Blended Diet Continues

Tuesday, Bryce and I made another trip down to Tucson to meet with Marsha Dunn Klein, OTR/L and Ellen Duperret, RD. This was our third visit and the first one since beginning to feed Bryce real food through the tube.  It had been 6 weeks since our last visit.  The first month we focused on getting Bryce off of his night feedings.  That went well, so this past 6 weeks we have gradually transitioned his evening meal from formula to "real food" blended together and given through his g-tube. 

Bryce with Marsha.  A special bond has already formed between these two wonderful people.  

Some pretty amazing things have begun to happen in just 6 short weeks of eating a bit of real food.  First of all within a 6 week period Bryce gained 1 lb. 8 oz!  He is finally, once again, back on the growth chart.  Barely...but he is there!  He hasn't been there since he was two years old!   We were all very excited about that!  

Bryce enjoying his dinner of chicken, peas, squash, coconut oil, and a bit of water. 

Some of the other things I and others who work with Bryce have noticed since making changes to his diet include: 

1. He seems to be more alert, aware, and involved in what is going on around him.  One of his physical therapists today described it as him being more "present" in the world around him.

2.  He has had the healthiest November that he has had since becoming sick in July 2008.  I'm a little nervous to say that with a few days of November left, but so far, considering this is a month which I have come to fear and dread, he has done amazingly well.  The true test will be to see how January/February go.

3.  He gags less, has less reflux issues, and has good, regular bowel movements. He is also willing and wanting to taste more and more foods by mouth.  This first started when I began giving him green smoothies, but over the past month, we have all seen an even bigger improvement in this area.

4.  His sleep has improved.  Less waking up during the night!

5.  Has more energy.  He is trying to roll over or scoot around more often and with more purpose in the movements  i.e. to get his feet to his sister so that he can kick her (seriously, he does this intentionally...typical big brother!)  or to knock over a stack of blocks that someone has built just out of his reach.

6.  Starting to copy more sounds that others make.  The staff at Ryan House last week really noticed this.  Liz, one of the nurses there, kept commenting on how much better Bryce seems to be doing now than he was during our past visits.

7.  He just seems to look and act better overall.

These are the changes that I can think off right off hand.  It's interesting because the people who have commented about these changes say things like, "I can't pinpoint exactly what is different about him, but he just seems better, or more with it and capable than he has been in the past", and then give some example.  

Is all of this due to him getting some real nutrition into his body as opposed to the canned formula he has had exclusively for 3 years now?  I don't know for sure.  But what I do know is that I have been led by the spirit to make these changes to Bryce's diet and it certainly appears to be the right choice for him at this time!

Bryce loves being involved in his own meals.  He often wants to help me push his food into his belly.  The only trouble with that is that he tends to want to push it much faster than it should go.  We have to take it slow or he has trouble keeping it down.   As of now, it takes about 30-40 minutes to get one meal of about 240 calories into him.   We currently feed (or give him water) 8 times throughout the day.   It's a lot of work to prep, feed, and clean everything, but that boy is definitely worth it!  

Next step:  Starting the day with a Real food breakfast!  Wish us luck and please keep Bryce in your prayers  that these changes may continue to be for his good and that we may continue to be led to know what is best for our son!  

Whatever!

Annalise's funny of the day...during OT today, her therapist was trying to get her to button and unbutton a vest.  Annalise said that she wanted to put it on.  Her therapist said that she could if she would then do 4 buttons.  Annalise agreed and they put the vest on.  She then began to play.  The therapist reminded Annalise that  she owed her 4 buttons.  Annalise looked right at her and with the hand and all the attitude in that little 3 year old body she said, "Whatever!"  She then giggled and went on playing.  We could not stop laughing.  Yeah, she's 3 going on 16, the little stinker.  I think the poor therapist finally got 2 buttons out of her today!

