The Tolmanator's New Textbook

After a year and a half of long, hard work, Justin's Pharmaceutics textbook has now been published and is for sale!  Here's a link to the Amazon listing for it.  On that page you can click "Look Inside" to see several pages.  Feel free to purchase it, read it, and write a wonderful review! ;)

This textbook (written with two other co-workers of his) was the highest selling textbook from this publisher at the last American Association of Pharmaceutical Sciences (AAPS) conference.  Way to go, Justin!!

Annie Update

Annie is still sick.  Today is day 9 of this nasty illness.  She hasn't been this sick in a really long time.  She has been coughing nearly non-stop all day and night for the last several days and has been having a really hard time with thick congestion in her throat.  She's been requiring suctioning regularly around the clock, but today was the first day that we went more than an hour without suctioning!  VERY small progress, but I'll take it and am happy to see it.  I think this illness would have slammed even a typically healthy child, but for Annie it's always much worse.

Annie has been continuing to have a persistent rattle in her breathing and after we listened to her lungs and heard the rattling there too, we became a little nervous, especially since her fevers are still hanging out around 101-102 degrees.  Fortunately her seizures have remained fairly well controlled since her scary episodes 9 days ago--they are not completely gone, but back to their usual frequency.

It's hard to tell if the rattling in her lungs is from pneumonia or is just being reflected from her upper airway.  So I took Annie back to the pediatrician again today to have her checked out for pneumonia.  She's never had pneumonia before since she's always maintained a good, strong cough, but her cough hasn't always been productive this week as she's grown so weak from coughing so much.  And with pneumonia being the most common cause of death with Aicardi Syndrome, we don't treat it lightly.

The pediatrician thought Annie's lungs sounded fairly clear, and her O2 sats were around 95-97.  Not bad, but she's usually always at 100.  If her O2 sats had been in the 80s he would have hospitalized her immediately.  We've always thought it would be convenient to have our own pulse ox machine at home, and our doctor today agreed, so he ordered us one and we'll get it soon, which will bring some peace of mind.  So then we went to the hospital for a chest x-ray.  It was torture for Annie getting the x-ray, but fortunately it was completely clear!  Whew!

Annie also had a Respiratory Viral Panel done at the hospital--just a nasal swab to check for about two dozen different viral infections.  And surprisingly, that came back negative as well.  We suspect that she had some kind of terrible viral infection last week, but all the phelgm and congestion she's struggled to clear has turned into bronchitis or a sinus infection.  So we'll start her on yet another round of antibiotics, hoping and praying that this girl can finally catch a break, get healthy and strong in plenty of time for her spinal surgery in 3 1/2 weeks, and sail through without any problems.  Once she has her spinal fusion, her lungs should be healthier and stronger at times like this since her left lung is currently being crushed by her scoliosis.  We've just got to get her healthy for surgery!

For now, I'm most grateful for a husband who could work from home yesterday so I could take a 4 hour nap.  We're still exhausted but I think Annie will turn the corner really soon with the antibiotic, and hopefully we can all start getting some sleep again.  We really appreciate thoughts and prayers on her behalf.

Postponing Surgery

Annie is keeping us on our toes this week. She was scheduled for her spinal fusion surgery on Monday, but on Wednesday this week she had terrible seizures for 3 hours requiring two rescue meds, developed a temperature of 104.4, and was in the ER last night. We've kept her in isolation the last 2 weeks to prepare for this surgery, yet she still managed to develop a viral infection. With this surgery being so risky anyway and requiring 8-9 hours under anesthesia, the risks are way too high to proceed on Monday with her in this condition. The surgeon has rescheduled her spinal fusion for December 9th. I'm hoping we can survive 4 more weeks of high anxiety and stress leading up to this and that Annie can get (and stay) healthy and strong this time!

Final Spinal Surgery Testing and Info

Today I took Annie to the hospital for a series of tests and consultations in preparation for her spinal fusion on November 11th.  First we had a series of x-rays taken of her back, and met with the orthopedic surgeon for more than 30 minutes.

