Woah! It's been 2 months!?!

Hey all!!

I am so sorry I haven't blogged in such a long time. I had to read my last blog just to see where I left off. So here we go:

Anabelle still has her NG tube unfortunately. I absolutely hate the stupid thing (except that it feeds her) because we constantly have to change it out, it gets clogged, and the tape on her face only gives people something else to stare at. She is not gaining as much weight as she did initially she has leveled out quite a bit. However, her surgery for the G-Tube is coming up on the 30th of this month. I am so excited to get this thing placed, but I'm also a little scared for her first surgery. The surgeon assures us it is a relatively simple procedure and he feels really good about it. It should take about an hour to complete and I think we will be staying overnight. She is having the surgery done in Portland at Doernbecher's. Today she had an x-ray to make sure her intestines are working properly. I havent heard the results from that yet. We're hoping that with the G-Tube we will be able to do some more calorie dense foods to help her gain weight.

Anabelle is starting to really struggle with sleep. She is now on two different medications to help her sleep at night. When she struggles with sleep, we struggle with sleep. Now that we have the medications it is really helping. We also had to up her meds for her muscles and her pain. I'm not really a fan of that but whatever makes my baby comfortable.

Unfortunately, Anabelle is no longer talking. She doesn't have any words. However, on the plus side she still laughs so much! And I love making her laugh. I talk to her all day and love to talk to her about things that make her happy. She is also no longer moving. She can mover her arms but not with any precision. She is doing really well with being content with her situation.

Our Car Show fundraiser got cancelled unfortunately. We just didn't get enough cars to put on a good show. However a friend of our family has put together a great new fundraiser called Anabelle's Angel Glow 5k. For more information about this event visit www.angelglow.org. We are so excited about Bend's first glow run!

Now for the fun part! We went to Orlando for Anabelle's Wish trip and had a blast! We got to go to Disney, Sea World, and Universal. We went to Universal our first day and got to see the Barney show and meet Barney. Anabelle was so happy to meet Barney! We also got to see a few other shows while we were there. We went to Disney 3 different days. First day we went to Magic Kingdom (identical to Disney in So Cal) and went on all the rides they would let us take Anabelle on. She absolutely loved Dumbo ride!! She also liked the tea cups (mommy and daddy left a little nauseous)!! She also loved meeting the characters! Mickey was her favorite!! Duh! Then we went to Sea World and we got to feed the dolphins. I think most of you got a chance to see the picture. She was crazy excited about the dolphins. Shortly after that a huge thunderstorm rolled in and they shut down most of the park. The place we stayed at called Give Kids the World was fantastic! They had meals available for us 3 times a day. They had an Ice Cream Parlor that served ice cream from 7:30 in the morning to 9 at night. We were able to place a star on the ceiling of the main tower that will be there forever. She also made a cute little pillow. They also gave Anabelle a different toy every day. We came home with a whole extra duffel bag full of stuff. Our family was treated fantastically! Make-A-Wish did everything they could to make us feel really special.

I know I say this every time but I am truly thankful for all of the support we have been given over the last almost year (I know crazy right?!). You guys are so great!! God has big plans and big outcomes for all of us through Anabelle's life. You are one of Anabelle's Angels!

Quick Update

I am happy to report that not really a lot is happening with Anabelle right now...  She has had her NG tube for 3 weeks now as of Thursday and I will weigh her again. So far she has been gaining a pound a week which has been fantastic (two pounds total)!! I think about a pound on me and it doesn't seem like much but on her it is much more! She looks great. She seems to be feeling a lot better! I'm going through WAY more diapers! She is babbling at me more which I love. It brings me joy just to hear her voice! Our fundraiser Cars for C.A.R.E has turned into so much more than we thought it would. We are in the process of turning into a non-profit charity so we can give to other families in need and make this thing a real business! Check out our Facebook page at www.facebook.com/carsforcareMLD Ronn and I are really excited for the Car Show in September! Also we got our dates for Anabelle's Make A Wish and we will be going to Orlando Florida from Aug 20-26 and will be going to Walt Disney World, Sea World and Wild Animal Kingdom! It's going to be so much fun. Just Ronn, Anabelle, and I will be taking the trip. We were really hoping to take all of our family but MAW just didn't have the budget for that. It's okay though because I know were going to make some great memories!! That is really all I've got on Anabelle's situation. Isn't it so great that my blog is only a paragraph long! However there is a lot on my heart... and if you head over to my new blog "A Mother's Heart" (www.anabellesmommy.blogspot.com ) I am going to start blogging more about what's on my mind for me personally. Thank you all for your kind words and endless support! "Therefore encourage one another and build each other up, just as in fact you are doing" 1Thessalonians 5:11

FINALLY SOME GREAT NEWS!!!!