Botox and other New Stuff

Friday morning Bryce had his first round of botox shots.  They are used to loosen the spasticity, tightness, and pain in certain muscles.  We have tried other medications, but he can only tolerate a very small dosage of these as they lead to increased breathing trouble.  His Physical medicine doctor has been encouraging us to have Botox done for a while now.  I have been very leery about it...I mean, really, the idea of injecting a toxin into my son really did not sound like a good idea at all.  I did a lot of research and spoke to multiple specialists about the procedure.  I also spoke with many parents who have had this done for their children.  There are definitely some risks involved that we have to be aware of, but after months (almost a year) of prayerful consideration, it seems that the benefits may outweigh the risks at this time so, we decided to give it a try.

One of the worst parts for him was the waiting time before getting called back.  It was really only a few minutes, but Bryce just does not like to sit still...ever!  (Or maybe he is just mad that I still put a bib on him.  I really need to figure out a better, more grown up way of keeping him dry!)

All the supplies

He received 3 shots in each of his upper arms.  He did not cry for the first arm, but did on the second.  I think he was just tired of getting poked!   Poor kid.

After the procedure.  He chose this frog from the basket of stuffed animals.  

We didn't really notice anything different until Sunday.  He actually sat in his chair and was able to reach out for toys and play with them a bit more than usual.  Then later, while he was lying on the floor I noticed that it was much easier for him to raise his arms to reach for things.   At one point, he was just lying there raising his arm up above his head and back down while looking at his arm and smiling.  I still don't notice a real drastic change, but he does seem to have a bit better range of motion and a bit less pain.  It will be interesting to hear what his therapists have to say about it after working with him tomorrow.  

Both kids have also received some new equipment this week.  

Annalise got some new AFO's.  Since her surgery we can actually get her feet into them!  She can still only tolerate them for about 30 minutes at a time, but it is a step in the right direction.  

Bryce got this Hensinger Head Support.  As you can see, he likes it.  If you ask him, he will tell you that he doesn't like it, but he does it with a smile and doesn't complain at all!  He has such poor head control and continually tips his head backward.  With this, he can concentrate on things other than trying so hard to hold his head up.  It can also help with breathing and swallowing...both things Bryce struggles with at times.

Bryce wearing his Hensinger and standing in his stander.  He has had the stander for about a year now.  With the head support he is able to get into a 80+ degree instead of a 60 or 70.  He loves standing!

The Casts Come Off!

October 30, 2012

After 7 weeks and 6 days, the time has finally arrived!  The day that the casts, aka boots, come off!

We got up early and made our way to the CRS orthopedic clinic.  While we waited in the waiting room, I met a lady who was there with 3 of her children.  Two of them were in power wheelchairs and one in a regular push wheelchair.   As we spoke, I found out that these were 3 of her 6 children.  5 of her children are in wheelchairs, 1 of the five is tube fed, and they will be adopting 2 year old twin boys, one who is blind, in the coming months.  The first three of these children are their biological children, the other 5 have come to them through foster parenting/adopting.  It was so neat to talk with her for a few minutes and learn how each of these children have come into her life.  Caring for these special children, who are often just passed by, is definitely one of this sweet lady's missions while on this earth.  What a great person.  I wish I had been able to talk with her longer.  I'm sure she has a lot to tell!

Once we got into the casting room, Annalise kept saying, " my boots come off.  The doctor take my boots off and I have a bubble bath in the big mommy bath tub." 

 The casts being cut off.

Annalise did really well, no tears at all until he began cutting on the second one.  It was just too much and the tears began to flow.  This wonderful child life specialist whom we have met and gotten to know over the past couple of months came right over to Annalise and showed her some games on the iPad.  It was a great distraction! 

While the tech was taking off the casts, I was told that Annalise may have some muscle spasms due to her high tone and having the legs in one place for so long.  The tech looked up and said, "she will have some for sure.  I can already feel it in her legs".  Poor girl.  Any time she tried to bend her legs, they would hurt her which always led to tears.   

The casts are off.  Just waiting to be cleaned up a little and for recasting for the AFOs.  She wasn't so sure about the casting.  She kept telling me that she did not want more boots.  She calmed down once she saw that they would be taking them right back off! 

Home, happy, and cast free!   

Annalise has begun twirling her bangs.  She does this a lot when she is tired or needing extra comfort. 

