Not the life I chose.

As I sit here watching my girl seize I am brought to tears.

In spite of valium, clonazepam, ativan, clobazam, and phenobarb (some multiple doses) she still seizes.

This means Joe and I sleep in shifts and more than likely we won't be going to church in the morning.

I have a very close knit group of lifelong friends on facebook in my infantile spasms group and tonight I learned of a sweet girl at the end stages of her disease.

Yesterday, I heard of a sweet little boy that passed away, most likely, due to a seizure in his sleep.

Brave Liam is still in the hospital on bipap now. He has been on a ventilator for some time now. His last MRI shows atrophy in his brain stem.

These families have to make horribly painful decisions for their children.

I did not choose this life, nor do I want it. I would do anything to heal my daughter.

I don't want to have my family so fractured that I go a year without seeing the people you think would be there for you the most. I don't want Emily to go the rest of her life not knowing some of the closest family members.

I don't want to have so many people in and out of my house during the week. I don't want to know all the best medical supply companies.

I don't want to have to have a neurologist, cardiologist, opthamologist, and a nurse case manager.

I don't want a feeding pump on a pole next to my daughters crib.

I don't want to have to drive 2 hours to have Emily fit for a wheel chair.
I don't want to have to fight over a parking space in front of my apartment and I don't want the handicapped tag checked with the word permanent that comes with said spot.

I begged and pleaded for this cup to pass by. God wanted us to have all of this. I don't know why, I may never know.

What I do know is God is the same before all of this and He will be the same after.

I run my fingers through her fine, wispy hair and I know she is what I want.

I love her more than I ever thought possible.

Some days are harder than others. Today is hard. Today I am mad at the crazy parking space lady. I am mad at selfish family members who I will never be able to change. I am just mad.

I have that right.

So, for now I will cry and vent and cuddle with my seizing girl.

Tomorrow is a new day and it may be better.

I hope.

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!!



update time

First, our day started crappy yesterday.

At 7am I hook Emily up to her feeding pump for an hour because usually she is asleep and that way I can get an hour of sleep to. So I got up put her extension on started the pump and back to sleep I went. The pump was down an hour later and Emily was stirring around. I put my hand on her and she was soaking wet! My first thought was that she peed, I turned her to her side and the bed was soaking wet too! I instantly grabbed her extension and I did not hook it up right so for an hour 6 ounces of formula ran into my mattress!!!

Then I get her up and start getting her meds together, the nurse comes in and I get a phone call from the nursing agency. Medicaid has denied my nursing coverage. The nurse has to leave immediately. Mind you I have had 1 hour of sleep so far and Emily has a neuro appt at 1:30pm.

So quickly before the nurse leaves I took a shower and got ready and made some more phone calls and then off to the appt we go.

The appt was great. The result from her last eeg was so much better. She did not have any seizures during the eeg. This is the first time she has ever done that! We are also weaning her clonozepam (klonopin) too. Hopefully in 10days she will be completely off of it.

We also go the results of some testing she had done in Cleveland. Emily has a vitamin D deficiency which if common in kids who are not in the sun much, so we need to add that supplement. Another one of the genetics was normal, another they did not have enough blood to do it so we had to go the lab after the appt and get it redrawn. We were at the lab for almost 2 hours. I was so tired at this point. I did not leave the hospital until after 5pm. I rushed home, Joe had some dinner ready for me, I shoveled food in and fell into my bed for a few hours and now I start my day all over.

 

I am so thankful we met emily's nurse. She is more than Emily's nurse she has become a friend! Besides Joe, myself, and her nurse we have no one that can take care of Emily. No one knows anything about her diet, meds, seizures, g-tube, and therapies. So we are so thankful for her because if there is ever an emergency with Joe or myself I know we can call her.

It is hard when the people you are supposed to count on choose not to be the people you always thought they would be or should be.

It's amazing how having a sick child really filters the people in your life. At the end of the day you really know who you can count on.

Well I am hoping we can get this medicaid issues resolved quickly and get her nurse back. We are looking forward to a fun weekend!

Emily laughing in her sleep.

Cleveland-day one

We are in Cleveland now and Emily is all hooked up to her eeg for the next few days. They want to see all the different types of seizures she has on the monitor so they may back off some of her meds temporarily and see what happens.

It is defiantly a different world here. Don't get me wrong it seems like a fantastic hospital and they have an amazing reputation for the most modern technology it's just not our little,everyone knows you, hospital.

The accommodations are a def lacking comparing to our home hospital. We have a very tiny room and we share a bathroom with another room. There is one shower on the unit and the door does not lock (ha!) Honestly I think we are just spoiled with what we are used to.
On a positive note, they left the hospital bed in the room so we have that to sleep on and a pull out chair/bed thing. The Ronald McDonald house is full so we could not get a room but they are letting both of us stay here so we don't need it now. They have a Ronald McDonald room on the floor for all the pediatric patients family's that has a couple computers, TVs, showers and laundry that we can use while we are here.

So enough with the complaining.....We met the neurologist up here and he is incredibly thorough, he knows his stuff. Joe and I were both very impressed. He agrees that something is going on with Emily's brain that is causing her problems, seizures, developmental delays, and low muscle tone, we just need to find what. He said he finds the cause in about 60-70% of cases but even if we find out what it is it may not change what we are doing. Finding a cause may just may be just that and not being able to fix anything.

That is pretty much whats going on so far, in the morning she has a PET scan done. Unfortunately, she has to be NPO (nothing to eat) after midnight and her test is not until 11am. I am freaking out a bit because I know she will be. We were 1 1/2 hours late feeding her earlier and you would have though I had not fed her all day! Also when she does not eat her blood sugar drops because of the diet she is on so we are going to have to watch the pretty closely. So needless to say I am a nervous wreck about this. She also has to be sedated for the test.

