Saturday, October 20, 2012

Our Story Continues...Enjoy the last weeks of the Summer

So after hearing pretty much the worst news ever, we decide that we need Hailey time. Just Hailey, Josh and me. Before leaving Salt Lake and heading back home we take Hailey to Hogle Zoo for an afternoon before we start back to Las Vegas.

 Hailey was smitten with the "Baby" Elephant
 Time for some music.
 Waiting for the Polar Bear
 Making friends with the Brown Bear.
 Up Close with the Tigers.
 I love how it looks like she was standing right next to Mr. Tiger.

It was a warm day, but we really enjoyed having that time together. Laughing at the animals and riding the train. After a whole afternoon with the animals we packed up and headed back to Las Vegas. We were going to do the trip in 2 days anyway so we weren't in any hurry to leave. We really enjoyed what we had seen while in Salt Lake even if we weren't in the best of spirits. 

We drove by the campus one last time before heading out. Some how I knew we'd be back. (Just not as soon as a month and a half later)

So we now only have a month left until school starts and we have 2 more "vacations" we want to squeeze in before then. First up is a trip to California. My older sister, Hailey's Aunt Pam, lives in California with her husband and 4 kids. They invited us to go up with her as she was going to be driving to California from Las Vegas.

On our way to Aunt Pam's house we stop at a working farm where there was a petting zoo and fresh fruits and veggies. There was also this HUGE pit filled with corn feed. The kids had no reservations about jumping right in and feeling the corn with their bare feet.
The rest of the car ride was pretty quiet. Hailey and her cousin fell asleep holding hands and watching The Little Mermaid. Ariel is one of Hailey's new favorite princesses.
We had loads of fun in California. Hailey had her cousins to play with and really enjoyed swimming in Aunt Pam's Pool.

We even had a lunch date at BJ's where we chatted and enjoyed the sun outside. It was so odd to me to be outside in the sun in August and it not be 110 degrees out.

Now while things are going great there is something in me that is worrying me. Hailey had been potty trained and re-trained several times. In California she started wetting her pants. At first I was in denial thinking that she's just having too much fun with her cousins that she's to busy to stop and go potty. At least that's what I wanted the case to be. Remember in January she was wetting her pants before an episode...
My sister did get Hailey to poop in her potty by telling Hailey that she was the poo-poo fairy and if Hailey poops in the potty the poo-poo fairy will bring a special present. This opportunity was too good for Hailey to pass up because lo and behold she poops in the potty. That evening a new friendship was made. Meet Lambie:

 Lambie was instantly loved on by Hailey, and Lambie will play a big part in Hailey's life in a few weeks.

Look at that happy face! Being with her cousin and having Lambie all in the same moment was pure bliss.
So I was able to put my fear aside for the remaining time we were there. After we got home I did mention my concerns to Josh and said that I was going to retrain her to potty but in the in between times and during the night we needed Hailey to go back into "diapers" or training pants. I was worried after about a full week of being at home and Hailey going from time to time diapers to full time diapers. It just wasn't working. She wasn't telling me when she had to go. Then another little bump in the road was that when Hailey is not having an episode she's a normal little kid that can walk, run, talk, and open doors. Our doors have round handles that you have to turn the knob to open. One morning I heard her attempting to open the door in her room but then eventually end up calling for help. She wasn't able to open the doors. My anxiety started to surface. Josh went straight to changing the door knob handles to levers so that Hailey could use them.

We received paperwork to have Hailey tested for Vanishing White Matter Disease. It was something that the neurologist set up and we agreed to have it done. If it was going to confirm anything we wanted to know. Maybe there would be a medical study and we would be able to get Hailey into that if that was the type of leukodystrophy she had.
It was time for the first day back to school. I am sure I was as nervous as most parents who put their 4 year olds on a bus and waves good bye. This was Hailey's second year of school and I trust Hailey's teacher that she'll makes sure that Hailey is safe. In some ways I feel like that over protective or overbearing parent that is always at school and trying to help in anyway I can and donate supplies when possilbe. I like to go to school when there is a special day like an ice cream social or I like to bring pizza for teacher appreciation day. What is odd is that I had never expected to be the only parent that  goes to school for those special days. I have been to Hailey's school too many times to even count and I have NEVER seen another parent there at the same time. I know we have special circumstances where we only have so much time with Hailey and I want to make the most of it. When I think about it I don't know exactly how much time there is, but no body does. Even if I had all the time world I would still be at the school spending time with my child. Not to say that other parents should participate more, I know parents have jobs and other children and you should let your kids do their own thing and be independent, it's just something I think about. I am thankful for those opportunities and for Hailey's teacher for letting me be so involved. I hope she knows how much I appreciate that.

