prayers for our family. We have so many wonderful friends who have come alongside us during this time; and really, ever since Littlest was born. People who have watched the older two, family who has come out and spelled me at the hospital/home, church family who bring meals and hugs. We are so appreciative. I know I haven’t been able to express it; I feel like my entire brain is numb 90% of the time, and it only goes into “lucid mode” when there’s an acute situation… but I have been so blessed and encourage by the notes people have sent, the messages on Facebook or my blog, etc. etc. etc.
I don’t know if we’ve actually met, Veronica. But our family prays for Baby Mac every night. And I know you must be a special family for the Lord to have entrusted you with that precious little boy.
You are soooo right, Michele. They are VERY special, and God is so good. I pray God allows you to meet them some day.
Michele, I don’t believe we have met, I think you’ve met Joel, while I was either on bedrest or in “germ-free isolation” with Mac. 🙂 God has given us wondrous amounts of grace to deal with the complications and struggles of parenting a medically fragile child… and He has seen fit to bless Mac with an amazing personality that takes things so well, which almost always shows a cheerful face despite the pain, and which allows him to cope with unimaginable difficulty with joy.
Thanks for the detailed update! We pray for all of you!