Lauren's update

2011-10-04T18:50:00.001-07:00

Yesturday I was looking at my dad's phone and found these videos of last year when Lauren was coming off the diet. She was in a bad state with her seizures, happening pretty constantly. It was so fun watching her eat the food and knowing what she ate for 2 years! We stopped the diet cold turkey because I wanted to try steroids or the ACTH. We were planning on starting the ACTH but the doctors changed their minds and didn't feel comfortable putting her on the ACTH. It took 9 months after this to find a doctor willing to try the ACTH on her.

It so amazing to see how far she has come since this video!

http://www.youtube.com/watch?v=CmQgpRLOP5Y&feature=mfu_in_order&list=UL

http://www.youtube.com/watch?v=TjhGOXKC-SM&feature=related

One Year Anniversary Being Seizure Free

2011-09-28T21:55:00.000-07:00

It was one year since Lauren had her last seizure!! We went out to eat tonight to celebrate. It has been such a miracle and we feel so blessed to have had such a good year with Lauren. We are hoping for more years of the same?

2011-09-25T22:25:00.000-07:00

Happy 6th Birthday Lauren! Last Monday Lauren had a birthday party with 11 other kids in our neighborhood. We used all her party stuff we had from last year because her last birthday she was in the hospital and was unable to use it. What a difference this year! It is so amazing and unbelievable to me to see where she has come in one year!

2011-06-10T23:00:00.000-07:00

Lauren is still doing amazing! Almost 9 month of no seizures! She just finished up Pre-school and will be going to the Special Needs Kindergarten in the fall. With school out we are increasing her therapy but she still has some time to enjoy summer.
Below is a video I put together going through these past few years. This song has always touched me, so I wanted to use it to tell Lauren's story. Just a little warning, there are some videos of her having seizures in it(in case you don't want to see that).

2011-04-08T00:12:00.000-07:00

Things have continued to go really well for Lauren. She is going on 6 plus months of no seizures. Last week she had a Neurophysiological exam. It was a long afternoon of testing! We needed to get a feel of her IQ and other developmental stages so we can evaluate if she is regressing over the years. I know she is very behind developmentally but it was still hard to hear it and see to what extent this seizure disorder has taken on her. I think of it like a storm that has hit and now that it has past (for now) you can see all the damage the storm left. Overall we are just so happy for how well she is doing, we feel so blessed and thankful.

I signed her up for preschool soccer. I have been stressed about signing her up for this, thinking I was pretty crazy thinking she could do this. Lauren was so excited getting ready for her soccer game (she has 3 older sisters who play soccer). She wanted a bag for her ball and a water bottle like they take. I was so surprised at how she did. I guess I forgot at this age nobody knows what they are doing!!!! As long as she doesn't need to talk I think it will work out?

2011-03-06T13:21:00.000-08:00

Lauren is still doing very good. I think I can say that she is now potty trained! She has gone over a week with no accident. That has been such an amazing accomplishment because we really thought that was something she would never do. We started doing ABA therapy with Lauren a few months ago which has helped her in so many ways. It is very intense and is usually used for autistic children. Lauren is not autistic but has severe developmental delays, and this program really has helped her a lot. Right now Lauren has about 25-30 hours a week of different therapies (Physical therapy, occupation, speech, ABA, and her school). She works so hard as we try during this good period to try and catch her up on 3 years of the biggest development period in her life. It is a hard and long battle-for example working daily for almost 2 weeks to get her to say the "t" when she says the word "cat". Our thoughts are that it is worth all the time and money to put the effort in now to try to get her to develop to her full potential, whatever that maybe?

A funny story this past week when she was doing ABA therapy downstairs in her therapy room which has a separate door with a key. She was down with her therapist doing a session along with a new therapist that was being trained. After therapy was done, they sent Lauren upstairs as they finished talking. My kids were home babysitting as I was gone to the store to get something when the phone rang. Our kids aren't supposed to answer the phone when we are gone but the caller ID showed the name of the therapist's family so Nathan thought it was okay to answer. When Nathan answered the phone, it was the therapist on the phone. She told Nathan that Lauren had locked her and the other therapist in the school and they could not get out. Nathan had to go unlock the school and release them! Hopefully the new therapist will come back!

2011-02-27T21:22:00.000-08:00

Here are some pictures from her overnight EEG last week. I can't find my videos that I took (I will post those as soon as I find my flip camera).

We had a good visit with Lauren's Neurologists on Thursday. Her EEG showed no clinical or electrographic seizures (which means no seizures you can see or seizures the EEG can "see"). Her EEG still shows slowing and epileptiform bursts (which means she her brain is still highly susceptible to seizures). Overall this is such an improvement and so amazing that she had no seizures detected. As you know deep down I was hoping for a normal EEG or at least no slowing but still so happy for how far she has come. As I understand it slowing in the EEG is not good and contributes to the developmental delays.

I tried to get a feel from her doctors of what I could expect with her going this long with no seizures and her EEG. All I got was...that they would not be surprised if she started to seize again (it could be tonight, 1 year, 5 years, 20 etc). That I needed to be prepared for that at any moment. I told them I was prepared for that! That is on my mind 24 hours a day, just waiting for it to all fall apart. SO being prepared for that I again asked for some sort of prognosis or what can I expect? ...He told me he doesn't know, that Lauren is in unwritten areas and that she is writing her own book. I HATE THIS!!! I know life is full of uncertainty and I understand that(I think) but a little insight or hope would be so nice to hear! I know they only want to tell me the worse case scenarios or what is most likely going to happen so they don't get my hopes up or expect to much from this good time. We have always felt/hoped that Lauren would overcome this and it has been a constant fight to keep the doctors believing in her, so I don't know why I am bothered by all this? I am going to try to Email Dr Kossoff at Johns Hopkins (Lauren's Dr while on the Ketogenic diet, he is world renowned in this field) and see if he would be willing to review her EEG and latest history and give me his insight. He has seen a lot of Lauren's previous EEGs and I think he would be a lot more open and upfront with me. He just sees a lot more of the difficult/catastrophic cases of seizures syndromes then Primary Childrens. Who knows if he will do it and it is probably silly for me to try but I need more information (even though I know there is probably nothing more, I just can't rest until I have tried every option out there).

I know that some of you that read this have had similar experiences or if you can add anything about reading the EEG please let me know. Fawn (or others), I think your daughter is seizure free now? Did she have slowing and if she did what does her EEG show now that she is seizure free? Any insight would be helpful ( even the bad info). Below is what her EEG report said (for those that understand)...

Impression:
This was an abnormal long-term monitored EEG recording because of slower then expected background frequencies and the presence of relatively infrequent predominantly generalized epileptiform bursts of brief duration, particularly during sleep. This record does suggest an underlying seizure tendency.

Background activity:
With patient awake, variable 5-6 Hz theta was seen posteriorly. Occasional slowing was also intermixed. Smaller amplitude mixed frequencies were seen in the frontal and temporal areas.

Sleep record:
The patient did become drowsy and fell asleep. There was an increase in the amount and amplitude of background slowing. Sleep spindles and V waves were noted.

Interictal Abnormalities:
There were several bursts of large amplitude irregular slowing with intermixed sharp or occasionally spikes. These were less then a second in duration and were not sequential. On a couple of occasions, a left frontal spike was noted.

Clinical events/electrographic correlates:
There were no clinical or electrographic seizures captured

2011-02-20T12:30:00.000-08:00

Lauren's 24 hour EEG went great. WE should get results this week at her Neurology check up.

The Neurologist that was on-call when she was admitted for her EEG this past week was the same one that was on-call over a year ago when she had gone into one of her really bad seizure episodes. (there are about 10 Pediatric Neurologists at the hospital and they all know Lauren through her many stays at Primarys) It was at that time last year I first wanted to take her off the keto diet and put her on steroids/ACTH. I had brought all my medical journal articles with the experimental research on steroids/ACTH on children with uncontrolled epilepsy . This doctor would have nothing to do with trying it and seemed a little put off that I was using these medical articles to try to get what I wanted (the attitude of .....I know what they say you don't need to tell me, and it is not going to work-probably nothing will). It was about 9 months later that I found a doctor willing to try the ACTH. Even though the ACTH almost killed her I honesty feel that it is what helped her, it has been since then her seizures have been in control. Well when this doctor came into the room he was in complete shock (just hearing that Lauren has been seizure free for 5 months, they hadn't even hooked her up to the EEG yet). He stayed in the room for about 20 minutes and all he kept saying is "all I can say is I am completely shocked and I am so amazed!"). He even started asking what dosing was used for the ACTH. I asked him if he had ever seen this before (someone with seizures as bad as Lauren go this long being seizure free), he said he had seen it once when he was a resident. He continued to say, that when you see children having seizures this bad and uncontrolled you know there is really no hope. He then said, " I am just going to hang my head and walk out quietly backwards and do nothing." So I guess from his response it is truly amazing/miracle that she is doing this good! But along with that I always have to say and be aware that this could all end today, and I still live every moment wondering when it will end.

Here are some videos from the group called CURE. A lot of other organizations with epilepsy give help with support and information on seizures (which is great) but in the end we need a help finding a cure, which seems to be their main goal. I am going to keep looking into this organization and see how to best help.
http://www.cureepilepsy.org/video/2007_CURE_video.asp

http://www.cureepilepsy.org/video/2008_CURE_video.asp

2011-02-13T20:17:00.000-08:00

Lauren is still doing amazing (which translates into no seizures)!
This Tuesday I am taking her to Primary Children's for a 24 hour EEG( brain waves). They will hook her up and monitor her for 24 hours (it will be so fun to entertain her that long, she is allowed to move about 2-3 feet in bed). The main thing we want to see is what is going on with her brain through the night or while she is sleeping. These past few years the night has been one of her hardest time. Even during somewhat good periods of seizures (1-2 granmals a night in her sleep) as soon as she would go to sleep her seizure activity picks up and she has lots of sub-clinical seizures (seizures you can't see but the monitor pick up on). When she is in a bad state she will have almost constant seizure activity showing on the EEG at all times. Overall her EEG for her age has been very abnormal. Her first EEG she had when this all started was normal but quickly it went very abnormal. If I understood EEGs better I could better explain all of it, but I don't. I know that she has back ground slowing for her age which probably goes along with her developmental delays. So I am very interested to see if there will be any improvements in her EEG that go along with this good period she is going through.
I am so nervous about this! I know I will be up all night watching the monitor (even though I don't fully understand it, I do know the waves go crazy when she has any type of seizure). I know it should not matter what the EEG shows and I should just focus on how good she is doing clinically. But over the years the EEG has served as such a guide into how she has been doing. I am telling myself to not get my hopes up that most likely it is going to be very abnormal still, for that is what the doctors have told me is always going to be her future. But I keep letting the words "what if its a normal EEG?" creep in my mind. "No that is sooooo crazy and unrealistic" I think and I quickly get mad at myself and try to prepare myself for the worse case, because that has been the reality of Lauren these past 3 years. I don't know why I do this, it is a constant battle going on in my head.
One of the reasons we are doing the 24 hour EEG is decide if she should have the VNS surgery (vegas nerve stimulator). Her Neurologist thinks it would still be beneficial to have it implanted because he doesn't believe this good period will last and her brain would still benefit from the surgery. So that is what we will be talking about at our next Neurology appt after we get the results from the EEG. I am praying so hard that there is improvement in her EEG and this is not a decision I will not even have to face.

2011-02-06T20:05:00.000-08:00

Lauren is still doing amazing! She has an 24 hour EEG at Primary Children's Hospital in a few weeks where we will be able to see what her brain is doing. Hopefully seeing an improvement in the EEG like we are seeing in her.

Below is a link to a video I recently watched from a leader of our church. As I thought of life and many of its challenges this message really helped me and I wanted to share.

http://old.mormon.org/mormonorg/eng/videos/come-what-may-love-it?tabId=beliefs&index=1&autoplay=true

2011-01-30T23:24:00.000-08:00

I am really behind but I have wanted to post some pictures of when Lauren was in the hospital for 2 weeks this past September. This is when she was having the very rare and serious side-effects of the ACTH. The pictures do not even show how big she really was (or all the hair that grew all over her face). All these pictures were taken after coming out of ICU where she was for almost a week prior ( I have no pictures of her while in ICU, I guess my mind was worrying about other things!). These pictures she was a lot more responsive, awake, and able to interact some. It was pretty bad while she was in ICU (really low potassium, heart problems, blood gases off etc).

Her stomach was so big! The nurses had to measure it multiple times a day to see if it was going down and not getting to big.

She had a Birthday and turned 5 while in the hospital. Family came to help celebrate. The hospital brought her a cupcake with paper candles (the video is of her "blowing" out her paper candles). She was still pretty unhappy and miserable on her birthday but was able to sit up some (which is more then she had done in the last 10 days).

This was a hard time for us. Lauren was pretty bad and we were losing all hope that she would ever get better. But we are gong on over 4 months and counting of no seizures. Her last seizure was on the day before we left the hospital. Do the doctors know what helped or caused this good period? No. Do they know how long or if it will last? No. But we are enjoying every moment while it lasts! She is doing so good!!!

2010-12-26T15:57:00.000-08:00

Lauren on Christmas day 2007. The day before her seizures started.

It was 3 years ago today that Lauren's seizures first started. I tend to relive the trauma of the day so I will put it on here, then I can also have a record of it too....

We had just had a wonderful Christmas and the kids were up in the morning playing with all their new presents. Lauren was so happy because she had received a new kitchen set from Santa. She was playing with her sisters in the family room and next thing I know Marc is yelling at me to call 911. We were not familiar with seizures at that time and had no idea what was happening. I really thought she was dying (Her lips were completely blue). The dispatcher was trying to keep us calm and telling us things we should do. The ambulance, fire department, and police arrived(we had told them she hit her head when she started having the seizures so the police had to come investigate) . Her seizure had stopped and the EMT checked her out and reassured us that she was fine and would now sleep in a postictal state for awhile. They told us to just take her into her pediatrician to make sure things were ok. We took our 4 older and very shaken up children to my parents house and took Lauren and Ryan (just 3 months old) to the doctors office. It was the holidays so it was not normal business hours but luckily our neighbor was on call. He looked her over (she was still in a very deep sleep) and sent us over to the hospital lab to have a blood test done. While we were waiting at the lab (which was located in the hospital) she woke up and started acting like her normal self. Within 5 minutes she started having another grandmal seizure. This one sticks out so big to me because I felt like we were in the middle of some movie. A huge crowd gathered around and Marc was asking anyone if they knew CPR (she was turning blue again and that was before we knew you weren't suppose to do CPR while having a seizure). I was just watching and crying in disbelieve, wondering what in the world was going on. Staff at the hospital were requesting emergency help over the intercom and phones. Next thing I know a team of doctors and nurses come running through the halls with a stretcher and other medical equipment. They loaded her little body and pushed her to the ER. As they were going to the ER the questions began, "did she get into poison, is there any medical problems, were their problems with your pregnancy???etc.. My mind was racing trying to think of anything that might of happened to her, something I might of done while I was pregnant to cause this. Soon the seizure was over and she went back into the deep postictal sleep. In the ER she had a ct scan and the blood work done (all were normal), and slept through it all. While waiting for the results she woke up and within a few minutes she had another grandmal seizure. In a matter of a few hours she had 3 grandmal seizures so at that point they loaded her with medication and put her in a drug induced coma like state. At this time they preformed her 1st EEG (brain waves) which came back normal, (this would be the last and only time she had a normal EEG, within 1 week her EEG was very abnormal). She slept for the next 24 hours, and that was the beginning of our journey/battle with epilepsy. It seems like yesterday this nightmare started but in the same moment it feels like an eternity.

Lauren waking up for the first time after her 3 grandmal seizures and drug induced state. Little did we know what she was waking up to and how her world (and ours) would change!

Christmas this year -2010! 12 weeks and 6 days (and counting) of no seizures!
Our hope and prayer is that 2011 will continue in the same way!

On a side note:
The other day I sent Make a wish a link to the video I put together and a thank you note for the wonderful trip our family had. They posted it on their blog page. There are a lot of really neat stories of other wish children on the blog. here is a link to it. http://makeawishfoundationofutah.blogspot.com/

2010-12-15T20:43:00.000-08:00

Here is a movie from Lauren's Make a Wish trip. It was the most amazing experience, beyond words! Our whole family was treated with such love and kindness. The greatest part is that Lauren had no seizures and is doing so good. She is now at 11 weeks and 1 day seizure free!

Lauren's Star Party

2010-11-14T22:20:00.000-08:00

On November 5 Make a wish gave Lauren a party! It was a fun night to be with friends and family. They have the parties so we she can raise a star and as a send off before our Disney world trip. Every child that has been granted a wish from the Utah "make a wish" chapter has a star on the ceiling. I have a video of it but-again-I can't figure out how to get it on here? The song "as you wish upon a star" played while Lauren (with the help from Marc) raised her decorated star up. The whole experience has been amazing!

The next night with the extra cake we celebrated Lauren's 5th birthday. She was in the hospital on her real birthday (sept 19) so we never had a chance to really celebrate.

2010-11-14T11:49:00.000-08:00

Lauren is still doing really good, going on almost 7 weeks now of no seizures! We met with the Neurosurgeon about implanting the VNS this past week. We were fully expecting her seizures to be starting up again by now, so we are putting the surgery on hold for now. As soon as they start up again we will schedule the surgery and give the VNS a try.

Last week we also had Lauren's star rising party at the make a wish building. It was such a fun night. They make everything they do so special. She came home with almost every Disney character stuff animal (over 10) and a really big Minnie mouse. They gave each of our kids a bag of treats and toys to take on the plane for our make a wish trip to Disney World. I really need to post some pictures of it all, it was such a great night. We feel so grateful for everything they have done. I am not able to post pictures or videos, every time I try only a mix of letters and numbers show up? So when I get a moment I will have to figure out whats going on and get some pictures up.

