hard day

I wish this were a happy, fluffy, everything is great post-it is not.

14 weeks spasm free came to a screeching halt this morning.

The first arm flailing, heart sinking jerk was obvious and very painful to watch.

We are heartbroken and devastated. We knew it could come back but were praying it would not.

So far she has had two clusters.

She does not have many other options left.

We will keep fighting but tonight we are weak and hurt.

Good days

We have had several good days now. I think we are at 5 or 6 really good days! Thank you Jesus!

By good I mean little to no seizures, minimal spasms (but still there) she is alert, responsive, visually tracking us and toys and babbling like crazy!

She has been such a joy the last week! I don't think she has really cried much either. She is sleeping at least 5-6 hours at night. Most nights she does not go to sleep until 12:30 or 1am but I will take it!

Previous to our really good week she had probably some of her worst weeks, hence the no blogging.

She was seizing like crazy no matter how much meds we gave her. You could tell she was just so out of it and "off". Long story short-low grade temp, strong smelling urine, extremely fussy and increased seizures= massive urinary tract infection (e coli to be specific) Things got worse once we started the antibiotics and I was so nervous because she has never needed them before so I did not know how she would react. Just a couple days in and she was doing SO much better.

We noticed the weather forecast was calling for mid to upper 70's last Friday and Joe and I really wanted to get some family/18 months/Christmas picture taken so we asked our good friend Rebecca to take them for us. Also Joe's dad, step-mom (Brenda) and his grandma Mae were coming in that week too so that was a bonus. They were originally coming next week to celebrate Thanksgiving but Brenda and Lonnie are going to be grandparents again and they were cutting it close to the due date. Can't wait to meet Ava!

We got some great pictures! Rebecca is so talented and I am so thankful to have these pictures to look back on. I don't think she understood how grateful we are! To capture such great pictures on a beautiful day and one that Emily was feeling great, we will always have these to look at on bad days to remind us that it is not always going to be bad. Thanks again aunt Bec!! You can follow her here on facebook.

So I will leave you with a few of the pics! Have a blessed week!



One of my favorites!



This color was amazing!



She amazes me!



off

I don't really want to blog right now but I am making myself.

Things have not been good lately.

Emily's seizures have picked up in spite of everything we are doing.

She has what we think are seizure-free days once or twice every week to two weeks but I am not sure that they are really seizure free.

The past few days have gotten really bad.  She is having so many seizures and we are giving her a lot of meds to TRY and slow them down but they are not working well anymore.

She is weaker than she has ever been and has not been herself in a few days.

She is twitchy and very seizurey looking.

We took her to the ped yesterday just to check labs and urine to make sure it's not something simple like an infection or off labs we are dealing with but they were normal.

I really don't want to go the hospital (especially during cold and flu season) we have oxygen and and a monitor at home and we have been using it lately. She has only needed oxygen once but we are thankful we had it.

We are taking her in to see the neuro the beginning of next week and hopefully we can figure something out.

I want my happy smiling baby back.

lot of bad, little bit of good

Since my last post Emily has had some pretty bad days.

Her seizures just went nuts.  We made it through that rough patch with only one phone call to neurology, no hospital visits this time. We did get very close one night though.

I have had a very hard time ,emotionally, lately. I can handle having a child with disabilities, special needs, developmentally delayed-whatever you like to call it. I can not handle that fact that I have a child with very serious medical needs. It is hard knowing that her life if so fragile. Every attempt to stop or even slow seizures have failed and we all know that 100-200 seizures per day plus her 10+ clusters of spasms she still has daily is not good for her.

I have mentioned before besides our nurse who works 4 days/week we have no one that can help us take care of Emily. There is not one single person who can care for her if something, God forbid, happens to Joe or myself. I know that sounds morbid but that is something we have to think about. Don't get me wrong, we have a couple family members and some church members that have and continue to help us out financially and bring us delicious meals or bring over groceries. We are incredibly thankful for all of the assistance we get! We love getting cards in the mail or phone calls to check on us. Those things we truly appreciate!

Some days, especially bad seizure days, it is hard to deal with all of our daily responsibilities on top of everything with Emily. Dishes don't do themselves and the laundry just does not jump into the washer just because we are having a bad day. Life must go on.

So now that we are done with the bad stuff and complaining.... on to the good!

Emily has not had a seizure since Thursday at 2am. When she has seizure free days she does so much better with holding her head up and assisted setting.

