Big boy car ride..video of head control post mestinon

Ok, we've never been able to have Luke ride in little cars where there is no support or especially trunk support. Well, this past weekend we tried his big boy car and guess what? He's doing great in it and loves, loves it! YIPPEEEE

Now, your going to die laughing when you see the video below as it will totally contradict the picture below. When we put Luke in this car for the very first time......this is what we got....

Huge big boy car melt down

Daddy....get me out of this....what are you doing?
See video below...long story short: Melt down went away within minutes! Happy boy!

Make an on-line slide show at www.OneTrueMedia.com

Look at me! I'm driving my car.

Daddy.....keep going!
Mommy gave me big kiss after my big boy ride!
Thanks for lookin. He's just so funny now isn't he? We are loving all of it.

MELT DOWN's and all.

Dallas world aquarium...head control amazing!

Yes, we actually went this am. (Sunday) Lulu...we decided to go. Luke was thrilled to be there.

Yes, Yes, Yes I know family. We are still keeping Luke away from everything....but I cracked. It's the only place that is AC'ed and not HOT and where Luke could enjoy a 'zoo' atmosphere.

My intuitive mother instincts proved correct, LUKE LOVED IT. We went when there was little crowd..but it got super busy around 11:30am and we left quickly. Good news is...it's wide open and very airy.

We took pics and video taped. The video tape is long and it's ok if you don't finish. I just want it for historical purposes. Can't wait to scrapbook this theme Deb. So fun.

Make an on-line slide show at www.OneTrueMedia.com

Leaving out....baby is tired! Thanks for viewing our MAJOR adventure with us. I wished we could do more of this stuff because Luke is just soo good. And, mommy and daddy are really wanting OUT of the house. Hugs, A

Luke has gone bolus..

Ok, in the tube feeding world there are terms that I've had to learn. One of which is a term called "Bolus feed". Meaning, you pour the formula in a syringe and gravity the entire feed in over a 5 minute time frame. That's called Bolus.

So, in the baby food feeding world....if you put a drip of baby food on a spoon that's called "a drip". (lol) But, if you dip that puppy in the jar and pull out a whole spoonful...that's called a BOLUS spoonful.

So, after giving Luke the mestinon and trying baby food with Vital Stim...guess what? BOLUS SPOONFULS of baby food with ZERO SUCTIONING! He's swallowing the food people. My therapist is amazed and says he's double swallowing now and she's convinced he's really swallowing. If this gene is 'the one'..then we are scheduling a modified barium swallow test like SUPER QUICK.

If he passes it......you guessed it. BABY FOOD TIME. I can't even begin to think what that will be like. Crazy isn't it? All I've ever known is a gtuber. Wow? Pray loved ones

The food of the day was "SQUASH". Yummmmyyyyy. Luke loves it.

Peek a boo---post mestinon

Unbelievable...post mestinon

I can't say much. It's just unbelievable.

Sitting up more than ever in stroller...to the point where he scoots all the way at the end and swings his legs! Video coming!

Luke is smiling so big....it's all I can do but not cry...we are in awe

Our child has never held his entire arms UP so high. Not only are they up high...he's holding a folder!
And..to top off this miracle...he's holding up the folder with his arms AND LEGS! OH MY LORD!

Ohhhhh, Jesus. Abba...it's hard to speak.

Quite expressive now...post mestinon

Very wide eyes, scrunchy face and side profile view of his sucking his lip and making funny noises! So cute.

Overnight milestones - post mestinon

I have to post these overnight milestones. We got home from the Mayo late this afternoon.

We put Luke on the living room floor. (STARTED ROLLING)

We put Luke in the bath. (SO CRAZY and SPLASHING that bathtime was cut short and momma drenched)

We put Luke in his stationary play exersaucer (TURNED AROUND FOR THE FIRST TIME!)

Here is a glimpse of him turning around!

big talker and loud - post mestinon

Luke is very loud now. Takes after his momma!

Day 4 EEG appt. and last day

EEG at 7:30am.

Baby cried and daddy held him entire time. They had to sedate him briefly....but all is good. (EEG TEST WAS NORMAL)

Set for the last appt. with the Neuro this afternoon.

Everything is packed....if all goes well....then we are off to Kansas City tonight.

Then....By Friday night....we'll be back in.....

Be back with closing pics!

Love ya'll

Day 3 observation evening

Many of you know...we have nursing. So, this post is for Wanda and Megan

Why?

Cuz ladies....no more leaving Luke on that couch! LOL!

