Christmas, vacation, surgery and teething oh my!!

Lots and lots of updates. I will be brief though.

I need to do several post to explain everything but I thought tonight I will catch everyone up.

Christmas was nice. Quite and calm. Just like we like it.

We had an impromptu mini vacation for New Years. The three of us went to Joe's dad and step-moms (Lonnie and Brenda) house Thur-Mon. It went so well. It was a 4 1/2 hour drive and Emily did fantastic! We all felt so relaxed the entire time we were there. It was so nice to have extra hands around to help with Emily. Of course Emily loved having her grandma and grandpa to love on her too!

I am proud of us for doing it. We only forgot one thing too HER KETO SCALE!! No worries though, I made enough meals to last a few days and then we bought another from Walmart.

It is hard to travel with Emily and we can't do it often but this trip was so worth it!

As long as she had her little puppy ,Violet, she was fine. :)

We had an appointment with ortho. Emily's left hip is out of socket and most likely has been for a while.

They will surgically fix it on Feb 14th. After the surgery she will be in a spica cast for 3 month and then bracing for a while after that.

This is what the cast will look like. She will be in the bilateral long leg hip spica.



She is off of topamax now!! Yay!!! Only on phenobarb and the ketogentic diet. This is the best seizure control she has ever had!

She is having a few-20 seizures per day BUT they are all very very short and only when she is falling asleep, waking up or after a very hard cluster of spasms. If we can get her all the way asleep or wake her up she stops.

She is incredibly alert and really looks for people. You can just see it in her eyes how much better she feels!

She is cutting her 4th tooth. Two on bottom and now two on top. This 4th one has caused her more pain though. Lots of tylenol and oragel and snuggles.

I will update more soon with lots and lots of pics!

Hope everyone has had a great New Years!!!

more updates

Two posts in one day!! AHHH!

Emily is doing much better the past few days. She is finally over the uti and her first TWO teeth have broken through. I thought this girl would never have teeth. She now has two bottom teeth that came one right after the other.

She is still having clusters of spasms and a few seizures daily. The past few days her seizures have been down to 3 or fewer.

So now we know that a uti and teething are seizure triggers for Emily. Now we are dealing with biting. Fun.

Visually, Emily is really progressing. She is seeing so much better and is tracking and following more objects. Used to you would have to introduce something to her from the side and hope it would catch her attention but now we can start closer to mid line.

Another big thing is she is interested in the TV! She does not care for therapy so her PT suggested we turn on cartoons, I laughed to myself because she has never been interested in cartoons before, well she proved me wrong! She loved it and we got through PT without any major melt downs!

A friend gave us some DVDs to try and she loves them!

Here are a couple pics of her watching TV.

She really likes baby Einstein.

Emily and her daddy watching videos on the computer.

I want to get her an ipad so badly. I know she would love it!

We are weaning her topamax slowly and when she is off of that she will only be on phenobarb. We are defiantly seeing more of Emily since we began weaning meds. She is pretty happy but she is very opinionated if she does not like something. Especially when she is tired.

She finally broke the 20 pound mark! She has always been on the small side and now that she is on the ketogenic diet she does not gain weight very quickly. She is small but proportionate.

We did get Emily's bath chair. We decided to go with the Rifton Blue wave bath chair with a tub stand to make it taller when we need it. Emily seems to enjoy it and it supports her so much better.

All of the support straps are movable. We don't use the chest and leg straps because she does not move enough to use them yet. She loves kicking her legs in the water.

We also got her stroller. We decided to go with the Special tomato EIO. I don't have a great picture of it yet. There are many reason we chose it. It holds up to 90lbs and it reclines all the way back so she can sleep. We also got the special tomato soft touch liners to support her better and they can also be removed and used in a chair for more support later.

So far I am very pleased with it. I wish it had a bigger basket under the chair to hold her diaper bag. We carry so much stuff when we go anywhere.

So that is pretty much it. Oh and I turned 30.

Looking forward to some more good days.

general update all things Emily

I just thought I would update everyone on Emily in general.

I talk about her seizures more than anything and it gets easy not to talk about the other things she has going on.

Lets get the seizures out of the way first.