She had her 15 month check up (it was actually 16 months though) and her head, height and weight at all way under normal but proportionate. The only thing we are watching right now is her hips. Emily legs tend to fall to the right when she is laying flat and she is going to be prone to dislocation so we have to try to keep her hips rotated to the left more.

Trying really hard to set up



Giving her PT the "get off off me" look.





Holding her head up so much better





Emily loves her sensory cat!! It has 3lbs of rice in it to help with her sensory issues.



Thank you Colin for the wonderful gift!!



She loves it!

Praying that this seizure-free streak will continue!!



still not good

After Emily's most recent hospital stay she had a couple of good days but it has went right back to bad again.

Everyday she was going down in seizures by at least 50% and then Wednesday night she started and has not really stopped. She seized pretty much most of Thursday so I am holding my breath for this morning.

I am just so tired. So tired of seizures, so tired of being tired. I feel like I can't sleep because she has started having seizures in her sleep now.

It is hard because when she was having a couple of good days she could see so much better, she was so much stronger and was awake a lot more through the day. I makes me so sad to see her start seizing again and stop doing those things as much.

I did get a couple of pics of her holding her head up. She was not happy about it but she did it.

 She was watching football. It is the only thing she will watch on tv. Makes daddy proud!

 I moved her to see if she was really looking at the tv and you can see she leaned her head back to see it.

She is done holding her head up. Time for sleep!!

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!

Another hospital visit

So Emily made it back to the hospital...again.

This time for some IV steroids. Her seizures have been relentless and we just can't seem to stop them. When we do it is not for very long.

So lots of tylenol (in case it is teething) and a couple med changes we are hoping to do the trick.

It has been a rough couple of days. We NEED to get these seizures to stop.

We are supposed to go home in the morning so I will try and do a better job at updating everyone then.

Thanks for all the prayers!!

raw

Seizures returned tonight.

sigh.......

Emily had gone over 3 weeks with no seizures, just lots of spasms.

It was nice. Nice to not have to count seizure after seemingly endless seizure.

I got to get out of the house for a day, I almost put my guard down. Almost.....

I watch Emily like a hawk. Every movement every twitch and eye flicker. I watch other people kids for seizures. I look at kids in the grocery store afraid one of the will have one.

It sucks. Their is no other way around it. It's not fair.

It's not fair to watch her not develop with other babies her age. It's not fair that she slept through her birthday party because she could not process all of the noise. It's not fair that I have to sacrifice any sleep I might get to watch her because the seizures have screwed up her sleep cycle and during the day she has therapy 3 days/week. Today , even with a nurse, I got woke up 3 times with phone calls for appts, testing, insurance.......

It's not fair that people have removed themselves from our lives. I have very little family support. Those who do help us financially and emotionally we are incredibly grateful for and they know who they are. Our church has been a HUGE source of help, honestly we would have lost everything without them.

Others are just too busy with their own lives. I get it but it is still just not fair.

I am not good. If you ask and I say yes I am lying. Things are ok, I am just ok. Most day are good enough and we do get by. Some days are easier than others. Days like today not so much. One day I will be good. I know I will, just not today.

I know to some I sound like an whiney ungrateful brat but this is how I feel. My emotions are raw and I am just being honest. Things tonight are not ok.

Tomorrow is a new day blah blah blah I know but right now I can't stop crying. I hurt for Emily. I hurt for me and Joe.

45 seiuzres in and I am praying they will stop soon.

I debated posting this. It was very theraptic typing it all out. Maybe it will give someone else the nerve to be honest with themselves too.

Brakes

The brakes have been slammed on Emily's good seizure streak.

On Saturday we noticed she was having very subtle spasms again, well we thought they were. As the night went on it was much more clear that they were spasms.

Right before her 9pm meds she was asleep and she started shaking her head back and forth, again very subtle, like she was saying no. We were pretty sure it was a seizure and luckily it was the only one and it only lasted 10 seconds.

Joe and I took shifts through the night to watch her. She did not sleep much through the night and in turn she has slept most of the day.

Today, more spasms, they are still very subtle. Sadly she has two seizures, well we are almost 99% sure they are seizures. Both were less than 15 seconds and were the same as the day before and both while she slept and they never woke her up.

Joe and I have been racking our brains to see what it could have been.

There are several different things it could be. She has been constipated ( sorry if this is TMI) she has not had a good BM in 2 days in spite of miralax.

