RareJul

In the last 18 months we have learned more about the medical field than any of us had ever dreamed about!

We were talking about how last June when Juliet had to have an NG tube for feeding how hard we thought that was going to be, if we had only known then what we do now of how easy that step would be.  It’s crazy how we can pick up on facts and procedures when we are so heavily vested in something, that we had never before even had an interest in.

We met a new family today that has been in a very familer (to us) situation, and just meeting and talking to them we had a instant bond, they were talking about things that just a few years ago probably had no idea what 95% of it was.  And I just thought to myself on how we talk to people and use all these medical terms now that most people have no idea what they mean and they are now just second nature. Please pray for “Sweet AlexusJo” when you get a moment! And her beautiful family.

When we started this journey we were only aware of 21 people ever having been diagnosed with Vici Syndrome.  That number has now grown to around 50, several diagnosed since Juliet.  We have also noticed there are similarities to other more common diseases, one being CF (Cystic Fibrosis).  A lot of respirotory and pancreatic issues, as well as several of the same treatments.  Vici still being such a new and unknown to most in the medical field we feel there will be several more Vici diagnoses in the coming years and we already know of one or two in Nebraska but have not been able to contact the family to this point.

The exciting news is there is starting to become a Vici Family of several other Vici kids and we have begun to share info to help us info our families, doctors, and others about the cares and treatments for our Vici kids.

My oh my! Talk about slow days!

We are very slow at posting.
So we are checking IN.

Juliet continues to go IN the hospital and OUT of the hospital. July was a major UTI and kidney infection treated IN Omaha.

Recently, we were able to battle another virus close to home at Good Samaritan Hospital in Kearney. She was let OUT, within a week! Only to be rushed IN to the ED by mom and dad 30hrs laters! Bacteria infection. OUT a week later.

Summer has moved faster than we have wanted. We are clinging to each day, of warm bright sun. Hoping our emotions will settle and check back IN. Since February, our hearts and emotions have slowly began to check OUT. Due to overwhelming stays IN the PICU, repeated intubations, very critical and tramatic situations, hard talks, exhaustion, confusion, family seperated by distance, uplifting moments, closeness, hope, dashed hope, love given, Friends………IN OUT IN OUT IN OUT ….heart mind body soul.

IN our hearts we are weary. But we are living on lived OUT love! Juliet is still a “Jul” IN the rough.

P.S. Juliet got a new chair.

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Continue reading In and Out!

SLOW

thats the way Juliet likes it! This has become a humorus statement around the medical team!

The Doctors, one in particular, say it with quite a chuckle! Some just shake their heads.

Juliet will give hints. And after awhilllllllle, if you haven’t figured it out, she will give one big one! “This is what’s up, guys.”

Rounds with the team: “just taking it slow with her today, nothing new. Not going to change anything.”

Slowly she is improving! After 19 days on the vent she was stable enough to take her off of it.

Today, On day 28 of This admission, she is still in the PICU here at Children’s. She is working slowly to kick the issues with withdrawal from sedation drugs. Once she conquers that then; she will move on to weening the oxygen support.

One thing at a time. Again! that’s the way she likes it.

When LIFE throws you into the trenches, you begin to dust and dig and get down and get dirty to survive. You search and look.

In those rough and gritty ruins you search for beauty. Hope.

Juliet’s journey has thrown us into those trenches. Juliet is our beauty. Our found treasure.

Our search is not over though. We keep searching for pieces of treasure,-hope given, love shown, laughter, encouragement, to add the beauty she is. We keep finding these pieces and clinging to them.

*you have to get dirty in the ruins to find the treasure within it-a RareJul.

Last Tuesday we took Juliet into our pediatrician, we were then sent on to Omaha, this time just for a short stay (2-4 days) or so we thought, Juliet always has her own ways of being defiant.  Within hours of being at children’s, she was moved down from fourth floor to PICU, spent the day in PICU, then that night wanted to keep the medical team up on there training and gave them a workout for a few hours.  They were great and got her stable.  At this point she is still in PICU and has been sedated for a few days, she is slowly doing better with the hopes of getting off of the vent tube in next couple days, but as we all know, Juliet sets her own schedule.

We have been receiving overwhelming support from everyone, Juliet has been in three papers recently and a fundraiser is set for this Sat., Feb 28, which is also, International Rare Disease Day.  It is very humbling how many people truly care!

On a side note, there are posters all over the hospital on how they were voted best hospital for almost every area of the hospital (which I would not for a minute, disagree), but I think a category they should add is, Best Hospital for Family Support.

