Why does time seem to be going by so fast?!

 Not even sure where to begin, have 3 years of updates to fit in. Lots have happened, most importantly back in January of this year we lost access to Julia's life saving medicine Cyclodextrin, partly because the FDA failed to approve it & the pharmaceutical company filed bankruptcy & partly because after 142 lumbar punctures Julia was starting to have back pain after each biweekly LP that stole her independence for 2-3 days. Without the medicine Julia has begun a slow but steady decline that seems to be picking up speed as it goes. It's scary to see your baby girl, yes she's 21 now but she's still my baby,  lose her mind. Her long term memories are still quite in tack, but her short term is fading & she is often confused & can't remember things. Right now she still thinks we are in October & asks almost every day if we're going trick or treating tonight. Sunday in church she told someone it was her birthday & we were having a party after church. Her birthday is in February. So we are on a mission to get her back on the Cyclodextrin. So starting tomorrow we will be flying to Rush medical center in Chicago every month. As a compromise Julia has agreed to receive the medicine via lumbar puncture once a month instead of every 2 weeks.  Please pray for us as we begin this next part of our journey, in some ways it feels like starting all over from scratch. Except this time we know she's receiving a medicine that has a track history of keeping her & many other kids stable. Praying for safe travel & pain free lumbar punctures 💜

Surgery Date

 So a lot of you know for several years now Julia has had to have her drinks thickened to a nectar consistency, thicker than water but not as thick as honey. This is to prevent her from aspirating the liquids (aspirating is when the liquid goes into the lungs instead of down the esophagus & into the stomach where they belong). In patients with NPC the flap @ the back of your throat that your brain is responsible for closing when you drink(to keep liquids out of your lungs) doesn't work properly. Either it doesn't close off the airway in time to keep the liquids out or it doesn't close completely, either way it lets liquids in where they shouldn't be which leads to infection or pneumonia. Thankfully Julia has only had one bout with pneumonia & hopefully it will be her last. She is scheduled to have surgery this Friday November 30th to have a feeding tube (also known as a g-tube) placed. She can still eat whatever she likes by mouth as she is not having trouble with solids currently but all of her liquids will then go through the tube instead of her drinking them. She is nervous about the surgery but has adapted to the idea very well as she always does. I'm so thankful for her sunny outlook & easy adaptability to whatever this disease throws @ her. She is teaching me how to be flexible so I can bend & not break, just like her. She will have her surgery @ Shands in Gainesville & will have to stay several nights before coming home so if anyone wants to come visit let me know, I know she would like that 😊

Next Chapter- 7 years since diagnosis

Hard to believe it but October 7th marked 7 years since Julia was diagnosed with this horrible disease. October is also the 5 year mark for the restart of the trial. While we have experienced a lot of ups & downs in these last 7 years there is still so much to be thankful for. Julia did some on the job training recently @ a local restaurant, she got a $1 tip from a customer during her 2 hour shift & was thrilled. Although she was tired when she came home she loved it. On the other hand I recently was laid off from my  work from home data entry job. It would be very difficult for me to get a job outside the house with Julia's schedule of appointments, not to mention I can't leave her by herself. So we have decide to expand our home based sign business to include custom t-shirts, screen printing & our own brand, Marker 28 Apparel. Please say a prayer for us if you would as we embark on this new venture. Julia & I also just returned from our yearly visit to NIH. She is still mostly stable, I feel like I say this a lot but it's the only way I know to describe her condition. We do see small bits of decline both physically & cognitively but for the most part things haven't changed much with her condition over the last year. The most notable change is with her swallowing. The folks @ NIH do a swallow study on her every year to monitor this. Recently Julia had a case of pneumonia, which we thought was just caused from a virus. Turns out it was aspiration pneumonia. So it was recommended that we immediately begin thickening her drinks to honey consistency  & when we came home find a surgeon to place a feeding tube. Not exactly news we wanted to hear, but in the course of this disease not uncommon or totally unexpected. I'll post more about the surgery after meet with a surgeon & get everything scheduled. One thing in life is always certain, changes happen, good & bad. I've learned if you're not flexible enough to bend you'll end up breaking.

Graduation!!

Totally unsure how this day has crept up on me so quickly. Today Julia received her High School Diploma. I couldn't be more proud of this girl, she continues to amaze me! I can still remember a day a short time ago when I was sitting in the back of a freshman meeting crying as they talked about all the wonderful things that these students would be doing & accomplishing over the next 4 years. I wasn't sure how Julia would be in 4 years, yet here she is, graduated & still trucking along amazingly well. We continue our biweekly trips to Shands & the medicine seems to be keeping her mostly stable. Hoping in the near future for FDA approval of the drug, but time will tell. Thanks so much for reading & God Bless!

