We have been home from our second hospital admission for about 6 days now. Drake had to be admitted, twice in two weeks, due to complications from two pretty mean respiratory viruses, and him not being able to maintain his own oxygen. Weeks prior to the viruses Drake has been in a very poor state: keto acidosis from an accidental ketogenic state, seizures at an all time high due to his glycine levels getting extremely high, and then dipping too low due to a sudden weight loss, and then while in the hospital fighting the viruses his nutrition started lacking, and he began showing signs of muscle wasting due to the nutritional deficit. To say these past six weeks have been the most challenging six weeks would be an understatement.
Eric and I have learned to keep our minds protected and keep our eyes on Jesus, when going through extremely taxing hours that turn into days and months…. it is so easy for the devil to attack then, because physical exhaustion soon aids in the mental exhaustion. We have learned, two years in now with Drake, to be every so mindful to guard our thoughts, make sure we are spending extra time with the Lord, and find different scriptures and songs that bring focus and purpose to the suffering and nail them to our foreheads…because we simply have to. This is not a battle of just physical aspects with Drake…so much is fought spiritually.
The devil doesn’t want Drake….he wants our joy.
Right now we are in a really uncertain, but purely amazing place. I am going to share all the details with you as you have truly walked this hard road with us and Drake.
Drake right now is DOING AMAZING…BETTER THAN WE HAVE EVER SEEN HIM.
But why? This is where God really shines through….. you ready to be blown away?
Drake had to be admitted to the hospital for a second time due to a returning fever and a blood sample that showed his white blood count had went from 8,000 to 32,000. Our doctors were all very concerned that our viral infections had turned to bacterial, and we had to get ahead of it quickly as Drake was extremely weak and depleted. We went back into the hospital to be closely monitored and also to start an aggressive IV form of antibiotics. We stated cultures, drew 15 cc of blood, and ran multiple, multiple panels to make sure we knew what was going on inside Drake’s little body. The Thursday before Drake went back into the hospital for the second admission, he was seizing, once again, every fifteen minutes due to his glycine getting high. The muscle wasting was causing his body to break down his own tissues for the lacking nutritional needs, and that was releasing more glycine into his body. Due to his disease, NKH, he can’t not break down glycine effectively so it builds, floods his brain, and causes relentless seizures that WILL NOT STOP until we get his glycine under 400.
We got Drake on the IV antibiotic, and also made an adjustment to his “medicine” to lower his glycine. We have to be careful with that “medicine” as it causes so much trouble in an already broken body. Drake started to sleep Saturday night after we got admitted, and we prayed it was due to his glycine level coming down, or him being purely exhausted. We were able to also get glycine levels drawn Saturday night as well, and we would have to wait until Monday to send them out. I had packed a cooler full of vegetable/fruit juice that we had prepared right before we came in. Again, trying to get Drake’s nutrition in the hospital setting is challenging as so much of the food we give him is made fresh, and has to be pureed to go through his g-tube.
This past Sunday, we started getting Drake’s labs back and everything was coming back in range. The fever had went away on it own with no medicine, and the culture for the bacterial growth was reading negative at the 24 hour mark. Drake was still having about 9 to 10 seizures but overall, he was sleeping and 15 seizures, and under, a day is a good day. With the blood, and cultures coming back normal, the talks of us being able to go and manage him at home started. Our metabolic team was monitoring his nutrition very closely as we had been on the phone discussing calories, and protein needs, etc. We decided the best for Drake, until we understood what happened to cause his white blood county to spike, was to keep him on a antibiotic and continue monitoring him. We went with a higher dose to make sure we were targeting his lungs, as those would be our concern if there was bacteria in there the culture may not show with out a tissue biopsy.
We brought him home, weak and gray, on Monday afternoon. We have been doing shaker vest treatments Q4, and all the other maintenance care and medicine needs to try to keep him protected and healing. We keep extensive journals of every medicine, seizure, food, urine acidity, temperature, blood drawn for Drake and have since he was about a week old. It helps us correlate symptoms to the blood levels that take sometimes a week to get back. As we were filling out our journal, we noticed that we had not recorded seizure activity in two days. We all were discussing ( Eric, myself, and Pam- Eric’s mother who helps us care for Drake), and we had not SEEN ANY SEIZURES IN ALMOST 48 HOURS!!! But how can this be? Drake has seized every day of his life, since he was 5 or 6 days old. The lowest number of seizures we have ever seen was five in a 24 hour period back when he was under six months old, and that is considered the diseases' “calm” period. He was currently at 15 and under for total seizures in a 24 hour period.
So, you know me, I started researching the only two things we had added to his diet… an antibiotic and a probiotic he had been on in the past. And miraculously I found MANY published research articles that stated that the “beta-lactams” (active ingredient in the antibiotic) could IN FACT INCREASE OR CAUSE SEIZURE ACTIVITY in a neurotypical brain. So, that gave me the answers I needed as 1.) this medicine can make it past the blood brain barrier into the brain, and 2.) if it could alter someone’s normal brain activity so much so to cause a seizure, that it could be possibly be balancing Drake’s abnormally functioning brain to clam it.
I immediately reached out to our friend who is a pharmacists at the hospital and started talking about compounds and the differences between the two antibiotics Drake had been on (IV and by mouth), I wrote a PhD friend that has been closely following Drake and his disease to discuss our findings, I also wrote two of our NKH researchers (ND and the UK researcher) to talk about what we are seeing, the medicine, and Drake’s current glycine level.
OH, OH, that is another thing… we drew blood on Thursday night to send to the lab Friday morning. All this research we were finding is very promising but THEN, I though maybe we are seeing the reprieve in seizures because Drake’s glycine is just in a really, really good spot… YOU GUYS… our metabolic doctor went to the lab on Saturday morning to check our level for us….DRAKE’S GLYCINE IS CURRENTLY UP NEAR 600! The child should be seizing NON STOP RIGHT NOW, but instead he is a cool as a cucumber and awake and aware!
We are currently are fervently trying to make notes, and adjustments, look at the effects of long term antibiotic use, and how to balance his gut to allow him to stay on it until we can figure out what and why it is working so well. Things can always shift, his body could not tolerate long periods of antibiotics, and honestly we have no idea what it can be doing in his diseased body, due to the research not yet testing antibiotics in the NKH mouse models…..But DEAR LORD… it is truly amazing to see him!! And regardless of our fear, and all the unknowns….. we are PRAISING GOD for giving us this new direction, and to see how “healthy” Drake looks right now is truly miraculous. He is up and moving, AND he has gained right at a half a pound just this week from being home from the hospital.
THANK YOU JESUS for renewing our hope and strength in what has felt like a spiraling down few months. Thank you all for praying so passionately on Drake’s behalf as God hears our prayers. Please continue to pray that we can figure out what and why this is working for Drake so that we can get it onto paper to help many other children suffering with NKH.
TO GOD BE THE GLORY WHO GIVES NOT AS THIS WORLD GIVES…. HE IS ABLE AND WE ARE SO HUMBLED.
All our love,
Eric, Tarah, littles and Drake
Ephesians 3:20 Now to him who is able to do far more abundantly than all that we ask or think, according to the power at work within us, to him be glory in the church and in Christ Jesus throughout all generations, forever and ever. Amen.