Daddy's 40th Birthday!

We had such a blast celebrating Jason's 40th birthday. His dad took him & his brother to drive a Lamborghini! The whole family went to watch & cheer them on & most of them ended up getting to ride too. Jason had his Julia's Journey shirt on & the guys that run the business asked about it. When he told them about Julia & NPC they asked if she wanted to go for a ride for free. Boy did she ever!!!!! She got about 5 laps around a closed course driven by a professional drift card driver. She had a blast & was smiling from ear to ear. If you ever want to drive a luxury car please check out Gotham Dream Cars. These guys were great & went out of their way to give Julia an awesome experience. We ended the day with some go cart racing @ an indoor track in Orlando. It was an awesome day!

Santa Claus came to NIH!

Julia did awesome as usual during her LP. I wish I had 1/2 of her bravery. We had kind of a lazy day yesterday, just laying around afterwards, but as Dr. Porter says boring is good. Boring means there is nothing unexpected going on. Today we had a couple of blood draws but otherwise a lot of free time. We spent it hanging out with our friends & going to a Christmas party hosted @ the clinical center for the kids. Julia enjoyed it & got to see Santa Claus! He even had a huge gift bag for each child. It was really cool!   :)  We only have one more blood draw tomorrow & one Friday morning & then we fly home Friday evening.

On the Road Again

We arrived this afternoon back @ NIH to a beautiful snowfall. The high today was 32! Julia will get dose # 4 Tuesday morning. She has informed us that she does not want to be fully sedated this time. I am excited about that. What it means is  they will give her just a little something to relax her & then a local anesthetic. It also means that I get to be with her the whole time! Very exciting! Better go for now it's just about dinner time. Will update more later in the week :)

Dose # 3 Tommorrow

We arrived safely back @ NIH yesterday afternoon. Had a nice relaxing day today & gearing up for Julia's procedure tomorrow. Julia is anxious to return home this time as quickly as possible because daddy picked up her pig for the FFA show in Feb on Sunday afternoon. All she has is this one picture of her & she can't wait to meet her. Julia has named her "Ansley", so cute!!

Home Sweet Home

We made it home last night. I am happy to report that everything went smooth. So happy that we are able to continue in this trial!!! Looking forward to our next trip around Nov. 10th. Julia feels awesome as usual & life returns to normal for a few weeks until we head back, wonderful to be home!!!! :)

All Went Well

Julia had her LP yesterday & everything went very good. She hasn't had any problems & feels great. She has a few blood draws left between tomorrow & Friday but we are still on schedule to go home Friday evening :) In the meantime, we enjoyed dinner out tonight @ Ledo's pizza, a local restaurant that generously treats the residents of The Children's Inn to dinner free of charge. Their pizza is delicious!!!  Tomorrow afternoon there is an outing scheduled to Buttler's Orchard, a local apple orchard & pumpkin patch. Julia is really looking forward to that, especially the hayride :)

Nice Refreshing Weekend

Julia & I had a wonderful weekend. We left the hospital Fri. afternoon & didn't have to return until tonight. As usual we met some new friends. So Julia has spent lots of time socializing, like the social little butterfly that she is :) We took a bus to the mall, went  for walks, did a little baking. It was very refreshing, so now we are ready for the next few days :) Tomorrow morning she will receive the cyclodextrin during her LP & then we will probably spend most of the day resting in the hospital.

Back On Track

Sorry to keep everyone wondering what is going on. Truthfully, we spent most of the time since my last post waiting to hear news ourselves. The FDA finally released the hold & we are back on track with a lot of revising of the protocol. For starters they are no longer giving the medicine directly in the brain via the Ommaya. They are going to give it via a lumbar puncture into the spinal fluid.
So we arrived back @ NIH this past Sunday. Our flight was great! Julia had some baseline testing on Monday & then the first of 2 lumbar punctures during this visit, on Tues morning. Everything went awesome. during the 1st LP she only got saline, but next Tues she will get the cyclodextrin. Between now & then she will have a few more tests & blood-draws, but we should have quite a bit of down time this weekend to enjoy this beautiful weather :)

Postponed

So it looks like I posted a day too soon. I received a call yesterday from Nicole @ NIH to say that they are temporarily postponing our return. As things unfold with little Cassie Hempel the NIH is taking the opportunity to take a step back & consider all the information available to them to make sure they are doing everything possible to minimize the risk the surgery poses for our NPC children. I appreciate this about the team of doctors so very much, that they are not rushing recklessly through this trial but stopping every so often to make sure they are seeing all the pieces, so to speak. While I am slightly disappointed because we were ready to go back, I know that they are absolutely acting in the best interests of Julia & every other patient involved in the trial.

