Update

Lots has been going on. We had an MRI of the brain and spine to see if the Chiari is getting worse. Its recommended mri's every 6 months. While that is alot of radiation, so much can change in his head in 6 mo. So its a decision that is hard to make. With Jase still not making much progress, we decided to see what was going on in there. Also from day 1 of this journey, drs. told us by 2, we will know whats going on with the myelnation.  So this is pretty close to 2 and Im anxious to see whats going on with mylenation.   Genetist also ordered an MRS. Different type of MRI to look at the brain tissue to see how its metabolizing things. (i hope that is right)

Well both MRIs (head and spine) looked good. Spine was totally normal. Brain remains unchanged. not better but not worse. The meylination looks just fine. So that is excellent news. The Chiari is still 9 mm and drs. are not convinced that Jases symptoms warrant surgery at this point. I am so glad we havent' had to go into that sweet, special head of his.

Until today.  As we went on a wonderful trip to California, I was able to hear and watch Jase sleep very closely.  I noticed he was gasping and waking up a lot, for no apparent reason.  He has also had 2 episodes of waking up in what seems like a night terror. Totally inconsolable. Which is VERY VERY out of character for him.

I captured this video of him sleeping.  you will notice that he stops breathing a lot, but not for too long. I sent the video to his Dr.  She was quite concerned because he is not in respiratory distress. That means there really is no reason for him to stop breathing so much. Unless something in his brainstem is causing the problem. Thats exactly where the Chiari is. Brainstem.  So she is thinking that Chiari is causing his apnea to get worse.

After Jase had tonsils out, his apnea had improved alot. He still had apnea, but dr felt good taking him off oxygen.    Now he's back on oxygen and more than before.  Its disheartening to  have this happen, because we had just gotten to a good place where I was ok with what the drs. were telling me.

Speaking of.....what the Drs. told me.   We had another appointment with the Geneticist last week. To review the good MRI results.  She also said that looking at everything she is confident saying that he doesnt' have a degenerative brain disease, or metabolic/mitochondrial disease. This was the biggest load lifted off my shoulders. I was so relived. I couldnt' imagine anything worse.
I asked well then what? whats going on? why cant' he crawl, or walk, or even make a sound other than hummimng and an ahhh here and there.  and hes almost 2.  She basicly said that Jase will be the equivalent to a mentally handicapped person.  He learns so slowly and always will. She also said he wont speak. He should walk, maybe not till 5 or 6, but he should walk eventually.  I was so relived to hear this news. I have known for along time that Jase will be mentally behind and will  need help his whole life.  So I actually left in great spirits.  

So now that this is coming along its another bump in the road that hopefully doesnt' lead to a brain surgery.  Its discouraging and upsetting.  Im SO over drs and tests.  and Im sure Jase is as well.

Now I Can did such a great job putting this video together. Just watch how hard he worked and how strong he  has become.  I cant' recommend Now I Can enough.  We are headed to the Now I Can 5k/1 mile run on Sat. I cant wait to celebrate Jases accomplishments.

 http://animoto.com/play/K6TqUmUNAUG1395Bn2CjsQ

Jase was so lucky to go have a 3 week session at Now I Can. It was so much hard work, with such great people. Jase gained some good strength and learned a few new skills:)  We are now going to do the Now I Can 5k walk run, with our whole family. It will be so fun. Can't wait to posts pics of that.

easter

Here are some fun easter pics. It was a great fun day!

Summary of Jase

Ok, so you know when you have those reality checks....like when you know something is just not going to change, and you have to accept the way it is? I guess that is what this post is....I guess.

Confession: I hate to update my blog, becuase its always heavy news, and putting it on "paper" is so final. You know. Like so final. If i dont realize this problem on paper, it wont exsist.

I think i am just going to summerize all of Jases symptoms and issues and see what happens. It might make me feel better....who knows???:)

Jases head:

Jases head is now 53.5 cm. at 16 mo old that is REALLY BIG!! So understanding our latest MRI report, and a 2nd opinion from radiologiost, Jase has a Chiari Malformation of 9mm. That is a pretty signifigant measurment. 9mm is alot. He has enlarged ventricles, Left more so than the right, but now they are in better porportion to his head size. What does that mean? I dont know. I guess since his head is huge, the ventricles are not as big as they used to be in comparison to his head?? Make sense...i know, me either.
He has an adult sized vein that runs along the top of the head. (not there previously at 6 mo.) The Vein of Troler. Yes very frightning, I am terrified of the risk of stroke or something bad. Our Neurosurgeon was so confident that none of this is causing a problem for Jase. He was delayed at birth, and so Chiari is not to blame. *sinking pit in stomach and i cant breathe....*
He DOES NOT have Hydrocephalus. One thing Neurosurgeon made clear.....NOT HYDRO. So I guess he does not have hydro.