Watch this....he almost rolled off the hotel bed. Kinda freaked us out. Off to Lowe's when we get back to baby proof the house. (God willing)

Megan: We got your message honey. Thanks soo much. Your work on Saturday was most needed. (thx for ignoring our drama) Hugs.

Day 3 observation Afternoon

Check out this video below. He's reaching and grabbing things. WOWSA! These are little miracles right before our eyes. Please pray that this is THE MED. that fits the gene that is suspect. hugs again!

This is just so fun. We find ourselves wanting to take long walks with Luke today because it's just amazing. The little things are really becoming so joyful for us.




He's looking left and right at all the cars and people. It's been so fun to watch him as he stares 'down' some people all the way down the street and as far as his head will turn. LOL!
Little Luke going left to right!


Here's a glimpse of how straight his backline is. It's amazing.

Day 3 observation amazing

Ok, can you say "STROLLER BOY" sitting up BIG and TALL.

In the past, Luke has been able to sit up for a brief period in his stroller and then after 2-3 minutes (literally), he would fall back. All day on Monday, he sat back in his stroller as we walked to and from the Mayo Clinic.

Today: We set out for breakfast and a walk. From the minute we put him in his stoller he was able to sit up and KEEP THAT POSITION with us pushing him and turning him. (this is amazing) Typically, if we strollered off while he was in sitting mode...he would fall back. He's never been able to maintain a 'balanced' sitting position in walking mode. EVER.

So...By God's grace in inventing this drug......watch this! *turn your volume up...you can get a glimpse of us desperately trying to act normal for the camera. he! Hugs

Other exciting stuff:

1. No secretion issues....Praise God!

2. A increase in motility...meaning, no problems with constipation! Yipppeeee. (trust me...like clock work..we get ready to leave the room and then a huge smell comes over us.....back to room, fix the problem...then back out the room! he,he

3. Verbal talking. Dah,dah....bah bah, and yah, yah (our therapists will freak!)

4. Playing with toys aggressively! I'm talking throwing across the room. (so funny)

Ok..that's it. thanks for joining us again today.

Love you!

be back

Day 2 observation by Neuro

Ok ya'll....huge. (video footage below)

After Luke's nap and 1 hour after the 2nd dose it was temper tantrum and wild man central. He was a wild man. Verbally and physically. The neurologist was amazed and very impressed that Luke responded to such a small amount. She started him super low. His neck control was sooo evident. And, she said "I can see him today". Luke has a huge tendency to lower his head when tired. However, he would look up at her and then turn his head left and look right at me. Amazing.

Currently, we are experiencing like itching of his eyes, and rubbing his head, his feet, etc. Not sure if he's just got more strength that he feels this now? The Neuro asked if that was normal and I said that he usually rubs his eyes a lot and have never been able to figure that out. However, he's been super alert. On High Alert. Like....staring at people with these wide eyes, and has a huge expression in his brows, mouth and eyes. The Neuro noticed that right off.

Ok..UPDATE NOW:
1. Our Neuro cancelled the EMG. (Glory to God..enough said!)

2. We went down to the blood labs to get Luke's blood. Anyone want to take a guess as to the outcome? YEP----Only got 1cc! Meaning, they only got enough to test that one gene that is suspect. Everywhere we go...we tell them "he's a tough stick". Sure enough....they did all they could. So, our Neuro said it's just enough to test that one gene they really believe is the cause.

3. However, this is where it gets complicated. Luke's blood has to be tested FIRST and then ours. We thought she said we would know tomorrow about 'me and Jonathan'. We misunderstood greatly. They have to test Luke's DNA first (which takes 5 weeks) and then ours. If Luke's gene is not the one they suspect, then off to do more blood work for Luke at Children's in Dallas. (ohhhh, I'm tired already)

4. Now, to make things more complicated is the following: Some CMS patients respond to medication and then DIVE (meaning...it stops working overnight-usually in 1 week or 2weeks) and at this point, they know it's not the gene they suspect it is. What does this mean? Means since Luke's testing will take 5 weeks....we may find out based on his symptoms if it's not that gene prior to the final diagnosis of the suspect gene. (NICE HUH?)

I hope this makes sense. It's gotten a smidge more complicated today. We felt pretty clear yesterday and now we are in the 'waiting mode' again.

I did give him his last dose of the day just now. Jonathan is getting us some din din. So, be back with more results from that. All in all, we are feeling very good about it.

We are scheduled for a '2 day trial' and it looks like we may go home Friday AM. Yippeeee
We will know more tomorrow. Tomorrow we have an EEG scheduled per the Neuro's suggestion. So more info tomorrow.