The whole way home, Annalise kept talking about her bubble bath in the mommy bathtub.  She was so happy that her boots were off and she was going to be getting a bubble bath.

However, once we got home, I ran some bath water full of lots of bubbles.  I put her in, but she instantly started freaking out a little and crying that she wanted to get out.  I made sure the water temperature and everything was fine.   I'm not sure why she did not want to be in the bathtub. I'm guessing that the water just felt really weird on her bare legs and that it was hurting.  I got her out and after a few cuddles, I handed her over to Sarah who was here to care for her while I took William and Jacob to the dermatologist.  I gave her some pain medicine and headed off to pick up the boys.  It was hard to leave her!

I guess all afternoon, Annalise wanted her legs to be covered with a towel or blanket.  She did not want to look at them and kept saying that she didn't like them and that they have owies.  I guess 8 weeks in a cast is a long time.  Being out of them is going to take some getting used to.  Hopefully she will get used to that soon and be able to enjoy her bubble baths again!

Answers?

I found out tonight that Bryce's entire genome, which has been sequenced, was one of the 10 that were picked up from San Diego this week and brought here to TGen in Phoenix to be analyzed.  What that means is that within 6 months, we could have some answers!  This is what we have been waiting for!

I have been asked to be on the parent advisory committee for the new Center for Rare Childhood Disorders.  Tonight Denise Hauer and I attended the first meeting. Here I met and talked with the scientists who will be doing the analyzing.  I'll keep you posted!

Watch this amazing video to see what our wonderful neurologist, Dr. Narayanan, and TGen are doing to help children with rare diseases.  I am so excited to be a part of this team.



Today when Annalise was eating her lunch, I handed her cup of milk to her and ask if she wanted a drink.  She instantly lifted her dress, stuck the straw to her belly, and said, "I'm drinking it from the tube in my tummy."

She then gave her butterfly a drink through his tummy as well. 

We are working hard to keep Annalise from needing a tube in her tummy, but if she ever does need one, she will know exactly how to use it!  

Without Them

We went to Schnepf Farms tonight with some friends.  We have gone to the Pumpkin and Chili party almost every year for the past 8 years.  I loved spending time with our friends, but tonight was tough for me as well.  Tonight was the first time in 8 years that our entire family was not there.  We left Bryce and Annalise home because we knew they could not handle the heat, they can't ride the rides, run around or even eat the food. They would not have enjoyed it.  That makes me sad.  I missed them the entire time.  I hated seeing other kids their ages running around and playing in the dirt, hay, and grass.  Usually it doesn't bother me so much, but tonight it did.  It just wasn't right without them.

Annalise dancing to the music at Schneph Farms October 2010.  

Moving Forward with a Blended Diet

I can't believe I haven't told you about this yet.  As I mentioned a few posts back, I was taking Bryce down to meet with Marsha Dunn Klein.  Well, we went and the visit was amazing!  We met with Marsha and Ellen, the nutritionist that works with her.  We talked for a couple of hours.  We found out all about doing a blended diet and set up a plan to get Bryce moving in that direction.  While we were there, Marsha even asked me to write up our story about feeding Bryce his "green love"  (green smoothies) and the difference it has already made in his health.  I still need to write that up!  She is planning to use our story in her pamphlet that they hand out at her lectures.  She lectures all over the world.

Marsha also told us that of all the places around the world where she teaches people about using a blended diet with tube fed kids, Phoenix has been the most difficult to get anyone to listen to her...as far as doctors and nutritionists.  They aren't even open to talking to her.  She said she believes it is because some parents up here have tried a blended diet on their own (because they do not get the support from the doctors here), their child has had problems or become more sick, and then they go back in to the GI doctor for help.  The doctors have seen these kids who have become anemic and had other problems and blame it on the blended diet.  She said that she really wanted to work with us so that we could be a success story and help the doctors here see that it is a good thing...if it is done correctly and with supervision.  I am all about that.  I have known all along that I did not want to go out on my own with this one.  She of course fell in love with Bryce and seems very excited to help us make this work.  I came home very excited to start the process.