The good news is that her curve hasn't worsened since August--the largest curve (of her 3 curves) is still around 85-90 degrees.  But her spine isn't quite as flexible as he had once thought in the past.  The flexibility x-ray where they bend her against her biggest curve showed that they were able to bend her to 50 degrees.  Generally a 50% improvement is expected after surgery, so the surgeon does think he can get her down to about a 40 degree curve with the fusion.

However, since her spine is not only curved, but also twisted, she has developed a very large rib hump on her right side, and since it is so difficult to un-twist her spine with this surgery, he anticipates that she will still have a rib hump, but hopefully it will not be quite as prominent.

The orthopedist will need to study her x-rays more thoroughly to decide exactly which vertebrae will be fused together, but her fusion will definitely start at T2 (just below the neck) and go to L3 or L4 (below the waist) or maybe down to her pelvis.  Her worst curves are fairly high on her back, so he might be able to avoid fusing her pelvis.

The doctor went into great detail of how the surgery is performed which was helpful.  After making the incision from her next to hips, he first inserts the screws into the vertebrae down the length of her spine.  They have screw knobs on the end of them that they then begin attaching one metal rod to, working down the length of her spine, on the concave side of her curve.

As they attach the rod to the implanted screws, they tighten them so as to pull her curved spine towards the straight rod.  The surgeon watches to see how far he can pull the spine before the screws in her vertebrae start to slip, and then he knows that that is only as far as he is able to correct her curve.  At that point, they will shape the rod to fit the remaining curve of her spine.

After the first rod is placed securing her spine, they place the second rod on the opposite side of her spine to further secure it.  As they attach the rods to the spine, they will also try to un-twist her spine and rotate it back to a normal position as much as possible, but that part of it is very difficult to correct.

They expect the surgery to last 8-9 hours, which I know will be the longest and scariest 8-9 hours of my life.  We have been told of all the serious risks involved with this procedure, but today we were told of even more risks.  The biggest risk during the surgery is a fatal loss of blood.  They plan to have two units of blood available in the OR for her, but sometimes if there is an accidental nick of an artery or they can't find a source of blood loss, it can be life-threatening.

Also, since Annie will be laying prone for 8-9 hours, there is also a risk of blindness.  It is rare, but it occurred at our children's hospital a few years ago during this same surgery.  There is a risk of cardiac arrest during surgery, as well as risks of paralysis when working with the spine.  Yes, Annie doesn't walk, but paralysis would still affect her rather dramatically in other ways.  They will be inserting needles under her skin all over her body and have leads on her head and one person will constantly be monitoring her neurological activity during the surgery.

After surgery she will be in the ICU for a day or two.  She might remain intubated and on the ventilator in the ICU also. The biggest risk after surgery is pneumonia, so they will work extra hard with us to get Annie sitting and up-right as soon as possible after surgery to help avoid a potentially fatal case of pneumonia.  There is also a risk of infection, which is often very difficult to treat in spinal fusion patients and can be fatal.  Annie will be having cadaver bone grafts placed in her spine as part of the procedure, and the doctor will be inserting antibiotics in with each bone graft, and she will also be receiving IV antibiotics as well.

After surgery she'll have a drain coming out of her back for a couple days, and will probably receive at least one more blood transfusion.  She is expected to be in the hospital for a week if all goes according as planned.  Once she's home, she will be recovering and out of school for about 4-6 weeks.

After x-rays and meeting with the orthopedist, we went down to the pre-op unit and Annie had blood drawn for lab work and to type her blood in prep for her blood transfusions.  They also took a urine sample, making sure to check for any sign of UTI.

Then I met at length with the anesthesiologist.  She again stressed all the major risks that the orthopedist talked to us about, stressing the risk of fatal blood loss during surgery and cardiac arrest.  She also talked about the added risks of being under anesthesia for such a long time.

We often feel overwhelmed and incredibly nervous and scared about this procedure.  It's such a major surgery, but when we expressed our anxiety to the orthopedist, he reminded us that he does one of these spinal fusions every single week, and that it really is a rather safe procedure!  I know they have to legally tell us all the risks up front, but it sure does scare us.  The orthopedist reminded us that they know exactly what all the risks are and what complications can arise, so they are always prepared for those.  He gave us a little more sense of peace, and we find a lot of peace knowing that so many of our friends have gone through this successfully.  We are trying to keep a lot of faith in Heavenly Father knowing that Annie will truly be in His hands, and have complete trust that He will watch over her.