Once we get home we have Hailey set up for Summer School. I can't express how much this child loves going to school. Riding the bus is the highlight of her day, seeing her friends and her teachers puts a huge smile on her face. Summer school was only about 2 weeks long for her but she really enjoyed the time she went. The first thing she does when she gets off the bus and into the house would be to sit down and open her backpack to show me that she got a green light for the day. Then she'd put it aside to show dad when he got home.

One week into school, we notice Hailey is really tired. Her teacher even notices and lets me know Hailey is pretty lethargic at school. It was a muggy August and it was super hot. We had an upcoming trip to Washington to see Josh's family and we were hoping the cooler air would help. Still having my mommy senses buzzing I call her Neurologist in Utah and let him know that Hailey is always sleepy, and she is starting to walk slower. He said that maybe it's the weather maybe it's an episode, maybe it's the disease. He mentioned again that part of her disease is losing ability to walk and the possibility of her being wheelchair bound are very high. He says to keep in touch, go ahead on the trip and see how it goes.
We go to Washington and Hailey is walking on her own, slowly but surely. She isn't falling down or bumping into things, but that doesn't last long.
We were there for 5 days I think and when Hailey had an episode in the past it happens like 0 to 60, this is happening very slowly and I have to remind you she is still on steroids. All this time I felt in my heart steroids are what's helping...So why is this happening?
Josh and I believe this is the beginning of anther episode. We just had an episode, why is this happening so fast? It's not stopping Hailey though. She is still having fun and enjoying every minute of family and fun. I love this kids smile! (You will hear me say that a lot)

This was our first trip to VooDoo Donuts in Portland. We all pack up in the car and drove over for some super yummy donuts!

Once we get home things just go down hill fast. Hailey is walking, still baring weight but I have to hold her up under her arm pits to make sure she doesn't fall. She is talking, she is eating, but not walking on her own. We talked to her Dr. and he advised to wean Hailey off the steroids. It was obvious they were not helping. It hurt my heart to do it, but I could see that they weren't helping.

When we were last hospitalized in June we had ordered a wheelchair/stroller for Hailey. We didn't get it until Sept 6th. We understood this is what her disease was and this was going to happen. I didn't realize a child in a wheelchair was going to be so challenging. I still wanted her to go to school and since she takes the bus I just thought that they would see she was in a wheelchair and adjust accordingly. The school busses wouldn't take Hailey to school in her chair since it wasn't written in her IEP. I asked when we could have that adjusted and I got the date of Oct 19th. Basically a month and a half later. I would have to take Hailey to school. We don't have a wheelchair accessible vehicle so I would have to wheel her to the car, carry Hailey to put her in the car seat, pack up her chair, put it in the back and take Hailey wherever we needed to go. Hailey is 52 pounds and half my size. Carrying her is not an easy task.
Slowly Hailey started to not want to eat as much as she used to. Then every time I would help her to walk her legs would shake like crazy before calming down. This was something new. I have never seen her legs do that. She also started to clench her fists and the eating had slowed even more. It was Josh and mine's 10 year wedding anniversary and Josh's mom came down in hopes that Josh and I would go away and have our day, but we couldn't leave. Well maybe I couldn't kid was sick.
I get a call into the neurologist and he calls me back pretty quick. It was a Tuesday. He started off by wanting to tell me that he got the test results back from the vanishing white matter disease blood test. He said it came back need to test 5 specific genes to get an accurate and true yes or no. We only tested 1 but it came back normal? Now what? Well, before we get to now what I interrupt and tell him about the walking which we understand is part of the disease but what do I do about the leg spasms? The conversation kind of went like this:

Me: These leg spasms are new. They are freaking me out.
Dr: I don't like hearing that. Can you bring her to Utah?
Me: How soon should I have her there?
Dr: I can have a room for her tomorrow at Primary Children's Hospital in Salt Lake.
Me: Guess we'll see you tomorrow...