Ready to Try Again

So here we go again. We have talked to the doctors @ NIH & are ready to begin our journey again where we left off. Julia is a little nervous as am I, but we can't just stop here. If we do then everything we have been through has been for nothing, & we can't live with that. We are not ready to give-up our fight yet, we just had to regroup & renew our strength to continue on. Julia & I will fly to Md & she will have surgery on Apr 22nd to implant the new Ommaya so we can continue on.

Home Sweet Home!

We made it home finally!! Today is actually 1 week since we arrived home. I am very surprised how easy it has been to slip back into our "normal" routine. I think Julia is made of rubber because she has just bounced right back with no problem. We have about 4 more days of antibiotics left & then we are clear. We have not yet made any decisions about going back to continue on in the trial. The doctors are sorting through a few things & making a few changes to the protocol. In the meantime we are taking the approach of sitting back & watching how things play out before we make any rash decisions. The one thing I do know is it is so good to be home & God is still good!

2 steps forward & 1 step back

Julia had her surgery this morning to remove her bubble. The doctor said it went well & after a little vomiting from the anesthesia, she has recovered nicely. With any luck we will get released from the hospital tomorrow & get to go back to The Children's Inn. Hopefully we will get to go home on Tuesday. We certainly have a lot of soul searching to do from there. While it is definitely a possibility to place another Ommaya after she has healed we have to decide what we feel is best. Right now Julia is against the idea & I think if I had to choose right now I would be inclined to agree with her view. What we have been through to get here has simply been an emotionally taxing roller-coaster. I think a little time & distance will go a long way towards helping us see the big picture with more clarity. I have to give a word of praise to to all the doctors & staff up here caring for Julia. I hold everyone of them blameless in all of this. We knew coming into this that it is a trial & things can & will go wrong sometimes. They have done their very best & are giving Julia & me the very best care.

Dissapointment

What a disappointing day. We had such high hopes this morning that her labs would be good & we could go home tomorrow. Julia was even doing her go home dance. Instead Julia is scheduled for surgery tomorrow @ 8:30 to remove her  "bubble" (ommaya
reservoir) .She has another 14 days of antibiotics in store as well, but we will start them here & finish them @ home. Her white blood counts went up instead of down so that shows that the bacteria is still there & her body is trying to fight it. The doctors gave it their best shot @ clearing the bacteria without removing the ommaya but knew it was only about a 50% shot. Julia of course cried when Dr. Porter told her we couldn't go home yet. Hopefully we will go home this Tues. I"m not counting my chickens yet though, one day @ a time. That's how I'm making it through.  

Snow Storm :) & Hopefully Coming Home soon!!!

This is what we woke up to this morning. The snow came down almost all day. Already though it is melting & likely will  be gone by this time tomorrow. That's ok with us though because if all goes well Julia & I will be on a plane headed home this sat!!!! I don't want to count my blessings too early, but I can't wait to be home! Julia is still feeling great & her labs have finally been steadily heading in the right direction. She has finished all her antibiotics, now they are waiting until Fri to make sure they are out of her system so they can pull one last csf sample. As long as the gram stain & white blood count from that sample are good then we get to come home Sat. Praying & Hoping it's good :)

What a Weekend

The weekend flew by. Usually it's just Julia & I here but this weekend we had visitors, not once but twice! What a nice change. On Saturday my cousin Wendy & her husband Jason who live about 45 min away in Va, drove over & took us to lunch & we out with them for several hours before they brought us back to The Children's Inn & we showed them around a little. They also had a goody bag for Julia for her birthday filled with all kinds of stuff that made her smile :)  Then on  Sunday Ms. Cindy from home flew in to visit her daughter & son who live in Va. Cindy & her daughter Kim came & picked us up & took us to dinner @ her sons home. We had a lot of fun! During this visit we have met a 20 yr old young man named Chris & his parents. Like Julia, Chris is also fighting NPC. Tonight we went to dinner in a nearby town, Frederick @ Fosters Grille to support Dillon's Night with Chris & his mom. Every 1st Monday of the month this restaurant donates 30% of it's sales to Dillon's Fight For a Cure. Dillon is a local 10 year old boy also fighting NPC who became Julia's friend when we met him during our last visit to NIH. I was blown away by the support shown @ Foster's Grille, the place was packed!!  It was very nice getting out & away this weekend. Thanks to everyone that visited us & helped us get away!!!