2010-10-31T13:58:00.000-07:00

Another parent with a child with Doose Syndrome shared this website.

www.ice-epilepsy.org.

The ICE stands for intractable childhood epilepsy, it looks like a great site! I wish I would of been aware of it sooner! Their slogan and more info from their site...

“ICE the Inferno” , the slogan ICE Alliance has adopted, reflects the devastation, grief and pain suffered by the children who experience uncontrolled and life threatening seizures as well as the families who love and take care of these children while epilepsy destroys their developmental abilities and often takes their lives.

Drug resistant epilepsy in children can lead to brain damage, developmental delays, psychological abnormalities, and even death.

ICE Alliance is working to propel research on the intractable epilepsies of childhood in order to better understand the cause of seizures and streamline therapy towards a cure. We strive to provide pertinent, professionally written and evidence-based information to parents, caregivers, and healthcare professionals in order to improve the outcome of children faced with drug resistant epilepsy.

Lauren's syndrome is in the class of a "catastrophic drug resistant epilepsy". There is little research being done on this group. Most people (70%) that have seizures have them controlled with medication so the remaining 30% are left with only a few options, and when those options don't help there is little hope left. What I like about this website is they are searching for a cure specifically focusing on this group of children that have drug-resistant epilepsy.

Just wanted to share.

2010-10-20T12:10:00.000-07:00

I hate saying anything about how Lauren is doing when she is having a good period or honeymoon from a new drug. So all I am going to say is she is doing really good since coming home from the hospital. While there she was started on Keppra in addition to her other drugs-felbatol and vimpat. I am not going to say the number of days/weeks things are good because this road is a complete roller coaster.
As much as I love seeing Lauren doing good it is a hard time for me. I am always wondering when I am going to start hearing the awful sound that comes with a grandmal seizure. At night I have anxiety wondering if this is going to be the night the honeymoon ends. I know I should focus more on the positive and enjoy the good times when they come, but I don't. As soon as I think "maybe this is going to work!" the seizures start back up. This has been the story for the 2 years and 10 months this nightmare has been going on. I have learned to function better in Trauma and hospitals then in "normal life". I tell myself to stop worrying, stop fighting to make her seizure free and go on with our "new life", make the best of it. I tell myself to give up, you have done the best you can to help her. But I can't. I am consumed with finding something to help Lauren. I see/remember the child that she once was and that comes out during good periods. I can't give up even though I want to. But I am tired of reading medical journals, searching the internet, flying to doctors across the country in hope that I might find something. Some doctors tell me that this is going to be her life but can't tell me what if anything is wrong with her. I can't accept that. But I am tired and weak, this past 2 week hospital stay tested my determination, faith, strength, and hope. Many people tell me how strong I am, I am not the strong one, Lauren is. What she has and continues to go through is so much for a little 5 year old child. Her strength and strong will is unbelievable! She bounces back time and time again, although it has taken a huge toll on her abilities-she still fights. So how do I give up when she doesn't? So we continue to fight and experiment...hoping everyday. But I have to keep reminding myself that Lauren is in the Lord's hand, I can only do so much.

We had to cancel Lauren's Make a Wish trip to Disney World when she was in the hospital. We just rescheduled a new date and they are planning Lauren's wish raising ceremony and farewell party at the Make a Wish building in Salt Lake. As soon as we get a final date I will post it, and all are invited if you are available to come.

Also, I will post some pictures soon.

2010-09-28T21:50:00.001-07:00

Lauren is home from the hospital! Yea! It feels so good and she is doing pretty good. She is so fat it doesn't even look like her and she has acne all over her face-it is so sad. As we wean the steroids over this next month those symptoms should improve. When she was discharged the doctors explained more of what happened to her from the ACTH. They told me they had to fluid resuscitate her in ICU. The main thing that was of concern was her PH (along with her potassium-but not so much). That once her PH was not so high they moved her out of ICU. Then trying to maintain it all was the hard thing as she continued to have the access urine. It is really so much and new I can't really comprehend all of it.
As far as her seizures. We started her on Keppra (this is the 2nd time we tried this drug. It was the very 1st drug she was on when the seizures started almost 3 years ago). We are hoping to get another honeymoon period to get us through this wean and bad patch. I have no hope that any medicine will work. We have an appointment on Nov 1st with Neurosurgery to look into implanting the Vegas Nerve Stimulator(VNS). I will post more info about it when I get a chance. It is something that has been mentioned before but quickly passed over. It possibly helps shorten or improve but most likely not stop the seizures and if it helps/works can take up to a year to get it to the right level.
Thank you for all the kindness and prayers!

2010-09-26T13:17:00.000-07:00

Things have been getting better. We actually were going to get discharged today (sunday) but the endo dr came in and wanted her to stay a few more days as they reduce the high dose steroid a little tomorrow. But everything was going good and were just hanging out playing then she started to seize again. We are getting ready to load her with the IV keppra again. So hopefully just a little step backwards?!

2010-09-25T18:12:00.000-07:00

Yesterday was such a roller coaster of a day! Lauren seizures went out of control. They gave her iv Valium and she continued to seize. In an 1 1/2 hour period she had 3 iv doses of iv valium and was continuing to cycle grandmal seizures every 20-30 minutes. The pediatric resident doctor (not a Neurologists) told me that there was probably not much more the neurologist could be doing. I was so frustrated and told him that they better do something fast or this is going to get a lot worse. He left and didn't get the neurologist. I lost it (emotionally), to hear that there was "not much they could do", something i know deep down but to hear out loud hit me the wrong way. Lauren's nurse was totally shocked at the doctor and the next thing I know I have a social worker in the room, the charge nurse on the phone insisting that Neurology get down immediately to talk to me. Things then quickly turned around (thanks to good nurses!). The neurologist came and we decided to load her with IV Keppra (the 2nd drug Lauren tried which did nothing-but time and keto diet can change the brain) to stop this bad seizure cycle. We also had a long discussion about trying the VNS (Vegas Nerve Stimulator) when she stabilizes. So her seizures quickly stabilized for now. Next the Endocrinologist came in and told me she was us late researching and consulting the National institute of health (NIH) to see what more they could add to this situation. They told her we could wean the ACTH faster and transition her to a high dose steroid. Again she added how rare this response is(this coming from the main consultant from the NIH). So we stopped the ACTH today and replaced it with high doses of steroids that we will wean over the next 30 days. Then the attending Pediatric doc came in next to address her high heart rate (160-185), she said she has no idea why? So at the end of a very long and intense day Lauren is doing pretty good. I guess we will see what tomorrow will bring!

2010-09-23T21:05:00.000-07:00

Yesterday Lauren's heart rate started going in the 170-180 range. The are wondering if she is getting dehydrated again. they gave some fluids and her heart rate went went down some. The doctors involved with this part of been somewhat frustrating. They want to see if her body will just regulate her urine output on its own. We know that on the ACTH at this level she was having problems. we have gone back and forth with the drs these past few days in how to approach this. Her heart rate has continued to be really high, they are now worried her pic line is infected. BUt gave her more fluids as we wait for the blood cultures to come back, her heart rate did come down a little. This evening I feel that I can finally trust the doctors because they finally admitted that they are worried and don't know exactly what to do. we are ok with that-actually very use to that. So they called in the endocrinologists to help, and I had a great conversation. The endo dr said that this reaction/side-effect is very rare, in fact she has never seen it and only read about it in some medical literature in a few cases. (So I guess the rare gets even rarer(is that even a word). The endo dr said she is going to research tonight and will have more answers some things we can do in the morning. Her seizures are not doing too good right now either, most likely the wean of the ACTH.

2010-09-21T21:30:00.000-07:00

Lauren is pretty stable right now. They stopped replacing her fluids to see what her body
will do on its own. Thinks looked ok but she is starting to show the signs of being dehydrated tonight. The drs are coming by at 11pm tonight to reevaluate. Otherwise she is up and more interactive and playing some. Her seizures are still clustering, (had 3 gradmals in 1 hour time-with them every 2 hours during her sleep.) so time will tell if she goes in a bad state with her seizures. But right now she is still recovering from them. In the morning we do another drop in her wean- so hope all will go well.

2010-09-18T23:00:00.000-07:00

Lauren was moved out of ICU yesterday. She has moments where she is somewhat happy and interactive, but most of the time she lays pretty out of it. We reduced the ACTH from 80 units a day to 40 units on Friday. Her urine out put has gone down a little, but still over 3 times the normal amount. So they are still replacing fluids most of the day. She is also still on the oral and IV concentrate potassium. Last night she had 4 grandmal seizures through the night and then had another one when she woke up this morning. That usually is a sign to us that things are going to get bad. She was treated with IV Valium and has been good (seizure wise) the rest of the day. Coming off the medicine is probably going to be bad on her seizures even though the seizures were not that many before, its just any changes with Lauren and her brain freaks out. So we have to get her off the ACTH fast but it is probably throw her in a bad seizure cycle. The idea was thrown out to start her back on the ketogenic diet when her body stabilizes, but I am to tired to think through that right now. Tomorrow (Sept 19th) is Lauren's 5th birthday, so we will go up and try to celebrate it best we can.

2010-09-15T21:43:00.000-07:00

Things are still the same. They are keeping Lauren in the PICU until they can get her levels in good range-which they are having a hard time dping. The main thing being her potassium. They have her on normal IV maintenance fluids, plus concentrated IV potassium, and an oral potassium. Her numbers still stay low, she is just peeing everything out. Tomorrow they are going to put a pic line in because they have to keep giving her new IVs because all the fluids and potassium are hard on her veins. They are going to try to wean the ACTH a little faster (every 5 days) and see if her body can handle it. She is awake most of the day but still not doing much but laying there (and eating tons of course).

2010-09-14T22:26:00.001-07:00

Lauren is still in the pediatric ICU. They were hoping to move her to the regular floor today but her blood levels are still off. The main thing they have come up with is that the ACTH is lowering her potassium and causing these other side effects. Today they have been giving her fluids almost constantly. So for example, they gave her 250ml of fluid so they would expect to get a similar amount in urine but she was having over 700mls. Then they would make up the difference in giving her more fluids to compensate. They are trying to figure out how much fluids she needs to stay hydrated. Her heart is looking good today, all tests looked normal. her heart rate was in the high 50s low 60s last night and today it has been in the low 100 (normal range). She was awake most of the day, although just not able to move or do much. It will take us at least 3 weeks to wean of ACTH and they told us to expect at least 5-6 more days in the hospital. Overall it seems she is doing much better today.

2010-09-14T00:43:00.000-07:00

A lot has happened these past few weeks. Just wanted to give a quick update. We started ACTH about 4 weeks ago. The first week was great but then she started sleeping a lot and her stomach just started getting huge and she was peeing tons. I took her to the ER 2 weeks ago and they ran tests which all came back normal, gave her some fluids and told me she probably just had a virus. I told them I have 6 kids and this is not a virus-she was sleeping all of the time. I have been told these are not symptoms of ACTH so we continued on them (because of improved seizure control). I continued to express my concerns to the neurologists and began feeling like I was just being a"paranoid" mom. I couldn't shake the feeling something was wrong so took her back to the pediatrician today who ran some more tests that showed some abnormalities. So tonight I took her to the ER at Primary Childrens and they just put her in ICU. Her potassium is low, her gases are off, and her heart is slightly enlarge, etc(that is the best I can come up with). Her heart rate is beating low but her EKG was normal. They are trying to figure out what is going on with her body. We are working to wean her off the ACTH but that will take time-up to a month (we will go over a plan in the morning). We are just hoping that once we get her electrolytes normal and get her off the ACTH she will be fine. I guess the positive is that her seizures control has been better, but she just can't function. I guess I can cross ACTH/steroids off my list. I feel so bad for Lauren, she is just one big experiment.

2010-08-06T15:06:00.000-07:00

Lauren is still up and down. She has seemed to stabilize a little these past few weeks. We are changing plans for her treatment. She was in the ER a couple of weeks ago after we started her on the topamax. It seemed to aggravate her seizures so we quickly weaned the topamax and started her on Vimpat a brand new drug that was recently FDA approved. That is what is probably giving us the stable period now, but not expecting that to last too long, and besides she is still having a lot of seizure activity. We went to Primary Childrens on Tuesday to meet with her Neurologists. I brought some research articles about using steroid treatment that I have been looking at for the past 8 months. Her doctor was very open to try, pretty much saying there is no wrong choice-all trial and error. The first type of steroid treatment we prefer to try is ACTH. The cost is over $100,000 for one treatment period. We don't know if insurance will cover it but they put in the request today. We should find out in the next week. I put some information about ACTH(a very strong steroid) below. All I know is that it is given by injection twice a day. They will teach us how to give the injections and depending on how Lauren responds, she would be on ACTH for a few months.

Also, when Lauren was in the hospital last December the doctor on call came in to her room in the middle of the day (which does not usually happen) and told us that she found something that may be wrong in one of her tests that had been done two years before when all this all started. It was a Neurotransmitter test and it appears that the person who read the results from Baylor University marked it normal when it may of been high. We don't know if it was a mistake or if the test is abnormal, so to retest Lauren they needed to do a lumbar puncture (spinal tap). The doctor did not want to do the test back in December because Lauren was not stable enough. The doctor told us to have our regular Neuro redo this test. When I brought it up at Lauren's follow-up appointment a few months later, the doctor dismissed iut as being nothing important. This past week I brought it up again with her new Neurologist that has taken over her care (the other doctor retired) and he looked up the notes for Lauren's inpatient stay last December. Our new doctor quickly had her lumbar puncture scheduled for the next day - which was Wed 8/4. The results will take 3-4 weeks to get back. I don't know what it means (the neurotransmitter stuff was all over my head), and the doctors didn't want to go in detail until the new results are back.

So, a lot of waiting, hoping and praying for the coming weeks!

Some info on ACTH:
Adrenocorticotropic hormone (ACTH), also known as 'corticotropin', is a polypeptide tropic hormone produced and secreted by the anterior pituitary gland. It is an important component of the hypothalamic-pituitary-adrenal axis and is often produced in response to biological stress (along with corticotropin-releasing hormone from the hypothalamus). Its principal effects are increased production and release of corticosteroids and, as its name suggests, cortisol from the adrenal cortex.

Andrenocorticotrophic hormone (ACTH) is a first-line treatment for infantile spasms and it can be used in other childhood epilepsies including Lennox-Gastaut syndrome, Landau-Kleffner syndrome, and MAE. ACTH is a peptide hormone produced in the anterior pituitary gland that is administered by injection.

ACTH therapy has caused fatalities and serious complications in the past so it is regarded as a high-risk treatment and used only when it is judged that the benefits (seizure control) outweigh the risks.

Side effects of of long-term or high dose steroid therapy
These side effects are common but are dose related,
* Stunted growth
* Weight gain (cushingoid effects such as puffy cheeks/moon face, tummy bulge)
* Increased appetite, food cravings
* High blood pressure
* Low potassium in the blood
* High blood sugar
* Loss of bone density
* Behavior disturbances (eg, temper outbursts, irritability)
* Sleep disturbances
* Lowered immunity -> lowered resistance to infections
* Excess bodily hair growth (eyebrows, limbs, etc)
* Stomach ulcers
* Constipation
* Fluid retention

All of these side effects are temporary and will go away once the steroid is stopped: growth should resume and catch up, excess body weight and hair will fall away, and the immune system will return to normal, etc. Some of these side effects may be prevented; calcium supplementation may assist bone density, an antacid such as Zantac may help prevent stomach ulcers, and increased fiber intake should alleviate constipation.

There are some dangerous risks associated with long-term or high dose steroid therapy. Some risks are more serious than others.

* Eye cataracts
* High blood pressure -> stroke
* Severe bone thinning -> osteoporosis
* Induced diabetes
* Kidney stones

2010-07-12T08:28:00.000-07:00

Lauren is having up and down days lately. Last week after the 4th of July she unexpectedly started having seizures during the day. We ended up using diastat (emergency meds) and has been pretty good this past week (until today, she just had a big seizure while I was typing this! Ryan my 2 year old just said "Lauren having seizure"). The past two nights her nocturnal TC (grandmal seizures) have been increasing so I am anticipating that as the diastat wears off she is going to get worse again. This has sent me into-what are we going to do next mode? I am starting to work on getting back east to Cleveland to see Dr. Tuxhorn. In searching the internet for any new research on epilepsy (I know I do this way to much, I just want to make sure I know as much as possible in case there is something new that could help Lauren) I found this article in Newsweek. I thought it was a good article. As we have been thrown into this neurological world we have felt that the advances are so little and there is so much unknown in this field.