We stay at what they call “The Rainbow House”, it’s pretty much a hotel for the immediate families of children that are impatient.  The workers there have always been good, but the last couple days they have risen to the great level.  Today, Feb 22nd was Juliet’s big sister Piper’s Birthday, she turned 6.  We asked if we could use part of the Kitchen area for the party and they agreed to let us, keep in mind we live over 4 hours from Omaha, anytime we asked for something they would just tell me to go enjoy your family we will take care of it, it was very sweet of them to go out of their way for our family.  So for that reason they are getting my new award, even if it’s not official.

With all of our great Dr’s, nurses, and all other healthcare pros, here’s to hoping, we can soon get off of the Beaten Path!

Also, HAPPY BIRTHDAY PIPER!!!

All these links are from my phone so they may only work on mobile devices, I haven’t tested them.

http://m.nptelegraph.com/news/local_news/baby-only-st-case-of-disease-in-world/article_29863cf3-d5b4-5b9b-91e8-2985e47d02c5.html?mode=jqm

http://www.gothenburgtimes.com/index.php?option=com_content&view=article&id=8593:juliets-story&catid=1:local&Itemid=2

Click to access Arnold_Sentinel_2-5-15.pdf

Jailyn’s class is organizing the benefit this coming Sat. Feb, 28 at the Arnold Community Center.  A go fund me has also been set up.

http://www.gofundme.com/n0kxco

As of last Friday we have now got to be impatient at Children’s Hospital seven times in seven months with each stay being at least a week, the longest 2 days shy of a month.  Juliet has taken two chopper rides and one via ambulance, one thing we can say is she has definitely had the best flight attendants in the skies.

We have started to become known at Children’s to the point, Dr’s and Nurse’s will come and visit even if they are not taken care of Juliet at the moment, I guess it’s good to have them on your side when you need them (or all the time).  We have however spent nearly as much time with them as we have at home, so they truly are our second family.

Children’s has a mixed emotion feel every time you’re there, on one hand you have the “My child is getting the best possible care in the best possible facility there is” on the other you have the “I’m in a hospital where it’s never just the common cold”, you pass other parents in the halls or elevator and we all have that same thought or look in our eyes wanting to ask the question, “what is your child here for?”, sometimes you do, but most of the time it’s just understood that no case is better or worse than the other, we all have a sick child and no parent wishes that on anyone.  We wish we could just take it away and make it be us instead of them, at times feeling helpless.

The hardest part of all of these journey’s has been to understand why God chose our little girl to be diagnosed with Vici Syndrome?  Of all of the things that could happen to a child why this?

“Cast your burden to the Lord and he will sustain you – Ps 55:22”

If you want to know more about Vici Syndrome: http://www.nature.com/ejhg/journal/v22/n3/full/ejhg2013142a.html

Here are some resources we are trying to get awareness elevated, Vici is a fairly new Syndrome and not a lot of research has been done, the diagnosing gene was only found a couple of years ago.  We are trying to start the process of helping to raise awareness.

http://www.rarediseaseday.org/

http://www.liftinglila.org/

http://globalgenes.org/world-rare-disease-day/

Juliet Perri was born on the morning of May 15, 2014, she came to us very similar to our older two children.  We had a nice drive in to the hospital, in the middle of the night, just like her siblings, she did hold on a little longer to be born as it was mid-morning.  Her newborn screen was normal except for the dots in her eyes, our pediatrician, wasn’t concerned, but thought that they did not look normal.  Fast forward a 3 weeks and they were a confirmed cataracts, however our Eye Dr. was not worried and just wanted to see us again in 2 months.

Around the 4-5 weeks of age, we noticed that Juliet didn’t seem to be gaining any weight, although she ate, or tried to eat, like any other newborn.  We got a Dr. appointment, and were admitted to the hospital for, “Failure to Thrive”, it was at this point that they did an MRI on Juliet and we learned about her, ACC (Agenesis of the Corpus Callosum).  It was at this point that our real journey began.

On December 11, Juliet was officially diagnosed with Vici Syndrome.

“Vici Syndrome (VS) is an extremely rare multi-system disorder characterized by an absence of corpus callosum (part of the brain), low muscle tone, significant developmental delays, cataracts, heart muscle disease, abnormalities of the immune system, seizures,recurrent severe infections and in some cases loss of hearing. Life expectancy for children with VS varies between ages three through eight.” – liftinglila.org

Doctor’s at Childrens are not familiar with the disease due to it’s rarity, but are in full research mode.

This is the information in our hands at this moments, more background from the day Juliet was born until present will eventually be making it’s way to the blog.