Today started out pretty normal with Physical Therapy at 9 am and then Anabelle had a appointment with the pediatrician at 9:40 so we had to boogie over there, and of course I got lost! Finally we made it and the doctor was still willing to see us. They weighed Anabelle and this time she was 2 pounds under her regular weight so I was super bummed. And previous to the appt. the g-tube people called and said it wouldn't be until August 9th that we get her g-tube. I was just spiraling down and the day just felt like it was getting worse! I was discussing some options with the doctor and she said that we could do an NG tube which goes in her nose and then down to her stomach. Sounded like great news but I just felt like it was going to be another 3 weeks until we could do that. And then she said "We can do it today here in the office" I just starting crying. I think after all the waiting and the let downs and Anabelle losing weight I was just overwhelmed with "happy tears." God is so good isn't He! I knew there was going to be some great bright shining light soon! So they didn't have the size tube they needed at the Westside clinic so we met her doctor at the Eastside clinic at 4:30 and Anabelle got her NG tube! We then went to the Home Health Nursing office to get a pump for her tube and some other supplies. We got home and Anabelle got to have her first feeding and my sweet little baby went to sleep with a full tummy. Praise the Lord!!! I cannot even begin to express how excited I am. This will be just a temporary fix until we get her g-tube. Oh man I am out of my skin so excited that my baby is going to enjoy the feeling of being nourished and maybe put on some weight. YAY! Such a great day! Also we picked our Make A Wish and will be taking it around the second week in August. We will be staying at Give Kids The World in Florida (look it up it's awesome!) for 6 nights 7 days and we will get to go to Disney World, Sea World, and Animal Kingdom. This will be such a great trip for our family we are so excited. After such a heavy road of big blows it's nice to have some treasures along the way. Thank you all for your consistent thoughts and prayers. We love you all soo much! Until next time...

Over the mountain...

And through the woods to Shriner's Hospital we go...

We are back again from Portland and it was a productive trip but still harder than I want it to be. I wish these trips would give us some better news but I know that it just doesn't happen that way with Anabelle's disease. This trip was beneficial though. We were able to get Anabelle's Botox injections done. She got Botox injections in her adductors (inner thighs) to help with diapering and comfort. We decided not to use orthotics after all, so they didn't do her calves. Apparently any one person can only have a certain amount of Botox injected at one time, so they felt it was best to use the amount she could take in her biggest problem area. The PT said that with her thighs relaxed it may help loosen everything else. We decided against the orthotics because it was not going to be that helpful in the grand scheme of things. Honestly, I know that I only have a limited time with my daughter and I would rather spend it with her comfortable than upset because I'm making her wear these things. Between her swim therapy, daily stretches and regular PT there isn't much more the orthotics could do. And she hates having them on and I hate fighting her on them. Also while we were there we were able to get her wheelchair fitted. With my last two blogs we were considering a stander and then we were considering a kid kart. Unfortunately neither of those things happened. According to the PT and the wheelchair fitter Anabelle just didn't have the body support for either of those things. But we got a sweet wheelchair with some awesome add-ons. Hopefully she will be so comfortable she will love it. Also its going to be pink! Which of course makes mommy happy. Hehe.

On her G-Tube... I called Doernbecher's and they said that her referral is in "Medical Review" which apparently means that based on Anabelle's abilities and history they are trying to figure out which doctor is going to do Anabelle's procedure. To me, it seems like that would be a one day thing but of course it never works the way we think it should. We should hear back from them sometime this coming week. It is getting harder and harder to feed her. And what I was afraid of is happening. Anabelle has started losing weight. I guess expressing to these life saving professionals that my daughters case is emergent is not enough to get things moving. So frustrating!

It's been really hard to go through all this stuff with her. Every time we make a trip up there things become so much more real. I have a hard time realizing my daughter is different. I don't want anything to be wrong with her. I know that she is different and I get that, but I just want her progression to slow down. It's starting to feel like the end is sooner than we thought. I am often up at night because I am afraid if I go in her bedroom she wont be breathing. A lot of those fears you have when you first bring them home are starting to come back. I have to watch her floppy head, make sure she doesn't choke, and keep her fed. All without being able to communicate with her. A lot of me feels like that's been the hardest part. I think it would be easier for me to deal with her motor loss if we could still have quality conversations. Not knowing what she is thinking just kills me.

On a brighter note, Anabelle has been approved her Make A Wish and we are just waiting to hear from the volunteers to schedule a visit to the house to set up her wish. I am really excited for this time for our family!

Please don't take my words as a note that you need to call in an intervention to save me from my thoughts. I have a God who can handle all of them and always has better advice. I stand firm in the thought that God has Anabelle in his hands and is cradling her as we speak. I love how strong my God is. He lets me be mad and angry and frustrated and just cuddles me deeper into His chest. My God is an Awesome God!