Here is the article....
The toll of epilepsy has been overlooked—and the research underfunded—for too long. A call to action.
The statistics are stark and sobering— and for the uninitiated (which is to say most of us), startling. Epilepsy in America is as common as breast cancer, and takes as many lives. A mysterious and widely misunderstood affliction, epilepsy is a disorder in which the brain produces sudden bursts of electrical energy that can interfere with a person's consciousness, movements or sensations. Up to 50,000 Americans die each year from seizures and related causes, including drownings and other accidents; one in 10 people will suffer a seizure in their lifetimes. By some estimates, the mortality rate for people with epilepsy is two to three times higher—and the risk of sudden death is 24 times greater—than that of the general population. There are 200,000 new cases each year, and a total of more than 3 million Americans are affected by it—more than multiple sclerosis, cerebral palsy, muscular dystrophy and Parkinson's disease combined. Between 1 and 3 percent of the population will develop some form of epilepsy before age 75. There is also a rise expected in the incidence of epilepsy among the veterans of the wars in Afghanistan and Iraq who have sustained traumatic head injuries. Yet public and private funding for research lag far behind other neurological afflictions, at $35 a patient (compared, for instance, with $129 for Alzheimer's and $280 for multiple sclerosis). It is time to remedy that gap, and to raise epilepsy to the front ranks of public and medical concern.There is cause for hope. Science is unraveling more and more of the mysteries of the brain, and perhaps the source of the cataclysmic electrical storms of epilepsy will yield its secrets. Such, at least, is the aim of Sen. Edward M. Kennedy, who is considering a major bill to support enhanced research that will fund more work toward a cure, and of a resolute band of advocates that includes the man who sits nearest the Oval Office in the West Wing. White House senior adviser David Axelrod and his wife, Susan, are the parents of Lauren, a 27-year-old who began suffering seizures when she was 7 months old. Mrs. Axelrod, who contributes a piece in the following pages, is a founding board member and president of CURE—Citizens United for Research in Epilepsy. The group's mission is clear from the acronym: to learn everything we can about epilepsy in search of a cure
Epilepsy is an ancient brain disorder, and different cultures at different times have veered between considering it a disease or thinking of it as a sign of demonic possession. Around 400 B.C., Hippocrates defined it as a physical, not a spirit, affliction, writing of what was then called "the Sacred Disease": "It appears to me to be nowise more divine nor more sacred than other diseases, but has a natural cause like other affections." Julius Caesar suffered from it; Jesus cured people with epilepsy in Gospel accounts of his ministry. The word itself is derived from the Latin epilepsia, which means "to take hold of." In 1604, Shakespeare has Othello suffer a kind of seizure as Iago works him into a frenzy of jealousy: "My lord is fall'n into an epilepsy," Iago tells Cassius. Seizures played a role in convicting suspected witches through the ages. Well into the 20th century, some states had sterilization laws that applied to people with epilepsy, and several more forbade those with epilepsy from marrying. In 1956, Roscoe L. Barrow and Howard D. Fabing published "Epilepsy and the Law: A Proposal for Legal Reform in the Light of Medical Progress," a book that helped lead to the repeal of many sterilization and antimarriage laws.Though the most overt examples of discrimination and demonization have faded with time, epilepsy still receives too little attention, either from the medical community or the public at large. Why? One reason is that advances in drug treatments have created the popular impression that epilepsy is now an essentially manageable condition. (Which, for two thirds of patients, it is. But that still leaves a third for whom it is not.) It is thought to be rarely fatal, controllable by medication. There is a terrible irony here: because most people with epilepsy are not in a constant state of seizure—they are, rather, in perpetual but quiet danger—their condition can appear less serious than it truly is. It is all too human, but all too true, that a problem, including the problem of a serious medical affliction, stays out of mind when it is out of sight.

Because so many of those who must endure it do so valiantly, and with grace and grit, it is more difficult for those not directly affected by it to grasp that epilepsy can kill. Put harshly, we need more of a cancerlike sensibility around epilepsy. We cannot usually see our friends' cancer, but we do not hesitate to invest the search for a cure for different cancers with the utmost cultural and political importance. We must now do the same with epilepsy. "We want complete freedom from seizures," says Susan Axelrod. "We want future families to be spared what so many other families, for so many years, have endured. Lives should not be defined by diseases." No, they should not—which is why all of us must focus on understanding epilepsy. And then we must defeat it.

2010-07-03T07:19:00.000-07:00

Lauren is back to baseline as far as her seizures. Still having the nightly grandmals but not any we are seeing while awake. She is off the clobazam for a few weeks now and are slowly working up the topamax levels. It will be a couple of months before we can tell if this helps.

2010-06-20T22:45:00.000-07:00

Lauren continued to get worse this past week and started having drops and grandmal seizures during the day. It had been over 5 months of no seizures while she was awake. We had to use diastat(her emergency med) last week. After she was great for a couple of days then as soon as it wore off she went right back into her bad state. It has been so hard to see her in this state after a pretty stable 5 months.
Two days ago she started to stabilize a little and the drops and daytime seizures have appeared to stop. She is up and about doing stuff but not talking much and very out of it. I am just happy that we have not ended up at Primary Children's yet. Last week we also started her on Topamax again. It will take awhile to get her blood levels in a therapeutic range so we will have to give it a couple of months to see if it will work. I really hate this drug, she is so out of it. I know the point of the medicine is to slow down her brain but with the topamax it just seems worse then the other meds. If it works I will take the side-effects, but if it doesn't I really want her off it. I don't know why I am even holding out hope that something is going to work? It has been 2 1/2 years of this and so many drugs I can't keep track, why do I think something will work now?! I guess it is just a my hope and prayer that someday we will find the right combination to help Lauren and give her brain a chance to develop.

2010-06-06T14:35:00.000-07:00

We finished weaning the clobazam this past week. When we got down to a 1/4 of a pill she started going into non-convusive status. She started drooling, staring, her hands and eyes having constant twitching. She has still maintained functioning (eating, walking etc), so it is not an emergency right now. We were expecting to have some withdraw from the clobazam but still hate to see it. The benzo class of drugs are pretty hard to wean (valium, ativan, clobazam, klonopin and more). The brain has a hard time letting go of the drug even though the brain has built up a tolerance to it. We will wait out this week and see if she gets back to baseline (which has been the nightly grandmals). If not we will add back the clobazam.
I am also undecided about the bromides right now. I can't make an appointment in Cleveland yet to see Dr. Tuxhorn. We have been thinking of trying topamax again while we wait until we can get to Cleveland and have Dr. Tuxhorn oversee Lauren on the bromides. Topamax was one of the first drugs she was on before starting the keto diet. It help control the grandmal seizures for over a month but caused her drop seizures to increase greatly. The side-effects of Topamax are not great either. Non of them are, but this one effects her expressive language which is her greatest delay. It also takes away their appetite and their ability to sweat- to name a few. The hope would be that with her drops under control that the topamax would help with the grandmals? I don't know what we will decide to do, It's all just trial and error, so I guess it doesn't matter which one we try?

2010-05-23T09:04:00.000-07:00

Fawn,
The link to the video is not that bad as far as showing seizures. It is a radio show talking with a mom that has a daughtor that has been having seizures for since she was 2 years old. She wrote a book called "seizing the day" and is putting her effort into finding a cure for epilepsy. I just put it was awful because I hate seizures so much, but the clip has a good message. How is your daughter doing? Last I remember is you were going up to a 5:1 ratio to try to stop the nocturnal TC? Did it help, how are her seizures?

2010-05-22T20:56:00.001-07:00

Well the Bromides arrived yesturday. We are going to finish weaning the clobazam before we start. We don't want her on to many medications at once. Last week I talked to some doose syndrome parents who were also starting their son on the bromides and they had to stop because it caused 3 really bad unexpected status episodes. I am always nervous starting a new medication but hearing that made things feel more scary. We will still try them because we really have no other options and it is all just trial and error. We should be ready to start them in about 2 weeks and then we will have to make a trip out to Cleveland to see Dr. Tuxhorn sometime in the next few months so she can monitor Lauren's care while on the bromides.

Here is a video link about a mom trying to help find a cure for epilepsy.

http://media.causes.com/808538?m=acb56307

2010-05-14T11:02:00.000-07:00

It has been awhile since I updated. Things have been the same these past few months. Lauren continues to have 1-3 grandmal seizures in her sleep nightly. We actually weaned one of her drugs with no withdraw seizures or worsening of her seizures. We are still waiting for the bromides. I called Caligor pharmacy (this pharmacy is in NYC and can import drugs from outside of the US). The pharmacy told me that the bromides arrived from Germany but the FDA have held it up and sent it to another one of their departments for further investigation. The pharmacy said the FDA does this occasionally and they just happened to pick this one. They told me that it is a government agency so that is why it has taken so long, it will just be a matter of time before they release it. So we are just waiting.

2010-04-13T13:11:00.001-07:00

Lauren is still pretty stable during the day and having 2-4 grandmal seizures in her sleep at night. The bromides are ordered and should take 7-10 days to come from Germany. Then it depends on how long it will take them to get through customs, which might take weeks. Then they just need to be sent from Caligor pharmacy in New York City to our house. I also just found out that Dr. Tuxhorn is leaving the Cleveland Clinic. I don't know where she is going for sure. We met with the new local Neurologist at Primary Children's yesterday. I was kind of hoping that maybe we could have him take over all the care for Lauren, but I don't think that is going to happen. He has had 2 experiences with bromides. One over 30 years ago when he was under another doctor and one time a few years ago, and the patient developed boils all over his body so he stopped the bromides. He thought it was a fine option to try but not wanting to look over her care on them. So we are probably going to have to make another trip out to where Dr. Tuxhorn moves. Right now we Lauren is a patient of the Cleveland Clinic so have to re-establish Lauren as a patient at the new hospital.

We are in the process of weaning Lauren off zarontin, so I really hope she remains stable through this. History has shown that she will probably go through a rough time for awhile, even the the med didn't do much.

That is all for now, and just wanted to add a side note to an earlier post about the cost of Lauren's medication. We have been very blessed to be able to afford all her medication, my comment was just pointing out the huge difference in cost compared to the diet.

As always we appreciate everyone's thoughts and prayers.

2010-03-28T21:34:00.000-07:00

We are still trying to figure out what the next step will be for Lauren. Dr. Tuxhorn finally called last Friday and told me that she has missed placed the formulation that she has been working on for the bromides. So we are going to work on getting the drug from Germany (because they are not FDA approved for use on humans in the US). Right now we already get another drug from Europe (Clobazam) and are some what familiar with how things work ( I call it my "drug dealing"). Unfortunately when you get drugs that are not FDA approved insurance doesn't pay for them. The felbatol med she is also on is rarely used and is pretty pricey even with insurance and the other drug Zarontin we are using the brand name which costs a lot more then the generic. Are monthly drug bill for Lauren is getting pretty high. The cost of using butter and cream as her "medicine"was pretty cheap and had way less side-effects! I really hoping to wean some of the medication, but I am also scared to mess with anything right now. I hate this!

Next month we are going to meet with our new Local Neurologist because are current one is retiring. I am working on putting together some videos of Lauren's different types of seizures. It is very helpful for the doctors to see the seizures because they seem to never happen when they are in front of them or hooked up to an EEG. And different seizures can mean different things. SO below I am posting some of Lauren's seizures. I know that there are some that read this blog that have children with seizure disorders and for me it has been very helpful when I can see what others look like, to know what an atonic drop seizure looks like for example.
I also want to have these videos through the internet if I need to show a doctor at some time. Which would of been nice to have when we were at the Cleveland Clinic. The doctor had asked to see videos of her seizures and we didn't have them with us. I am putting them on here to have a record of them if needed.
So I just want to give a warning that these are not fun or enjoyable videos to watch!!!! Some may not like to watch-including my husband. When I showed him he stopped and said he sees enough of it in real time.

This is the first seizure type that Lauren had on December 26, 2007. Right now this is the main seizure type that she is having. They are usually in her sleep between 5-8am and during her nap. She is having between 1-8 a day but always when she is asleep. Before we started the diet she would have them only when she was awake and they would come every 3-4 days and they were much longer then what she is having now.

Grand-mal or Tonic-Clonic Seizure

Shortly after her first grand-mal the "drop" seizures, myoclonic seizures and absence seizures started. In this video she has all three of these seizure types. I posted this video in the beginning of this nightmare but wanted to post it with the rest. Drop seizures are pretty hard to video tape, they happen so fast and are over just as fast, and I was usually wiping up blood or comforting her because she would usually hurt herself after a drop seizure. This is why she had to wear her helmet. I really hate these seizures, whenever I hear a load thud my heart drops. These became the main seizure type Lauren was having before we started the Ketogenic Diet, anywhere between 20-100, depending on the day. The syndrome she has is call
Myoclonic-Astatic Epilepsy (Doose Syndrome), this is a myoclonic-astatic seizure-hence the name. Right now we see these only when she is in a very bad period
(like she had this past Dec -09).

Drop Seizure

Lauren has has many periods were she starts having constant myoclonic seizures. There was a period where she was going into this state a lot. This is usually when we have had to use diastat-her emergency rescue medication. It is very hard to watch her in this state because of the constant "interruptions" she is having. A lot of people of asked what I mean when I say she is having "constant seizures". This is what it looks like sometimes a lot worse-sometimes not as bad.

Constant Myoclonic Seizures

We usually always have to use Diastat, which is rectal Valium to stop her seizing in this state. We have has to use diastat more times then I can count. I was so afraid the first time I had to use it, the EMT (ambulance man) made me do it -instead of him-so I could learn. Now I could do it in my sleep and it has saved us from many 911 calls and hospital trips.
Below you can see the seizures stop shortly after we administer the valium, but you can she is completely "drunk". As funny as it sometimes watching her in this "high" state it is also so depressing seeing your young child so drugged. The periods we end up in the hospital is when the diastat stops working (her body builds a tolerance after it is used too much). So we usually know to go to Primary Children's is after we have used diastat 3-4 times in a couple of days and the constant seizing continues despite the medication.

After Using Diastat to stop Myoclonic episode

Other times we end up in the hospital is when she goes into Non-Convulsive Status (NCS). That is when she not having clinical seizures that you can see but her brain is constantly seizing. As you can see she can't move well, her speech is slurred, her eyes are slightly twitching if you look close, she is drooling, she can't eat or even sleep when she is in this state. I have tried to put her to bed hoping sleep would help and hours latter I check on her and she is still staring off drooling. If you hooked her up to an EEG you would see constant seizures going on. Usually mixed into this she would start having drops and grandmal seizures. These have been very scary times for us in the hospital when we can't get her out of this state, it has gone on for days with no medications working. And honestly these have been the times I wonder if she will ever come out of it or will this be the time that she is "gone". These episodes have taken a toll on her. After we finally get the seizures to stop it takes days for her to be able to walk and function again. Luckily is has been since this past December since she has been in this state.

Non-convulsive Status

2010-03-22T08:58:00.000-07:00

Lauren seizures continue to be the same for now. She has between 2-8 grandmals at night and around 2 during her nap. I have not yet heard back from the Cleavland Clinic, which has been very frustrating. I have called almost daily and have been told that I should hear back by the next day, but no on calls. I can't do much with out their help because they are one of the only place that knows how to use the bromides. I know I am probably holding on to much that bromides might work, but some studies have shown that it is effective in resistant grandmal seizures. We don't have that many choices of meds left to try, and right now I doubt that any medicine is going to work. Right now she is still doing pretty good while she is awake. In the past though after the grandmals go for awhile then she will eventually loose control during the day.

Lauren has caused some pretty interesting things this past month. A few weeks ago we were trying to get all the kids ready for school. Lauren and Ryan were on the coach playing and then we hear Ryan (our 2 year old) start screaming out of control. My first thought was that Lauren was bugging or hitting him which is something that happens all day. But then Ryan comes into me and has blood all down back of his neck and shirt. Lauren had found a little key chain Swiss army knife and got him on the back of the neck with the little knife. It was a little crazy morning with some of the kids screaming and running around because of all the blood. It was a deep cut but after awhile we got it closed with some butterfly bandages. Probably should have taken him in to get stitches but now a days it takes a lot for me to go into the doctor.

The next week I had Ryan and Lauren strapped in their car seats and I had to run inside for a second. Lauren got out of her seat and got the bottle of sanitizer I keep in the front of the car and dumped the whole bottle on Ryan's head. When I came back Ryan was hysterical! The sanitizer had gone down the front of his face and was all in his eyes. My first thought was he was going to be blind-it was so much sanitizer. I tried to rinse it out but he kept rubbing his eyes making it worse. I called our pediatrician and they told me to call poison control. They told me to put him in the bath tub and rinse his eyes for 20 minutes. While I did this I strapped Lauren in the high chair so she couldn't cause anymore damage. When I got Ryan all taken care of I found Lauren asleep in the high chair and when I went to get her out she had a grandmal seizure. It was quite the afternoon.

Then last night Lauren got into Megan's pink grape fruit body spray and started drinking it. We had no idea how much she got but had to call poison control again. They told me what to do and said they would call back in an hour, they then said my phone number. I just told the guy, "oh great you are tracking me, you know my phone number because I had to call poison control last week." He laughed and said no the phone number shows up when we call.
Last night when we were "watching" for the drunk like symptoms from drinking the alcohol from the body spray we couldn't stop laughing. How do we tell the operator she walked like she was drunk before drinking the spray. Luckily everything turned out okay.

Now I can look back and think it has been a funny couple of weeks!

Lauren's make a wish

2010-03-15T14:50:00.000-07:00

This past Saturday we went up to Salt Lake to the Make a Wish center for Lauren to declare her wish. It was a wonderful day for her and our family. Since Lauren is not verbal enough they talked to us about the things that she would like to do. They tried asking her questions but she just smiled and said "yes" to whatever they asked. We really thought that she would really love the Disney World Wish. Some of the reasons why we thought she would like this wish is she loves things that go fast (bikes, boats, spinning etc) she really has no fear, also (unfortunately) she spends a lot of her time watching Disney Movie, and the last thing is that she enjoys giving people a "five". So her official wish is to give Mickey a high five.
We had a time for each of us to express our personal wish for Lauren, of course we all want Lauren to get better and live as normal a life as possible, it is so nice that Make a Wish does this to help"heal" such amazing and strong kids! Lauren was smiling the whole time we were there.

2010-03-11T17:29:00.000-08:00

A neighbor called this morning and left a message that Lauren was on the front page of the Utah section of the Deseret News! We went and bought the paper and sure enough there she was.
http://www.deseretnews.com/article/700015549/Beauty-contestants-organize-princess-party-for-Make-A-Wish.html
Yesterday we went up to Salt Lake to the Princes Party and the kids had so much fun. They each had there own princes that spent the whole time with them. It was a great experiences. It is such an amazing organization that does so much.