****I have forwared on Jases MRI reports to 3 of the top Chiari surgeons in the nation. They all emailed me back the same night as my inital Email and are curious to see his records. Dr. Lazareff in UCLA, Dr. Frim in Chicago, and Dr. Dobyns in Seattle. I should be hearing from them each SOON*********

SWALLOW STUDY: Jase aspirates liquids into his lungs when he drinks. My amazing pulmo knew this from the first day she saw Jase one year ago, and we now have confirmation through a swallow study, and all the other symptoms when he eats. This could be from the Chairi, (that is known to have swallowing issues) or from Low tone? He did have Larangomalacia (floppy airway) but ENT said that has resolved itself. Anyhow, because he chokes, coughs, watery eyes and "wet vocal quality", lungs sound junky, and has to come up for air after every few sips, we know he is having trouble. We thicken his liquids, and hope he is ok with food. We just have to watch him carefully and hope it doesnt' get worse. If it does, the Chiari could be getting worse.

SLEEP APNEA: Jase had 1st sleep study in Nov. and it suprised me. He has a VERY high number of periodic Limb Movments. I had never noticed any movment with hands/arms feet/toes when he sleeps, so that was suprising to me. Although now, he twitches in his sleep. Fingers/hands move alot, and toes/feet and sometimes legs. A concern for seizures in his sleep. We will assess that at next sleep study, Mar 29. I worry that is getting worse.
Jase had 116 obstructive breathing events, 54 hypoapneas, 22 central apneas, and some use of using intercostal muscles to breathe. REPORT: moderate to severe level of obstructive sleep apena. Suggestion Remove Tonsills and Adenoids.

We removed "HUGE" adenoids and enlarged tonsils.

REFLUX

REACTIVE AIRWAY DISEASE aka ATHSMA:

6 mo EEG: slightly abnormal, intermixed slowing not clear of signifigance.

HEART: Murmur and slightly enlarged heart. Murmur was heard, so had EKG. One hospital said heart mildly enlarged, Primary Childrens reviewed and said it looks to be fine. I hope it is fine. I still worry about that. Murmur is innocent.

Jase was born with a Strange birthmark on his lower shin. It is red and blotchy. Seems to be lightning.

Found Low growth hormone in blood over this last stay. Something to watch.

Jase development: Jase has now reached "severe delay" in his reports. He is able to sit and roll. He can get out of sitting by droping his head, but is not able to get into sitting yet. He can stand if leaning on something. His leg strength has gotten SO MUCH STRONGER! He is still working on balance. and moving those feet to try to walk.
As of Last week, he will lay on his back and arch like a worm and slide backwards on the top of his head to try to get around. Its so cute to see him try to problem solve:)
He no longer says Ma, or gives Kisses, or Claps like he once did. That is troubling as he may be losing skills. That would not be good.

NOW I CAN: I am so excited for NOW I CAN! http://www.nowican.org/. They are going to help jase with everything. It is a 3 week session, 5 days a week, 4 hours a day. Because Jase has fatigue issues and lethargy, I am curious to see how long he can last. He has always had "hypotonia" but it seems to be getting better.

I am done.
love you all.

Jase Hospital stay

Oh my I guess it is time for an update!! Lots going on over here and keeping us very busy!

Jase was in the hospital for 5 days last week. He actually was in 2 different hospitals..here is how it played out. So since his tonsils/adenoids removed he hasnt really wanted to eat much, he will drink anything. So I would just gently push solid food, aka mac and cheese, with really no luck. He was slowly getting sicker. After debating and debating if he was really "sick" or just feeling yucky, and should I take him in, should I not....sunday afternoon we took him to instacare.

Instacare told us to go to primarys, he was probably dehydrated. We went up to primarys ER and they drew blood to check. The dr was suprised on how dehydrated he really was because he looked "ok." So we would need to stay overnight to get enough fluids to hydrate him. It sucked, Jase just loves to watch barney, so he was ok with it. For the first day:)

He continued to puke and be sick through the night, so once we got him to keep fluids in we could leave. I ended up leaving about 3 that afternoon.