Until next time...

No news is bad news

We are almost back from Portland and unfortunately it just seems things are continually going downhill... The Neurologist didn't really have any good information for us.  Except he has prescribed her another medication to help with her comfort. Other than that it was all bad news. He said we should start seeing a pulmonologist to monitor her lung function because kids with MLD can get chest infections very easily. He also said with her steady decline in speech it's a pretty good indicator the disease is starting to affect the cognitive part (grey matter) of her brain. We were really hoping that it would be further out but what can we do. She is still very happy playing and being engaged she just may not be on the intellectual level we thought she was.  We also went to pick up her new orthotics but of course they don't fit because Anabelle is just too stiff. We are going to wait and get them in June when we go in for her botox injections. Hopefully the botox will relax her enough to be able to wear her orthodics and maybe use a stander. Speaking of... We were considering getting her the stander in our last blog but since then we have decided to go with getting a kid kart. It looks like a stroller but is actually a wheelchair. I would attach a picture but I'm blogging from my phone. We as usual are feeling somewhat defeated but I am confident she is recieving excellent care. She has her g-tube consult in a week and I'm very excited about that (never thought I'd be excited for such a thing) but having her tube in will give her and us such a better quality of life. Anabelle is almost 2.5 and I will be calling Make-A-Wish on June 11. I really want to get her MAW done before she loses the chance to enjoy it. Still not sure what we're going to "wish" for but I will keep y'all updated. Also Ronn is working on a fundraiser for Anabelle and the MLD Foundation that will be held on Sept 14th at the Deschutes County Fairgrounds. We are planning on a car show. We don't have a name or any of that just yet but please be praying for a successful outcome.  I think that's it for us! Thank you all for your continued support. We couldn't do it without you!!

"I have told you these things, so that in me you may have peace. In this world you will have trouble. But take heart! I have overcome the world." John 16:33

Man, its been a while...

Hello friends :)