Julia's Birthday

We took a trip to the Smithsonian Zoo @ Julia's request for her birthday. It was cold & Julia got tired before long so we only stayed about an hour & a half, but it was a lot of fun. She said her favorite animal was the Giant Panda. Earlier that morning her team @ NIH brought her a goodie bag full of birthday gifts that she loved & a cake. Then she got another goodie bag from The Children's Inn. Even though celebrating her birthday here instead of home hasn't been ideal I have to say a huge Thank You!! to everyone who has done their best to help put a smile on her face. She has received cards & packages for several days from friends & family both near & far, the farthest came from Alaska! She even received an angel from the Angels for Angels organization, thanks to Mrs. Stephanie :)

Turning 13

 Finding it hard to believe that tomorrow Julia will be 13! Her birthday is technically Feb 29th, but with no leap year this year we call it her non-birthday :) We are going to make a trip to the Smithsonian Zoo in DC tomorrow to celebrate. I was reading her bday list & it broke my heart to see on there, "To get this disease away". Wouldn't it be awesome if that was something we could truly give her. That's why despite the obstacles placed in our path, we still feel like we are doing the right thing by continuing on with this trial. 
    We got the chance today to meet the next patient in the trial & his family. If we keep up this pattern they are going to just have to make Julia the ambassador of the trial. She was patient # 1 & now she has met & talked to patient #s 2 & 3 & answered any questions they had & showed them her battle scars so they know what they can expect. It's very cool for her to be able to do that. It's also wonderful for me to have other parents to connect with who are going through the exact same thing as we are.
    Julia continues to feel good & finally I can say that I think we are seeing her lab results tip in our favor. It's still too soon to do a victory dance, but it feels good to see things going in a positive direction. Still alot of things in the air as far as decisions go, but by monday we should have a much clearer idea of what's next. Really hoping to get an all clear & be able to get her next dose of the study medicine as it was originally scheduled for mar 5th anyway.If that happens then we can expect to come home mar 9th. Feels like such a long way away. We miss home & everyone there, but we are well taken care of here. :)

Trying to Hang in Here =]

This sucks :( We are trying to hang on & be upbeat but the truth of the matter is that over the last 2 months we've been here in Md more than home with family & Julia & I are both over it. Having said that Julia is still feeling great, no headaches, no fevers, nothing. She handles everything so well though, I don't think I would be as good a patient as she is :) The doctors have her on 2 different antibiotics, one she gets by iv every 12 hours, the other they inject right into her ommaya reservoir in her brain every 24 hours. As of this morning they are discussing adding a 3rd antibiotic she will be taking by mouth every 24 hours. They are not getting results as quickly as they would like out of the other two. They have been monitoring her white blood count & they expected to see it stay stable or go down, instead it keeps creeping up although very slowly. As of right now she will remain on the antibiotics until @ least Mar 5th with monitoring going on daily & possibly plans changing as well. If the antibiotics don't clear the bacteria to the doctors liking then they will consider removing her ommaya, which means another surgery :( Obviously this is not top choice as she needs the ommaya to continue in the clinical trial so if they remove it we would have to look @ placing another one @ some point in the future for her to continue in this trial, but every step between here & there just delays her getting her next dose of the trial medicine :( We need prayers that the antibiotics completely get rid of this bacteria so we can continue on in the trial without delays.
Mean while we have a lot of free time on our hands here. Today we are filling that time watching the Daytona 500 :)

A Rough Week & Now Back in MD :(

So a lot has gone on this past week, Julia & I got back to work & school & tried to find our rhythm. Unfortunately her doctors called me from MD as they were concerned with some of her csf samples. It seems when they studied them in the lab they where growing some bacteria in them. It was a slow growing bacteria that is normally found on the skin. They asked me to take her to Shands for a csf draw there so they could see what was going on. I took her on Tuesday & they did the draw. Our Md doctors communicated with the lab @ Shands all week & by Sat we all breathed a sigh of relief when no bacteria grew in her sample. They looked @ it again Sunday & still nothing. However on Monday morning they called @ said it had started to grow :( I was shocked, as were the doctors. I think I'm still in shock honestly. So what does this mean? Well we went to our local hospital mrmc yesterday, Monday & they gave her some antibiotics through iv & then we were on a plane last night headed to Md. They gave her more iv antibiotics last night shortly after we arrived & now she will be getting them every 12 hours for the next 7-10 days. The goal is to knock this bacteria out before it turns into an actual infection. That is the silver lining right now, this thing has the potential to cause meningitis, but as of right now she is not having any symptoms of an infection.  So for now we sit & wait & keep up the antibiotic schedule until the doctors decide the rest of the plan :)

Home Safe and Sound!