A quick update on Lauren's seizures. Yesterday during her nap (right before we drove up to the party) she had a grandmal seizure. She has had 4 more since (last night while asleep and again during her nap today). I called the Cleavland Clinic today to start working on putting her on the Bromides, I have not heard back yet. Currently she is on 3 different medications, we can't (or I don't want to) add a another one without weaning one of her current medications. Four medications will have such a huge impact on her cognitively and functioning. Three is already too much for a little 4 year old. I am pretty stressed on what to do because Lauren does not handle weaning the medication very well. Even if the medication is not doing much to help control the seizures her brain is so use to the med she always has more seizures during a wean. I am also very nervous about putting her on the bromides. Just the thought of working with a vet to have them check her blood levels makes me sad. I am just so tired of all of this! I am tired of experimenting with Lauren. It is all trial and error. I don't know if a change will make her worse or help and if it does help then I wonder for how long. I have I mentioned that
I HATE SEIZURES!!!!!!

2010-03-07T21:27:00.000-08:00

I just wanted to give an update on Lauren. She has been doing really well these past 2 weeks (No seizures). Two weeks ago we increased her felbatol medication. Dr. Tuxhorn from the Cleavland Clinic had us give her Diamox in addition while we waited for the felbatol blood levels to increase-which can take many days. The Diamox is an anti-convulsive medication that we have used on and off during periods when her seizures are bad-used like an emergency medication. We have been slowly weening the diamox this past week. Tonight was her last dose of the Diamox so we will have to wait a couple of days while that med gets out of her blood to see if the increase in felbatol is helping or if it was just the diamox that was helping. Time will tell! But overall she has had a great start to 2010. I was looking over her seizure log for these past few months, she has had around 25-27 grandmal seizures for this year (mostly between Feb 4-Feb 21st). I guess it is all perspective, when she was in a really bad status episode in the hosptital a little over a year ago she had over 28 grandmal seizures in one day.
This past week Lauren had a follow-up appt with her neurologist at Primary Children's. No new changes or information. I have to get refills of her medications but other then that nothing too productive. I did ask his thoughts on the likelihood of Lauren's development progressing if her seizures stop. He said, "kids like Lauren will progress to some extent but will never be "normal". I don't know why that is still hard for me to take in. I have witnessed her go from a total "normal" 2 1/2 year old to a very behind 4 1/2 year old. Her local Neurologist is really a kind and caring doctor, I just need to learn to stop asking questions that they really don't have the answer to. Then I can just focus on Lauren reaching her own potential and stop comparing her to "normal".
This week Lauren and her sisters have the princess tea party and on Saturday she get to go make her wish at the wishing well. I will post pictures, it should be a fun week.

2010-02-19T21:51:00.000-08:00

Last week we did another IVIG infusion and there was no change in her seizures. She is having between 2-3 short grandmal seizures every morning in her sleep (between 6:30-8:30am). Sometimes when she takes an afternoon nap she will have very short ones while sleeping. She is still doing pretty good during her awake time. We increased her felbatol med at the beginning of the week to see if it would help, so far no change.
I talked to Dr. Tuxhorn at the Cleveland Clinic today to get her input. Her thought were to try to keep working with the felbatol increase for the next 2 weeks. If that doesn't help to try bromides. From my understanding bromides were one of the first drugs used for seizures many many years ago, before anti-seizure meds were used. It is no longer FDA approved and used only in the US in vets on animals. It is still used in Germany were Dr. Tuxhorn originally practiced before the Cleveland Clinic. She is pretty much the only doctor in the US that has experience working with Bromides. It is a pretty tricky drug to use and you have to have someone who knows how to use it. When we were at the hospital at Primary children's in Dec and I mentioned that we were thinking Bromides the on-call neurologists mouth just dropped, it is just unheard of to use now. The reason she wants us to wait 2 weeks is she is currently working with another doctor on a formulation of the bromides so we will be able to get a local pharmacy to compound it. The reason she is thinking Bromides is they are very effective against grandmal seizure type. With Doose syndrome a lot of patients continue with these nocturnal grandmal seizures that are very resistant to medication. Dr. Tuxhorn is working with a lot of other Doose families and is trying to come up with a possibility to help these kids. We feel so blessed that we are able to see a doctor that is doing on going research and knows so much about such a rare and terrible disorder.
On a positive note! Lauren got approved for Make a Wish. Her physical therapist kept mentioning that we needed to apply. After our last hospital stay I put in her application and forgot about it. Make a wish contacts her doctors and they are the ones who decides if she is "eligible". We should be meeting soon with the volunteers to go over what Lauren's wish would be. Since she is not verbal enough to say on her own what her wish would be, so we help think what she would like. They told me that they send half of the children to Walt Disney World, they say that it is a trip that can't be created on your own. They have a place called -give kids the world-where they stay, it is an amazing place (http://www.gktw.org/). From what I understand they have villas where the family stays, an ice cream pallor, miniature golf, movie theater, pony stable, etc.
Along with the wish they grant she is considered a "wish child" and we are a "wish family" for 5 years. That means she gets invitations to different events during that time. We just got our first invitation in the mail, it is a "Princess tea party" with the junior miss contestants. Lauren and her 3 sisters all get to dress up as their favorite princess. As you can imagine we have some very excited girls. It is such an amazing program.
Tami

2010-02-08T21:51:00.000-08:00

Well we had a very nice 31 days of no seizures. That ended last Thursday, I was laying next to her asleep and heard the awful sound that comes when one of her grandmal seizure starts. My heart just fell. Knowing that one seizure means more are on the way, her brain has worked its way around another drug-the 11th drug to be exact. This time the "honeymoon" just went on a little longer, almost long enough to make me feel that we might have found the right combination?! I am trying so hard to not get too discouraged. There are not many options left.

She has continued to have short grandmals in her sleep yesterday and today. Her days are still good so far, I feel that it is a matter of time before her seizures build and she will be in a bad state again. It could be in 1 month or maybe 3-4 months, who knows?

Right now the plan is to see what happens when we go to Primary Children's on Wednesday and get another IVIG infusion. Our hope is that the IVIG "ran out" of its effect before the next infusion, which we do every 4 weeks. If the IVIG helps then we would double the infusion (2g per kg over two days) every 3-4 weeks. If it doesn't help then we will stop the infusion (it is to expensive to do unless we see very definite results) and look into increasing her current dose of felbatol. She is at the maximum dose right now but our local Neurologist says that we could push it a little more if her CBC blood test improves. Right now her white blood cells are still low so we are cautiously checking it weekly.
Our next step after that is to try putting her on steroids. I want to be pretty aggresive, since we have seen this pattern too many times. I don't want to wait until she is hospitalized to try something new. I don't know if our local doctors agree with this plan? After this, I have no idea what to do. That is where I have to remind myself that this is not in my hands. I just have to keep praying that there is something out there to help Lauren. It was very nice getting to "see" my daughter again this past month. It is amazing what happens in a month of no seizures, I know she is in there if the stupid seizures could stop long enough to let her develop. Anyway, she is such a strong and determined girl such a blessing in our lives.
Thank you for all the support and prayers.

2010-01-14T13:13:00.000-08:00

Lauren has been doing very well for over a week. She has had no seizures night or day for the past 11 days. As you all know by now that we have been in this situation numerous times with starting a new drug, so we are still cautiously hopeful. This new drug does have a better "record" then other drugs for intractable epilepsy but because of the side effects is only used occasionally. We have had to get blood tests weekly because her red and white blood count have been low for the past few test.
Aplastic anemia is one of the bad side effect this is the warning we get about the drug....

WARNING

1. APLASTIC ANEMIA

THE USE OF FELBATOL® (felbamate) IS ASSOCIATED WITH A MARKED INCREASE IN THE INCIDENCE OF APLASTIC ANEMIA. ACCORDINGLY, FELBATOL® SHOULD ONLY BE USED IN PATIENTS WHOSE EPILEPSY IS SO SEVERE THAT THE RISK OF APLASTIC ANEMIA IS DEEMED ACCEPTABLE IN LIGHT OF THE BENEFITS CONFERRED BY ITS USE (SEE INDICATIONS). ORDINARILY, A PATIENT SHOULD NOT BE PLACED ON AND/OR CONTINUED ON FELBATOL® WITHOUT CONSIDERATION OF APPROPRIATE EXPERT HEMATOLOGIC CONSULTATION.

AMONG FELBATOL® TREATED PATIENTS, APLASTIC ANEMIA (PANCYTOPENIA IN THE PRESENCE OF A BONE MARROW LARGELY DEPLETED OF HEMATOPOIETIC PRECURSORS) OCCURS AT AN INCIDENCE THAT MAY BE MORE THAN A 100 FOLD GREATER THAN THAT SEEN IN THE UNTREATED POPULATION (I.E., 2 TO 5 PER MILLION PERSONS PER YEAR). THE RISK OF DEATH IN PATIENTS WITH APLASTIC ANEMIA GENERALLY VARIES AS A FUNCTION OF ITS SEVERITY AND ETIOLOGY; CURRENT ESTIMATES OF THE OVERALL CASE FATALITY RATE ARE IN THE RANGE OF 20 TO 30%, BUT RATES AS HIGH AS 70% HAVE BEEN REPORTED IN THE PAST.

THERE ARE TOO FEW FELBATOL® ASSOCIATED CASES, AND TOO LITTLE KNOWN ABOUT THEM TO PROVIDE A RELIABLE ESTIMATE OF THE SYNDROME'S INCIDENCE OR ITS CASE FATALITY RATE OR TO IDENTIFY THE FACTORS, IF ANY, THAT MIGHT CONCEIVABLY BE USED TO PREDICT WHO IS AT GREATER OR LESSER RISK.

IN MANAGING PATIENTS ON FELBATOL®, IT SHOULD BE BORNE IN MIND THAT THE CLINICAL MANIFESTATION OF APLASTIC ANEMIA MAY NOT BE SEEN UNTIL AFTER A PATIENT HAS BEEN ON FELBATOL® FOR SEVERAL MONTHS (E.G., ONSET OF APLASTIC ANEMIA AMONG FELBATOL® EXPOSED PATIENTS FOR WHOM DATA ARE AVAILABLE HAS RANGED FROM 5 TO 30 WEEKS). HOWEVER, THE INJURY TO BONE MARROW STEM CELLS THAT IS HELD TO BE ULTIMATELY RESPONSIBLE FOR THE ANEMIA MAY OCCUR WEEKS TO MONTHS EARLIER. ACCORDINGLY, PATIENTS WHO ARE DISCONTINUED FROM FELBATOL® REMAIN AT RISK FOR DEVELOPING ANEMIA FOR A VARIABLE, AND UNKNOWN, PERIOD AFTERWARDS.

IT IS NOT KNOWN WHETHER OR NOT THE RISK OF DEVELOPING APLASTIC ANEMIA CHANGES WITH DURATION OF EXPOSURE. CONSEQUENTLY, IT IS NOT SAFE TO ASSUME THAT A PATIENT WHO HAS BEEN ON FELBATOL® WITHOUT SIGNS OF HEMATOLOGIC ABNORMALITY FOR LONG PERIODS OF TIME IS WITHOUT RISK. IT IS NOT KNOWN WHETHER OR NOT THE DOSE OF FELBATOL® AFFECTS THE INCIDENCE OF APLASTIC ANEMIA.

IT IS NOT KNOWN WHETHER OR NOT CONCOMITANT USE OF ANTIEPILEPTIC DRUGS AND/OR OTHER DRUGS AFFECTS THE INCIDENCE OF APLASTIC ANEMIA.

APLASTIC ANEMIA TYPICALLY DEVELOPS WITHOUT PREMONITORY CLINICAL OR LABORATORY SIGNS, THE FULL BLOWN SYNDROME PRESENTING WITH SIGNS OF INFECTION, BLEEDING, OR ANEMIA. ACCORDINGLY, ROUTINE BLOOD TESTING CANNOT BE RELIABLY USED TO REDUCE THE INCIDENCE OF APLASTIC ANEMIA, BUT, IT WILL, IN SOME CASES, ALLOW THE DETECTION OF THE HEMATOLOGIC CHANGES BEFORE THE SYNDROME DECLARES ITSELF CLINICALLY. FELBATOL® SHOULD BE DISCONTINUED IF ANY EVIDENCE OF BONE MARROW DEPRESSION OCCURS.

So we have to get her tested again this Friday and see if her levels are still dropping. We really hope her test results look good because we will have to stop the drug if they go lower. This Friday we are going back to Primary Children's to get another dose of IVIG. We just have to go to the rapid treatment unit and stay for the 3-4 hours it takes to do infusion and not be admitted. So we are very hopeful that the combination of the IVIG and Felbatol will be what Lauren needs.

On a side note...The Charlie Foundation, which pretty much brought back the Ketogenic diet into practice throughout the world about 15 years ago, put a video together to try to earn votes for the $10,000 that the movie Extraordinary Measures is giving away to the wining charity. Many of the children in the video have Doose Syndrome (which is what Lauren has) the diet is one of the most effective treatments for that syndrome. Through the internet I felt like I have gotten to know some of these children and their families that have similar struggles. If you have a moment take time and watch the videos from the different charities, some are very touching.

Even though The Ketogenic diet didn't make Lauren seizure free it did so much to "heal" her brain. Because of the diet we were able to put her hockey helmet on the shelf, we had 6 months of no drugs, we were able to leave our house without her having 80 drop seizures a day. It pretty much allowed us to see the sparkle in Lauren's eye that had been taken away by the many drugs and daily seizures. It didn't heal her completely but the almost 2 years on the diet did so much to help her. So take a second and vote for the Charlie Foundation video. I have to admit that I voted for many of the other videos as well, so much to be grateful for!

http://extraordinarymeasuresthemovie.com/#/quilt

Tami

I am going to try to post some videos to show how well Lauren has been doing!

2010-01-03T20:40:00.000-08:00

Lauren is doing very well during her awake hours. Whenever she goes tosleep she is having lots of short grandmal seizures. Last week she was having about 3 an hour and I was sure we would be back in the hospital. Dr. Tuxhorn had us give a high dose of an emergency med before bed for 3 nights. So things slowed down for a couple of days. But overall she is doing really well, but this happens with every new med we try on her. This new med (felbatol) is suppose to work better with intractable epilepsy, so we will give a few more weeks and see. The list is pretty short of new meds to try, so I really hope this will be more promising. Our next step will be to try steroids in pulse therapy-3 days on then 3 days off (hoping to minimize some of the side-effects).

2009-12-26T21:25:00.000-08:00

Sorry I have not posted for a couple of days, we were pretty behind for Christmas. Lauren was able to come home from the hospital Wednesday night. We were so happy to be able to be home for Christmas!
The plan right now is to wait and see how she handles the new drug felbatol. We had to stop depakote cold turkey because of the high risks of the new drug on the liver and bone marrow. Depakote also effects those areas, although not as bad. They also wanted to see if the IVIG did anything to help, we will probably do it again in 1 month. Primary Children's couldn't get a hold of our Dr at the Cleavland Clinic until Wednesday so when they did she pretty much agreed with what had already happened and to wait and see.
Right now Lauren is doing okay during the day, her nights are filled with lots of seizure activity. She is loving not being on the keto diet anymore. I don' t think the reality has sunk in for me. No more measuring, preparing, and forcing her to eat! Almost 2 years of doing this it almost seems to weird not always worrying about what she might be getting into.
Thank you for all the support and prayers it has strengthened us so much. I hope everyone had a wonderful Christmas.

2009-12-22T20:39:00.000-08:00

A new doctor came on shift for the week. She didn't want to do anything more until we can talk to Dr. Tuxhorn, hopefully soon. We are using ativan to keep her stable for now. Let you know when I know more.

2009-12-20T22:59:00.000-08:00

Lauren is still strugling but doing a little better for the past few hours. We gave the second dose of IVIG today, it may take awhile to see if this has helped. We had to use Ativan (like valium) 2 times because she was getting worse, so she has improved temporarily. We also started Felbatol today. This is a pretty high risk anti-convusant, I actually had to sign a release before they would give her the medicine. We had to cover the fact that the depakote was not working or making her worse. I don't know how Dr. Tuxhorn of the Cleveland is going to feel about this, of course she is out of town until the 23th. Tommorow we are going to talk about starting some kind of steroid treatment. I would really like Dr. Tuxhorn's input on this because Primary Children's experience using it for cases like Lauren is limited. In preperation of trying steroids we took Lauren off of the diet abbrutley (reccomended from Johns Hopkins). Her doctors at Johns Hopkins felt okay doing this because Lauren can't really get much worse then were she is at now. You can't do steroids and the diet at the same time. Steroids release glucose which would take you at of ketosis on the diet and stop the effects of the diet. So today Lauren in a half drunk like state ate chiken nuggets and mozzeralla sticks. She would not let me take the plate away. I wish she was not in such a bad state so she could really enjoy her food. They were getting ready to stick an ng tube in to feed her but thanks to the ativan we got a small moment where she could eat (it had been 2 1/2 days of no food because she was seizing so much). I think that is all for now. I will try to post the video my dad took of her eating.

2009-12-19T12:14:00.001-08:00

Lauren has had a very tough week since last Friday. She is currently at primary Children's hospital not doing to good. Last week we had to use 3 doses of diastat (emergency meds) and eventually they stopped working so yesterday we brought her in. We don't know what caused such a change, I have my theries ...
1. She ate almost a whole banana on the Thursday before she lost control of her seizure. The first 24 hours nothing happened, so i emailed her Drs at Johns Hopkins at they told me to start weaning the diet. I was very happy! but was going to wait to start the wean in a few weeks.
2. We also were increasing her Depakote at that time. As strange as it sounds sometimes medicine makes her worse.
3. We had also been doing a slow ween of another drug over the past 2 months. Maybe it was actually doing something?
4. This is just another one of her bad cycles she goes into?!