The first hurdle we had was the appointment with our GI doctor.  I love the GI doctor that we have right now, but I knew that he was not thrilled about a blended diet when I first brought it up a few weeks ago.  It took him a while to even be okay with me feeding Bryce a green smoothie.  So, I wasn't sure how he would take the news that I was now also working with Marsha and her team, and that I will be working toward a blended diet for Bryce.

We went in to the appointment and Bryce had gained a bit of weight which was good.  Dr. McOmber checked him over and said that everything looked good.  We discussed the amounts of formula and water (and green smoothie) he is currently getting.  Then he began to talk about where we wanted to go as far as his feedings over the next few months until Bryce's next appointment.  That was the time to let him in on our little plan.  I told him that we had been down to see Marsha Dunn Klein and he instantly rolled his eyes.  I tried not to laugh, because that was pretty much the reaction I was expecting.  I said, "oh, not good, huh?"  Then he told me that it isn't that he has anything against her, but just that she is not a medical doctor and he thinks she often pushes too fast.  He said that he would have to show our plan to his nutritionist and make sure they think it is okay.  I assured him that I want to continue to work closely with him and the nutritionist...along with Marsha and her team.  I don't want to cut anyone out.  I want to do what is best for my son and keeping track of how he is doing in all areas is my number one concern.  I am totally aware that this may not work for Bryce and if that is the case, we will go back to what we have been doing for the past 3 years...but I have to try!

Dr. McOmber still seemed concerned, so I showed him the plan for our first step of transitioning Bryce to a BD.  He looked it over, and started to get excited.  See, our first step is to just get Bryce off of his night  time feedings.  To create more of a normal "eating" schedule for our 5 year old son.  We didn't mess with the number of calories or add any more real food to the plan...yet.  He totally changed his attitude about it and said that it sounds like a great idea and at this point he doesn't even need to talk to the nutritionist about it.  He will want to once we start adding food in, but for now, he was very pleased with what we are doing.  That we are taking it slowly.  He wished us luck, told us if we had any questions to call.  And then he said something that made my day.  He said to tell Marsha that if she ever wanted to talk to him about what we are doing, or to ask any questions, to just give him a call!  YES!!!  Success!

I was so excited!  I meant to call Marsha right away, but still haven't.  We go back down to meet with them again a week from Friday.  I will take Dr. McOmber's contact information and let her know that he is now on board with what we are doing and very much wants to be a part of it!

It has not been easy changing Bryce's feeding schedule around.  It has had to be done slowly and I had to watch for signs of intolerance or aspiration.  He has had a couple of challenges along the way, but for the most part, he is handling the change well.  He is now given 4 "drinks" of water a day that take about 10-15 minutes each time, one serving of "green love" and 4 "meals" through the day which take about 40 minutes to get into him, plus the prep time.  There were a few days that I was very overwhelmed by it all, but things have smoothed out a bit and we are finding our flow.  Having him on Fall break and at home has helped a lot.  Hopefully I will be able to get his Para at school on board pretty quickly once he is back.  I'm not worried about that though...they are great at his school!  It's a lot of work, but I honestly think that it will all be worth it for him.

I have more good news from today, but it is late.  It will have to wait until tomorrow.  Thanks for reading and thank you always for your prayers!  Good night for now!

Latest Test Results

Yesterday we got the results from the SAMHD1 deletion testing that was done in Denver after our trip last April to see Dr. Vanderver.  It was normal.  So, two different tests looking for deletions have now come back normal, specialists in AGS aren't convinced AGS is what is causing our children's regression...the questions continue.  Could it really be that Bryce and Annalise are just carriers of this rare disorder and their symptoms are coming from something else?  It just seems so unlikely, but who knows.  Well, I know someone who knows, but for some reason, He just isn't telling right now so we will have to wait a little longer!  It is just so hard not knowing...not knowing what to research, what to look for, what to plan for, etc.  I'm really beginning to wonder if we will ever know.