New Year Reflections

The start of a new year is usually a time we find ourselves reflecting on the events of the past year & making plans for the year to come. We definitely have a lot to reflect on from the past year & also a lot of upcoming events to plan for during this year. We are so thankful for the trial continuing to be accessible so close to home & that Julia continues to tolerate the treatment so well. We have some amazing doctors @ Shands & have developed a routine that feels like normal to us now. She continues to do well & is looking forward to the Tim Tebow Foundation "Night To Shine" in February. This will be her 2nd time attending & she is very excited. Also I neglected to mention back in 2016 Julia became an Aunt for the 1st time & I became a grandma. It was a very exciting time in our family & now in a little less than a month Julia's sister Rebecka is due again. I can't tell you how thrilled Julia is to be an Aunt, she takes her job very seriously. Julia also has a birthday coming up @ the end of February & it's a BIG one, 18. Some days it seems unreal that we've made it it this far & she still seems to be in such good shape physically. If that's not enough excitement, Julia is also on track to graduate high school in May. She is really looking forward to entering adulthood, she is hoping to work with vocational rehab & do some on the job training after graduation. I wonder how long her enthusiasm for adulthood will last? Knowing her it will last, she continues to amaze me every day & I am so thankful for every single day God chooses to bless us with her :)

Update & changes

Happy Memorial Day Weekend to everyone! Just wanted to give an update since it's been awhile. Julia has had 6 Lp's @ Shands now. It has been so nice to be so close to home. When she's done we get to drive home & she can do her resting in her own bed, she has been especially happy about that. Things haven't been all roses for her though. About a month & 1/2 ago she had an Lp headache afterwards. It got pretty bad, she was vomiting for about 48 hours & just plain miserable for about 4 days. I'm glad that hasn't been a common occurrence, I felt so bad for her. Two weeks later when she had her Lp they hit some scar tissue & had a hard time getting the needle in, it took 3 tries, so afterward she had a backache for a few days. She is a tough kid, not sure I could go through what she goes through & keep such a positive attitude.
I also wanted to let everyone know that Julia recently got a power wheelchair. I've kind of put off posting about it because it seems like such a big change, but I didn't want people to worry if they saw her in it. It's not because she needs it all the time, she hasn't gotten to that point. But there are days that she just can't keep up or is too worn out. We use it on those days, mostly the day after she has her Lp or when we have big plans or long days involving a lot of walking. It helps to conserve her energy so she's not too worn out from walking to enjoy the day. She's a little embarrassed by it but also thinks it's kind of fun learning to zip around & navigate in it. She definitely agrees that it is a big help when she's tired.
All in all things are good all things considered. Hope everyone has a blessed weekend!

Good news!

We finally got the good news we've been waiting for. Next week we get to go to Shands hospital in gainesville which is only a 45 minute drive from our house. We will be going there for the trial now instead of NIH in MD.  Our dosing schedule will change to every 2 weeks instead of every 4 but that still cuts our travel in by more than half. So excited to start this next chapter but we will miss all the doctors, nurses & friends we've met through the years. We will still go up to MD once a year for check ups but she will be getting the trial medicine @ Shands from now on.

Still Moving Ahead

We are A NIH right now for our 25th consecutive monthly visit. Crazy! What's even crazier is that this could possibly be our last visit for awhile. They are working on Phase 2 of the trial & when it gets up & running we will be going to Gainesville for the medicine & only coming to NIH yearly! Julia continues to do well, she had her first dose of 1200mg on Tues. She only had a slight affect on her hearing, & we are hopeful that it will bounce back. She is still in school full time when we are home & loves school! She's in the 10th grade & got A/B honor roll on her first progress report of the year. The picture above is of her most recent fishing trip with her Daddy & friends. The last time the guys went out without her they didn't catch anything & they told her daddy they wouldn't go again without her. I guess she really is their good luck charm because they had a good day with her on the boat :)

Trial Update

It's been awhile since I posted an update on the trial. Things have been going fairly good. Julia has slowly moved up in her dosages over the last 6 months. She is currently @ 900 mg a month. When she reached 600mg she had a small shift in her hearing that recovered slightly & again @ 900mg she had another shift & a small amount of recovery during the month following. Other than the hearing there has been really no changes either good or bad. We fly up again tomorrow for our March visit. I will try to do another update following this visit.

Thank You

Have to say a huge THANK YOU to Mr. Bob Hampton @ Bi-Lo sales. Mr. Hampton assembled a small army of people that pooled their money to buy Julia's pig @ the Youth Fair. Below is link to an article our local paper ran about the purchase of Julia's pig. We appreciate each & every person that worked together with Mr. Hampton to make the purchase happen. The money raised goes a long way in helping pay for Julia's medical bills.

http://www.ocala.com/article/20150303/ARTICLES/150309923

My Little Prize Fighter

Julia looks like a prize fighter after her run in with a weight machine @ school. She tripped over a mat on the floor & went head/face first into a weight machine. Thankfully she has an extremely high pain tolerance. Her coach was so worried & said she hit so hard it should have knocked her out. We took her to the dr & she got a little liquid stitch on her jaw & she is good to go. Now it will just take some time for the bruising to heal.