So as you can see we have no Idea. Right now we are just working on getting her out of Non-convulsive status. Last night we loaded her with IV Depakote to get her blood levels up. It didn't do much good. Right now we are trying IVIG therapy. It is a long shot but at this point your options are pretty limited. From what I learned so far IVIG is..

Intravenous immune globulin (IVIG) is a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months. It is mainly used as treatment in three major categories:

Immune deficiencies such as X-linked agammaglobulinemia, hypogammaglobulinemia (primary immune deficiencies), and acquired compromised immunity conditions (secondary immune deficiencies) featuring low antibody levels.
Inflammatory and autoimmune diseases.
Acute infections.

As you can see Lauren does not fit into any of those categories, just something to try based on some people success with it. Here is more info I found.....

Immunoglobulin therapy was first reported to be effective in epilepsy in 1977, when children with epilepsy were given intramuscular IgG to treat recurrent upper respiratory tract infections.93 Improvements in seizure frequency and behavior were noted. Subsequently, 16 children with Lennox-Gastaut syndrome were treated with intravenous IgG (IVIG), and significant improvement was seen in 8 of them.94 Since that time, several additional clinical trials have been published with similar results, reporting significant improvement in about half of cases.93

There is emerging clinical evidence that IVIG may be of value in several refractory seizure conditions. The details regarding which patients and at what point of the illness it should be used need to be investigated further.

They are going to do one infusion today and another one tomorrow. We just have to wait and see. Even if this does not work I am happy that we have a chance to try this therapy. Not every doctor would give this option, the doctor on call this weekend (which we have never met) is young (which worried me a bit) but I like the fact that he will try something out of the norm. It will be something else I can cross of my list. I will post how she does.

We appreciate everyone prayers on her behalf, Thank you!
Tami
-Can this count as our Christmas card? I will even post some pictures next time!

2009-11-28T14:33:00.000-08:00

We went to Parent/Teacher conference this past week and Nathan's (my 11 year old son) teacher gave us some of his work. Below is an assignment he had to write about giving the most amazing award, I thought it was interesting to see things from his perspective even though some things are not totally accurate he did a pretty good job explaining things.

By: Nathan Austin
I think my sister Lauren should get the most amazing award because she has seizures. On December 26, 2007 (the day after Christmas) she started having seizures. A seizure is when you start shaking and twitching, you can barely breath so your face turns blue and purple. And while this is happening you are unconscious. She has other kinds of seizures, one is when your legs just stop working and you fall. If you are going down the stairs and your legs stop working you would fall down the stairs and kill yourself. She has had a lot of big falls so she has a lot of cuts. When she started having a lot of those seizures my mom and dad bought her a hockey helmet and she wore it all most every where. It has probably saved her life many times. We call those kinds of seizures drops, because she just drops. She also has flinch seizures, that is when her arm just jerks. She can hit other people or herself with whatever she is holding. She has a lot of smaller seizures that are harder to see, like when she just starts staring for awhile.

So on the morning she started having seizures I was upstairs reading then when I came down stairs I saw fire fighters and doctors in our living room. Lauren had been on the couch and fell over and started twitching. My mom had called 911 and a firetruck and ambulance had come. They did not take her to the hospital but she got a doctor at Primary Children's, he gave her medicine but it only worked for a little while. Then he gave her new medicine but the same thing happened over and over.

We were planning a trip to Disneyland in February. We already had the plane tickets bought but then she started having seizures, so we could not go.

When her seizures are really bad she goes to Primary Children's, once she stayed their for almost a month.

Then my mom and dad found out about a diet that helps seizures. On the diet you have a 4 to 1 ratio butter to protein. She has to have so much butter and cream and only 9 grams of bacon.

I think Lauren should get the most amazing award because she has seizures and can not have normal food. Also, because she can not do things that kids can normally do. Therefor Lauren should get the most amazing award.

I agree Nathan, Lauren should get an amazing award!

*All of my children have
been so wonderful and
helpful during this
difficult time!

2009-11-25T15:37:00.000-08:00

A couple of night ago we went out to eat to Chick-fil-a as family. On the Keto diet we have a program we have use to calculate her meals, they list a chicken nugget from chick-fil-a in the protien section. In over a year in a half we have never given her anything that seems "risky". We give her just plain chicken, beef, eggs for her protein. Everything is calculated to the gram so it should not matter what we give her as long as it is calculated. We have always been nervous to try anything new because of how sensitive Lauren has been, and some kids on the diet can't tolerate any "extra" additives or their seizures get worse. Well, it has been so hard with 5 other kids to always leave her out of everything we do involving food. So we went out to eat and I brought our scale and the right measurements of cream and butter along with us. We bought her a kids meal and I quickly took out her fries and measured 20 grams of nugget (which was about 2 small nuggets) and put them back in her little box and her drink of course was just water. It was so fun to see her eating those two little chicken nuggets, it has been 21 months of this diet of pretty plain food, she was so excited. It was a fun night!

2009-11-24T18:10:00.000-08:00

This past month has been very up and down with Lauren. About 2 weeks ago she became "toxic" on the new medicine that we started while at the Cleveland Clinic. She was not eating for days and sleeping all the time and her seizures got a lot worse. We then found out that her blood levels for the drug were really high and we had to stop giving it for a couple of days, then reduce the amount down to try to get her blood levels down. She was not on an abnormally high dose of the drug, her body is just so sensitive to medication sometimes. Being on the Keto diet also tends to make you more sensitive. So we got her stabilized again with lots of Valium and diamox.

Last week we added another drug, Depakote. This was one of the first drugs she was on when her seizures started, it didn't seem to do much back when this all started. Dr. Tuxhorn really wanted to try it again because it is one of the best drugs for Doose syndrome. Also, sometimes trying the same drug at different stages can have different effect. Since starting this drug this past week Lauren has been doing very well. She still has 2-3 small grandmal seizures a night in her sleep, but her days are great. She is more interactive, talking more, playing etc. It has been awhile since she has been this engaged. We are doing another increase tonight of Depakote so hopefully things can improve more? The real test is to see if it can last longer then 3-4 weeks. We have gone through this "honeymoon stage" so many times, so we remain cautiously hopeful.

Right now she is on the Keto diet and 4 drugs -Depakote, Zarontin, Clobazam, and Banzel (we are in a very slow process of weaning this drug).

2009-10-23T21:35:00.000-07:00

I just wanted to share some information. I just learned about a 60 minutes report this sunday

on CBS. I hear it will have part of its show devoted to Epilepsy and what it is like to live with this and why it isn't so easy to control.

http://www.cbsnews. com/stories/ 2009/10/23/ 60minutes/ main5414456. shtml

Not much of a difference with Lauren lately. She had another bad couple of days this past week. Her doctor says it is to early to tell if this drug is going to work or not.

2009-10-17T13:25:00.000-07:00

I just wanted to give a an update of our final meeting with Dr. Tuxhorn before we flew home on Friday. It was so much information that Marc and I wished we recorded it. I am going to write everything I can remember even though it may not make a lot of sense to everyone. So beware this is long and probably more information then you want to know!

She first started by explaining the results of Lauren's 3-day EEG. In the past we have never had too much of an explanation of her EEGs. We mostly would get "very abnormal" , "looks worse or better then before", "it is generalized", "there is slowing in the back ground" etc..
Dr. Tuxhorn explained that she did have slowing in the background (which is not good at all) but that there were times she had a normal background for her age (the benefits of doing a long-term EEG). That sometimes she did have good spikes when she was sleeping (which is not the case with some). That most of seizure activity was happening when she slept. She is having brief myoclonic seizures throughout the night, (all we see visually, if we are awake to see, is her opening her eyes and then her eyes roll slightly back in her head). It shows that these brief myoclonic seizures build then she has her grandmal seizures in the early part of the morning. She did identify the convulsive seizures that she is having as Tonic-Clonic or Clonic seizures not Tonic seizure (which is not a good prognosis usually). Her feelings or impressions is that the Keto diet is helping control the day seizures but she is having these break through seizures through the night, then these nightly seizures build and she begins to cluster them more and go into a status period where we have to use her rescue meds or go to the hospital. She also feels that looking at her EEG that her epilepsy is of a genetic nature. Katie our 8 year old had 1 febrile seizure (seizure with a high fever) when she was 3, and I have a cousin with controlled grandmals. It is believed that Doose syndrome is genetic, even though the other examples are so different. She told us Dr. Doose (which she worked with in Germany) would travel through different villages and towns in germany with children he suspected had this syndrome and he would hook up EEG monitors to their whole family. He found that even if family members who didn't have seizures they still had "markers" on their EEGs, thus making it genetic. This is all they know now about the genetic nature of Doose. Another severe seizure sydrome "dravet syndrome" they have found a genetic link on the sodium channels in their brains, so now they have a genetic test that can confirm for sure they have this syndrome-this is a big discovery for dravet Syndrome which will help them in their treatment and research for a possible cure. Hopefully someday that will be found for Doose syndrome? (In my research I have read that Doose syndrome can skip up to 5 generations and it usually has to have something from both parents-don't know if this is true or not?)
So Dr. Tuxhorn does believe that she has Doose Syndrome. In the past we have been told she could have Doose or Lennox Gastaut Syndrome (LGS). Both are rare and extremely severe seizure disorders/syndromes but with LGS the prognosis is really bad. With Doose syndrome the outcome or prognosis is varied they can have complete remission (normal) to moderate to severe mental retardation. So we don't know what Lauren's abilities will be but knowing Lauren has Doose Syndrome gives us more hope of a possible better outcome.
*As an example, I can think of Downs Syndrome. There are certain things that all people with Downs Syndrome have but their abilities can be varied.
*A side note about syndromes. A syndrome is a group of signs and symptoms that together are characteristic of a particular disorder. Many times "syndrome" is used for a group of characteristics that occur together, though the reason why they occur is not known. This is true for many rare syndromes. Sometimes the syndrome is named after the person who discovered it.

Dr. Tuxhorn went on to talk about Lauren's treatment options. Knowing she has Doose Syndrome there a certain treatments that have shown to work better then others. Children with Doose usually respond very well to the ketogenic diet (which she has, but not completely), other treatments with good response has been ethosuximide and steroids. Steroids and the diet can't be used together because they counteract each other, also steroids can't be used long term without many side effects. Ethosuximide is a drug that is used for absence seizures (staring spells). That is the only use most doctors will use the drug for, not for grandmals or other seizure types. Of the 8-9 drugs that Lauren has tried this drug has never been used. Dr. Doose found that Doose Syndrome sometimes responded to Ethosuximide with good success even though that is not the typical use. This is why it is good to go to a epileptologists (neurologists with advanced training in epilepsy) that specializes in Doose Syndrome! So that is the drug we started while in the hospital at the Clinic. Their are gastrointestinal side effects with this drug. Lauren has had some stomach problems and was throwing up in the night. Hopefully she will get use to the drug and they will go away soon. Since most of her seizures are at night we are going to start off giving it only at night (which would not be done by a lot of other doctors). If this med doesn't help she is thinking of using bromides to stop the grandmal seizures. Bromides was the first drug used in the 1800s for seizures, once other drugs were "invented" it stopped being used. It is no longer available in the US except for animals. It has a very hard therapeutic range to get and only very specilized doctors know how to prescribe it and feel comfortable using it. (It is used more widely in Germany). We are also keeping an open mind to using steroid if this fails. Dr. Tuxhorn also prescribed us Diamox for her "bad episodes" to try instead of using Diastat (rectal Valium). It may work just as well without drugging her up so much with valium. If that doesn't break her bad cycle we are still to use diastat. In almost 2 years of this going on I have never heard of this drug, just having another option to use is in an emergency is huge for us!

As you can see we have a game plan an organized game plan! I feel that in the past few months we were getting back into the cocktail of drugs of try this and try. This will still be trial and error but with direct reason for each trial.

We feel so blessed with the opportunity to go to the Cleveland Clinic. Before we left I kept questioning myself of the benefit of going across the country,again? Will a doctor really do anything different? Is it worth all the sacrifice on our family? The only thing I regret is that we didn't do it sooner, it was an amazing experience!

Again I want to thank everyone for your prayers on Lauren's behalf. Each step of the way we have felt guided and blessed. She has been blessed by the many prayers and blessings she has received. We know that Lauren is in the Lord's hands and that she is loved by her Father in Heaven and He is going to direct her path, whatever that may be. We feel so blessed to have Lauren in our family, through her we have experienced many special moments. There are days when you can see Heaven in her eyes.

Some more pictures while at the Cleveland Clinic.

2009-10-16T22:33:00.000-07:00

We just got home from Cleveland and it has been a long day of doctors and travel. I will post more tomorrow when I am not so tired. I will just say that it was very worth it.

2009-10-14T18:31:00.000-07:00

Lauren has been doing such a good job being hooked up to the EEG monitor. She is doing better then me, I am starting to crazy! Dr. Tuxhorn stopped by for a short time this evening. She wants us to have her hooked up until Friday morning and then we can talk more about what she thinks and our plan with Lauren. So far we know that tomorrow we are going to start a new drug called ethosuximide, and start weaning the drug banzel when we get home. She feels that banzel is such a new drug and doctors know little about it. She also stated again that she wants her to stay on the diet feeling that has been the best treatment for her. I also agree with the diet helping but I think in the back of my head I was hoping she was going to stop the diet. It is so hard and time consuming, but I will do anything that will help.

Below are some pictures of Lauren today.

This is Lauren on one of her 15 minute breaks painting. The girl next to her is waiting for a transplant of her stomach,
pancreas (sp?), intestine, and many more things I can't remember.
She was hooked up to so many machines and feeding bags. She was so nice and happy!

2009-10-13T21:02:00.000-07:00

We arrived in Cleveland last night and had a good trip. Lauren had a pretty rough couple of days before we left and we had to use her rescue med Sunday night. I think Lauren knows when we are leaving to see a doctor-this is turning into a pattern!
This morning we met with Dr. Tuxhorn for over an hour. It was a great visit, she listened and asked a lot of questions trying to understand Lauren's history. She expressed the importance of trying to find out what syndrome she fits into, maybe knowing what is the better treatment plan.
After our appointment Lauren was admitted to the hospital in the Children's epilepsy monitoring unit at the clinic. Lauren has had many many EEG but never a long term EEG except when she was in a status situation and monitoring her immediate situation. They are going to monitor her for a couple of days. It is very difficult to keep Lauren in bed for this many days, she has maybe 3 feet to move in the bed. We are allowed to unhook her a couple of times a day for 15 minutes so she can walk around. Tomorrow Dr. Tuxhorn is going to stop by and talk more about what she sees in her EEG. We already feel like this trip has been worth it!
I just have to say the Cleveland Clinic is so amazing! It is such a huge hospital. It goes on for blocks and the different hospitals are mostly connected or built connected. The floor that Lauren is on is monitored by armed police. Maybe that is because she is on a children's floor? Marc and I have to have pictured badges to get in. The people in the hospital have been so helpful and friendly. We feel like they are so happy to have us here (coming from Utah), compared to Johns Hopkins the feelings seemed reversed. We are very happy that we went to Johns Hopkins for the diet, they really are the best in the world for doing the diet (confirmed again today at our appointment by Dr. Tuxhorn). But today we feel we are getting attention of the overall picture for Lauren. Dr. Tuxhorn definitely wants to keep her on the diet and not change anything with that. I will update more tomorrow.

Thank you for every ones continued support and prayers. We have felt so blessed through this experience and know it is from the many prayers offered on our behalf.

2009-09-21T11:39:00.001-07:00

I know that it has been forever since I have posted an update on Lauren! I doubt anyone is still looking at this blog, regardless it makes a great record for us. Overall Lauren has been pretty stable since I last updated. July was a tough month for Lauren, she had been sick and it really made her seizures bad. We also had another trip to Johns Hopkins at the end of July. Unfortunately the day we landed in Baltimore she had some bad seizures and was really out of it having tons of little seizures going on. The doctors seeing her in that state suggested taking her off the diet, after talking further we decided to keep her on the diet and add a new drug that is not available in the US but is used a lot through out other parts of the world. So since the end of July we have been slowly adding this med. She still remains stable and doing good during the day but suffers with multiple seizures in her sleep every night.

We still feel that the diet has been the biggest help in her improvement but that it is not enough to make her seizure free. Right now we are working on getting an appointment for her at the Cleveland Clinic in October. There is a doctor (Dr Ingrid Tuxhorn) their that is an expert on Doose Syndrome and worked with Dr. Doose in Germany. We are still unsure if Lauren has Doose syndrome (myoclonic Astatic Epilepsy) but we are hoping that Dr Tuxhorn can give us more insight and ideas on additional treatment options to get her seizure free, which is still our hope and desire.

Lauren had a birthday this past Saturday-Sept. 19. I wanted to post some pictures. It was such a good day! Last year the night before her birthday is when she started into one of her bad hospital stay with non-stop seizures. We never really celebrated her 3rd birthday. This year she had a great day! Happy 4th Birthday Lauren!

2009-05-14T21:59:00.000-07:00

I never know how to give an update on how Lauren is doing?! She had been doing so well these past few weeks with only an occasional grandmal in her sleep. Then this week she started having numerous (4-5) grandmals a night. She also is sick with a little cold so we are hoping that is the cause. It is so hard with 6 kids to keep her from getting sick, but it really has such a huge impact on her.
Overall she is still doing so much better. This past week I was looking at pictures of this past fall and found some pictures of Lauren in the hospital when she was in "status" (non-stop seizures). We felt like we lived at Primary Children's hospital with 10-11 day hospitalizations every month.