So for now, with surgery about 2 weeks away, we are working diligently to keep Annie healthy.  As the doctor said, all the stars must be aligned before going into this surgery.  It is a huge procedure and can be life-threatening, so if even one little thing isn't quite right, they will cancel surgery.  She can't have any kind of cold or illness, infection (UTI), or not even diaper rash or an ingrown toenail!  
Needless to say, this is a major surgery--definitely the biggest surgery of her entire life.  We really appreciate prayers and fasting on Annie's behalf, and also in behalf of the surgeons and surgical team that will be working on her.  And prayers for our own peace during such a scary time are greatly appreciated as well!

Make-a-Wish Trip Day 7: More GKTW, Universal Studios, and Heading Home

I can’t believe this is our last day here. We’ve looked forward to this amazing trip for so long and it’s hard to believe it’s nearly over. We took this morning a bit more slowly and finished packing up all our things. We got breakfast at the Gingerbread House and then went to the Candy Land playground again for Justin to see it this time and to be able to take some pictures in the daylight.

I still can't believe we packed so lightly and got all our stuff in just these few bags with NO checked luggage! The red and orange suitcases, as well as the pink backpack are all full of just the gifts we received!

Molly checking to see if she'll be able to carry this backpack through the airports for us.  It's HUGE, but it's really just filled with the 3 Magic Pillows the girls got at the beginning of the trip, and Annie's extra pillow for positioning her on the plane.

It's been wonderful spending so much time together as a family!

Enjoying our villa porch one last time before leaving.

The Candy Land Playground at GKTW

This playground is really awesome!  It's all wheelchair accesible, and it's a huge Candy Land game board.

On Sunday nights at GKTW they do an actual live Candy Land game here where the guests themselves are their own pawns as they move around the game board.

Here are some of the other villas in the village.  There are about 150 villas and each of them is uniquely designed on the outside.  The whole village feels so magical.

 After letting the girls play for a while we headed to the House of Hearts to check out. Again, it was humbling to be presented with more wonderful things at check out. We got a CD of all the photos that were taken of us around GKTW by their photographers. We also got a certificate that, as a wish family, will get us into most amusement parks and attractions around the country for free over the next year! We got a nice printed picture of us with Mickey Mouse when he was in the village earlier this week.

House of Hearts at GKTW

We also got a certificate for Crops of Love—an organization that will put together a complete scrapbook for us of all our pictures from our trip, for free. Our MAW wish granters are also putting together a scrap book for us, so we will be blessed with lots of ways to remember this wonderful trip!

We also got another golden star like the one with Annie’s name on it in the Castle of Miracles to remember exactly what her star looks like there. We have definitely received an amazing outpouring of love here at GKTW. It has been by far more magical than any Disney park could ever be. There is such a wonderful spirit about the people here—the volunteers as well as the wish families. I wish we had more time to stay!

After we checked out from GKTW, we drove to Universal Studios to go to the other half of the park we didn’t see yesterday. We headed to first aid to change Annie, got our free stroller rental as usual, then went to the Despicable Me ride. It was basically a 3D movie with moving seats which was fun, but that also made it so Annie could sit and watch the movie part without being jolted around in the ride. Then we went on Shrek 4D which was nearly the same way.

After that we went to an Animal Actors show since we loved the Pets Ahoy show so much in Sea World, but this one wasn’t nearly as exciting. However, the worker grabbed us before the show and told us to stick around afterwards, after everyone else has left the auditorium, and they’d bring out the animals and let us pet them and take pictures. So that was kind of special and the girls liked it.

Being silly waiting for the show to start

Annie's private time with the animals after the show.

Next we headed to the E.T. ride, which again, was accessible for wheelchairs, but they wouldn’t let Annie ride because her wheelchair supported her trunk and she couldn’t support her trunk herself. She could have totally gone on it. It’s been frustrating that there hasn’t been much in Universal Studios for Annie to participate in like she did in the Disney parks.