Huge hugs and thanks so much for the comments. We love ya'll. I am off to post to this P2P lady that has a child that's on Mestinon. And...our CMS friend emailed me! Yippeeeee

Video footage below...enjoy. P.S. I asked our Neuro permission to post her on our blog. She said 'no problem'. We love her!!

Day 2 observation of med

Ok, we walked to the Starbucks 2 blocks over. Definitely we can tell.
1. WIDE EYES---like..super expressive wide. That is huge!
2. HUGE TEMPER TANTRUM----I mean HUGE. Jonathan walked out with coffee, and Luke saw it and started banging his head back on the stroller really firm. It scared us and Jonathan had to get him out.
3. His scream! He screamed at the top of his lungs......it frightened us.
4. His cough. He coughed several times and we've NEVER heard a loud or aggressive cough come out of this kid.

We are back in the room and we are freaking out because we do notice the little stuff. Of course, we are worried it's just psychological because we are dissecting every sound, noise, move, you name it.

One more dose at 1:30pm.....I'll blog post that appt.

Oh ya'll......could it be our baby is being healed through meds? Oh Lord...could it be?

Crying now....gotta go.

A

1st day at the Mayo

Oh wow! What a day. We literally just got in and it's 7:00pm. We are exhausted. I'm just going to get right to it. I'm going to put some medical details in here as I'm hoping our CMS friend "K" will be reading this.

Ok, we have nothing but great things to say about the Mayo in EVERY WAY. They are amazing.

Now, our appt. with Dr. Duygu Selcer (from Turkey), Pediatric Neurologist, was right on time. Amazing. This lady was fantastic and asked so many questions that we were ready for her to finish. After about 30minutes of asking many questions, testing Luke's reflexes and watching him interact with mom/dad....she called in Dr. Andrew G. Engel.

Now, Dr. Engel was in his 70's and was just super. Aside from being super knowledgeable..everything he said just permeated the room. He was 'experienced' with CMS. We asked tons of questions and he was so patient with us and laughed with us and was so delightful.

Ok...I'm going to desperately try to bottomline this. Here's what he said. It takes 1 gene from me and 1 from Jonathan to make CMS happen. They had to take a blood test from me and Jonathan at that very moment to be sent off to the labs and believe it or not.....they will know tomorrow what gene it is! (she said it used to not be like that..but technology has flown in!)

Jonathan got weezy and I told him to suck it up! (LOL) And of course, I flew right through it. (big smile)

Ok, so LUKE. Dr. Engel explained that synaptic or post synaptic CAN BE TREATED. He said that it's 'trial and error', but that most forms can be treated.

Now, there are 2 medications that they want to try on Luke to see if he responds. Sorry y'all this is just so confusing. They said there are 2 ways to determine the DNA from Luke and what will work and that is the following:
1. See if he responds to the medicine #1 first...if so, then they know what type of CMS it is.
2. If he doesn't respond to medicine #1, then they proceed to another EMG with sedation along with medication #1 and/or #2. (they determine that after clinically observing him after medicine #1)

Medicine #1: Mestinon ("K"...this is your drug)
Medicine #2 and please note...this is not FDA approved yet..but they are currently treating CMS patients with it and with great success. 3.4 draminopyridine is Medicine #2.

Ok, so, the plan:

1. We got the prescription tonight for Mestinon. It's in our room and in liquid form.

2. At 9am tomorrow morning, we give him Medication #1 (Mestinon) 1ml through his gtube and watch him. Yes, that's right. Me/Jonathan sit here and watch our child. Dr. Selcen said it takes literally 1 hour or less and we will either notice a huge difference in his physical activity...or, we won't.

3. At 1:30pm, we give him another 1ml of Medication #1 (Mestinon) and then we proceed to Dr. Selcen's office at 2:30pm and she wants to clinically observe Luke herself.

Now, at this point...if Medication #1 Mestinon works, then we scrap the EMG that is set for Wednesday as they will determine exactly what strand Luke has with this result as well as our blood work and Luke's blood work.

4. If the Mestinon does not work (and we are praying IT DOES BLOG FAMILY) then we are set for an EMG with sedation AGAIN on WED. am. At this point, they will have to do further testing to determine exactly how to treat this.

They (both the neuro's) mentioned that they just saw a 15 year old patient (female) and that is totally active and productive with taking BOTH MEDS (med#1 AND med #2) at the same time.

NOW --- THE PROGNOSIS: We asked the question. Can Luke live out a productive life and will I see grandchildren: Dr. Engel (the elderly doc) said "I feel very good about saying he can...but let's see how the next 2 days go with his meds) GLORY TO GOD.