Once I heard the results, I emailed Dr. Narayanan and let him know.  He said that he is still waiting on results of the genome sequencing and will let us know as soon as he hears anything.  He also asked how Bryce and Annalise are doing.  Overall, they are doing pretty well.  Bryce has been having some issues with croup and his breathing, but nothing too serious (meaning we haven't ended up in the hospital) for now.  We are hoping to keep it that way!

Annalise continues to heal.  She finally slept through the night last night without pain medicine!  That is a huge step in my book!  She still asks me to take her boots off, but she is learning how to get what she needs/wants without the ability to move around.  She is a trouper!  Her eating, or lack of, and her speech have us pretty concerned at this point.  It is just so strange, it is like she has almost no appetite at all.  She will eat some, but only when we distract her with something else and we feed her.  It will take up to 45 minutes sometimes to get a small amount of food into her.  She is still drinking okay, but is not getting nearly enough liquid into her. It's kind of scary.

She has also become much more difficult to understand.  She still talks up a storm, but more and more she is slurring her words and we are all finding it hard to understand a lot of what she says.  It is the worst when she is tired.  She gets really frustrated when we can't understand.  She has begun using signs a lot more lately.  I need to get back into teaching/learning more of those so that she can get her message across a little easier.  I'm still hoping and praying that the medication she was on after her surgery is to blame for some of this change and that she will gain it back soon.

Oct. 30th will be a great day!  That is the day Annalise's boots will come off.  We are all looking forward to that day!

I Believe in You!

My son, Jacob, was just watching a Pokeman video and at the end this song came on. It made me think of so many of you...my friends, my family, my Leukodystrophy family, my church family, all of you who support us even though we have never met, and my Father in Heaven. I had to look it up and post it here for all of you to enjoy.  What a great song. Who would have thought something from Pokeman would ever touch my heart! :)  Okay, back to work.  Thank you all!

Trip to Tucson

Annalise continues to recover.  We came home Friday evening.  The first night home was great.  She even slept through the night.  However, the nights since then have been a different story.  She does pretty well during the day as long is she is on the pain medicine, but at night, the muscle spasms seem to continue to come right through the meds.  She doesn't want me to leave her side, so we are spending some quality time together reading, coloring, and playing with toys that she got from my wonderful friend Bobbie and my Mom!  If you want to read a more thorough description of how she is doing and see pictures, feel free to check out our family blog.  I will be posting more there as I get some time.

Today, Annalise will be spending the day with Denise, one of our respite workers.  Hopefully she will do well.  I will be heading to Tucson with Bryce to meet with Marsha Dunn Klein to learn more about what type of blended diet will work for Bryce.  Laura Hinners, Bryce and Annalise's speech therapist, will be going with us.   We will be meeting with a nutritionist as well.  Although I wish I did not have to leave Annalise here, I am really looking forward to meeting with Marsha and learning from her.  It is a great opportunity!

Annalise's First Surgery and Hospital Stay

Wednesday morning Annalise and I headed up to Phoenix Children's Hospital nice and early for her surgery.  They had called the night before and asked us to come 2 hours earlier than we had planned, so I made a few arrangements and got to PCH by 8:30

 Our home away from home for a couple of days. 

All ready to go see the doctor 

Annalise was excited to get her "bracelet" that had two princess stickers on it. 

With Dr. Duck.  They gave her this animal when we checked in.  The man who gave it to her said it was a goose.  She said, "It's not a goose.  It's a duck!"  Dr. Duck 

 Wearing pants for the last time in 6-8 weeks.

I had to take pictures of her cute, soft, little legs before they got covered with hard heavy casts. 

 We then spent time checking out all the toys.  

Annalise's new friend, a very sweet little girl who was waiting for her surgery also.  

 They played Candy Land and then tea party together.   So cute!

Once they called Jersey back, Annalise played with just about every other toy there was. 

We actually ended up having quite a while to play with toys because Dr. Shrader had two ER surgeries that came up.  Finally at about noon, only a half an hour before our originally scheduled time, the nurse came to get her.

 She had her dog (Mya) and her duck ready to go with her.  This was the hardest part for me...always is.  I walk with Annalise and the nurse to a door.  Then I give Annalise a kiss and they head one way and I head the other.  