She went 7 days with no seizures and had a 50% reduction in spasms. On the 7th day her spasms increased and she began to seize again. This time it only lasted 3 days and only 2 of those days required minimal extra meds to control them. Right now she is at almost 36 hours with no seizures and her spasms are super short and very weak and are down about 50% from what she normally does.

We changed one of her her fat sources on the ketogentic diet from canola oil to butter and we have seen an improvement with seizures. Don't know if it is just a coincidence or not but I will take it!

Developmentally she is right around a 4 month age level. I look back at video of her at 4 months old and she is doing things all over again. She is moving so much while she is laying down. She wants to badly to set up and I think she will get there soon! Her head control is getting pretty consistent, it of course is weak when she is tired but she is doing really well with it.

Emily has always been a clingy baby but lately it has gotten worse. She knows who she wants and will let you know if it is not you. Sometimes she only wants me and other times she just wants her daddy.

She has never liked any type of seat. She hates everything we have tried so she sets on the couch with us a lot on her boppy with a pillow under it to set her up but the last couple of weeks she just gets fussy and won't take her passy and it gets hard to hold her all of the time. One day when she was not happy I just set her up on the couch next to me and she was happy as a lark! Now that is all she wants to do. I think it is great because she is tolerating setting up and it is making her core stronger.

Her vision is getting so much better as well! Before I would have to say something to get her attention but now I can just walk in the room and she smiles because she sees me and recognizes my face. This makes me very happy. I have noticed her looking at the computer and the ceiling fan most recently too.

She still does not have teeth. At her most recent ped appt she said not to worry about it until she is 18 months old (she is 16 now) and then she would get x-rays. We have seen her teeth on an MRI before so I know they are there, just don't know why the won't break through.

She has no desire to eat ANYTHING. I try but she acts like I am feeding her acid. She especially dislikes water. She always has. Thank goodness for a g-tube. :)

We still have a long way to go with her sensory issues. She completely shuts down when we go out. Her pediatrician does not know her well, thankfully we don't go often, so last week she looked at Emily, who just got finished crying her self to sleep, and she asked if she looks at things or me. I am sure she did not believe me when I told her everything she is doing but I can't make her like going out and I can't make her tolerate a million sounds and smells at one time. We are working on it.

Last but not least...sleep.

Clearly there has not been an improvement (hence the blog at 4AM)

When she we seizure-free for 7 days I was beginning to get her on a better schedule but one seizure can ruin all of my work. Right now she is laying next to me kicking her legs and "talking" to me. I am giving daddy a couple more hours and then I am going to bed.

I could go on for days but I will stop.

I have another blog to post tomorrow. We have a friend ,Christy, who is doing a small fundraiser for us. It is more for locals but I will tell everyone the details tomorrow.

Soooooo

I did not quite make it to the women of faith conference.

I did get to go shopping with the girls at an outlet mall and check into the hotel but that is as far as I got.

Joe took off work to stay with Emily and he called while I was shopping and told me she had been seizing and he had given her just about all the meds he could. I told him to give her the last dose and call the on-call neuro.

So I am sure you can guess the rest of the story. I made it home pretty quickly  and met him in the ER and they admitted her.

IV steroids and Valium did not do a lot to help but we did find out that her labs are off. Because of the zonegran and the ketogenic diet she has metabolic acidosis the short description is-----metabolic acidosis is a condition that occurs when the body produces too much acid or when the kidneys are not removing enough acid from the body. If unchecked, metabolic acidosis leads to acidemia, i.e., blood pH is low (less than 7.35) due to increased production of hydrogen by the body or the inability of the body to form bicarbonate (HCO3-) in the kidney.

We are now taking her off the zonegran, she has been on it for several months and have not seen enough improvement and we added sodium bicarb.

We also are going to retry topamax. The 1st time she tried it she stopped eating and at that point she did not have her g-tube.

On Friday she had over 166 seizures and yesterday she had 83. That is a 50% reduction.

We are also going to adjust her ketogenic diet to allow more protein and see if that helps with some more seizure control.

Joe did such a great job on Friday, checking into the ER with Emily is no small task! I am thankful to have such a hands on husband. I just hate that we feel so confined to home. I was so afraid and nervous to leave and all this did was make those fears worse. So no trips for momma any time soon!

