Jack’s Story

Site created on July 13, 2019

Jack the eldest of Da Krakens! He was born at 33 weeks and 6 days and his brother Jesse was out 30 seconds later.  Day 1 he was a fighter.  Both of the boys had to fight and we were more concerned with  Jesse’s health as he was having more issues.  On day 26 in NICU we found out there was something wrong with our son’s brain.  At the time we did not fully know what it was or how this would affect us and him for the years to come.  But Da Krakens got to come home on Halloween.  We learned that he an arachnoid cyst in the choroid plexus of the left ventricle. and we were trying to get to him being 6months old before we had a procedure done.  However; due to the increasing size of the cyst Jack has a fenestration on 1/13/2017 followed up with a shunt install on 3/1/2017, and Revision on 4/21/2017.  There were further complications in July 2018 but we were able to get them solved with no procedures and thought we were in the clear just before their first birthday in 2017!

About a year later jack started having issues again.  In late September 2018 with "episodes" consisting of vomiting, lethargy, paleness, irritability/appears to be in a lot of pain, and bradycardia (numbers dip to the 60's; his normal is about 90 - 113). The first episode, he vomited 13 times overnight, and then went to sleep for 62 hours straight.  Additional hospitalizations that Fall, had him asleep for 48 hours, 54 hours and 24 hours. Because Jack acted normally between these episodes and his cyst had gotten smaller compared to his checkup scans earlier in the year, the neurosurgeon did not think it was due to a shunt malfunction. Thus, we went through extensive genetic testing and a 48-hour EEG to check for seizures. During his 4th hospitalization at the end of Nov, on the 4th day in the hospital, his scans showed increasing fluid in the brain compared to the day he checked in for that stay. That prompted them to do a shunt tap and then a shunt revision because it appeared to be occluded.

Jack was great and normal afterward until Feb 13, 2019 when the episodes started again. This spring & summer, the episodes were shorter in duration, but more frequently occurring.  They went to once a week and then almost daily.  Again, because he got better between episodes and the scans didn't show fluid buildup, the neurosurgeon said it wasn't a shunt/brain issue.  We were then referred to GI.  GI thought it could be cyclical vomiting syndrome (CVS) , so we started medications for that. However, the symptoms persisted. We called the GI doc to see about different medications and he said Jack's extreme pain/irritability was not normal for a cyclical vomiting patient. He felt we needed to revisit the brain, so he called our Neurosurgeon. The surgeon agreed to do an Intracranial Pressure Monitor (ICP) on June 10th. The ICP his numbers hovered in the 30's-50's and spiked as high as 89 (Normal is 0-15). We also saw negative numbers (-7) when he was sitting. Thus, the surgeon changed the shunt valve to an OSV II which he thought would better regulate the pressure. For 2 weeks post-op, Jack was a new kid: he had the stamina, no vomiting, no pain, no issues at all. However, on June 29, he started having symptoms again.

We have created this site to help keep people up to date on Jack as well as possible for our own sanity.  We are not sure how this will evolve or how often update will occur.  Thank you for all of the prayers and well wishes!  

We know that God gave Jack a twin, Jesse, to help him through this and the fact that Jack has hit every milestone is a testament to that!

Newest Update

Journal entry by Jenny Schaudies

I’ll start with the great news: we’ve seen huge improvements after Jack started on the cortisol supplements! We are thrilled! Not only have his episodes subsided but he has more stamina than he used too. A few weeks ago I baked cupcakes with the boys after dinner one night ... that small act would have been impossible months ago as his grandfather pointed out because Jack almost always tanked in the evenings. Even getting to eat dinner as a family is a gift we now get to enjoy. We have even been able to increase our outings and join in with some social activities. Yay! We are so grateful for our doctors we have now! There are times when we increase his dose because of activities that would likely trigger him in the past and he has handled it beautifully! We go back to Vanderbilt in a few weeks to follow up with the endocrinologist. 

I’ve become a stay at home mom to better accommodate Jack’s needs and spend more time with the boys while they are little. I get to enjoy being with them and it makes the logistics a lot easier. I’m so grateful to everyone who has made this possible. I absolutely loved my work team and was there for nearly 10 years. They have been so supportive all along and I will miss them greatly. They have always been such amazing friends and a second family for us. 

Today we had a follow up with Neurology. We were running late and as we rushed into his office I had completely forgotten that we were due to discuss the results from the mitochondrial testing they collected samples from us for way back in October (apparently it takes a really long time to run tests on your DNA). So when he pointed to papers on his desk and said we needed to discuss them,  my heart sank. They would have called if it was all clear. Back in October as we were leaving the office after collecting the cheek swabs the doctor said to us: “Pray that it’s just migraines. You don’t want it to be a mitochondrial mutation.” Super. I managed to stay away from google for about a month after that until curiosity got the best of me. What I found was really scary but by then Jack was starting to turn the corner. Then come December when the cortisol started kicking in and he was really improving we pretty much just assumed we had found all the root causes and I wrote off the mitochondrial stuff...until today. I will try to convey what I understand of the results but please forgive my limited knowledge. He does indeed have a mutation in the gene the neurologist was concerned about (the MT-ATP6 gene). However, it doesn’t seem to be severe based on how he is functioning (and maybe the level of variation? I’m a little fuzzy there since my adrenaline was so high when trying to have this conversation while also trying to keep two toddlers happy). The testing revealed I too have this mutation but I have been asymptomatic. Good news!  From here, it gets really “grey”...what does this mean for the future? We don’t know. Could this be the root cause of his episodes? Maybe. Or could be the migrane + adrenal deficiency. Or a combo. No concrete way to know. Could he get worse down the road or could I develop symptoms at some point? Maybe... maybe not. What do we do about this? There’s nothing they can do. Apparently very little is know about this. The doctor said based on my lack of symptoms and how well Jack is functioning for someone with this, the long term outlook is “probably fine”. That’s good news, but my pessimistic side goes to our history of hearing “it will be fine”, and then it not being so fine. So, I’ve called his geneticist at Emory to try to get an appointment to discuss further in depth but was told the earliest appointment was in September (I had to ask twice to be sure I heard that right); I put his name on a wait list in case of cancellations and am going to keep trying to get in sooner. For now, we just try to stay positive and focus on Jack greatly improving. Maybe for he and I this will be no big deal. I’m a big worry wort by nature though so I'm not the best at not stressing. And in my experince, when the doctor asks you multiple times if you are okay and then hugs you before you leave the office, things aren’t that great. I’m going to have a big margarita and what my work buddies call “shitty day queso” tonight, and then try to refocus tomorrow, keep looking forward and stay grateful for the good days we are having. I know all things are in His time and His plan so I will try to get better at resting in that. 

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