Then we headed to the kids play area and the girls (and Justin) LOVED the ball machines where they could vacuum up balls and make them travel through tubes and collect them to shoot out of air guns. We finally pulled them out of there when the noise was becoming way too much for us. Universal is definitely the loudest theme park I’ve ever been to in my life—every ride is super loud and even all the music playing through the walkways is way too much.

The girls could have played in here all day!

I think Justin had just as much fun as the girls did!

This was the huge water area, but we didn't get very wet since we were headed to the airport soon.

 Justin and I took turns going on a kids’ roller coaster with Molly and Lexie, and Molly even went on a water slide in an inner tube, but didn’t even get wet. It probably would have felt good with the hot temperatures. (I think each day got just a little warmer. Highs were around 85-90 degrees with heat indexes in the mid to upper 90s. The lows only got into the low 70s, so the evenings were really comfortable and nice.)

Justin, Molly, and Lexie are in the front two cars.

 

Molly on the waterslide

On our way out of the park, we stopped to have pictures with Curious George and Woody Woodpecker since they were just right there.  We were ushered to the front of the line of people waiting, and when we were done, the worker asked who else we wanted pictures with and she would just call them and have them come to us—anyone we wanted. We stood there stunned, but we weren’t sure who all the Universal characters were, and we were on our way to the airport anyway, so we had to turn down the opportunity. Too bad we couldn’t have taken advantage of that sooner in the day had we known!

With Curious George

Woody and Winnie Woodpecker

 

Winnie Woodpecker was super nice to Annie

We picked up our free 8x10 picture from the photo shop. Again, I’d never consider buying pictures like this, but when they are free as a wish family, why not?

The free picture we received as one of our MAW perks in Universal.

We enjoyed Subway so much for lunch yesterday that we hit it again today. But this time, the kind young man behind the counter gave us free cookies after seeing us in our MAW shirts. People everywhere are all so kind!

Then we headed to the airport and met the van rental lady right at the curb for departures to retrieve her van. The whole van rental process worked so smoothly and flawlessly without us really having to lift a finger. And it was a really nice car!

We got to the airport around 3pm which several people had recommended to us, even though our flight didn’t leave until 5:30pm, but Orlando’s security lines were much longer than Omaha’s and we needed that extra time to get through.

As we boarded our first flight of the night, the pilot came back to us in the cabin as we were getting Annie situated before anyone else was on the plane, and was so kind talking to us and telling us he’d get us whatever we need. Lexie slept through most of the first flight but woke up long enough to eat the hot fresh cookies our stewardess brought us after she served them to first class!

Those Magic Pillows from GKTW sure were great to have!

Lexie was a sound sleeper on the flights home!

Molly enjoyed browsing the airline catalog and got a kick out of some of the stuff in there.

Annie did great on the flights home.  Her chest strap and extra pillows worked great to keep her sitting up in the airplane seat.

 Justin was trying to figure out a way to hang up her beads for her when a total stranger stood up and came over to show Justin a way he could get them to hang! 

 
We landed in DFW for our layover and had about 2 hours, so we grabbed some dinner and let the girls watch a movie.

Before leaving DFW a family that had just come from Orlando had a daughter who wanted to give Annie her huge Mickey Mouse balloon. We were so touched by her thoughtfulness and generosity, but with one more leg of our flight ahead of us, we just couldn’t manage to take it with us. But even still, I continue to be amazed by all the kindness that has been shown to our family on this trip. Often Annie is overlooked or not considered in so many ways, and to see her be the center of attention on this trip, even from total strangers, has been so touching and continues to bring tears to my eyes as I consider all the genuine kindness she has received on this trip. She has truly been treated like a princess and she SO deserves it!!

Our flight to Omaha left around 10pm and all 5 of us quickly fell asleep and remained asleep for the entire flight. We landed at 11:30, and by the time we got our van from long-term parking, and got home, we all quickly fell into bed around 12:30. And fall definitely arrived while we were gone!! It’s SO cold! It’s going to be hard to be home and get back into our routines! But I am so grateful for this amazing week. It was truly a once-in-a-lifetime trip.

