We took Vivian into the ER Wednesday night to have her evaluated. Her body was on day three of fever, hundreds of seizures, and throwing up. We had tried everything we normally do to get her system to reset, but nothing was bringing relief and she desperately needed to rest.
Eric took her in at our local hospital that knows the children. Although we know a lot of the nurses, and respiratory therapist from our visits, we always have to pray that God will bring the right physician on call. Having a disease that causes hundreds of seizures, coupled with no treatment options, quickly can become hard to navigate safely. We bring all our notebooks, disease knowledge, and research and try to quickly explain what the children are experiencing. We have to try to explain to the specialist the last 6 years of discovery and research within about a 3 minute window. It is imperative that we are all on the same page, as if we give the wrong medicine, it can actually be a fatal mistake for the children due to the metabolic state and little known about their disease. The reason that we know some seizure medicines can be lethal, is from parents of other children with NKH that had to learn that information the hard way.
The ER agreed to run diagnostic labs to try to help us navigate why Vivian had taken such a hard downward turn. We ran blood cultures, blood gases, CBC with differential, comprehensive metabolic panel (CMP), magnesium, manual differential reflexed, phosphorus, procalcitonin, respiratory pathogen panel, urinalysis with reflex microscopic, amino acids, chest and abdomen X-rays, if we could test it we ran it....Vivian did amazing while she was poked and stuck all over. I hate the endless labs, but it is the necessary evil to give us insight into what is happening in her body. Her poor little arms already had bruises from the infusion she received just last Friday.
As we waited for the panels to result, praying they would give some insight, Eric sat with Vivian as I navigated getting the rest of the house in bed and Drake’s nighttime meds made. He would call each time the attending would round so we could go over the labs as they resulted.
One after the other came back...
...clear...
...clear...
...negative...
...elevated by one point...
...no significant findings...
...clear...
...in range...
...negative...
...negative...
...negative........
Nothing.
After 8 hours in the ER and three days of seizures and vomiting every 5 mins....we found nothing....nothing substantially raised to explain why Vivian had suffered the way she has.
With nothing there to guide the next steps and no medicines available to treat her disease....we loaded her back up as there was nothing else they could do for her. Her body was pale and limp from the exhaustion. Her arms hang like a rag doll, and you have to be careful to hold her head so it doesn’t fall. Her little purple eyelids almost violet now from all the disfunction happening in her body. We all agreed that she would rest better at home verses the hospital, and as long as she was sustaining her airways with oxygen support, we would continue to care for her ourselves.
The attending physician apologized that there was nothing more they could offer and I truly can’t imagine what that feels like for a doctor to send families home...suffering....with nothing. The attending kept telling Eric how fascinating our children’s story was as Eric was explaining all we had learned, and the research we were leading at the foundation. It truly is miraculous all God is doing through the work and your support.
I had already fallen asleep when Eric got home with Vivian. He got her set up with all her monitors and oxygen and took his post with her downstairs. He popped in a frozen kids pizza as he had not eaten all afternoon from not leaving her side at the hospital. I woke up around 6 am and went downstairs to get Birk ready for Co-op, and check on the kids. Vivian and Drake’s medicines begin at 6 am so I took the baskets of meds and food into their rooms, so Eric could sleep a few more minutes as he was up most of the night.
Feeling the heaviness of another day...the weight of the “unknowns”. The weight of having to "find the answers” ourselves with a disease that is just brutal and never plays fair....it was just that...heavy. I want to be angry and go tell someone how unfair this is...go sit at someone’s office and refuse to leave until they listen to me and finally help us do something to stop this for these children. Rare should not mean disposable. Rare should not mean not valuable. I wanted to turn my eyes to Father and ask Him....WHY?.....why so hard Lord?....why “nothing" last night to offer bread crumbs to inch us forward?...
As the tears burned down my face, I rounded the bedroom door and saw Vivian’s bed lit up in the most unique colors. Her
whole bed almost looked bright like a rainbow. It took we a minute to figure out where the light was coming from as the children's bedroom was pitch black and this early the sun was not up yet. The closet light was on to help me see my foot steps but nothing that would have reflected the rainbow I was seeing. I finally found the reflection was coming from the pulse ox machine. We position it in a way that the baby monitor can read the numbers when we have to step out of the room. The faint gloss on the wood of the crib allowed just enough backdrop to span the red and green into the beautiful rainbow I was seeing.
Isn’t that just like Father....to take the very thing you think is killing you to create something beautiful for His glory. All in a moment God gathered me and whispered like He always does. He is faithful and He has not left me with nothing, quiet the opposite. He has given me a list of things it “was not” last night, so that our team can take Vivian and Drake's days of medicine/food/weights/labs and journals to try to figure out what it was...
Many days I simple don’t know why God chose us...I wish I could be mad at Him for asking us to carry such a heavy load, but I can’t even do that, as deep down I know He is doing something far greater than our family...far greater than anything I can dream up for Drake and Vivian.
Please pray with us that Father reveals in their journals the purpose of the last three days and what He is trying to show us. We constantly beg Him for direction in treatment, and it rarely comes in the neat package we had thought it was coming. Pray we have eyes to see His direction as their journals often set in motion the directions for our research.
Pray for Vivian’s strength as her seizures have slowed to only clustering now verses consistent activity. Her body needs to rest and heal so we can start to rebuild what has been broken. Pray for the research for NKH. Just next month we will get to see the fruit of a project that we have been working and funding for the past three years. Three research institutions, stretched across half the country working to push the project to this date. To say there is so much hope and fear all rolled into one trial period is an understatement. I hope to update with astounding results soon as it would be a curative approach for our disease.
Also, thank you for the donations that have flooded in. We have been praying about a project we needed to take on and you all have steadied our feet that God will bring the funding and we need to step forward.
Thank you for the hope God brings through your support.
We love you dearly.
To God be the Glory,
Amen.
If you would like to make a donation to the Drake Rayden Foundation, a 501(c)3, to help continue the research for rare diseases you read about in this post please click here.