Lately

It has been a pretty eventful week or so.

Emily's seizures were still pretty frequent after her last hospital stay so Last Monday I called her neurologist to talk about what is next and we decided to bring her in to do a couple days of IV steroids. In the past it has always worked very good for her.

It seemed that they got worse this time. We made a few med changes and sped up the process of switching her to food from formula. She is still on the ketogenic diet I just make her meals now instead of the ketocal formula. Personally i think that has made a difference. She is not nearly as gassy and hardly spits up at all.

She is eating at least a portion of each meal and what she does not eat I just put down her tube.

We finally have stopped her clonazepam and as of Monday she will be off her lamictal too. I never saw any side effects of the lamictal but we also never saw an improvement.

Stopping the clonezepam has been such a blessing though! Emily is so much more alert and you can see in her eyes that she is seeing more too!

On Saturday while I was sleeping Joe got a video with his cell phone of Emily laughing. It is the most awesome thing ever! She has not laughed (awake) in so so long! Joe said that he opened his pop can and she cracked up so he snapped his fingers and she laughed again. He then coughed and she was laughing so hard all she could do was squeal! She has done this two nights in a row now. I got a video but it is too long. I will work on getting a shorter one to upload.

She is also resting so much better. She is sleeping at least part of the night since we got home and just seems much more rested when she wakes up.

Since leaving the hospital her seizures have gone down to 5-10 per day plus her spasms. They are super short too.

It was a long hospital stay and it always sucks but since Emily has been in so many times we have really gotten to know a lot of the staff and it is nice to have friends while we are there. People that set down and pray with us and for her or just set down and talk. There are a couple of night shift nurses we have grown to love, Alisha and Stephanie kept me company many nights and I am thankful they love Emily and take such good care of her. There are so many people there that love her!

I had a couple rough days in the hospital watching my baby seize over and over for hours on end. Over the last week I have had a couple of wonderful emails from parents who understand, I know I am not alone in these emotions.

On a completely different note...

I was thinking about my post a little while back where I was just honest and raw with my emotions. While I am terribly sad that people have chosen to remove themselves from our lives I am much more sad for them. They are missing the opportunity to get to know an amazing little girl. Emily is such a blessing and to see her smile and laugh brings such joy to my heart.

Thank you all so much for the prayers

The past week

After my last blog post we had to take Emily to the hospital. It ended up being a short stay, just overnight. She ended up having over 100 seizures within that 24 hour time. We tried everything we could to stay home because the more she is in the hospital the more germs we expose her to.

We got some more of the testing back from Cleveland and all it showed was a vitamin D deficiency. More normal genetics. We just had another genetic test sent out and that will take several weeks.

We are currently trying to stitch her from the ketogenic formula to ketogenic meals. We are changing it slowly so her body can adjust to actual food. So she is getting one of her five meals in food and she is eating about half of that by mouth. She really does not like the meat baby food ( i don't blame her) so we are looking into different protein sources.

She is still having many seizures a day. I think it may be her teeth, she wants to bite and chew on anything that gets close to her face. We are giving her oragel swab and Tylenol pretty often and it helps a little bit.

So that is pretty much it. She still is not sleeping at night. We did get our nurse back (yay!)

Here is a video (if it works) turn up the volume, Emily is laughing in her sleep. Whenever she hears me talk she starts laughing again. It is the cutest laugh!!

haha Daddy! I took your chair!

cleveland update

First I want to start off by saying thank you.

Thank you all so much for all of the kind words, prayers, and very thoughtful messages. It really did help us get through a horrible day. So many people love and care about Emily, even complete strangers, and for that we are eternally grateful!

Emily had a MUCH better day today. We did not see any visible seizures but with that being said since she is still on an eeg we saw many on the screen, mostly when she is sleeping or when she is falling asleep or just waking up.

She was much happier today also. We got lots of smiles and some pretty good eye contact too!

She saw the ophthalmologist, who was also great, and he said she does have CVI. Her eyes are healthy and her vision is perfect, her brain does not let her see properly right now. When we get home with will start a vi son program for her to help her see better.