So tomorrow is going to be a long day. We also meet with the metabolic specialist tomorrow and they had me fill out a 10+ page packet to help them fill in on Emily. Maybe they will see something no one else has so far.

I will update sometime tomorrow.

Your Fired!

We fired our first nurse yesterday.

We really did not want to do it. She was so nice and sweet and seemed to be a good nurse.

She came in Saturday morning looking like she was ran over by a mack truck! She told me she went to a concert the night before and she was still pretty tired.

First thing she started filling out all the morning paperwork and a couple times she closed her eyes but just for a split second. We have all done that though, at least I know I have! So I gave her the benefit of the doubt. She was acting a little odd too but I just chalked it up to being tired.

We got the morning started and I was doing Emily's stretches and the nurse was setting on the floor with me so she could learn and she straight up fell asleep! I just stared at her wondering what the heck I do.

Joe and I were just going to run to the grocery and get some lunch together but since nurse sleepyhead was here I did not want to leave. My sis-in-law stayed the night so I asked her to watch them and we would be quick.

Honestly I should have just asked to leave but I am so not a confrontational person, it terrifies me, so I thought I would give her a couple more hours to wake up and since Cayla was there she could tell me what happens.

Joe and I went on and Cayla texted me and said she was charting and she fell asleep with her face in the notebook.

That was it, I called her boss and they called and told her to leave.

Cayla said the nurse lied and said she was setting there watching tv and charting! Watching tv in the back of her eyelids I guess! So she cried and begged but I just could not trust her anymore.

I know she needed the job but it is my responsibility to make sure Emily gets taken care of and there is no way she could have done that.

So we have to start all over again. Honestly Emily is not a ton of care. Just a lot of seizures , meds, and g-tube care. She is not on o2, does not need suction, she is not extremely fussy, and she loves to cuddle. What more could you ask for in a patient! :)

I can do all of Emily's care 24/7 I just need a break because most of her seizures happen at night and she has them regardless if she is sleeping or not. So I am up through the night to watch her because when she does have them she does not make any noise and her movements are pretty small so she would not wake me up.

We are trying to get a 3rd shift nurse just Mon-Fri but apparently that is very hard to find.

Emily's seizures were exactly the same from the night before. She just has 8 and all were under 8 seconds, I should play the lottery haha!

Hoping we get to go to church in the morning, I miss everyone there.

I will not sleep

What a day we had yesterday!

Emily started the morning out not going to sleep (again) she will normally fall asleep around 4 or 5 but yesterday was not the case. Close to 6am she began has seizures. She had a total of 6 (less than 10 second) seizures in 30 minutes. Two doses of ativan and they stopped.

Ok, after two doses of strong ativan and no sleep all night one would think this child would be really sleepy right? Nope, not Emily. She did not go to sleep until NOON! Thank goodness Joe's sister,Cayla, is here.

I tried everything to get her to sleep. Finally I gave her some gas drops and ahhh that was the trick! Cayla took her and rocked her to sleep and I finally got to sleep too.

Emily proceeded to sleep off and on for the next 12 hours and now its almost 1am and she is wide awake.

She has a appointment at 10am in Louisville for g-tube clinic so we can learn to take her g-tube out and put it back in if we ever need to so no sleep for me until we get home.

Again let me say thank God for Cayla!!! I wish she would just move in with us. Wink Wink!!

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!

Eh

Eh, is how I feel. Not great but not the worst that I have been.

Emily has been seizing everyday. Some days are better than others. She has had a few seizure-free days and those days are fantastic! It just makes the days when she does have seizures seem worse.

She has only been on the ketogentic diet a couple weeks and we have seen improvement in the length and frequency of seizures but no amount of seizures is ok.

Emily's seizures are mostly tonic-clonic and come in clusters. Some only last 2-5 seconds and others will last 30-45 seconds. The past few days she has begun to have single seizures lasting just a few seconds and not going into clusters. Yesterday she had 2 clusters one was 13 minutes that had about 7 individual seizures and the other cluster was over 2 1/2 hours and I have no idea how many seizures were in that one.

So some times I think things are getting better than she has one for 2 hours.

I have heard of some kids seizures getting better after they began to wean off some of their seizure meds. Emily is currently on klonopin, phenobarbital, and levocarnitine routine and lorazepam and diastat for prolonged seizures.

I would love to wean her off klonopin but she has been on it for a long time and weaning will need to be very slow so she does not withdraw from it.

Also if I may continue to complain for a minute, I am not very happy with Emily's home health company. Professional and helpful are the last two words I would use to describe them.

We had a fantastic home heath and a wonderful tiny portable feeding pump through them but fantastic home health can not provide Emily's ketocal formula so we had to go through crappy home health and they threw a hissy fit and said they would not provide her formula unless they provided her pump WHICH would be fine but they will only give us a hospital grade 79lb (kidding) feeding pump. GRRR! I tried to get the formula through WIC but Indiana chose not to provide that formula but KY WIC does.

So after all that complaining I compare my suffering to Jesus'. Kinda makes you stop stop complaining a little huh.

As I am typing this Emily has fallen asleep (for a little while at least) and she looks so perfect. She is perfect in every way because God made her perfect. Not our idea of perfect, but His idea of perfect. Just like to Mary Jesus' death was not perfect by any means to her, but to God it was perfect. The wonderful thing though is a few days later Jesus was perfect to us all! One day Emily will be perfect to us all too! That is the promise we have in Jesus. He died so that we can one day be healed and be perfect with Him. I keep my faith in Him.

Happy Easter. I pray that you celebrate the true meaning and find comfort in His resurrection.