These are the pictures of Lauren in the hospital on Halloween last year (Oct 08). Totally non-responsive, feeding tube, going from seizure to seizure.

Seeing these pictures makes me feel so grateful for how she is doing now. These past few months she has been improving and developing so much. The hard part for Marc and I is knowing how fast things can change back to "bad". Almost with no warning. So as much as we feel blessed and happy that she is doing so much better, the scared feeling in the pit of our stomach does not go away.

We really feel like we are getting closer to finding the right combination of treatmeant for Lauren. We know that ketogenic diet is working, it has just taken awhile to get her body use to everything. She is such an amazing child that is so strong. We are so hopeful that some day she will beat this aweful "beast"( another word used to describe Doose Syndrome) that took over her brain 16 months ago.

Here is a video talking about epilepsy
http://www.msnbc.msn.com/id/3036789/#30742751

2009-04-27T13:21:00.000-07:00

Lauren has been really good these past couple of weeks. She still has grandmal seizures in her sleep, but they have become less frequent this past week (she had two nights during this past week where she had 1 seizure while sleeping). Since adding the carnitine supplement she has become more stable and so much more aware.

This has been very nice but has made it hard in other ways. Lauren has been on the ketogenic diet for over a year now. During this time she has not complained for "real" food, if she asked I would tell her it was not good for her brain and when she was bigger she could have it. She has been okay with this answer until lately. She cried over one of Ryan's chicken nuggets for 5 min the other day. It really breaks my heart when she is like this, but I know it is the best thing for her. She has also started to refuse to eat her meals. This has turned into huge power struggles between the 2 of us. So we are having to kind of go back to how it was when we first started the diet-give her 20 min to eat and then throw it away. When we first started the diet she went almost a week without eating but since then she really has been great with eating her food, until now. I have become pretty creative in her meal planning! Last night I was trying to make chocholate truffles, we will see how it goes!

Along with this came a great blessing. Lauren started swallowing pills! This has been so huge for us. Most of her meds and supplements come in adult pill form since she can't have the sugar and carbs of liquids. We normally have to crush her pills and mix it in her butter. Getting her to take her medicine has always been a huge struggle. Before the diet when she was on 3 anticonvusants we use to have some of our neighbors come and help with giving her meds-we have great neighbors! It is so nice now, she lays down and she puts the pills in her mouth and I pour a little water in her mouth and its done! Nathan my 10 year old son just learned how to swallow a pill and that was quite a struggle!

So overall a great week!

2009-04-11T13:27:00.000-07:00

Lauren has been doing pretty good lately. She is mainly having grandmal seizures when she sleeps at night (usually 1-3 a night). Her seizures have not come back during the day but all the night seizures are effecting how she is functioning during the day. Right now we are re-introducing the carnitine supplement that we had stopped a few months ago. Her blood tests showed her extremely carnitine deficiencent (which is not unusual with this kind of diet). We are hoping this will help with her muscle strength and tone (and seizures). We can only make one change at a time, so we will work on this for a few weeks and then see what we can change next.

A lot of people have asked about Lauren's development. Before her seizures started she was totally "normal" as far as her development, if not advanced in some areas. The only thing we were starting to wonder about was her speech. Right now Lauren is delayed in most areas developmentally, mainly her speech. She is currently 3 1/2 years old and mainly speaks using one word and is starting to put 2 words together more. The words she used before she started having seizures are very clear still but a lot of the new words she has gained are very hard to understand. She goes to speech therapy weekly.

Cognitively she seems to do well and has tested to be "average" in her understanding. She just can't express herself very well, which leads to lots of temper tantrums.

She has definitely regressed in her fine and gross motor skills. She has lost a lot of muscle tone and coordination. She also goes to physical therapy weekly.

Right now our 18 month old son is starting pass Lauren in some areas developmentally. When they wake up from their naps sometimes I can't tell who is calling for me.

When we first learned of the severity of her seizure disorder I had a hard time comprehending the development part. Now my hope is that we can get her seizures stable and not worry too much about the development side. I know that she will be able to reach her "full potential", whatever that may be.

We can't thank everyone enough for your continued prayers and support, we hope you have a wonderful Easter!

2009-03-31T11:58:00.000-07:00

These past few weeks have been pretty good considering everything that has been going on. About 2 weeks ago the grandmal seizures started again in her sleep. We had a really good "honeymoon" on this medication for about 2 weeks. We noticed that with this knew medication her ketones were getting higher and higher. We had stopped the MCT oil last month because we were thinking that having her ketones so high might be causing all the problems. Since this medication is so new it has never been tested on the ketogenic diet so we don't know if there is relationship between the two. So we decided to reduce her ratio from 4.5/1 to 3.5/1 , this calculates into a little more "food"-carbs and protein- and a little less fat. We left a couple of days after this change to Southern California to go to my brother's wedding and also go to Disneyland and Sea World. We were nervous because in the past she has gone down hill really fast once the seizures started back up. We figured there are some good hospital in LA if we needed them.
She remained pretty stable for the first part of the trip to sea world. When we got up to Disneyland she got really sick (an aweful flu like bug that my other kids had). This caused some additional seizures during the day that went along with her fever. She was really out of it for a couple of days. Because of the sickness she stopped eating and so her seizures stopped also. Right now she is doing good-no seizures- but still does not feel like eating much. I hope things continue to do well as she eats more, that will be the real tests. I am very happy to have made it on our trip without a hospital visit! This has been the longest she has gone in awhile with out a visit to the hosptial-(everyone knock on wood).

Here are some pictures from our trip

2009-03-14T20:56:00.000-07:00

Lauren has done pretty good this past week. She did have a grandmal seizure on Thursday morning while she was sleeping. She recovered really well. Right now at 10pm I am having a huge power struggle to get her to eat her evening meal. You would think she would be so hungry! I don't have to many bribes left to get her to eat, it is so exhausting (but worth it)!

2009-03-07T20:18:00.000-08:00

This past week has been wonderful for Lauren. She has had no seizures (at least that we are seeing). She has been very happy and playful all week. We are cautiously hopeful about the new changes and medication we added. In the past the longest a medication has worked has been 3 weeks, we are now at 10 days. We are noticing a lot of bruising over her body- which might be a bad side effect of the medication. I really hope that if this medicine is helping that we will not have to stop it because of the side effects.
Another amazing thing has been her blood ketones and glucose levels. We have a little monitor (what diabetics use) that we test her blood levels with every morning before she eats and again at night after all her ketogenic meals. This past week her levels have been very consistent of good ketones and lower blood sugar-which is what we want. In the past we have had a hard time getting consistent numbers, they would fluctuate a lot. This is very good because the diet is the main "medication" that has helped the majority of her seizure types (drops and myoclonic seizures). Our hope and prayer is that we can find a good combination between the meds and diet to help her.
We have been so blessed by everyones prayers-thank you!

2009-02-27T17:04:00.000-08:00

I really feel that I need to post an update about Lauren. Things have been really bad for Lauren these past months. I stopped updating her blog because I just have no idea what has been going on with her. I will try to give a quick update of the past few months.

Lauren's grandmal seizures have continued to build and cause her to go into these non-stop cycles of grandmal seizures. We increased the last anticonvulsant (zonegran) a month ago to try to help but that increase put her into non-convulsive status. That is when she is not outwardly having seizures but having nonstop seizure that can only be seen on an EEG. The best way to describe her would be a "zombie". She could not walk, talk, or eat. She just sat and drooled and we felt like we were looking at a blank child-she was just not there! The seizures are just so frequent it interferes with all her abilities. So we spent some time in January at Primary Children's trying to get her out of that state. It is really hard to comprehend that medicine can make her worse when they are suppose to help seizures.

So, at the end of January we started taking her off this medication (the Zonegran). This makes the 7 th medication that she has failed (not worked or made things worse). Lauren is still on the ketogenic diet which has been a miracle for the first 6 months but now something seems to have happened. We will continue with diet but we're left with 2 options of medication left to try. One was just approved by the FDA in November and just available a couple of weeks ago (Rufinamide), the other one has known side effects that are not very good and is left as a last resort med (Felbatol).

We had no idea what to do, the new medication-Rufinamide- is just so unknown and none of her neurologist have used it before. As we were trying to decide what drug to use in the middle of February Lauren started again to go into a bad grandmal cycle (having grandmal seizures every couple of hours without waking up between them). All of her emergency meds (Valium, etc) were not working so we decided to get her loaded in the ER with dylantin. That is the drug that we have used a couple of times before to stop the grandmal cycle but this drug aggravates her myoclonic and drop seizures so it is only used in emergencies. When Lauren was given this drug the grandmals stopped but she instantly went back into the non convulsive status again with lots of little seizures. This time around none of the emergency drugs were able to get her out of this really bad state. We used so much of the benzo's (valium and ativan) and they would only help for a couple of hours. She had been in this non-convusive state for over a week and we were getting really scared. Because of her current condition I had filled a prescription of the new drug rufinamide ,but was very nervous to start it.

We were trying to get Lauren to a much anticipated trip to Johns Hopkins scheduled for February 24. When I emailed an update to her doctors at Hopkins a day before we were suppose to fly out they emailed me back and told me not to come, that a plane trip wouldn't be advisable in her condition, but to start the new drug.

Even though I knew she was not in a state to travel I was totally devastated and kind of fell apart at this point. I really wanted to talk to her doctors at Hopkins and have a face to face conversation. Marc and I had no idea what to do and felt very lost and alone in this journey with Lauren. We had put so much into doing what was best for Lauren, we felt like the diet was the right thing, but at this time we felt like we were not getting any direction or answers.

It was Sunday night and our plane left early the next morning. Lauren was having non-stop seizures-big and small- and had hardly talked or walked in over a week. At this point we knew we needed strength that was beyond us, strength that only our Father in Heaven can offer. Later that evening our Bishop from our church came over to our house and gave Lauren a Priesthood blessing. He also gave Marc and I a blessing. Words can not express the feelings and power that were felt as he spoke. It is one of the most amazing experiences of my life. We felt strength and hope again and knew that through the help of our Heavenly Father and the many prayers that have and are still being offered that things were going to be okay.

We were still undecided about what we were going to do the next day so we packed our bags and knew in the morning we would either go to Primary Children's Hospital and have her admitted or get on a plane. Lauren was really in no condition to fly on a plane but Marc and I both felt it was the right thing to do. So, as we headed to Salt Lake City we decided to go to the airport and we got on the plane. This was one of the greatest tests of my faith. She had improved a little but was still having little seizures constantly. We got many looks on the plane as we had this little girl that obviously something was wrong, she had a big black eye from a drop seizure earlier in the week, and could not do anything. I just kept praying that we were making the right decision. In making the trip I was so worried that her doctor would be upset at us for coming.

We went to bed at the hotel that night in preparation of her early morning EEG testing. When we woke up Tuesday morning Lauren sat up in her bed and started talking. It had been over a week with hardly hearing her voice, that morning it was music to our ears, a true miracle! Lauren improved as the day went on, and by the time her afternoon appointment with her doctors she was a new child. Her doctor was a little concerned that we had come but said that she was not as bad as I had described-How do you tell your doctor about a Priesthood blessing? We had a wonderful appointment with the whole team of doctors and dietitians. No changes were made because we had just added the new drug (Rufinamide) and they never make two changes at one time. We went over my theory that last September when things all started to go down hill was the same time we added MCT oil to her diet to help boost her ketones, and the possibility of having too high of ketones may have not been good for her.

Our next step after a few weeks of trying Rufinamide is to reduce her ratio on the diet which would lower her ketones and see if that helps. We don't know what to expect next. Right now Lauren is doing very well- talking, playing, walking, so happy. We are presuming that this is what they call the " honeymoon period" of anti-seizure drugs. With intractable seizures it seems that medicine works really good for awhile then stops working. Rufinamide is a new drug just approved so maybe there is some hope that it may do some good long term or that some new diet changes may help. It is all so unknown?! We just want to thank everyone for all the help, love, prayers, and support we have felt. We also are so thankful for all those who will be fasting this Sunday for Lauren and her doctors. We know that through the power of prayer and fasting as a family, ward, and friends that Lauren will be blessed with what she needs at this time.

2008-11-19T11:08:00.000-08:00

I can't remember if I have already posted this link? This is a slide show of a little boy that also has Doose syndrome like Lauren. There is a great yahoo group for families with Doose syndrome that we belong to. His parents started a non-profit foundation to help all families dealing with epilepsy. I think November is Epilepsy awareness month so they are trying to get the word out.

http://epilepsyoutreach.org/emmetts-story.html

2008-11-18T23:35:00.000-08:00

I am sorry that it has been so long since I have posted an update. Things got really bad for Lauren again towards the end of October. She had been doing great for about 3 1/2 weeks since we last left the hospital, then she started to have the grandmals again. At first they were only happening at night (6-8 grandmals a night). We tried taking her to our local hospital (Utah valley) to start the emergency plan that we had left the hospital with 4 weeks earlier. The ER doctor had no idea what to do with her, so he sent us home with an IV and home health care which had us giving her IV Valium (which I later found out should not of happened). I was very nervous, IV Valium can suppress breathing, so we had her hooked up to an oxygen monitor and had oxygen on hand. The Valium was not working and she was getting worse and started having grandmals every 2 hours (even through the day). We took her into Primary children's on October 28th after almost a week of trying everything to stop the seizures. I really didn't want to go to the hospital again!

Our choices were very limited on what we could do. The Valium, ativan, klonopin etc are "emergency meds"- her body at this point has built up a tolerance to them and they were doing nothing to stop the seizures, just making her very drunk. Because of the risks of aspirating during a seizure we could only give IV meds to her. The only anti-seizure meds that are in IV form are Depakote, Keppra, Phenobarbital, and dylantin. She is allergic to phenobarbital, dylantin is known to aggravate myoclonic and drop seizures, and she had already failed using depakote and Keppra before the diet. The Neurologist at Primary Children's thought loading (high doses) her with IV depakote was our best option because that drug did work for a short time back in January. So they gave her a loading dose and she got worse and pretty much was having periods were she was having grandmals every 20 minutes. At that point they put a feeding tube in her and she continued to get worse. We stopped the depakote and tried the Valium again at a higher dose and every 6 hours. That did not help, and at this point had gone about4 days with being totally unresponsive (not waking up in between seizures). There next step was going to try loading her with Keppra. I started feeling pretty hopeless at this point, when her seizures first started Keppra was the first drug she tried and it did nothing but make her seizures worse.
Her doctors at Johns Hopkins had stepped back not being able to help from such a far distance, but through many emails begged for their input. He suggested trying the Dylantin instead to stop the grandmals. So they loaded her with the dylantin and the grandmals finally stopped. But within 1 day her myoclonic seizures (small jerks) started and increased until she was having jerks constantly. She was at least conscience and somewhat awake though. We spent the next 3 or so day getting they myoclonics under control through the diet (the diet is very effective against that seizure type-where most meds are not). So after another 11 day hospital stay we finally got her seizures under control!

Our new plan from Johns Hopkins is to fast her every 5th day. This is something they have never done before, but like I have mentioned before, her body seems to "forget" how to go to her own fat unless it is "shocked" into it. We also started a small dose of Zonegren (anti-seizure med) to try to help with the grandmals. We have been home for about 10 days. It took her about 5 days to be able to walk again, she got pretty good at "crawling" around. But slowly things are starting to come back. All the drugs and seizures took a big toll on her little body.

We have fasted her 2 times and I have to admit it really is a nice break for me to not have to worry about feeding her-almost a day off. She seems to handle it better and better. I can hardly fast for 24 hours, but when we fasted her this last time she was playing all day and didn't even ask to eat!

So far she has not had another seizure since we started this BUT we know all to well this can change at any moment. I hate posting that she is doing well because I feel as soon as I do it all changes. So I will just say that she has had a good day, and we continue to take it day by day.

We are so grateful for all your prayers and continued support!

2008-10-21T21:13:00.000-07:00

Things have pretty much stayed the same lately. Lauren's doctor wants us to continue the klonopin and to keep adding the MCT oil slowly. She has had 2 grandmal seizures (both at 6:30am) since we have been home almost 3 weeks now. Her ketones continue to rise as we add the MCT oil. I hope this helps her keep high enough ketones to make it overnight.

2008-10-14T21:10:00.000-07:00

Sorry it has been so long since I have updated. We came home from the hospital almost 2 weeks ago. We have not yet heard from her doctors from Johns Hopkins on what the future plan is going to be. Lauren was sent home on Klonopin 3x a day. This is in the same family as Valium but used for more long term use. From what I understand it is better to use only short term because of the addictive nature. But she has done pretty good these past 2 weeks. I am just waiting for her brain to find some way around this and start having more seizures again. I have been gradually adding the MCT oil back-1 meal at a time, then waiting 3 days to add to the next. So far she has been tolerating it well. I am just doing my own thing till I hear from her doctors?!

2008-10-01T16:57:00.001-07:00

Lauren has improved so much these past few days. They stopped the Valium last night and we are waiting to see if it has broken the cycle. So far so good (knock on wood). If all goes well we can leave the hospital tomorrow? She has been able to eat by mouth for 2 days now. They took out her feeding tube this morning. She still can't walk very good and spends most of the day playing in her bed. We have taken a couple of trips to the great hospital playroom. Overall she is doing so much better. It is amazing the toll these seizures had on her body. Thanks for all the visitors, it helped passed a lot of time. We also appreciate all the help with our other children. We feel so blessed with all the love and support we have received.

*Rodney, Kim, and family thanks for the special delivery of flowers,balloon, and bear it was a fun surprise and very thoughtful-she loved it!