Wednesday, October 3, 2012

Our Story Continues...The UCLA/UofU Appointment

**I don't know if I mentioned exactly how Hailey was at this time. She is walking, and talking and it's only been 2 weeks since her episode.

When they call us back Hailey is super tired and pretty much falls asleep instantly. We spend nearly 2 hours between talking with a med student who needs our whole background history and then her relaying that info to the "Real" Dr. then waiting for him to come in. I will condense this as much as I can. Here is what happened: 

  • The Dr. looks at the MRIs and tells us that if he were to look at the MRIs from the very begining he would have NEVER diagnosed ADEM. He leans toward Leukodystrophy.
  • He thinks Hailey should be off steroids as well.
  • He wants Hailey to have a nerve conduction test
  • He wakes Hailey up so he can see how she walks and gets up off the floor and she wasn't happy to be waken up from a nap for that. 
We were there all together for 3 1/2 hours. We didn't get much out of him beside maybe getting that never conduction test. 
Since we didn't want to drive back to Vegas that night we spent the night there at the Tiverton house. We decided to take Hailey out to do something fun that night and there wasn't much to pick from. We decided on Universal City Walk.
No trip is complete without stopping in Hailey's favorite shop: 

and dinner with one of her most favorite people ever:

We end up driving home the next day still not knowing exactly what is wrong with Hailey, but we have knowledge of the nerve conduction test. When I asked if it was painful I never got a straight answer. They never had one, some people are too dramatic about it, some are super laid back. Who knows?
We head back to Vegas, and have some things to think about. When we talk to our Las Vegas Neurologist he doesn't recommend the nerve conduction test. He said that he could get the same results with a blood test as with the NCT. It's something to think about.
We end up getting a call from a Dr. in Utah that specializes in Leukodystrophy and he has an appointment available in 15 days from then. I think that if he specializes in this disease he will be able to tell us which one she has and what we can expect/prepare for. So now we need to plan a trip to Utah.
Josh's car is newer but he drives so much for work that he has over 170,000+ miles on it. My car is 15 years old but has 137,000 miles on it and needs a few things done and we wouldn't be able to drive it to Utah. We end up having to rent a car, book a hotel because we don't want to keep Hailey in the car for too long, and then figure out where we are going to stay. Luckily we have a few options. I have a friend that lives in Ogden, Josh has a friend that lives in South Jordan and I have a recommendation from a social worker for the Ronald McDonald House, but they are full for now.

The first half of the trip goes great. We drive to Cedar City and spend the night there. They have an indoor pool so Josh takes Hailey swimming and she is having a lot of fun. We go to bed early and head out to Salt Lake. We will be staying in South Jordan with Josh's friends family. They have two little boys that Hailey was excited to see. Our appointment was the next afternoon so we head out for dinner and we have a Vegas favorite, Cafe Rio!

I had some trouble sleeping that night. I kept checking to see if I had all her medical work, test results, and MRIs ready to go. I don't think I fell asleep until about 1 am and that was after I took a melatonin. This Dr.s appointment was nerve wreaking. He was going to tell us if he thought that Hailey had leukodystrophy. I hate that word. I hate typing it. I hate knowing what it means. I hate reading it. I can't read about it, but reading the word is just as bad.

We meet our neurologist and it's like starting over again. Hailey history, reliving everything all over again. Hailey falls asleep in my arms, but before she did the Dr. saw how she walked and ran up the hall and got her to talk. It had been less then a month since her last episode. One of the questions he asked us was  if when Hailey had an episode and then "got better" was she better then she was before the episode. She always did, and that was interesting to him. Since he can't just guess what she had with out extensive blood work, he gave us his best guess.

He said that looking at her MRI scans and know what tests they have taken he could take an educated guess on a few things:

  • The MRIs are conclusive with Leukodystophy. He wouldn't have diagnosed ADEM
  • In regards to what specific kind of (that L word) she has we could guess Vanishing White Matter Disease. There is a blood test we will do for that back at home.
  • In regards to the steroids "working" he says it's a coincidence. *I have to intervene real quick here which I did. 1, 2, 3 times I could say is a coincidence. 4 times...that's a pattern. He tells me to think about if Josh was walking down the street and then someone crosses the street and starts walking next to Josh someone might say that Josh and that person are walking together...but they are not*
  • I had said that they tested for this disease in January and those were the most common types, those were the types with some kind of treatment. There is no cure for any leukodystrophies, treatments for only some?
  • There are a few reasons to know which type you have. There are clinical studies done on specific types that you may be able to participate in. If you need a bone marrow transplant in the future this will help get the ball rolling.
  • The best way to get a diagnosis is to do a full genetic panel of 2500 genes. That isn't usually approved by insurance. If we have to order tests one by one the insurance will probably en cure less payment if they just opt for the full panel to get done and then it be over with.
  • So until we do a blood test or a genetic test to know what type she has what do we do until then?
The answer to that question sent me over the edge. The words that came out of his mouth were: "Enjoy the time you have left with her."I am sure he delivers this news multiple times on a daily basis so he might be a little numb to how devastating those words are for a parent to hear. I couldn't even type them let alone hear them. I really don't remember the rest of the appointment. I was just a sobbing mess. My chest hurt, it was tight. I think I had a panic attack. 

That night we had a room at the Ronald McDonald House. We had to start calling family to tell them what the news was. I couldn't call anyone, I emailed. Josh could talk about it. He called his family. In a way you have to think, it is what it is. You can't change anything, you couldn't have done anything different. It's just bad luck...or bad genes in our case. The Dr. also said the chance of Hailey having siblings with the same disease was 25%. No more kids for us, but even if there wasn't a chance Hailey needs 100% of both of us anyway. 

I don't think I slept that night either, I know I didn't eat. I stared at Hailey a lot. She was playing on the iPad on the sleep away couch. I couldn't take my eyes off her. So many unpleasant things went through my head over and over the tears wouldn't stop flowing. I had to relive the Dr. appointment every time Josh called one of his family members. One of the things he said was that, we'll give her the best we can, and do everything we can for her. I think that made me cry more.

The wall in our room had these words on it:

Honestly it couldn't be more appropriate. These words have FOLLOWED me numerous times. I see them EVERYWHERE! I feel like I need to know more about Hailey's disease but to this day I can't bare to read anything about it. I know the gist of it. Isn't that enough?

What are we suppose to do if she has another episode? The Dr. said that it's up to us, but the way the L word disease progresses it's inevitable, that Hailey may/will one day be in a wheelchair, may/will one day she'll need a feeding tube, she may/won't be able to control her bowels. Yes I know I put "may" and if she has this terrible disease I should say will, I can't yet. I haven't accepted this and I don't know if I ever will.

For now she's okay. She's doing good. She's walking, she's talking, she'll go to summer school. I can breath for now...well at least for the next month and a half, but I didn't know that then. In a month and a half, things will go really bad...


**Sorry if there were tons of grammatical mistakes. I couldn't bring myself to re-read this and correct anything.**

Tuesday, October 2, 2012

Our Story to UCLA

So Hailey's 3rd hospitalization was in April, late April. We see her new pediatrician who is getting us info on how to get to UCLA. I had been hearing that it's a great school/hospital and so many people were positive we'd get some answers. In the mean time we still have to see the neurologist here in Vegas. He has a new idea for steroids.

He wants Hailey to do a full week of steroids at 5mLs in the morning and 5mLs at night. Remember 5 mLs = 1 tsp. After one week he wants to take her completely off and go a whole week off steroids and try that for 1 month. I know the look on my face was dumbfonded. How could he not see that every time she is off steroids we are in the hospital? Well, he's the Dr. right?

Well a month goes by and Hailey is still doing good. She had her 4th birthday at a bowling alley. She had so much fun and was doing great. A month ago she couldn't walk and now she is holding a 6 pound bowling ball in a bowling alley.

In June we go back to the Dr. and now he wants Hailey to cut that dose in 1/2 one week on and one week off. Now, I'm nervous. Hailey goes a full week on at 2.5mLs in the morning and 2.5mLs at night. Then she goes a week off and things almost seem fine until the last day off and the next day she was suppose to start back on again. The next morning I wake up and hope Hailey is walking and walking well. I notice when Hailey walked into the bathroom she passes my bed and bumps into it. Am I over reacting? So I call Josh while he is at work and I know he hates when his phone rings in the middle of the day and he sees my phone number. It's one of those calls where I say, "I think it's happening again..."