Sure, we can always go back to Disney World, and we are also even welcome back to GKTW for a day visit whenever we like, but never again will we experience these places in the magical way we were able to on this trip. I am so grateful for the kindness and generosity of so many people—the volunteers at GKTW and MAW, as well as all the donors that contributed to make not only our trip possible, but the trips for over 120,000 other kids who have been able to experience what we have. It is absolutely amazing! The love and kindness we have received has been overwhelming and we are so grateful for the memories that we’ll always have of this trip.

Make-a-Wish Trip Day 6: Islands of Adventure, Downtown Disney, & Christmas at GKTW

This morning we grabbed an Express start breakfast from the Ice Cream Palace and ate in the car on the way to Universal Studio’s Islands of Adventure. We first visited Dr. Seuss world meant specifically for kids. I took the girls on the Cat in the Hat ride which was just like other Fantasyland rides, but they wouldn’t let Annie ride it because she couldn’t support her trunk by herself, even though she’s in a supportive chair. After getting off the ride, I told Justin that Annie could have EASILY ridden it and she probably would have loved it! Toy Story Mania was more jerky and spun more than The Cat in the Hat!

Had to grab a quick picture with Spiderman!

And Molly's goofiness continues in full today.

We went on another ride like the Dumbo rides and it was marked on the map as having a wheelchair accessible vehicle, but when we got there, they told us that vehicle was gone today and getting serviced. It was pretty disappointing.  But the girls loved it.

Little did Justin know how wet he'd get on that ride!

We next made our way to Harry Potter world which was pretty neat, but incredibly crowded! It was rather difficult to push our way down the street of Hogsmeade. We skipped the two huge roller coasters there since none of the girls could go on them and the area was so crowded anyway, and headed into Hogwarts for the ride in there. Molly went on it with me and then Justin went with Molly. I thought she’d be a little scared from it with the dementors flying down on us and other scary things, but she liked it and wanted to go on it twice.

I was convinced earlier this year to read all the books, so the whole Harry Potter world was much more interesting than it would have been otherwise. Molly and I rode Flight of the Hippogriff which was a smaller roller coaster. We saw the store where you could go in and have a wand experience—finding a wand that chooses you like in Harry Potter—but the line was huge and the wait time was 2 hours. That wouldn’t have mattered for us with Annie’s pass that allowed her to the front of all the lines, but I told Justin that Annie doesn’t really care about Harry Potter, and we moved on. I think Justin was really just wanting his own wand. So we did look at them later in a different store. He kept saying we should pick one for Annie, but I just couldn’t imagine what Annie would do with a wand, especially since she’s not a Harry Potter fan, but I do agree that they were pretty cool to look at.

Entrance to Hogsmeade

Hogwarts Express

Quality Quidditch Supplies Store--the Bludgers were actually moving around and rattling in their trunk!  So fun!

Lexie is ready to take off!

Hogwarts

Hogwarts--the ride inside takes you on a true Harry Potter experience!

 

So then we moved on to Jurassic Park world. They had one kids ride that was pretty fun—the Pteranadon Flyers—which I rode on with Molly and Lexie several times—just staying on the ride over and over. There were some other big water rides that looked fun, but after seeing how incredibly soaked everyone was getting off of them, we opted out. Although thinking about it now, we had our rain ponchos with us and probably could have stayed somewhat dry with them on on the ride. Instead, Molly had fun watching one big water ride and found the nerve to go get a little (or a lot) wet!

Waiting...

Waiting...

About to get wet!!

 We finished wandering around the rest of the loop of the park and were a little disappointed how little there was geared for younger kids. And the music even just walking down the sidewalks, as well as in rides, was just so loud that both Justin and I started getting a headache and sore voices from having to speak so loudly to one another! However, no headache could have held me back from riding the tower ride there! Justin waited with the kids while I rode it and it was awesome! Unlike most tower rides that slowly take you up to the top and then drop you, this one shot you up to the top super fast and then dropped you! It was great! I wish I could have stayed on it and gone a couple more times, but I knew everyone else was waiting for me.  We took turns going on a couple other rides like Spiderman.

