Not too much to report today. Jacob is stable & still sleeping a lot with the medication changes.

Overall Status: Good Day
Visitors: Kerry, Grandma & Sara D
Dinner Provided By: The Stillers

Noni & Papa picked up Amanda this morning for the weekend so Kevin & I could spend some time together alone as well as with Jake. Jake is having a rough day. He's struggling to breathe & his blood has a trace of carbon dioxide. They have increased his flow of oxygen which will hopefully help him out a bit. We've also been placing him on his belly which appears to help him breathe better.

Dr A. Johnson stopped by to tell us the results of the EEG. He's been able to review 15 of the 17 hours. Jake had 13 seizures in those 15 hours, however, the video portion of the test failed so he was unable to tell us what movements he was making at the time of the seizures. He also stated that most of them occurred between 11pm and 4am. Once he received his medication at 5am the seizures stopped & he was relaxed. He's ordered that the medications be staggered a bit to see what happens with the seizures then. He'll receive medication every 6 hours now.

He's also doubled the Keppra medication in hopes to decrease the Klonopin, which he believes is the reason for the respiratory distress and he's ordered another EEG be done on Monday.

That's about all to report for today. Hopefully it's just a little hurdle to get over & more good days are to come.

Overall Status: So-So Day
Visitors: Laura

Today was a mild day with nothing really to report. He was removed from the EEG & I gave him his 3rd bath, which he loved. He also proceeded to poop on the towels again, yet I escaped!! Still pending the results of the EEG. They weren't able to get the spinal tap done today because they were still awaiting a call from the lab in Houston to make sure it was done correctly. It has been rescheduled for Tuesday.

Have I mentioned lately how much I really hate this?

Overall Status: Good Day

Visitors: Barb & Nicole

Dinner Provided By: The Priddy's

I can't believe it's been 2 weeks today since this journey began. It's been full of ups & downs that's for sure. Today seemed to be a pretty down day. I guess after 2 weeks of trying to hold my head up high, I finally collapsed. It's been quite an emotional one for me, as I just can't seem to keep it together. He's so sweet & innocent, I just can't stand dealing with this. I'm not ready for what the future might have in store for us.

For the last couple of days, Jacob appeared to be doing well, however, I began to notice some increase in head & mouth movements. Today it escalated & my gut just told me something isn't right. I clocked him for 2 hours & 20 minutes; constant head movements, jerking back & forth as he tried to fall into a deep sleep. He looked so uncomfortable & at times would let out what I would call 'his cry' as if to say "make it stop, I'm tired & just want to go to sleep".

After he finally got into a deep sleep & all parts of him were relaxed, I called the nurse over & said I wanted to speak with the neurologist. It took a few hours to track him down. When he arrived, he could tell I was distressed about something. I explained to him what was happening since he last saw Jacob. As we talked, it aroused Jacob & he began to twitch again. Head jerking back & forth, eyes jerking, mouth twitching. Dr A. Johnson observed for a few minutes & then asked that I un-swaddle him so he could thoroughly examine him.

Although Jacob's arms & legs were fairly stable, his head wouldn't stop. It was decided to get an EEG hooked up right away to see what was going on, which will run for 17-18 hours. It's unclear if they are seizures or not & he also stated that it's possible his movements are just migrating to different areas of his body. Grreeaatt

He also ordered the spinal tap be done tomorrow, as well as some more blood work for additional testing. He also wants to look at the area of his blood that came back abnormal the last time to see what it looks like now. Apparently it should be zero, meaning you shouldn't see it in the blood at all; Jacob's level was 16, clearly abnormal. Looking at this again, might offer some more clues as to what's going on.

Once the spinal tap & blood work is done, he will add Keppra to see if it helps with the myoclonic movements.

The good news is Jacob was alert again today & did take another 3 cc's by bottle; still not much sucking going on though.

They also decided to increase his oxygen to 100% & decrease his flow to .1 liter. This is a more standard mix for him to go home on, should he need oxygen, which I'm guessing at this point will be a given. We also talked a bit about the G-Tube today. Although we still don't want to do it right now, most of us are getting prepared for the fact that it most likely will need to happen in order to bring Jake home.

I can only hope we'll have some answers soon...if only I had that magic crystal ball.

Overall Status: So-So Day
Visitors: Jared & Sara

I'm so happy to report another mild day in the NICU! Jacob had his usual alert times around feeding time & is continuing to show signs of sucking/swallowing. In fact, at his 5:00pm feeding, he ate 3 cc's by bottle! By the way, for those that are wondering, 1 cc=1 ml; 30 cc's = 1 ounce. So it's a very small amount but it is for sure a start. Although most of it was from the bottle dripping in his mouth, he did clamp down on it a few times & he didn't choke on any of it. I heard good swallows. I didn't see any Dr's today, so nothing more to report there.

While the occupational therapist was there today, he was alert & relaxed. He was doing so well that we had to decrease his oxygen & put him on room air for a while! Yippee!! Although when he went back to sleep, we had to turn it back on...he just gets too comfy while sleeping.

Jake & I rested in the lazy chair most of the day while visiting with our friends & family that stopped by. I did browse through some new scrapbook magazines while we hung out & even managed to strike up some conversations with a few NICU staff who are interested in attending Crop for Cancer this year...yahoo!! Speaking of, if anybody reading this lives near Anchorage & loves to scrapbook, I've got tickets & they go on sale August 15th! *wink

Well I think that's about it for this entry, let's hope for some more progress tomorrow!