THE OTHER QUESTION: Can we have more children? Dr. Selcen said there is a 25% change of our next child having it.

And, it's super SLIM that Luke's children will ever get it. (almost 0% chance)

Also: personal note to "K"---yes, they have heard of Ester neurosciences.

wow.

Ok, is this clear at mud? Or are you more confused? I hope I explained this and we are asking for great results tomorrow.

And yes, we will be video taping Luke if we start to see radical results. We are excited.

Ok loved ones....signing off with pics. Love you and may I just say thank you so much for riding along this awesome journey with us? We love you! So much.

Our lobby within a huge lobby before going in....

Another view of Lobby
Me and baby waiting for the prescription of Mestinon
Dad/baby
The big Mayo lobby that carries the little lobby's! LOL! It was beautiful. Atrium window looking up!
Attempt to get a cool pic.
Outside of big lobby...garden area
Dad/Luke
Big lobby view

Ok! See you tomorrow....and I know your praying...love you again.

A

Mayo Registration

Hey loved ones, top of the morning to you! All I'm going to say is thank God for Jonathan's Hilton points. We've got 2 nights here free of charge and can you say "major comfy beds". Wowsa. We all 3 slept soooo good. Woke up happy, chipper and little mister talked up a storm.

Luci loo got me this devotional daily reading book. Guess what my verse was today..?

2 Timothy 2:24

"The servant of the Lord must....be gentle"

(Thx sister) Thought that was appropriate considering our road trip attitude coming up. Thank you Lord for that sweet reminder and help me have a gentle spirit when things are going the way I expect. I so need to work on that dear husband. (thanks for letting me vent blog family...know my heart is one of willingness) big smile. (by God's grace for sure)

OK: We strollered out of the hotel, down the street a few blocks and there is this huge atrium where all the Mayo building's connect. It's actually easier to walk down the street in fresh air than to take the sky bridge. So, that's a great thing for us as long as the weather is good. (pics later of this area..it's very pretty)

We found the Starbucks right in the atrium across from the Ganda registration bldg. Convenient! Me/Luke hung out on the outside while Jon ran in to get us some coffee. Then, strollered to the registration desk (huge, huge area) and here's how it went. Waited in line 2 minutes, handed them my appt. card and she said "Ok..your all set". Wow? That easy? She said I took care of everything on the phone. I said "I did?"...and she said "yep, your set, see you on the 9th floor at 2:00pm". Oky doky then! Off we went back to the hotel to rest.

Me blogging and the men vegging out. We are waiting for our 1st appt. this afternoon.

Hi..
Luke, bus and sister
View of our hotel room...yep..a clutter bug mess
Luke is watching his video. Jon figured it out! Jon reading and me blogging.
Be back...love ya'll.

We made it!

Hi Blog family, whoosy. We made it! Ok, here's the bottomline.

Departure time: 3pm Saturday

2 pitstops

Arrived in Shawnee, Kansas at 11:45pm - in room by midnight

Ks. departure: 9:00am

3 pitstops

Arrived in Rochester, MN at 4:30pm today (Sunday)

This is me earlier in the car in the pic below. Why do I look like I'm about to go crazy? Well, here's the only thing I can come up with. When we got to Iowa..we saw the welcome sign that read "Welcome to Iowa, the field of opportunities".

Ok...enough said. ALL FIELDS, ALL FLAT, NO FUN STUFF, NO FUN ANTIQUE SHOPS off the hwy..just corn field after corn field. LORDY!

An opportunity to look at fields. Thanks Iowa.

I just took these pics. The time span of Luke playing with sister Cassidy and the picture below where he fell asleep when we got to the hotel room was LITERALLY 10minutes! I was shocked. Baby tired!!
Night Cassidy....asleep!
Ok..private reality now that I'm in the hotel room and having a glass of champagne. Yes...me and husband wanted to put some much needed romance in between this trip and not sure about the romance today, but hey! Jon is currently downstairs seeking out a warm meal down the street from this home cookin cafe joint.

So...me time and blogging about it!

Well, the trip honestly ya'll...was soo smooth. It's literally nothing but highway! Hwy 35 North and UP and then BAM..your here. It's amazing.

And, Jonathan and I are just giving Glory to God and your prayers because we are shocked at how good Luke did. NO CRYING EVER. Sadly, it's confirmed that he's a video junkie because he just loved his video's all the way up. It was great. He didn't want to go to sleep ever..but never cried about it. He would just sack out whenever. It was great. He's the perfect child and we were thrilled about that.