Yesterday we met with the neuro/metabolic specialist, he was so great! He explained what he thought and what we are going to look for. He thinks Emily may have an early onset of Rett's syndrome or cdkl5. She has actually been tested for cdkl5 before and it was negative but apparently the lab the preformed the test is not "the best" so if all of the other testing comes back negative we are going to re-test her for that one. He was explaining that finding Emily's disorder is going to be very difficult. He described it to us like this-finding a typo in a book with 20 trillion letters in it.

Finding what is "wrong" with her may not change anything, but if we do find out we can prepare ourselves for what is to come and some disorders have other problems that arise in the future like heart or kidney issues and we can already be on top of things if we have a heads up.

So blood and urine were sent off and we should hear something in 4-8 weeks.

Also we have decided to change her diet slightly. Stacy if you are reading this I am going to email you all of the new info this weekend :)

She is currently on a 4:1 ratio *4 fat to 1 carb* and the formula that she is on has artificial sweetener and some people are much more sensitive to it so we are changing formulas and ratios to see if that will help at all.

I will leave you with a few pictures.

This is Emily's very first hotel stay. She looks pretty darn comfy.

Her eeg leads on. She did so great with them, all 4 days worth!

hello food!

Today I gave Emily her first taste of food in over almost 2 months. She was in heaven!

I could not get it in her mouth fast enough.

I honestly did not think she would like it because it was 15 grams of sweet potatoes and 10 grams of canola oil. It looks really gross!

It was not a lot of food either so she was still pretty hungry. Luckily she was tired so she fell asleep shortly afterwards.

I think she may start teething soon. She still does not have one tooth but she is drooling soooo much. I had to take this picture it looks like a drool beard. haha!

I love you

So I think we are going to start Emily on baby food again! Before the horrible side effects of her many seizure meds Emily was a fantastic eater! I made all of her baby food and she was not picky at all. She loved her veggies most though.

She slowly quit eating and it was so sad for me to see her not be able to eat. The past few days though she had tried to bite whatever comes close to her mouth! It is kinda funny. So I think she is ready.

So I got several ketogenic recipes from our amazing, wonderful, and newly engaged ;) dietitian, Stacy. I am a little nervous about it though. Change in anything with a seizure kiddo can bring on seizures so I am praying for a smooth transition.

I just wish there was something I could do to get her to sleep at night. Currently it is 2:10AM and she is squealing and kicking with delight. Don't get me wrong I am to the moon happy that she is in a good mood, I would just love to sleep at night because though the night there is less care to be done for her. In the morning, when she sleeps the best, I have to do her meds, g-tube care, feedings, make phone call, appts, deal with crappy home health companies (haha! can you tell i don't like them?) and it does not make it very easy for me to get good sleep.

I got a video of her last night, I say I love you to her probably a million times a day and last night it sounded like she was trying to say it! Just ignore me repeating myself over and over.

I was trying so hard to get a picture of her smiling but she would only smile after the flash went off. Also I understand that there are a few students in Mrs. Rollins class that check-up on Emily and like to see her pictures so I put this one on her for you all today. Thanks for thinking about Emily I told her that you all think she is cute and she smiled!

bad news

I have been holding off blogging about this just because it is hard to type.

We got the results of Emily's follow up MRI of her brain.

Compared to the MRI in Dec. her brain has suffered mild to moderate atrophy, which means it has shrunk.

We are getting a 2ND opinion within a month at the Cleavland clinic.

Another thing that could have happened is that her brain just did not grow with her skull, which is what we were hoping.

We were just so taken aback because she has actually made PROGRESS the past few days. She rolled to one side and brought her hands together (like praying). She is smiling more and laughing appropriately. She is even making more eye contact too.

So needless to say we are pretty upset with this news.

I am holding out hope though, this is one doctors opinion, and as we all now Emily is a mystery so we can't compare her to anyone else.

She started the ketogentic diet and tonight it seems to be kicking in. We have to test her blood sugar and check for keytones in her urine. The goal is to get her blood sugar somewhere in the 70's (normal is over 100) today she got done in the 80's. She is already having moderate keytones in her urine too.

We are only 2 days into the diet so I have not seen a huge improvement in her spasms. Yesterday was a very bad day for spasms but today they are not nearly as long, so maybe that is the diet kicking in.

So please pray for Emily and us. This is so hard to go through.