The surgery lasted just over an hour.  She did great.  She woke up for about 2 seconds in the recovery room  

and before long they had us up in her room.  Room 8147.  This is the first time that we have been in the new part of the hospital.  It is so much nicer than the times we have stayed with Bryce.  The rooms are much bigger, we have the room to ourselves, and there is a bathroom and shower that I can use as well.  So nice not to have to walk down the hall every time nature calls!

The view from our room is also pretty nice.  

Annalise slept until about 3 pm.  Then woke up and was ready to play.

Aunt Sheri came and brought Annalise a balloon and a book.  Annalise enjoyed playing peek-a-boo with Aunt Sheri. 

Annalise also loves holding on to the balloon and then letting it go and watching it go up to the ceiling. 

Also, my friend Ann stopped by to say hello.  I met Ann and her family one time when we were staying at the Ryan House.  She is here at the hospital with her son Jack.  She is the one who I first heard refer to this place as hotel PCH.  I like that!  When she came by, she gave me this necklace.   I love it!  

After work, John came by to stay with Annalise so that I could go to Ryder's End of Life Celebration.  It was beautiful.  Yesterday would have been Ryder's 3rd birthday.  It posted sideways here, but this is the program they had there.  Denise wrote the poem.  While at the celebration I saw many mutual friends.  It was a lovely night and I am glad that I was able to get away for a bit and be with Les, Denise, and their friends and family to honor such an incredible little boy!  

 While I was away, Annalise colored with her Dad.  

Also, her pain medicine began wearing off and she began having some pretty bad muscle spasms.  She threw up twice, once with John and once with me.   

Annalise's boots.  She choose pink and the lady who casted her decided to add the purple around the bar.   

 She keeps telling me that she wants her boots off!  It is so hard to see her so uncomfortable.  

 My poor baby had a really rough night the first night.  They ended up having to give her Morphine in addition to all the other pain medicine she was already on.  

It did help though and finally she was able to get some rest.  

It is now about 3am, Friday morning.  Annalise woke up about an hour ago in some pain, but other than that she has had a pretty good night.  Luckily they are keeping on top of giving her the pain meds and that is helping a lot.  I really like the night nurse tonight.  Not trying to be mean, but in my opinion she is much better than the one we had last night.  There is definitely a difference between nurses!

After getting Annalise settled back to sleep, I have not been able to get back to sleep, so I figured I would get up and finish off this post...then hopefully get a little more sleep before the sun and my daughter wake up.

Annalise woke up yesterday morning around 9 o'clock.  I figured she would take a nap during the day, but she ended up staying awake until almost 8:30 last night.  She had a few rough/painful moments through the day, but overall she seemed pretty comfortable...relatively speaking.  

Regression is always a concern with these kiddos because of the Leukodystrophy.  The only thing so far that I have noticed with her since her surgery is her speaking.  She is still babbling away just as much as she was before, but I am really having a hard time understanding her.  I hope and pray that it is just because she is on medication and that soon she will be back to her normal.  

 Watching some t.v.  I think that keeping this girl entertained is going to be the toughest part of the next few weeks.  She doesn't really like to sit and watch t.v. or movies a whole lot, so I am going to need to get a little creative.  Today we spent time reading books, coloring, and playing with some  toys.  

 We even got a few smiles out of her today.  

 She also discovered how to raise and lower her bed.  

She thought that was pretty entertaining.   

She also took lots of pictures with my phone.  Most were pictures of the t.v. 

Our view at night.

We were hoping to go home on Thursday, but they decided to keep her one more night to make sure she made it through the night without the extra pain medication or any more throwing up.  So far, she has done well, so hopefully we will be heading home later today.  I have friends who have had to spend many days/nights at a time in the hospital with their children.  I admire them and their strength so much.  We've only been here two days and I'm already going stir crazy.  Thanks to everyone for the visits, texts, prayers, and well wishes.  We have felt your love and thank you for that!   The prayers have been especially appreciated!  The strength and comfort that comes when so many people are praying for you is impossible to describe.  It has been a huge help to my baby girl and this very anxious mom!