Julia & I arrived home around 3pm Friday afternoon. Let me just say, it is sooo good to be home! Sat evening we had an awesome sweet 16 party for Rebecka. We even surprised Rebecka with live entertainment courtesy of our wonderful friends Clifton & Theresa who drove all the way from Louisiana to be here! We owe them a HUGE Thank You! We hosted around 66 people & I think everyone had an awesome time.  I know Rebecka, Julia, Jason & I did :)
Now tomorrow we all get to try to get back into a normal routine.

The Big Day!

Julia finally received the  medicine yesterday morning!!! Once it happened I got just a slight bit emotional realizing how long we have waited & prayed for this day. To think of all the people who have been working to make this trial a reality & all the time & money to get to this day. I know that gods fingerprints are all over this. Dr. Porter & his teams of people have been working on this for years, long before Julia was even diagnosed. For her to be the first patient in this trial has been an amazing experience. I want to say a huge thank you to everyone @ NIH & The Children's Inn. They have taken such good care of Julia & me. We are  now more than 24 hours out from when she 1st received the medicine & there have been no reactions or side affects to report, thank goodness. Just a few more days & we will be on our way back home :) Till next month.

Update

Sorry to anyone who has been looking for updates. I've been neglectful in that area. It doesn't mean anything bad though. Just the opposite, everything has been going absolutely perfect! Julia is incredible! She came through surgery beautifully, on Wednesday morning they began accessing her ommaya to draw cerebral spinal fluid or CSF for preliminary testing. This involves sticking a needle through her skin into the ommaya & using a syringe to draw the fluid. I'm not sure I could sit still for them to do it on me but Julia sits as still as can be & takes it like it's nothing. I admire her strength.
 This weekend we were able to come down to the Children's Inn. We sat this morning & watched the snowfall & the deer outside our patio again :) Tonight we will head back up to the hospital again to Skype with Jason & Rebecka while we watch the Super Bowl. We have a friendly rivalry going on, we are rooting on the local team "Go Ravens!" while they have claimed the 49ers. Whoever's team loses has to cook a full course meal for the others :)
Tomorrow morning Julia is set to receive the cyclodextrin for the first time. We are extremely excited about this, but also a teeny bit apprehensive. I know that God has her wrapped firmly in his arms though. Still it is the 1st time in the US that a child will receive this medicine directly to the brain.
I will be adding some recent picture later today so go check those out if you want on the family photos page. While it hasn't been all fun & games, we have managed to really make the most of our free time while here :)

With Flying Colors

That's how my baby came through surgery today! Praise God! This is a picture of her an hour after her surgery, sitting up eating eggs & bacon. She was talking & moving all of her parts. Everything seems completely normal & she is recovering as expected if not better! The doctors & nurses are taking excellent care of her & she is the "Rockstar"  of the ICU :) We  will stay here a few days (3-4) & then go back downstairs to her regular room. Hopefully we will spend the weekend back @ The Children's Inn across the street. I am adding pictures to the family pictures page daily so check there too if you want.

Starting the Next Chapter

It  feels kind of like we are starting the next chapter in our journey. This chapter is full of hope! We made it to Md safely, it was 25 degrees outside when we landed, brrrrrrr. We got checked into our room @ The children's Inn about 8:30 tonight. All is well & we will head up to the hospital tomorrow to start all the consent & admissions paperwork & meet with doctors. It will be a busy but good day. Will check back in when I can.

It's a Go!

So for the past few weeks we have been awaiting the FDA's decision on the clinical trial for cyclodextrin. The wonderful folks @ NIH have been in constant contact with them trying to work out all the details. They were finally given the green light today! The trial has been approved & Julia & I are scheduled to fly up to MD Jan 22nd for a 2 1/2 week stay. Julia will have surgery to install the Ommaya reservoir on the 28th & then receive the first dose of medicine before we return home. We are soooo excited to begin this I can't even begin to describe it. Keep the prayers coming because they have kept us going. That & the knowledge that God didn't bring us to this for nothing, he's been here every step of the way & he's teaching us through it to always rely on him :) Hope everyone has a great weekend!