She was out in the heat on Saturday. We did a good job on keeping her cool though. I did forget to give her MCT oil with her 10am feeding which brought her calories down slightly but her urine ketones and blood sugar remained perfect the past 2 days.

She has also been super gassy. When I vent her g-tube air rushes out so I am trying to vent her more often.

For the life of me I just don't understand her seizures. She has a virus with a fever of 103.7 and no seizures and now she has had seizures out of the blue with no reason what so ever. I guess that is just the nature of stupid seizures.

She had 22 days with no seizure or spasms. She is capable of doing it again! I keep telling myself this.

they're back

We have had a rough night.

Emily started seizing around 6:45pm and has not stopped. She had 49 seizures yesterday and since midnight she has already had 9. They around 5 to 30 seconds long. I have given her all of the medicine I can give her for now.

Since we switched to oral Valium it really knocks her out, unlike the ativan.

She is not on her full dose of zonegran yet, we just increased it again last night so hopefully that will kick in. Until then we started her on another round of steroids last night but those usually take a few days to work.

I have to take her to the hospital in the morning for some blood work and I pray that she stops seizing before we leave.

Stupid seizures!

bad to worse

I am blogging this because it is too hard to tell 35 people the same thing over and over. Please do not get your feelings hurt because we did not call you and tell you personally. We are choosing not to call anyone right now. It is too hard to say what we are going to say. If you would like, you may call us later tonight and ask questions but right now this is how we are choosing to update about Emily.

Last night she had a really bad night. Really bad seizures that would not respond to ANY meds and they were very strong. They were getting ready to send her to the PICU-(pediatric intensive care unit) when she finally stopped.

So that was our night-BAD

This morning the neurologist-neurosurgeon/epiologist came in to give us the results of her PET scan. Emily's seizures are from everywhere in her brain. She is not a surgery candidate. He also said from monitoring her on a constant eeg the past few days she has a very severe form of epilepsy. We already knew that but we had hoped that the PET scan would show that she was a surgery candidate so that was a huge blow.

She does not respond to most meds so we are adding some meds to her already extensive list. Once she is on the new drugs well we will begin to wean her off her klonopin and then use klonopin as an emergency drug instead of ativan and diastat because neither of those are working for her.

Our new plan, since she does typically respond well to steroids (for a short time) is when she starts a big cluster of seizures we will begin a round of 3 days or so of oral steroids-starting now because as I am typing she is seizing.

We are also starting a new drug zonagran (tonight), there are only 3 drugs left he thinks could work for Emily and this is the first one we are choosing to try. We will also be increasing her phenobarb because her phenobarb level is still a little low and in higher doses she does respond for a little while.

We will also be meeting with the keto dietitian to see if there is any tweaking we can do with the diet because she is still not in large ketones. That meeting is tomorrow.

Our new goal with Emily is less seizures, seizure freedom is not realistic right now.-WORSE

The doctors here are amazing and incredible. Sadly there is just not much else to do for Emily. Only time will tell what the future holds.

This is the hardest thing to deal with because there has always been a next step, many other drug choices and tests. Now we are down to 3 drugs and that is it. We have hit a brick wall and it hurts.

So please be understanding that right now we are grieving again. It may sound horrible to say that but we are. We are grieving the hope that we had in new meds, new doctors, new tests, new outcomes for Emily.

The reason we chose not to call anyone is because this is hard enough to type let alone tell so many people. Emily does have a lot of people who love her. Again, it is not personal. It is actually selfish and right now that is what Joe and I need to worry about, ourselves and Emily.

We will be coming home on Friday probably. She is not stable for travel right now, so hopefully we will be home this weekend. We love you all so much and are so thankful for all the support we get. Please keep our family in your prayers. This is just more difficult then I thought it would be.

I love Emily so much and to see her suffer like this is beyond fathomable.

rough night/morning

It's 6am and I still have not been to bed.

Emily started seizing shortly after midnight and I had just given her her dose of sleep medicine, which we increased. She was so so sleepy but her seizures just would not let her little body rest. She was laying there with her eyes closed but she was not asleep.

I had to call the on-call neuro and she instructed me on what meds to give and to call her back if she did not stop. She had not stopped and it was almost 2 hours later and now she was wide awake!

We gave her more sleeping meds and I can give her one more dose of ativan before I have to call back. So far she is asleep but I need to monitor her because she has gotten A LOT of very sedating meds. Every time she wakes up she has a seizure so right now I am just trying to keep her asleep.

We are pretty much out of options right now (med wise) and I desperately want to keep her out of the hospital.