Josh rushes home and we go to the neurologist office without an appointment. The front desk girl tried to have me schedule something for next month. I told her to either bring the doctor out here to the waiting room or we'll go find him. He comes out after about 20 minutes. He sees Hailey is off balance and his suggestion is to have Hailey go back home and up the steroids to the 5 mLs she did fine on. If by Friday she's worse go to the ER. We had hoped that would work, but on Thursday I didn't wait any longer before we took her in and admitted her with ATAXIA...again...

She is barely walking on her own when we are finally admitted. The neurologist meets us there with the pediatric resident. Here's where it gets super scary. Our neurologist the one that said repeatedly that she has chronic adem has a dreadful look on his face. He says to us:

  • I don't believe the steroids are working
  • What she has is something bigger then ADEM
  • She may have leukodystrophy
Whoa whoa whoa! I told him that he tested her for Leukodystrophy in January and you told me those tests came back normal. What's going on? 
He said that he tested her for 5 types of leukodystrophy those did come back normal BUT...there are hundreds of types of Leukodystrophy, some that they don't even have tests for.

Oh my chest was so tight and I was mad and I was sad and I wanted to pass out. He said that we need to take Hailey somewhere else. There was nothing more they could do for her here. They said that they would do the IVIG, hope for the best, but in the end that was it. In my eyes they were telling us not to come back. I told them I had an appointment to go to UCLA in 1 week. The neurologist said that they would be any help they specialize in epilepsy and seizers in children. I told him that maybe if he would have suggested a place that we should take Hailey we would have been there by now.
The did the IVIG treatment but Hailey is going downhill fast. She can't bare weight on her legs, her speech is gone. They did the IVIG over 2 days and I cried the whole time. I am thinking this is it. She'll never walk again, she has this horrible disease and our lives will never be the same.

My little girl is usually always happy. She will laugh at the drop of a hat. The silliest song, or at a funny face.

 They ask us if she is having problems chewing and swallowing her food. She's not. She's eating like a champ.
 The third day when we were suppose to go home Hailey woke up and ate her breakfast and then vomited it all up. Well after that happened we weren't going to be going home. They gave her some zofran and she slept most of the day. They kept her on an IV and tried to get her to eat crackers but it didn't work. By the middle of the night  (3 am) she had woken up and wanted some crackers. I got the nurse and she ate a cracker. She held it down and the next day they were able to discharge us. Josh missed work and had to return. I had Dr. appointments to schedule, chores to do, but I wasn't going to leave Hailey upstairs in her room and I didn't want her to sleep on the couch in case she rolled off when I went to the bathroom so I made her a bed on the floor with a pad underneath and a puppy by her side. My dog is constantly next to Hailey. I mean ALWAYS. Such a sweet pup.
 After a few days Hailey's speech was coming back and she was able to bare weight but not walk. I remember laying down by her on the couch and crying. She looked at me and said, "mom, don't cry. Be happy, like me."Ugh...I LOST it! We looked into getting Hailey a bean bag chair so that she wasn't laying down all the time. Those things are pricy! We found brand new large one for $400+ Luckily my friend Christina found one on Craig's list and we ended up getting it for 1/2 the price of a new one. It's the kids one so it's small, but it'll work better then the make shift one we had for a day before we got the one she uses now.

 Once we did get the bean bag she was pretty happy with it. I would find myself always having a head ache. Hailey always helped with that.
Before we leave to UCLA we have a meeting with a medical supply company and we wanted to order Hailey a bigger kid stroller, but the insurance would only pay for a child transportation stroller (a Wheelchair) Even though we needed this ASAP we weren't going to be able to get it for a few weeks (or so I assumed). 

Again after a week she's back up and walking and talking. Not as well, but on her own for the most part. I still hold her had to help balance, but she can walk on he own. 

Now it's time to head to UCLA. It's not a long drive, about 4 hours and we decided to leave in the morning for our afternoon appointment. We get to our hotel with about 1 hour to spare. The hospital is only a 10 minute walk from the hotel but we take a shuttle. It's a nice hospital, very colorful and kid friendly. Our appointment is in 40 minutes and for some reason I am on the verge of tears...


Monday, October 1, 2012

Moon Face

We heard that the side effects of steroid use was increased appetite, mood swings, and moon face among other things. Hailey has been on steroids for so long that I forgot how skinny she used to be. These pictures are just 1 year apart. It doesn't look like the same little girl. Steroids have sure taken a toll on her little body. But her smile is the EXACT same!