That night, feeling better, not 100% but thats a hard thing to tell with Jase anyway. He didn't want any dinner, so I just kept him drinking. He had a bottle and went to bed.

Next morning, I went to wake him up, (not usual, he is always awake before us) and he was so sleepy. Barely able to open his eyes. So I took him downstairs and tried to wake him on the couch. No luck. Lips were white and he was so lifeless. couldn't lift head off my shoulders. So I told Jeff, "somethings wrong I have to go." I jumped in car and raced to riverton ER. Saw a cop in the church so I stopped him and said something is wrong with my baby....and hoped he would escort me up. no luck. He said drive safe.

So Im shaking Jases leg in the car and trying to keep him awake. 3 min went by and He stopped opening his eyes. I freaked out, grabbed my phone and called 911. I said I am on my way to ER, I am speeding in the middle lane with my hazards on, my baby wont wake up, what do i do? Dispatcher was trying to get me to stop, or something....I really cant' remember. But in my rearview I see undercover cop following my in the middle lane and lights flashing. I told dispatcher to tell the cop I am not pulling over, I have to go fast. It really looked like a high speed chase. I was a flippin mess. I finally pulled over on Bangater highway. Ambulance was just around the corner and they came and took jase to ER.

ER scene was crazy and people standing around everywhere!! they seemed so unprepared. trying to get an IV in him, no luck and he is still "unresponsive" I remember looking at Jase asleep and tring to get him to wake up. I remember saying to myself, what if he doens't wake up and I didn't say anything...so I just yelled to all the people, "Someone help her!!! he is not responding...HE needs an IV, I dont care if you put it in his head FIND A VEIN!! and HELP HER!" It then seemed like people started to work. I called my dad and just said Get up here now and hung up.
A nurse said to me, you are handling this good. I thought, wait what? what does that mean? should I be bawlling? Is this really bad? Is this not going to turn out well? I was a crazy mess inside.

Anyway after about 20 min Jase came around and started to have a "look" back in his eye. The doctor came in and said how scared he was for a minute, and Jase has a blood sugar level of 35. That is really low. Really low. So once we get his blood sugars up he should be ok.

They decided to admit him and monitor how his blood sugar levels go, and do some controlled fasts for some genetic testing.

That hospitial was amazing, I loved everyone there, LOVED IT. (as much as you can love a hospitial!) Dr. Langston was awsome and very through. Jase sugars dipped again to 35 that night and rose to 365!

He left with a diagnosis of Ketotic Hypoglycemia. We basicly just have to keep him eating steadily and not to much sugars and no fasting.

Long week for us and It was so nice to know that so many people are willing to help. Seriously at a drop of a hat. They had dinners, rides, anything. We are so lucky to have people that love and care about us. There is not a day that goes by that we dont thank heavenly Father for you all! Thank you

Im sorry I didnt' update after our Neurosurgeons appointment. It went OK. The Dr. doesn't think the Chiari Malformation is causing any problems for Jase. That really caught me off guard. I thought for sure it was. Some of the symptoms are mobility issues for the lower half of body, low muscle tone, sleep apnea, voice issues, to name a few and Jase has all of those. He also said that there is probably no spinal issues. His theory is that since Jase is globally delayed, that this isnt' the cause. It is something else. The geneticist and neurologist need to figure it out. He said that he doesn't have hydrocephalus. Huh? Im so confused, the radiologist said he did....oh whatever. So no new developments there, and while I am glad there is no brain surgery, I just wish I knew more.

Jases new Gait Trainer

I have been so excited to get Jases new gait trainer. I can tell he wants to be mobile so bad. When he sees all the friends run through the house, his legs start to kick and starts grunting. He wants to play so bad! Jase loves to race Jordan from the couch into the kitchen. The bad part is, I have to carry him and race for him, as he kicks his legs. Those types of things tell me he understands more than I think he can. He is also leaning and looking for the basement to go and play. So I just knew he would be so excited and happy to be able to be more independent. As you can see, he was loving being one of the boys!

We are heading in tomorrow to have another hearing test. Friday Jase is going in to have tonsils and Adenoids removed, and a Bronchoscopy. (not really sure what that is :) He will stay overnight just for monitoring. We are removing the tonsils and Adenoids because they are growing rapidly and we think that is what is causing the Sleep Apnea. Im sure all will go just fine and we may get to now ditch the oxygen at night.

Overall Jase is doing so good, and is improving in many areas. Thanks for checking :)