Now..me and Jonathan. I wished I could tell you we had this 'grand' trip and talked about romantic stuff, laughed and enjoyed each others' company. Uugghhh, nope, didn't happen. Maybe on the way back. But..no go on this tripo to the Mayo. (LOL)

Honestly, we were at each others' throats most of the trip. It was a bummer deal. I think it started off with me feeling like he wasn't working fast enough, had plenty of time to do stuff earlier..but didn't and then him negotiating whether we should take the stroller or not? Honestly, isn't that baby 101? I mean...I could not get past the fact the he thought the stroller had to be cut out of the list? As Priscilla Presley was quoted after being asked by Oprah Winfrey how she thought of her marriage with Michael Jackson now that she's older...she quoted "HOLY MOTHER OF GOD".

Yep..that was me. Holy mother of God. Are you serious Jonathan? You really think leaving the stroller is a good plan. It kinda went downhill from there. I was annoyed because he decided to put his headphones on and tune out to music while I was in the back with Sesame Street music and the baby. I commented on that and of course.....'that went over well'.

So, then of course, I backseat drive and I think he swerves into other lanes and I had to beg to drive today because he wanted to drive the entire way and he retorted back that I take better care of the baby. Good answer..but translation: I want to drive and listen to great music, you can get all the feeds ready, meds, change out the baby dvd's every 30minutes and attempt to get any sleep because the baby wants another toy.

Side note for P2P ladies: I really thought I was brilliant and bought those little baby ozarka waters. Note to self: 60 cc syringe does not fit therefore forced to tilt bottle ever so slightly as you are pulling water out and then spilling it everywhere. Nice.

At any rate, I did my best to make Jonathan feel guilty and he conceded. I drove about 3 hours today in bliss listening to music w/headphones and loving it while Jonathan and baby were both asleep in the back. Isn't that the timing? Why can't baby sleep when I'm back there.

Here's my point: The job of a wife and mother is very hard. I believe we do most of the work and we just have to accept it or pray about it and hopefully an act of God will happen sooner than later. Maybe I'm wrong and some wife will comment on my blog and say "well, my husband does just the 'even' amount". Ok..praise God. But, for the sake of percentage here..work with me. Am I going crazy here?! Ok...enough said. Just wished it wasn't so hard sometimes.

Now, on the upside...we are here and the beds are comfy and we are in better spirits. Luke is fast asleep and Jonathan is back and all is well. We don't have a dvd player in the room and Jonathan's task tomorrow is to figure out the car DVD portable player to work in the hotel room. My task is the administrative part (again the hard stuff) and Jonathan and Luke will get to play in the hotel room all morning. So, in my rebellion, I will seek out the nearest Starbucks and use my gift card from "H" and get one and be very shhh about it! He,he.

Got the 9am registering to do tomorrow at the Mayo.

Dr. appt. late noon.

Blog then,

Love y'all so much,

A

Departure Day for the Mayo Clinic

Prior to departure yesterday for the Mayo, Luke and Lulu were playing a game. It was funny. She finally caved in and gave Luke her necklace. Gotta keep the peace before the long roadtrip.

This is Lulu. (my mom) Some call her Penny, some call her Hope, I call her mom, others call her chick. On this particular day...we shall call her "Prissy Pot". She so is!

Please note: Great eye pic of Luke. It's hard to catch his eyes from blinking!
Lulu giving Luke a kiss goodbye
She'll never admit this..but she posed with a slight tilt of her head.
Ok, this is the packing up part. It's probably a very good thing that 24 hours has gone before this blog post. Mainly because it's better to hold off writing any post due to stress. I was stressed out during this picture.

Right about this point...my husband quoted the following: "we have too much stuff, we need to leave the stroller, I'll just carry Luke".

I'll blog about this in a minute.

See our Minivan from Enterprise! Isn't it beautiful! (*we were so excited)
Departure picture taken by Lulu. Fake smiles. (trust me....a total posed shot) Like anyone can smile while packing, departing, screaming at each other? No way. We opted to fake it for the blog pic.

See Luke's DVD player! He was jazzed.

Ok, the time of this picture is about 7pm. We were getting tired already.

We brought toys. Luke wanted the ziploc bag from me.

Pitstop: Braums!

Braum's break. Luke was driving and Jonathan was eating Beef Jerky. (note: beef jerky is the greatest road trip food!)
It appears that we are just vegging out. But, in reality...my child just got laxed and was constipated. He needed to poop. He does much better in this position. (TMI)

P2P Girls: You know the gig - feeding bag on the go for Luke's button.

Yes, the yellow bus had to come with us.
Be back!