I just wished we had answers. None of her genetics are saying anything. We are currently waiting for the 3rd round to come back.

I love her so much. She is all we ever wanted.

One last thing. Emily's daddy ,Joe, has started blogging about his princess. Go check it out.

Good?

We are in the hospital....again. I am pretty sure I have said that a couple of time before.

This morning around 3am she started having her spasms and they lead into a massive seizure/spasms chaotic hour and a half.

After tons and tons of valium and klonopin and calls to the neurologist she still did not stop. After her last big seizure we called 911 because we could not give her any more meds without suppressing her breathing and we live over 30 minutes away from the hospital and it was almost rush hour at this point.

Honestly this was the best ER visit we have ever had. We were in the ER around 6am and we were in our room by 9am. Of course Emily did not have anymore seizures or spasms until we were admitted to the unit.

She had 3 more of her big seizures and a few more clusters of spasms. More meds and still no sleeping baby. I do not for the life of me know how she was awake.

Since midnight she had 6 doses of klonopin, 2 of valium and 1 IV ativan and this is what she looks like

It's almost 8pm and she fell asleep about an hour ago..finally.

So listen to how great everything is working for us!

We were hoping to start the keto diet and get a g-tube placed in the next week or so. It takes a little time to get everyone coordinated for all of this.

To get a g-tube it requires a couple of tests, both require sedation. To get the keto diet started requires a dietitian free for almost a day to do education and get her started on the formula.

Drum roll please...............................................................................

WE STARTED THE DIET TODAY!!!!!!

We are scheduled for all of the testing first thing is the morning!!!!

Everyone keeps saying " I can't believe how everything is falling into place!" Well I can believe it(wink to GOD) ;)

If all of her tests are OK then she will get her g-tube on Wednesday.

Just please keep Emily in your prayers. Lots of test tomorrow, lots of sedation and then possible surgery on Wednesday. Pray this diet helps her.

I want her healed so badly.

p.s. Dear seizures......I HATE YOU!!!!!

thanks!

little bit if this and a little bit of that

Let's start this off with some cuteness!!

This is Emily's new thing, when she is getting mad or irritated with us or what we are doing she says "neen". I am pretty sure it's a warning, like if you don't stop what you are doing right now I am going to get mean. Or she could be telling us we are mean! Either way it's just so darn cute!!

Next, I would like to say that out in this massive blogging world I found a family that has a little boy named Collin, very cute I must add, who has infantile spasms too. They live near us and their journey has had quite a few similarities. He is on the diet Emily is getting ready to start. Click here to go check them out! Please pray for this family. I know how hard this is and every added prayer does help!

On to the seizure stuff. I have not updated on Emily's seizure activity over the weekend because we wanted to make sure what we tell everyone is actually what we are going to do.

On Friday evening Emily had one of her big seizures (aka scary) in her sleep. I was holding her and she never even woke up. If I was not holding her I don't think I would have even heard her. Not that we ever let her our of our sights. It was incredibly scary. It only lasted about 30 seconds, we did not even have to give her medicine. I was on the phone with the on call neurologist with in minutes. Luckily I got to talk with one of my favorite people, one of the nurse practitioners, and she knows Emily well. I told her what had happened and she agreed that it is time to start the Ketogentic diet and to place a g-tube. She is eating less and less by mouth everyday. Today she ate 5 ounces.

To do a much better job at explaining the ketogentic diet click here . This site has been very helpful to me.

Next, I know some of you don't know what a g-tube is. It is a feeding tube that goes directly into her stomach. On the outside all you see is a little "button".

Something similar to this. By the way I do not know who this is. Just googled it.

This was the only picture I could find on google of a smaller kid. My point is that it is not some huge tube coming out of her belly.

The nurse practitioner is going to call me on Monday evening to let me know the plans.

It will take probably close to a week stay in the hospital again. We will meet with the dietitian and she will lay out Emily's course as far as the diet goes. We have to slowly switch to the keto-cal formula and check her blood sugar among other levels to make sure her body is tolerating it. Some kids it works right away, others it may take a couple weeks. The goal with the diet is to take her off all of this medicine and obviously stop her seizures.

I just pray this works. So far none of the medicine is working 100% and one seizure is one seizure too many!