2008-09-28T22:43:00.000-07:00

As many of you know Lauren went into the hospital last Monday and she is still at Primary Children's now (almost a week).
The night after we started the MCT oil Lauren went into a bad grandmal cycle. She was having one then going postictal then having another. We gave 3 doses of her rescue meds (diastat) and it had little effect. After 20 or so grandmals we took her to the ER and determined she was in some sort of "status" (non stop seizures). It took till 3-4am to determine that she did not need to go to intensive care but could be admitted on the regular floor. She spent 2-3 days in "brain fog". She looked like she was in a coma-but it is not the same thing. They have preformed numerous tests (again) _MRI, blood, urine etc to see what the change could be. SO far everything has come back normal (again). She has come out at of the "fog" but is still in bed unable to walk or talk very well and is on a high dose of Klonipin and Valium every 4 hours (rotating drugs). We are hoping this will break the cycle. We are having a conference call on Monday morning with hopkins and primary childrens neurologists to see what the game plan will be. We may have to start the anticonvulsant game again, which is really hard to swallow. We know the medicines don't work and sometimes make the seizures worse along with the bad side effects. We have no idea why she went into status-THe nature of the syndrome? We don't know if the diet will continue to work. They say it usually doesn't stop working all of sudden-who knows? Time will tell. I will try to keep this updated, I think we will be in the hospital for sometime. Some have asked if it is okay to visit Lauren-we would love the visits, but we can't predict the state she will be in. She is in room 2037 atPrimary children's hospital-for those that would like to come. Thanks for your prayers and support.

2008-09-20T13:43:00.001-07:00

We got back from Hopkins late Wednesday night. Overall it was a good trip and a good meeting with her doctor and dietitian. We all pretty much agree that there is something up with her metabolism making it difficult to get the diet to work. They made 2 changes to her diet. The first change is adding MCT oil to her meal. Here is info to describe it.....

MCT stands for medium chain triglycerides. Most naturally occurring fats are triglycerides - tri meaning that each molecule have three carbon chains. In the medium chain triglycerides each chain has 6 - 12 carbon atoms, and for the medically refined grades of MCT oil each chain has 8 - 10 carbon atoms. MCT oils occur naturally, and the most abundant source is coconut oil. Most MCT oil is refined from coconut oil.

MCT oil is interesting because, when it is metabolised in the body, it behaves rather more like a carbohydrate than a fat. The fuel of preference for the body is carbohydrate, and the body will use up its store of carbohydrate before using other fuels.

Long chain fats (ie the normal varieties) are converted into chemicals called chylomicrons by the digestive system, and these are then transported around the body by the lymphatic system before entering the circulatory system. This is a relatively slow process, and so fats metabolise more slowly than carbohydrates. Unlike other fats, MCT oil does not go into the lymphatic system; instead it is transported directly to the liver where it is metabolised, so releasing energy quickly, just like a carbohydrate, and creating lots of ketones in the process.

The oil has to be introduces slowly to avoid the side effects so we have added 5g to 4 of her meals and in 2 weeks we will increase to 10g per meal.

The reason the added the oil was to boost her ketones especially to try to help with her ketones dropping during the night. Well after the first day of adding the oil her ketones went pretty high but it has done something to effect her seizures. She went on to have 8 Grandmal seizures over the next day and half (It was a great birthday for her yesterday!). So I don't know if her body has to get use to it or if she can't handle it? We need to give it 2 weeks to see.

The second change was to add carnitine. Some of her blood test showed she was carnitine deficient. Since I barely understand all this here is more info to explain...

Carnitine is a protein that is essential to the metabolisation of fat. Long chain fats are metabolised by the mitochondria within the cell. Carnitine is essential to this process, because fat by itself cannot penetrate the membrane of the mitochondria. Each molecule of fat has to be transported across the mitochondrial membrane by binding with a molecule of carnitine. After the fat has been metabolised in the mitochondria, and generated the energy rich ATP, the carnitine is again required to transport the waste product out of the mitochondria as an acyl-carnitine.

Since the ketogenic diet has reduced protein and increased fat this may be the cause of her deficiency but we are wondering if she had this deficiency before she started the diet and it is just worse now?

It has taken a lot of work to locate the sugar free carnitine and thanks to Mike at Edgemont Pharmacy he had it overnight shipped to us last night.

So we are waiting to see if these 2 things work. If they don't her doctors are going to try a
24 hour fast every few days to a week. This would be an experiment to see if our theory of her body "forgetting" how to use her own fat until we shock it back by fasting her would work. We are really hoping our first option will work, it is not very fun fasting her, especially that often. But obviously we will do anything to try to control these seizures! Thanks again for all the prayers and support!

2008-09-06T21:49:00.000-07:00

Since we fasted her last Saturday and increased her calories by 200 Lauren has completely turned around. It took a couple of days for her abilities to come back (walking, talking, etc.) but they slowly did. Her doctor described her as being "encephalopathic" or in a brain fog for this past week. For a couple of days after her coming out of it she was having hallucinations of some sort and was going "crazy" for periods of time. Just like a switch she would start screaming and her eyes and head were darting back and forth like she was seeing things. She would wonder around calling for mama and I was right next to her. If I picked her up she would get worse, like she did not know me. She was acting like she was being killed. They would last for 1 hour or so and then like a switch again she would stop and was totally fine. They were happening 2-3 times a day at first and now they have slowed and hopefully have stopped. These were really scary to watch, I felt like she was going crazy-literally.

Well for this past week she has been totally seizure free during the day. She is still having her 6am in t/c on some mornings. But overall she has been so good. The roller coaster of emotions are so big. A little over a week ago I really thought we had "lost" Lauren to the seizures and tonight we just gone done playing soccer with her. I think I am the one who is going to go crazy!

We are really feeling there is something wrong with her metabolism that is causing such big swings. Our theory is that she can't switch over to her own body fat when the fat we feed her is gone? But if we feed her too much fat she gains weight which is then harder to maintain ketosis. We know the diet works it is just keeping her in the right balance with her body. I think if we can just figure out some of this we can get the seizures under control.

We are so grateful for all the continued prayers for Lauren and our family. Thank you!

2008-08-30T23:19:00.000-07:00

Lauren has had a very bad week. She has not fully recovered since she had the 6 t/c's in her sleep last week. We used her rescue meds again last Sunday and they didn't do anything to help, just added more drunk like behavior but still having hundreds of seizures. Monday I took her to Primary Children's Er . They were a little nervous when they asked how long she had been like this and I told then since Saturday morning. They pulled out the oxygen and gave her the fastest IV I have ever seen and gave her Ativan (more valium). Then they wanted her admitted to the hospital. I was not planning on staying, I was hoping they would give her some rescue meds then I could wait till I heard from her docs on Tuesday morning.

It was a very frustrating day in the hospital (having medical students ask me if she was full term, any problems with her delivery, etc....! Questions I have answered about a hundred times!) If I did not have a brother in medical school I think I would have hit one of them! It is so hard to explain the diet and a severe seizure disorder to them when I really don't get it. Anyway by that night I was asking to go home (thinking the 3 days of continues Ativan valium her doctors at Johns Hopkins had ordered) would work and she would be fine. They said I could go if I felt okay taking her in that state. Looking back now I should of stayed because she didn't improve. This past week she has hardly walked or talked. Most of the time she is being held with her head hanging sideways, drooling, eyes rolled up, jerking, and twitching. There has been some moments where we have seen "her" come through but not to many. We have been in constant contact with her doctors at JH. They increased her calories by 200 hoping to increase her ketone levels. I feel like we are going in circles, up and down with the calories. They have done hundreds of patients on the diet at JH and they said she has been one of the harder ones to figure out. She shows that the diet has worked but figuring out her metabolism has been very hard. But they have not given up yet. It will take awhile to see if the change will be enough.

Today after a tough morning and feeling pretty helpless we gave her diastat (it had been 6 days and 24 hours since her last dose of the other form of Valium-ativan-.) and amazingly she came out of it. For the first time in 6 days her body stopped seizing. But I am very concerned about her development. In these past 6 days I felt like she has lost a year in development. She can hardly walk or talk normal, she did say some words, but she had been doing so good before this happened. I really hope these things will come back once her brain can rest.
Anyway, since she had refused to eat most of the day we continued and did a 24 hour fast, hoping for something to work. So it is just a waiting game to see what tomorrow will bring.

2008-08-23T22:17:00.000-07:00

Lauren is still doing pretty bad right now. Last night she had 6 grandmal seizures in her sleep- a new record. She woke up a mess and we had to use more of the rescue medicine, which we have had to do a lot of lately. She still has not fully come out of it yet, we are wondering is she is still a postical from all the seizures or having lots of little seizures going on. I talked with her doctor today and they still want me to collect the info till this Tuesday when they all meet together before they make a change. We are just praying that they may beable to "find" something that is causing this aweful cycle.

2008-08-13T22:49:00.000-07:00

Lauren has had a very hard time this past week or so. Things just kind of fell apart. We had to use her rescue medicine 3 times in 8 days to keep her out of a constant seizure. She is starting to do better but is still having drops, myoclonic, absence, and tonic/clonic seizures daily. We have no idea what happened or why she is having such a bad time. Here is our latest email from her doctor at Johns Hopkins...

"Lauren has been hard to figure out, and it has been frustrating for all of us. My opinion is that her metabolism is such that at times it appears she is calorie overloaded, gains weight, and needs to be fasted or shocked into ketosis. At other times like you are now describing she appears to run out of "fuel" (calories) in her system. If she were a diabetic (which she of course is not) I would. describe her as "brittle.
All that said we need very detailed information from you, in the form of 7 days of information documented on an hourly basis, in chart form, to include calories in, activity level, any and all sz specified by type and duration, any unusual behaviors, fluid intake by type and time, daily early morning weight in consistent fashion; early morning, mdday and evening ketone measurements in consistent fashion.
This is like a detective mystery and we need information (clues) to figure it out. So hang in there and collect this info and email it to both Z and me and we can go from there.
I bet we will end up on a very unusual and strict diet interspersed with fasting every few days that will get her under consistent control. Dr. Jim"

I don't know I already felt like we were on a very unusual and strict diet! Well its time for her midnight snack.

2008-08-02T14:54:00.000-07:00

This past week Lauren has had a tonic/clonic seizure in the early morning (6-8am) on most days. I think she is having a hard time maintaining ketosis overnight and that is way we are seeing the early morning seizures? Today (Saturday) she has had a few drops which totally caught me off guard. She has gone weeks without a drop seizure. I don't know if she got into something or this is just the course of everything! She has been recovering from being sick so her doctors don't want to change anything till she is better.

A lot has happened these past 7 months and we have received a crash course in Neurology and epilepsy. We still feel like we know nothing but we try to educate ourselves the best we can so we can better help Lauren. I put some of information that we have collected about Doose syndrome for those that have been wondering what it was and what she is going though. Beware-It is a lot of info!

Myoclonic-Astatic Epilepsy (MAE) was first described and identified in the late 1960s by Herman Doose as an epilepsy syndrome, hence its original label, Doose Syndrome.

Doose syndrome or MAE-is rare and accounts for only 1 percent to 2 percent of all childhood epilepsy.

MAE is an epilepsy syndrome of early childhood that is often resistant to medication and for this reason it is typically difficult to treat. It is usually characterized by difficult-to-control generalized seizures, and is idiopathic (no known cause) in nature. The generalized seizure types seen in this syndrome vary, but many of the afflicted children can experience large numbers of seizures daily, part of what makes this condition so difficult to manage. Onset generally occurs between ages one and five, usually in children with an uneventful history.

CLINICAL MANIFESTATIONS

Prior to the onset of MAE, 84% of affected children show normal development; the remainder show moderate psychomotor retardation mainly affecting speech. The seizures usually begin between 2 and 5 years of age. Boys (74%) are more often affected than girls (Doose and Baier 1987a).

*On December 25, 2007 (the day before her seizures started) Lauren was a totally healthy, active, and happy 2 year old. We were just starting to wonder about her speech, but we were not that worried.

Here are some questions that are asked about Doose Syndrome (MAE)

"Why aren't the medications working?"

It is not yet known why some children with MAE do not respond to medications. In many MAE cases, the medications can actually aggravate the condition and increase the child’s seizures and/or unmask new seizure types. Some children with MAE are more susceptible to this paradoxical seizure reaction to medications than others. Additionally, myoclonic (jerk-type) and myoclonic-astatic (drop type) seizures, the most common seizure types associated with MAE, are notably difficult-to-treat seizure types.

"Why is MAE so severe?"

MAE is characterized by difficult-to-control seizures that often occur frequently throughout the day. This can potentially impair and/or disrupt a child’s learning and development opportunities. The focus for parents and treating doctors is to reduce seizures as much as possible. While some children may not have 100% seizure control, their ability to learn and develop can improve with a reduction of seizures.

"Is surgery an option?"

Because the seizures of MAE are generalized, meaning the abnormal electrical activity occurs spontaneously on both sides of the brain – they do not originate from a focal point in the brain – surgery is not an option. Epilepsy surgery is only indicated for those whose seizures originate in a specific area of the brain that can be removed safely (eg lesions on a part of the brain).

Types of seizures described

MAE is a complex seizure disorder that presents as a mixed bag of seizure types. The myoclonic-astatic seizure (also called drop seizure) is the core seizure type associated with MAE. All children with MAE will experience either myoclonic or myoclonic-astatic seizures - or both -other seizure types include absence, atypical absence, myoclonic, tonic-clonic, episodes of non-convulsive status epilepticus (NCSE).

Myoclonic seizure

Most people have experienced an abrupt jerk as they are falling asleep (normal sleep myoclonus) or have jumped when someone gives them a fright and this is similar to the jerk experienced by a child who has a myoclonic seizure. Myoclonic seizures are like being jolted by a mild electric shock. They are like single jolts which may occur frequently throughout the day. They can affect the whole body or just part of it. The jerk can be severe enough to make a child fall. As is the case with all generalized seizures, the child is not conscious during the event but the seizure is so brief that the person appears to remain fully conscious.

*Before the diet and on 3 anti-seizure medication Lauren was having over 100 (to many to count) of these seizures daily. Since starting the diet and no medications this seizure type has reduced greatly. These are what "come back" when she has her bad periods and is in a "constant" seizure with jerks happening every 1-2 seconds. This is when we have had to use her rescue medicine (diastat) to make them stop.

Myoclonic-astatic seizures (atonic or drop attacks)

The myoclonic-astatic seizure is rare and unique to MAE, and is one of the most important and distinct features which helps distinguish it from other epilepsy syndromes.

Like myoclonic seizures, myoclonic-astatic seizures (drop attacks) are sudden, single events. Myoclonic-astatic seizures involve abrupt loss of muscle control causing the person to fall to the ground, often resulting in injury. If a myoclonic-astatic seizure occurs while a child is standing, the seizure may cause the child to slam violently to the ground, perhaps hitting his/her face, breaking a tooth, or causing a facial laceration.Sometimes a child may be mildly agitated or disorientated following an astatic seizure. If injury has resulted, he/she may be duly distressed. For any family and child, the sudden, violent impact and resultant injuries seen with myoclonic-astatic seizures makes them one of the most frightening and distressing types of seizures to live with.

As the label myoclonic-astatic epilepsy (MAE) suggests, myoclonic-astatic seizures are the core seizure type associated with the disorder. In MAE, myoclonic-astatic seizures can be serious because they may be difficult to control, occur frequently on a daily basis putting the child at persistent risk of injury, and because they are only one manifestation of this mixed seizure disorder.

*Before the diet Lauren would have 20-100 of these a day. This is the reason she had to wear a helmet and the cause of most of her injuries. This became her "main seizure". She couldn't be left alone for one moment -she was hurting herself constantly. There were many times where we felt like angels were watching and helping her when we turned away for a second (ex/ having a drop on the top of the stairs but landing away from the stairs and not falling down them, etc). This has been one of the hardest part of this epilepsy. There has been so many bruises, blood, and scrapes caused by these drops! The day we started the diet she had 80 plus drops, the second day she had 20-30, and on the third she had none. That whole experience was a miracle. To have one day where Lauren could walk around and not hurt herself was and is beyond words.Currently the drops have mostly stopped but return when she is having her bad periods. The more we fine-tune the diet the longer the drops have stayed away.

Absence seizures (formerly petit mal)

Absence seizures can appear to observers as though the child is daydreaming or switching off. Because absence seizures may be hard to distinguish from the child's usual behaviour, they may be difficult to detect. They can occur many times a day and are often mistaken for daydreaming.

These absence seizures often accompany myoclonic seizures, especially clusters of myoclonic and / or myoclonic-astatic seizures. If these seizures occur frequently throughout the day, they can interrupt the child's ability to function properly because his/her awareness (and the brain's processing) is being constantly interrupted.

* Lauren would have many of these types of seizures daily. There were times I felt like I was calling her name all day trying to get her "out of it" (which you can’t). These seizures have gone away since the diet. We sometimes think we might see one or two but it hard to tell because sometimes kids stare for short periods of time!

Atypical absence seizures

Atypical absence seizures are similar to absence seizures but, as the name suggests, they are unusual or not typical. The child will stare, as with an absence seizure, but during an event he/she will be somewhat responsive. Like absence seizures, it can appear to observers as though the child is daydreaming or switching off so it may be hard to distinguish from the child's usual behavior and therefore difficult to detect. However, unlike a simple absence seizure, the child may be somewhat responsive and also have more pronounced motor symptoms such as twitching of the mouth, eye blinking, mild head bobbing which may give the observer a clue that seizure activity is occurring. In the case of an atypical absence seizure he/she may be somewhat responsive to and aware of his/her surroundings.

The child may have delayed reactions, seem out of character, be easily confused or agitated. Sometimes the seizures may go completely unnoticed because the event is indistinguishable from the child's usual behaviour. When the seizure ends, the child is usually alert afterwards however he/she will have missed some of what is happening around him/her.