      I know its been quite a long time since I last blogged. Almost a month! Needless to say I've been extremely busy and very tired. I usually blog after Anabelle has gone to sleep and lately I've been going to sleep shortly there after. A lot has happened in the last month. Let me try to catch you up.
      Shortly after Anabelle's official diagnosis we visited Shriner's Hospital in Portland. I don't feel like they did too much more there than we could have gotten done in town but it was nice to see some orthopedic specialists and get an idea of where she is at; also it was free. We talked with them about ordering a bantam stander (pictured below) that will help her digestion, socialization and quality of life. We also had her fitted for some new orthodics that are hopefully going to be much better than the old ones. We also discussed getting botox injections for her legs to help them relax. Quick lesson on botox: when people get it injected into their face they hope to relax their muscles so that their wrinkles don't show. Well much in the same way it can help Anabelle relax her leg muscles. We will be back at Shriner's in the middle of June for her botox eval and injections.
      The other good thing that happened while we were in Portland was we got to meet the directors of the MLD Foundation, Dean and Teryn Suhr. They gave us a lot of information on how to move forward, what to get in motion now, and what to expect for the future. I have to say it was quite relaxing to eat dinner with someone who knew that Anabelle's behavior was "normal" and weren't at all bothered by it. They were a very sweet couple and are a great source of wisdom and resources for this process. Through their foundation they have created an email list that I am now a part of with over 250 MLD families and that has really helped me understand this life I'm living and not feel so alone.
      After we returned home we started speech/swallow therapy. We started with a clinical evaluation of her swallowing and found out that Anabelle's timing (from her mouth to swallowing) was a little off and it would be a good idea to start her on a mechanically soft diet. Nothing dry, no crackers, and sans anything else she may choke on. Anabelle has really been having trouble eating and I was really starting to get worried. After seeing Anabelle try to eat Teryn insisted that we get the ball rolling on getting Anabelle a g-tube. She will get a g-tube w/ mic-Key button (pictured below). Its a nice little button that they can't really mess with and you just open it up and attach tubing for feeding. The consult for that is the first week in June. We will have the consult here in Bend but she will probably have to have her tube put in at Doernbecher's because they can't put her under with gas anesthesia because it will accelerate the progression of Anabelle's demyelination (disappearing white matter). So we would like them to be able to do it with more of a local anesthesia, which may not be in a comfortable area for the Gastro Doctors here in Bend. It's a big step for us to get to this part of the process but at this point she is hardly eating anymore and having to drink three PediaSures a day just to get enough calories. About a week after the clinical eval we had an x-ray eval done called a modified barium swallow. They had her eat food with barium in it and watched where her food went on the x-ray. Unfortunately the results from that were worse than we thought. To give you a comparison, you and I would cough just as liquid was touching our vocal chords, Anabelle is letting food and liquid fully penetrate her lungs before she coughs. She is getting more particles into her trachea than we initially thought. So now we are having to thicken everything she drinks and also she is on an almost completely pureed diet. It has been hard to see her digress so much but we are taking it one day at a time.
     That brings me to today. Well, yesterday. Yesterday Anabelle woke up from her nap at about 2 and was sitting with me when I noticed she started to get really sleepy very suddenly. She was starting to fall asleep in my arms which is very unlike her. I called her doctor to make an appointment and their earliest appointment was 7:30 pm.  The nurse on the phone asked me if I had taken her temp and I said no, and she didn't feel like she had one. After I got off the phone with the nurse I checked her temp and it was 101. I gave her some medicine for her fever and again she started to fall asleep in my arms. Suddenly she arches her back, her eyes roll back into her head and her arms are stiff as a board out in front of her just moving back and forth. She was having a seizure, I called Ronn being the smarter medic in the family (and all my medical experience went out the window when it was Anabelle having the issues) and he said just hold tight that he would be home soon but I just couldn't wait. I called 911 and the operator walked me through everything I should do. Anabelle's face started turning blue really fast so she had me lay her on the ground and check her mouth for anything blocking her airway. Next Anabelle spit up a little bit and her color came back to normal. At this point (the seizure lasted about a minute) Anabelle was starting to come down from her seizure but she was unconscious. The cops arrived before the paramedics and started to assess her. First there was a moto cop, then a pair of cop,s then a fire truck and then an ambulance with three paramedics. (I wonder what the neighbors were thinking.) Anabelle was still unconscious as they were giving her oxygen and waiting for her to come to. She was out for about 15 minutes and then they decided to transport her to the hospital by ambulance just in case she had another seizure. Anabelle started to come to just as we got her onto the ambulance. After a little while in the ER, the doctor ordered a flu test, a chest x-ray and a urinalysis. Anabelle came back positive for the flu and RSV. The doctor came in and told me that the flu caused the fever and the fever caused the seizure. Unfortunately with Anabelle's brain condition her seizure threshold is lower. The doctor decided to keep Anabelle overnight for evaluation. They did an EEG after we got up to her room to look at her brain waves and they said that her brain activity didn't show she was seizing during the EEG (they were worried about seizures we couldn't see), but that her brain activity was a little slow and disorganized, which is abnormal for a normal kid but normal for Anabelle (given the MLD). We got home about 3 this afternoon and I am so glad to be home. Anabelle is on some meds to help with the flu and I am closely monitoring her fever. We already had a follow up scheduled with the Neurologist in Portland at the end of May and we will be following up with Anabelle's pediatrician next week.
    WHEW!!!! I'm exhausted! Not just from the last couple days but I think it is really starting to hit me how serious this is and I'm getting really frustrated! I watched the neighbor girls (Anabelle's age) today run around the col-de-sac pushing a stroller and talking to each other about all sorts of things and it hurts me to imagine what Anabelle would be like without this stupid disease. I HATE THIS!!!!! I am so frustrated with how awful this disease is! It just makes me so angry I want to scream! I know that God is good and He is faithful but shoot, this really really sucks! And its so hard to know that the best years of her life are behind her not in front of her! ERRRGGHHH!!! Sorry for my little rant but I think I'm starting to hit a wall on this. I am really running out of fuel. Thanks for listening (or reading). As always I will let you know more as I know more.

Alex

Bantam Stander:




G-tube with Mic-Key


      

The Blood Results are In...

First, let me just say thank you to all of the people that have really come along side us in this. You guys are a huge part of the reason we are still standing. So, thank you. Next, the results. This morning the pediatrician called me and said that the blood results came back and they seemed to be inconclusive. The levels of enzyme were low, however, the PhD doing the lab test did not believe this lead us to a conclusive diagnosis of MLD. The pediatrician then explained that it may be a good idea for us to get a genetic mutation screen. With Anabelle already getting the screen they would do Ronn and I's at the same time. I asked "so genetic mutation meaning that we aren't carriers and this is specific to her?" and she said both. That it could be a gene mutation specific to her or Ronn and I could be carriers of the gene mutation and passed it on. This screen will apparently be able to resolve all of that. We also discussed that the neurologist in Portland had done some other tests and that I hadn't heard back from him yet either. She said she would contact him and let me know. At about 3:30 this afternoon she called me back and said that after talking with the neurologist, he believes that with her clinical history and the low enzyme levels, he is 99% sure of a MLD diagnosis. He believes that the PhD at the lab didn't think the levels were conclusive because he did not know Anabelle's history. We are still going to continue with the genetic mutation screen and we also are still continuing with the treatment of the symptoms. Ronn and I are doing okay. We are not at all happy about the future for our daughter, however we know that God is going to use this for His glory and we know that He has something for us to learn from this. Thank you Lord for your light and strength in our lives. You are our Rock!