Drake is struggling a bit. His seizures have been coming about every 10-15 minutes all day. I will take him to our pediatrician tomorrow, for his follow up appointment, to make sure nothing is settling in his chest. It is a bit harder to balance his needs with all our other littles, and the normal demands of the house, but we are thankful to have our babies back together. Today it took myself, Eric, and three grandparents just to get everybody and everything done that needed to be done. Eric and I are a bit sleep deprived, but Eric’s mom is coming tonight to stay up with Drake so we can try to sleep more consecutive hours.
We got our glycine levels back that we drew right before we left the hospital, and for some reason they had jumped to high 400’s. For Drake, he needs to stay between 350-400 to really thrive. With all the changes and things pulling at his little system, coupled by 24-48 hour turn around time for gettin labs, it has been a HUGE fight these past few months keeping him in this healthy range. Currently, due to the toil the viruses have taken on his body, and all the throwing up the “medicine” and mucus has caused during hospitalization… he is struggling nutritionally. His amino acid panel is all over showing it is depressed. Drake needs protein to balance out all of these numbers, but the only problem is protein has large amounts of glycine in it. And, again, due to lack of funding for NKH, their is no nutritional guidance for Drake to follow as it does not exist yet. Making times like this, extremely difficult to move forward due to a very small window of error.
Glycine is an essential amino acid. 80% is made by your body, and 20% of your intake comes from food sources. Meats, cheese, beans, lentils, asparagus, avocados…. a lot of things contain glycine in high levels… it is good for you… again it is essential for your body and brain to grow. But, due to Drake’s disease, NKH, his body is thought to have zero ability to break glycine down. His system that breaks it down (Glycine Cleavage System) is wiped out, almost completely, by defects in his genes. Due to this, Drake not only deals with his glycine being extremely high and causing all the seizures, but his body lives in a state of starvation due to the lack of glycine being able to be used. Drake’s system is broken at the very first step of break down… so by default, all other steps of being able to use it’s parts are completely missed.
That is why…. the children as severe as Drake…have very short life expectancy. Their little bodies hang on as long as they can….until the disease over comes it, or sickness takes them.
Please pray for Drake and for healing. We are doing constant measurements, monitoring, chest percussion therapy almost every two hours, and extremely detailed measurements for every ounce of food given to him. We are stilling trying to do daily blood draws for glycine. I tried this morning and was unsuccessful, which hurts my heart to stick him and not get blood. His little veins are just so blown from being stuck multiple times from the hospital stay, and all the continuous labs. We have a good friend coming tonight to try to get blood, we have to know what his numbers are doing, and why our seizures are so elevated. If we could do a finger stick, or heal prick, or something that required less blood to test, we wouldn’t have to have a butterfly needle/vein every time to get one glycine reading.
Every time I hold him down to get blood drawn, or beg God to calm my shaking hands as I try to draw it, I am reminded why we push ourselves to the point of exhaustion for better treatment and monitoring for NKH. Please continue to pray with us. God knows the needs and the people that we need to connect with to make this happen, and we pray hourly He gives us His wisdom and strength to follow every lead, and to not let any detail slip past us.
Thank you for loving us, thank you all that have reached out on Drake’s behalf, and all that are helping us think of tangible ways to move this disease forward. I truly believe if we do not give up, God will honor our efforts… and our boy will know healing and childhood, and will be a beacon of hope for others fighting the “impossibles”.
We love you all, Eric, Tarah, littles, and Drake
Galatians 6:9 “And let us not grow weary of doing good, for in due season we will reap, if we do not give up.”