Prayer

We are preparing for the hospital tomorrow. Emily will be admitted for a day or two and have an overnight video eeg so see whats going on with her seizures and to start ACTH again.

Emily is weaned off the ketogenic diet now because we have decided to do another round of ACTH.

If you remember it was the very first medicine we tried when she was diagnosed with IS.

This is what it did.

She got very swollen, stopped smiling and well stopped pretty much everything else.

We are very aware of the risks but we also are VERY aware of the risks of uncontrolled spasms and seizures.

We have postponed her surgery until the swelling has gone down, so probably some time this summer is when she will have it.

So I ask you tonight to please pray. Pray that we can get our miracle. Pray that we can control these horrible, life altering, life ending seizures.

We are desperate for a cure.



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.

Infantile spasms awareness week

As I set here feeding my 16 month old daughter through her g-tube, I am aware.

This is infantile spasms awareness week. It is sponsored by the company ,Questor, who manufactures the steroid injection ACTH (acthar gel). The same company who makes almost $30,000 per vial (we used 3!) eye roll
This is what she looked like on the ACTH.

Anyway....
No other company or organization is doing anything to bring awareness. I had planned on this week doing several blogs, posting videos and stats but Emily ended up back in the hospital again on Monday night. So I have not had the time to do it all.

It was the quickest stay yet. She was admitted around 11pm and we were home by 3pm Tuesday.

She had been doing really well, Saturday and Sunday she had no seizures and then Monday morning it all changed. Her spasms are usually an indicator of how her seizures will be. Less and more mild spasms usually equals little to no seizures and when she has frequent, harder spasms her seizures are crazy.
She woke up to a lot more spasms and the clusters were lasting pretty long too.

Just as predicted she started seizing just a couple hours after waking up and they "seemed" to be under control. Never trust a seizure! :)
Joe got sent home early from work so we laid down and were napping when Emily's nurse hollered for us. We ran out to the living room and she said she had a new scary seizure.
She described it and right on cue Emily did it again. It was a tonic-clonic (grand mal) but she was screaming with it. They were not long but she quickly began clustering them. I gave diastat and called neuro gave some more meds.....they were not stopping. It was only sedating her and my biggest fear is aspiration. Once she could not clear her throat I get nervous.
There was really no changes made this time, they just monitored her and gave some IV ativan.
Today she has only had 1 seizure.

Were are working on getting an oxygen monitor for home so that we can avoid hospitalizations like this one. If we can monitor her oxygen at home when she is that sedated hopefully we can stay out of the hospital during cold and flu season.

I am aware, aware that infantile spasms is scary, destructive, and life altering.
Before the diagnosis

 After the diagnosis

We have had to make choices no parent should.  We have sacrificed a lot and will sacrifice more. We have gone days without sleep, food or showers. We lost half of our income when I quit work to take care of Emily. I have lost relationships with people who in most families are their greatest support system. People just don't knock down your door to visit, we don't have must else to talk about these days other than Emily and everything that revolves around taking care of her.

Dec. 3 2009 was probably the worst day. When I heard the diagnosis of IS I felt relieved. A name like that CAN'T be that bad, right? Then I saw the sadness in the neurologist eyes, he knew how hard this road was going to be. I am so thankful we have such a great neurology team. Our neurologist is kind and helps with whatever we need. I am pretty sure Emily is his favorite patient (wink wink) :) The whole office is always very helpful and most of them know us well. When we are in the hospital the nurses know us well and take great care of us.  We have a great relationship with the neurology nurse practitioner and Emily's dietitian also. While I wished with every ounce of my being that we did not have to have those people in our lives, I am thankful that we do have those people who take such great care of her.

I pray one day we can find a cure for IS and epilepsy in general. I have many friends I have met through blogs, forums and facebook and watching so many kids suffer daily from seizures and spasms is devastating. Since last Dec, I have seen 5 kids pass away from epilepsy, we NEED a cure!

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!

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.

I am aware!

Today is epilepsy awareness day and I never in a million years thought I would have to be dealing with this.

Emily's form of epilepsy is labeled as catastrophic. It is also rare. Some sites say 1 in 2500 are diagnosised.

Most of the time they find a reason infantile spasms begin.

Genetic, traumatic birth, premature birth, or different types of infections in the spinal cord or brain are a few of the reasons.

So far we have done 3 rounds of genetic testing, including a lumbar puncture and all have come back normal. She was delivered via c-section at 38 weeks and she just had her very first fever in the hospital after surgery a couple weeks ago.

We cannot find a reason for Emily to have this horrible disorder.

There are many other theories as to what might cause this such as vaccines and I have even read somewhere stress. I am not sure I believe those things though.

Emily's prognosis is poor. She has had brain atrophy (shrinking) and is delayed. We don't know if these things are caused from her seizures alone or if it is indeed a genetic issue that the doctors just have not found yet.

It is hard to watch my almost 10 month old not sitting or crawling, barely able to hold her head up, or be able to say mama or dada.

I know that I am partial but Emily really is a beautiful baby. She does not have one blemish or birthmark and her eyes sometimes seem green and other times blue. She has the most perfectly shaped lips that I could just kiss off and my grandma's little upturned nose.

I pray that one day Emily will be seizure free. I also pray that the seizures she has had have not caused too much damage. I long for her to run and play and sneak my fingernail polish and write on the walls!

God gave me Emily because He loves me. That I know. Why she has this horrible disorder is something that I just can't figure out. I trust and have faith that one day we will figure it out. Until then I have a beautiful, amazing blessing that has epilepsy, but epilepsy does not have her!

So I am asking all of you to pray for a cure not just for Emily but for all the people suffering with this nightmare.

All of these pictures where taken before her diagnosis. I will get our picture of us wearing purple up later on today, she has had a very rough night so far and is asleep from all the meds. Please also pray that we don't have to go back to the hospital.

Thank you all so much for your support!!!