The girls got rather impatient (and silly) waiting for Justin and I to take turns going on some of the bigger rides.

We headed back to Dr. Seuss land for the kids to ride the carousel—the only ride that Annie was able to go on in the park. The girls also wanted to ride the little train ride again too. Then we headed out of the park around 2:30.

It's amazing how fast all the carousels go in Orlando!

Molly really wasn't upset.  I think she was just running out of silly faces by this time of the day!

Of course each of the girls had to catch a wave on our way out of the park.

We stopped at Subway for a quick lunch and then went to Downtown Disney to do our shopping. We were given an incredibly generous amount in gift cards to spend at Disney stores, so we spent the rest of the afternoon shopping. Justin and I got zip-up hoodies, a hat, a picture frame, and some cookie cutters, while the kids got stuffed animals/dolls, a cute collection of 12 dolls of the Disney princesses, a shirt and frame for Annie, and art kits for Molly and Lexie. They got several other souvenirs as well. I wanted to make sure we used up our gift cards tonight so that we can figure out how to pack it all before we have to check out tomorrow morning!

Molly had to try on some more Mickey ears.

We thought these displays were fun with the dresses made out of dolls.  There was one for Cinderella and Belle as well.

More posing silliness.

It took us til 7:00pm to pick out all the things we wanted to buy, and finally got back to GKTW around 7:30 for dinner at the Gingerbread house. Tonight it was a turkey dinner with stuffing and mashed potatoes and all the fixings of a Christmas dinner because tonight’s party at the village was a Christmas celebration!

The Gingerbread House is really neat because there are hundreds, if not thousands, of stuffed animals and dolls on shelves around the ceiling!  It became a great place to play "I spy with my little eye..." while we ate dinner.

After dinner we went to see Santa and got our picture taken, then were ushered to a gift area with shelves full of toys for each girl to pick out. Lexie got a really cool My Little Pony set with a remote control car, Molly got a Strawberry Shortcake doll and toy, and Annie got a board game. Tonight we were again overwhelmed with all the generosity we have received on this trip. We are in awe of all the selflessness of others in granting so many wishes to all the children here. And they do this every week! In addition, the Star Fairy visits our villa every day while we’re away and leaves presents on our kitchen table. We’ve received board games, stuffed animals and other toys every single day. I’m beginning to understand why they told us to bring an empty suitcase with us!

Julie's Safari Theater where we met Santa (and other characters earlier in the week).

Julie's Safari Theater

Waiting to see Santa!

Waiting to see Santa.

Christmas is every week at GKTW!

Picking out toys from Santa

After seeing Santa and picking gifts, we went to the Castle of Miracles to see where Annie’s gold star was hung up by the Star Fairy. A volunteer helped us locate the coordinates and we found her star up there with all the others—over 126,000!! It was so awesome! It’s neat to think that her star with her name on it will hang there forever.

Annie's star

The Star Tower entrance--where Annie's star is hanging.

Annie's star is hanging in the Star Tower of the Castle of Miracles.  It is near the bottom-right of the round "constellation" I'm pointing at.

The constellation with Annie's star near the bottom right.

Looking up in the Star Tower of the Castle of Miracles.

We were lucky to catch the Star Fairy coming out of the Star Tower of the Castle of Miracles just after we found Annie's star.

On our way back to the villa we had to stop for ice cream one last time. Then we got the kids to bed and we started packing everything up. We opened all the packaging from all the toys in order to condense them. The family room of our villa looked like Christmas morning! We had two empty suitcases that had carried all of Annie’s diapers, formula, and medical supplies on our way to Florida, so since we’d used up all those supplies, we filled those two suitcases with toys and gifts, and then had to use the extra empty backpack that we brought along as well. I was impressed that we were able to pack everything in all our carry-ons and still not have to check any luggage! We got everything organized and crashed into bed just after midnight.

Trying to fit all our gifts in our suitcases!  We are truly humbled and overwhelmed at all the kindness and generosity that has been shown to us on this trip.  It was truly a trip of a life-time!