Thank you Catch A Dream Foundation!

Julia recently had the opportunity to go trophy whitetail hunting in Missouri where she harvested this beautiful 13 point whitetail. We need to say a heartfelt thank you to the generous Mr. Bill Roller, owner of Little Flat Creek Ranch where Julia had the privilege to hunt. We met some of the most wonderful Godly people & had an awesome time. I would highly recommend The Catch A Dream Foundation to any family with a child with a life-threatening illness. I have never met so many people that were willing to give so generously of their time to strangers. When we left them after 4 days together no longer felt like strangers, but more like family. We will never forget this experience.

Doing the Happy Dance!

Julia started High School last week. So far she is loving it!! Julia & I are @ NIH for our Aug visit. We actually got to come up a few days early this month to attend the wedding of my new sister-in-law. Kim was a beautiful bride & the wedding was beautiful too! We got to NIH Sunday morning  & we have had a lot of good news since we've been here. For starters, & probably the best news of all, NO More IV's!!!! We are so excited about this, Julia's arms were constantly in some state of bruising from all the IV's & blood draws. The other good news is that after our 1 year visit next month, our visits will be reduced to 2 days!!!! Also very exciting!!! I can't believe it's been a year since we started back in the trial, crazy to think we've been traveling back & forth for that long. We have met so many wonderful people in this last year & have made lifelong friends. Also I added a video below this post, Julia was asked to help some of the leadership team here @ The Children's Inn where we stay to do the Ice Bucket Challenge. Of course she was only happy to help, Julia had a lot of fun dumping that bucket of Ice water :) In the video she is 2nd from the left in the back row.

It's Summer Time!

Finally!!!! Summer is here! May & Junes visits to NIH were wonderfully uneventful. Julia's condition remains stable. We are back @ NIH, as I type, for our July visit. Julia got her cyclo infusion this morning & everything went well. Because it's summer, Rebecka was able to come with us too. We are enjoying showing her around. Julia is especially enjoying introducing all her friends here to her big sister :) Tomorrow & Wednesday we should have some more free time & Georgetown cupcakes in our list of places to visit as well as maybe the Smithsonian Museum.

April Update

April's visit to NIH was pretty uneventful. Julia got her drug infusion & all was well. Time is flying by so fast I'm having difficulty keeping up, between challenges @ work & the everyday busyness of a household with 2 children in school. I can't believe we are closing in on the end of the school year so fast. Julia starts high school next year!!!! And now we are staring down our next trip to NIH. The good news is last month was our 6 month mark so there was extra testing & everything is showing Julia's condition to be stable :)

Chugging Along

Things have been going really well. We have been @ NIH this past week & get to go home tomorrow. Julia got her cyclodextrin infusion Monday with no issues as usual. We got to meet another family this time, Cody & Kayla. Julia had really been looking forward to meeting another girl & they got along very well. Hoping maybe next month they will get to spend a little more time together. I didn't post anything in Feb but we did come up to NIH & Julia got the Cyclo. Feb was just such a busy month I got behind. Both my girls celebrated a birthday in Feb. They are now officially 17 & 14! And I'm getting old, lol. I'm going to go & try & do a little back dated updating because there have been some really cool happenings that I've neglected to post about.

Wrapping up Youth Fair Week

I am so incredibly proud of both of my girls. They both did excellent jobs showing their animals in the Southeastern Youth Fair. Rebecka placed 5th in her class showing her steer Tucker  and Julia sold her pig Ansley for a whopping $7.75 a lb. Thanks so much for the generosity of the kind people @ Wiechen's Realty who purchased her and thanks to Brian Terry vinyl for purchasing Tucker. Julia is donating all of her money to her trust fund to help with her medical bills. Those kids put so much work into raising the animals, I makes me so proud to see them show them. Rebecka also showed her 4H chapter pig & took 1st in her class. All in all it was a great week!

Snow Day

I almost forgot to post about all the snow we got during our Feb visit to NIH. 18" fell over night and made for great sledding & snowman building.

One Tough Cookie!

Yesterday was a little rougher than usual. Julia had her LP in the morning, but for some reason there was a little trouble getting the needle in & then when they did she could feel it. She cried because it hurt so badly. It's moments like that that make me question if we are doing the right thing by putting her through all this, but ultimately I know that answer is yes! It is absolutely worth it! While we are not seeing improvement by leaps & bounds we are seeing stability & that is huge. The good news is we also have ideas for next month to ensure Julia doesn't have to be in pain for next months LP. After her LP was done Julia had a visit from a therapy dog named Donizetti, or Doni for short. It really helped her feel better after the painful morning.

Quick Update

Back for our monthly visit @ NIH. All done with today's appt's, going to cut out of the hospital for the day & maybe go on the free trip to the movies this evening. Sounds like a good time to me. Tomorrow will be spent here @ the hospital for her medicine dosage :) Hope everyone stays warm!!!!