Overall Status: Good Day
Visitors: Heather, Grandma & Grandpa, & Becky
Dinner Provided By: The Cagley's

The increase in Klonopin appears to be working. Jacob's jitteriness decreased by late afternoon. He also had a big alert time from about about 11:00 - 2:00, where I worked with his suck/swallow quite a bit. I started using my finger so I could get a better feel for what he was doing. As I depressed his tongue, he would suck! If I depressed just behind his chin, he would give a good swallow. So I'm convinced that he has what it takes to do it, he just needs some practice! While I was working with him, his mouth area was less over-stimulated with no noticeable signs of jitters, big difference over yesterday.

Dr A. Johnson stopped by for another visit about 4:30, after he was very sound asleep. He was very impressed with the changes in his muscle tone over yesterday. The original plan was to increase his medication a little bit each day, after seeing him today, he's decided to leave it as is.

Jacob does appear to breathe a lot shallower while in a deep sleep, which requires him to have more oxygen. Dr A. Johnson said that we may have to make a compromise, see more jitters or have respiratory issues. We'll see what Jacob does over the next few days on the new dose.

He is feeling more & more confident that we may be getting to the bottom of this. He does feel that this is a 'recessive' mutation, meaning it took the exact 2 people to cause this. Like a 1 in a million chance of it happening again. He said the chances of it happening again to us is likely about 25%. It was just the right 2 genes at this given time between Kevin & I to cause the syndrome. Hopefully that made some sense to everything reading this! He explained it much better then I am!

He did mention that Jacob will most likely come home on a saturation monitor, so that we can keep an eye on his oxygen levels, which means he may come home with oxygen as well, maybe not for us to use all the time, but to at least have it here in case it's needed.

He's also been in contact with a Dr in New York that is very familiar with this disease. He's trying to find out where he can send a spinal tap for testing. Apparently, the spinal tap will give a more conclusive result then the blood test. Not sure how that all works, but that's what I know! Hopefully he'll get that information soon so it can be sent out & maybe return about the same time the other test does.

It was great to see him awake for such a long period of time. He cooed a lot & I think even let out some cries when it was around feeding time. There were a couple of other babies that seemed to cry a lot today & I think he reacted to that too. It was fun to listen to him!

Well that's all for today, more good news & more 'wait & see'.

Overall Status: Great Day
Growth Stats:
Weight: 6 lbs 12 oz
Visitors: Colleen, Mary M. & Celeste

Jacob had another good day today. His muscle tone was more relaxed over yesterday. He was stretching out his legs on his own & didn't give me much resistance when I stretched his arms over his head. His oxygen levels were very good with no dips & they may drop him down to .2 liters this evening. His feeds have increased again to 55 cc's every 3 hours. His umbilical cord is about ready to fall off too! Dr A. Johnson, the pediatric neurologist, stopped by today to ensure that the lab work was done for the 2 genetic tests, as well as to check in with us to see how we felt Jacob was doing.

After chatting with him a bit, it was decided to increase the Klonopin a bit higher then the original dose. The goal is to get the myoclonic movements a little more under control, without compromising his breathing ability. Dr A. Johnson feels that he may be having myoclonic movements in his lip/cheek muscles, which could be prohibiting him from wanting to suck/swallow.

The nurses have begun to nipple feed him with a bottle to see what he does with it. The first try was during the 2:00 feeding. Although he was very aware that something was there, he didn't do much with it. He moved his tongue around, made a very weak suck attempt & some funny faces. The nurse did consider it as having an interest though, which is very promising. During this attempt, he was stimulated quite a bit & his lips began to quiver. I wondered if it may be a seizure, but when we pressed our fingers on his chin, the movements stopped. Had it been a seizure, you wouldn't have been able to stop it. This proves the theory of the reason why he may be lacking in the suck/swallow category.

The next several days may appear to be a bit of a setback with the increase in medication. The hope is that after the medication levels out, he will be back to waking up, have less or no myoclonic jerks & continue to show signs of eating. Depending on how well he tolerates the increase in medication, he may need to be intubated to get him 'over the hump'. Time will tell.

Both the Dr & I agreed that it was much better to try out this increase with him in the hospital then at home since he has 24/7 nursing staff. We also decided we wouldn't try the Keppra at this time & see what the Klonopin does instead.

One thing that I keep forgetting to mention, after reviewing the CT scan & MRI again, the Dr's have concluded that since the amount of brain atrophy is so minor, they are considering it normal. If you were to look at a perfectly normal person's brain, you might find the same amount of differences.

So far, I'm very pleased with Jacob's progress, and even though there may be a few steps back over the next several days, I have hope that things are looking better. The doctor told me today, the only thing keeping Jacob in the hospital right now is his lack of eating, & since nobody is wanting to rush to the surgery room to place a G-Tube in his lil' tummy, we'll just need to give him some time to respond & learn how to eat.

"If you permit yourself to let go of your worries, placing them in God’s hands,
trusting HIM completely, HE can truly lighten your load."

Overall Status: Good Day
Visitors: Pam, Melanie, & Sara
Dinner Provided By: The Keffalos Family