I did find out that our insurance approved us for a private duty nurse 8 hours a day, 7 days a week. I did not want to tell everyone until we knew for sure but they start on Wednesday. I can't wait! This is going to be such a blessing. I will be able to rest or clean my house for a change!

Please pray that we don't have to go back to the hospital today. Thanks!

good streak broken...

We were back in the ER this morning.

Emily starting having her spasms at 3:35am and then very quickly started having clusters of seizures. It started out with very short seizures, 30 minutes in we gave her ativan, we repeated 30 minutes later because they were getting stronger and more frequent. I called the on call neurologist because it was now 5am and she was not letting up and she also began have clonic/tonic seizures, most people would recognize them as "grand mal"

We were told to give her diastat (rectal valium) and call ems because she had so much medicine in here and we live so far away from the hospital. By the time ems got here she had over 30 seizures with almost half being the "big ones"

*Let me take this moment and vent about the ems worker that came. We have had her before but Emily was not as bad then. First thing that made me mad was she refused to let me stay in the back with her. She said I could not "handle" some of the procedures (starting an IV). So I quickly told her NO SUGAR! No dextrose, glucose of any form!!! She did not understand (HELLO that is why I need to be in the back with you Einstein!) Not many health professionals know much about the ketogentic diet and those that have heard of it know very minimal info on it. I then proceeded to tell her that Emily is a very hard stick to get an IV started and unless you must please wait for the IV nurse in the ER (they know her) to do it. She again did not listen to me and tried. Yes I said tried because SURPRISE she could not get it! We wasted 10 minutes setting in my road doing this! She gave her 2 IM (shots in the muscle) shots of valium on the way and checked her blood sugar and I pretty sure she used the biggest needle they make to do these two things because of the marks they left on her!

So to sum up this ambulance trip-It sucked!!

The ER knows Emily pretty well know and they are always very pleased with our record keeping. We keep a daily journal of all of her medicine, feedings, diapers, spasms, seizures, ketones, blood sugar. We would be so lost without that journal!

They checked her phenobarbital level and it was low so they increased her dose and made us stay a few hours to make sure she responded ok to it. They said that feeding food was fine so tonight we gave her one meal instead of two so we will see if we stay home tonight.

Praying for a good night, now off to watch Glee!

*Let me add something to the above rant. First I am so thankful to have EMS be able to come and pick us up and safely get Emily to the ER. I don't want anyone thinking otherwise. I just wish that they would listen to me. I am the mother of a child with a rare seizure disorder and an even more rare diet. I know more about Emily than they do. I do not know more than her (medically speaking) but I can hold my own I worked in a hospital for 8 years, I picked up a few things. I am not the average "oh my gosh something is wrong with my child, what could it be!" kind of mom. I know what is going on with Emily. This is our 4Th experience with EMS and 3 have been ok, just in combination with everything else it was too much!

she's baaaack

Guess what! Oh I am sure everyone can guess by now,

we are back in the hospital.

She actually had a really great night when we came home, her last seizure was at 8pm and she did not have another one until 11am the next morning. Then she had 2 but they were under 5 min. each.

So fast forward a couple hours and BAM! It was over 3 hours worth of seizures. Emily is very talented and her seizures manifests themselves in a couple ways. One is her newest , twitching. And twitch she did. I gave her all the klonopin I could give her, she passed out for a few minutes and then started having big seizures (cloninc/tonic) We called EMS and they witnesses a few so....here we are.

Around midnight she started again, with the twitching. This time she gets IV ativan (stronger than klonopin). She got than 3 times and IV dilantin and still her seizures(twitching) are not stopping. By the was this whole time she is not even falling asleep and should have been knocked out with the 1st IV ativan dose.

So they gave her diastat which is rectal valium and it seemed to have done the trick. She is not all the was asleep but resting for now. I am getting ready to try and sleep myself.

I just really wanted to lay in my bed and relax for a few days. We are just pretty down today. Kinda feeling a little defeated.

I am just so tired and for someone to help us they would pretty much have to live with us because Emily ,by no means, fits any mold. She changes day to day. Joe and I questioned each other all the time "Is this a seizure". So for someone to come from the outside to help would pretty much be impossible at this moment.

I am just really scared and want this all to stop. I hate it for Emily so much. Sometimes in between seizures she looks at me with the saddest eyes and I can't imagine what is going through her mind. I am her mommy, I am supposed to protect her and keep her from hurting! I have no control over this and it is killing me.

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.