*Before the diet Lauren there were a lot of times she was in a constant atypical absence. She just wasn’t herself! With the atypical seizures I could call her name and she would respond or answer but then would go right back into it. She could function through them but might just walk around in a stupor. These seizures have also stopped with the diet.

Tonic-clonic seizures (formerly grand mal)

A generalised tonic-clonic seizure (GTC) is the sort most people think of when seizures are mentioned.

In 60% of cases, the first seizure seen in MAE is a generalised tonic-clonic seizure. So, for most parents, a tonic-clonic seizure is the first symptom of MAE they will witness and it will strike completely out of the blue.

Initially the child stiffens and simultaneously loses consciousness (so he/she is completely unaware of the event). Tonic (meaning stiff) refers to the stiffening stage and causes the child to fall to the ground. Typically, the eyes roll upwards, the head goes back, the back arches and the arms and legs stiffen. The brain cells are connected to other nerve cells through the spinal cord and during this tonic stage, all the muscles are contracted, including the chest muscles so it is difficult for the child to breathe. A combination of the face being flushed with the bluish blood of the veins and also the lack of oxygen causes the child to turn somewhat blue around the lips - a process called cyanosis. Excess saliva may cause a gurgling sound in the mouth or throat. The result of the muscles in the lungs contracting forces air out, sometimes making the child sound as though he/she is crying out. Occasionally, if the child's bladder is full, he/she may lose bladder control.

After the tonic stage of a tonic-clonic seizure, the clonic (meaning jerk) phase of the seizure begins. The limbs jerk because now the muscles contract and relax in rapid succession. During this phase, the child may bite their tongue or the inside of their cheek. The fists are tightly clenched, the arms repeatedly flex at the elbows and then briefly relax. The legs flex at the hip and knee joint in a similar fashion; the head may flex and then fall backwards. Initially these movements occur rapidly but then gradual slow. The jerking becomes less intense and occurs at a slower rate, finally ceasing. Usually a deep sigh signals the end of the jerking and normal breathing resumes.

The seizure is now over but the child is not awake and will not respond immediately. This recovery phase is called the postictal state when the brain can be thought of as exhausted from all its activity. In fact, the brain is quite active but its major activity is to inhibit (stop) the cells from firing. This inhibition has brought the seizure under control. This postictal stage or recovery time differs from child to child. The child will probably feel like sleeping but can be roused and may feel tired, confused, agitated or somewhat disorientated. Muscles will probably be sore. It is best to allow the child to rest until he/she is alert and fully recovered.

*This is the first seizure Lauren had on December 26, 2007- and she had 3 of them that day. Before the diet she had these mostly when she first woke up in the morning. In the beggining these would happen every 3-4 days during good times. This is the only seizure type we felt that medicine was helping. Right before we started the diet she had a period where she went 3-4 weeks without a tonic/clonic. This is now the seizure type that we are having a hard time getting to stop! She now mostly has them in her sleep in the early morning hours. These are hard because they affect her whole day, they make her so tired and cranky.

PROGNOSIS AND COMPLICATIONS

Epilepsy with myoclonic-astatic seizures (Doose Syndrome) has a variable course and outcome (complete remission in some cases, persistent epilepsy in others) and cognitive development (normal or delayed). Spontaneous remission with normal development has been observed in a few cases. Complete seizure control can be achieved in about half of the cases with antiepileptic drug treatment-but may continue to have learning difficulties.(Doose and Baier 1987b; Dulac et al 1990). In the remainder of cases, the level of intelligence deteriorates and the children become severely retarded. Other neurologic abnormalities such as ataxia, poor motor function, dysarthria, and poor language development may emerge (Doose 1992b).

Various studies over recent years have shown that the ketogenic diet is extremely effective in controlling seizures in children with MAE, and is now considered one of the most successful treatments for the disorder, even more successful, according to some centers, than the more traditional anti-epileptic drugs.

*It is amazing how perspective changes with certain things. When I read the above about Lauren’s prognosis it gave me some hope. In the beginning Lauren had been given the diagnosis of Lennox-Gastaut syndrome, which there is not much hope for a normal life or development. The outcomes for both syndromes can be the same but with doose syndrome there is hope that Lauren will have a chance at somewhat of a normal life. That is why we wanted to get her on the diet so fast. We could see her going down hill very fast. Even though the diet has had its up and downs -the downs are not as low and we never had ups on the medication. We are hoping that the continued fine-tuning of the diet will get Lauren seizure free. It is the unknown of her future that has been so hard.

2008-07-18T22:27:00.000-07:00

Our family just got back from Aspen Grove family camp up in the mountains by our house. This has become a tradition for our family and we were concerned we would not be able to go this year.

Fortunately Lauren recovered well after her two grandmal seizures and we packed up all her food and gear and even a microwave and headed up to the mountains (only about 20 minutes from our house!). We arrived last Saturday and the next day she had 2 more grandmal seizures in her sleep. Again she recovered well and was able to do some of her activities with her age group. The BYU college girls that were her "teachers" were amazing and very willing to work with her. I think we overwhelmed them explaining the diet and all her restrictions. I still get overwhelmed hearing myself explain it! She did really good at her groups but it still is a little hard for me when I see the reality of how far behind she is compared to other kids her age. The more days she goes without seizures the faster she is picking up new skills which is really encouraging.

Anyway she did have another granmal seizure on Thursday morning in her sleep, which put her in some what of a bad mood during the day. We were able to keep in contact with her doctors while up there and they reduced her calories by another 100 on Tuesday. We are still working on getting her to loose some weight. She has lost about 1 pound in the last month and she still needs to lose a couple more. This diet has been such a wonderful blessing in our life. Even though she is still having seizures it is bringing back our little girl. We have received so many blessing and feel big and small miracles everyday. We are so thankful for all the many prayers for Lauren!

We had a great week as a family and had some much needed time together without a lot of outside stress. I will try to post some pictures.

2008-07-09T23:24:00.000-07:00

This morning at 6:30 and 8:30 am Lauren had two grandmal seizures in her sleep. We have no explanations why-none of this makes sense. It seems she does better when we don't feed her! Her day was okay except for being a little irritable. Now we just have to wait and see what each day brings. In the past once her seizures start like this it is just a matter of time before she "falls apart" and is in a constant seizure.

2008-07-06T13:47:00.000-07:00

Lauren's week has been up and down. Right now we are enjoying the up. Last Sunday (June 29th) we had to give her more "rescue medicine" to stop the continues seizures she was having. Everything seemed to be falling apart again. I talked to her doctor on Tuesday and they want to reduce her calories again. They are hopeful that once we get her to a good weight and have her on reduce calories that the diet will work more consistently. So on Wednesday we fasted her for 24 hours then the following day she received 300 then on the Friday she received 600. She is now at full diet at 900 calories. I apologize to our neighbors for this past week if they heard her screaming. There were a few episodes where she was screaming for 1-1 1/2 hours straight and the baby was sleeping so we took her outside. It is so hard to not feed your child! There was nothing I could do to help her or distract her. On the second day after fasting her seizures stopped. She has been 5 days with no seizures. Along with no seizures she has been amazing in her development. An average 2 year old has huge progress during this year. These past six months of seizures has taken a toll on her overall development. She has not made much progress and had lost some skills she had previously had before the seizures started especially in her coordination and speech. These past few days she has started talking so much more and even putting two words together. I am trying hard not to get my hopes up to much, we know how quickly things can change. It is a much harder fall for me when she does so good then falls apart. But we are always hopeful. We are so thankful for the kindness and support we receive from everyone it really lifts us during the hard times.

2008-06-28T20:45:00.000-07:00

Lauren has had a tough week with her seizures. A week ago we reduced her calories by 100 -doctor's order-to help her loose some weight. The night we did this she had 2 gradmal seizures in her sleep. She then continued to have one in her sleep for 4 days in a row. By the 5th day she started having drop seizures and jerking so we gave her Diastat on Thursday to try and break the cycle before it got too bad. Because of the medicine she had a good day but then started having drop seizures again on Friday evening. She has had a few today, we will see what tommorow will be like when the medicine has worn off more. Next Tuesday her doctor is suppose to call and check on her weight and what the next step will be.

2008-06-19T23:34:00.000-07:00

Lauren in the airport on her way to Johns Hopkins

This is Lauren's 5th EEG-she does so good!

Lunch With Leo-The family we started the diet with.

Baseball game at Camden yards Baltimore vs. Houston-
we saw 3 home runs and seats 10 rows up from home plate!

At the game with Lauren's Doctor-James Rubenstein and Diana Pillas-who was the first person I talked to and helped me get Lauren on the diet so fast.

Paddle boat ride on Chesapeake Bay and Lunch at the Cheesecake factory.

2008-06-19T14:43:00.001-07:00

We got back late last night from Baltimore. It was a quick trip but so much less stressful then our last trip up to Johns Hopkins. Lauren has been doing so well this past week-we never fasted her. Our appointment on Tuesday started out with an EEG we then had lunch and a quick visit with one of the families that we started the diet with. In the afternoon we met with Lauren's doctor and dietitian. I am a little confused on the results of her EEG-but based on the 3 EEGs they have seen at Johns Hopkins they are pretty sure that she has Doose Syndrome. 6 months ago this news would of been devastating to us, but after thousands of seizures it is encouraging. It is so confusing to have two different doctors at two different hospitals look at the same EEG and have two different diagnosis. Anyway all her other tests looked good -her cholesterol is only 203! They are concerned about her weight gain (she has gained 6 pounds since she started the diet). She is not suppose to gain weight on the diet so they are adjusting her calories again. They are wondering if this has been the reason for her up and down seizure control. Overall they wanted to stress that the diet is working, we went over her seizure calender since starting the diet and she has had 36 seizure free days and she has been on no anti-seizure medications. Before the diet she was on 3 medications and never had seizure free days-so the fine tuning continues and hopefully we can achieve more seizure control.

When we were getting ready to leave the hospital after our appointment we mentioned to Lauren's doctor that we were going to try to go to an Orioles Baseball game at Camden Yards that night-since our plane didn't leave till the next day. Her doctor said that he was going to the baseball game also and told us to buy the cheap seat and come sit with them in section 40. After convincing the usher we got through and sat with her doctor 10 rows up behind home plate! It was so much fun.

We stayed with my brother John again, he has a nice apartment that is not to far from the Hospital. He is taking his boards for medical school next week-so he was nice to let us stay there even though he was so busy.

The next morning we had a little time before our plane left so we went to the Inner Harbor in downtown Baltimore. Lauren loved looking at all the boats so we took a paddle boat ride in Chesapeake Bay. We then enjoyed a great lunch at the Cheesecake factory overlooking the harbor while Lauren ate her butter and cream and watched all the birds on the patio.

2008-06-14T22:03:00.000-07:00

I heard back from Lauren's doctor at Johns Hopkins 6 hours after I sent him the email-so amazing! He wanted us to fast her for 24 hour then feed her 1/3 of her calories the next day then 2/3 the following day. (Pretty much what we did when we first started the diet.) Before we could do this she went into another bad seizure cycle in the morning (Thursday) and we had to give her diastat. The diastat works longer then the versed so we decided to wait to fast her till the diastat wore off. Well it is now Saturday night and she has been doing so good. I am just waiting for the seizures to start up again to start the fast. I will be so upset if she stays good till we get to Hopkins then goes bad when we get home! A lot of people have thought that we were going back to Johns Hopkins this next week because she has been doing so bad lately-this is not the case. We have to go back every 3 months for follow-up appointment then every 6 months after a year. We have had this appointment planned since we left last time. We will keep you updated of what happens at our appointment.

2008-06-11T22:04:00.000-07:00

Things are not to good with Lauren right now. Here is the email that I sent to her doctor tonight that summarizes her day.

Dr. Rubenstein,

These past two days Lauren has quickly gone bad. For most of the day she has been in almost this constant seizure state-Rhythmic myclonic seizures about every 1-2 seconds with weird eye blinking and staring-she had about 5 of these that lasted around 40 minutes. When she is not having these she is so irritable and screaming and she is constantly "twitching" everywhere. She has not been able to function today. She has been having drop seizures that have really hurt her -I think she will loose a couple of her teeth, it looks like someone hit her across her face, and I have wiped up blood numerous times. We gave her nasal versed at 8pm tonight, and the seizures stopped after 5 minutes. I don't know what to do when the medicine wears off tomorrow! I don't feel comfortable traveling on a plane all day with her in this condition next Monday. The only medicine we have to give her is diastat. Should I just keep giving that to her so we can travel on Monday? I feel like I should fast her till we get there because I know that would stop all this! I am still hoping that the diet will work because she has had so many good days. I am trying so hard not to worry and wait till we get to Hopkins, but I am very worried about her-she is so bad. If you could give me any direction on what to do for her during this short time till we come next week. Maybe it is nothing, I just need to know.

Thanks,

Tami

2008-06-06T23:12:00.000-07:00

Lauren has had a grandmal seizure every morning for three days in a row (Wednesday-Friday). Tonight after she was a sleep for an hour or so she had a weird seizure, one that she has not had before. It went on for about 5 minutes so we gave her diastat and it shortly stopped. So with the medicine hopefully she will sleep the rest of the night. Overall during the day she has been okay, except for being cranky from the morning seizures. Yesterday morning she pointed to her head and said it hurt, so I think the seizures are taking a toll on her. ( I think I would be cranky too!) I think we are going to fast her for 24 hours tomorrow and see if that helps.

2008-06-03T20:45:00.000-07:00

Things have continued to be pretty good lately. She did have another grandmal in her sleep Monday morning. This one was at 1:30 am- so we have no explanations of why. She never had grandmal seizure in her sleep before the diet. But overall she is doing really good. She has not had a drop seizure for over a week-which is really nice. We are getting ready to go back to Johns Hopkins in a couple of weeks for her follow-up test and appointment. I can't believe it has been 3 months since we started the diet-time flies and goes in slow motion at the same time! We are so thankful to everyone for all your continued prayers and support.

2008-05-30T21:48:00.000-07:00

overall things have improved so much since we increased her calories. She went 8 days without having an early morning grandmal seizure but she did have one Yesterday morning. She was still having drops till last Saturday but those have stopped for now. She has been so happy and playful. We were able to go to St. George over the weekend. It has been the first time we have been able to do something since her seizures started. It felt very nice! Who knows what tomorrow will bring but we will enjoy the good days for now.

2008-05-20T20:41:00.000-07:00

Lauren continues to have a tough time. She had 2 more grandmals in the night at 2 and 5am. Plus she is still having drop during the day. I had a good conversation with Lauren's doctor and dietitian this afternoon. We went over her recent history of her seizures and tried to find any pattern and what would be the best adjustment for her diet. They pretty much left it up to me because they said my guess is as good as theirs! So we increased her calories by 100 but redistributed them to 200 in the morning and 333 for lunch and dinner and a meal before bed. We will just have to try this out for 10 or so days and see what it does. I am trying to remain sane during all of these up and downs but I think I am going crazy! We really are doing okay it is just so hard when there are no straight answers.

2008-05-18T20:41:00.000-07:00

Things sure turned around from the last time we updated. Last Monday she started having drops again and has continued to have grandmal seizures in the early morning. Saturday everything seemed to fall apart and by the evening she was in her continuous jerking and twitching. We had to give her some medication and fast her on Sunday for 24 hours. Her doctors are going to meet on Tuesday and call us with any changes.

2008-05-11T22:35:00.001-07:00

Lauren has had a good week. Since the last post she has only had one drop seizure but has had 3 grandmal seizures all at 5 am while she was asleep. So overall she is doing so much better. Her doctors have redistributed more of her calories to the evening to see if that will help with her early morning seizures. The purpose of that is to see if her body is running out of fat throughout the night and causing her ketone level to drop. So giving her more calories of fat before bed may help her maintain higher ketosis throughout the night?
It has been nice to have the drop seizures go down. Those seizures really hurt her and have caused some big bruises and cuts.
Well overall the diet is getting easier to give and measure. Using the gram scale has become second nature. It is still hard for me to make sure she doesn't get other food in between her meals. It is amazing how much food there is everywhere. She has been so excepting of it. We just tell her its not good for her brain and she says okay. I have been amazed!
Thanks you for your continued prayers, they are so appreciated!

2008-05-04T20:49:00.000-07:00

It's been a while since we've updated the blog. Sorry. Just after our last update, Tami talked to the doctors at Johns Hopkins and they reduced Lauren’s daily calories by 100 - she now gets 1100 calories per day. Following this change, Lauren had two more grandmal seizures - one on early Friday morning and early Saturday morning - around 5:30am each day. Even though these types of seizures are pretty dramatic (and traumatizing for the parents), Lauren went seizure free for the rest of both days. She was able to go to church on Sunday last week and today. Lauren really loves going to Nursery class.

This past week has been good overall. Lauren still has a few drop seizures (2-3) each day and usually in the morning. It seems that she gets better as the day goes on. On Wednesday, she was seizure free and had a wonderful day. Lauren did have another grandmal seizure on Saturday morning about 4am, and one drop seizure in the morning. After this she did great. She played outside on the swings, went down the slides and played with her sisters.

She is doing well on her diet. A typical meal for Lauren consists of butter soup (just melted butter), cream that we whip or make a shake out of, grilled chicken, and a very small portion of a fruit or vegetable. She loves her meals and eats them right up. She also gets two snacks during the day which is usually a peanut butter/butter lollipop.

She is such a courageous little girl. In her own way she understands that she cannot have regular food and the treats the other kids have. This is so hard as a parent, but little Lauren just says ok when we tell her certain foods are not good for her brain.

2008-04-24T20:16:00.001-07:00

Well like I said things can change very fast. Lauren was doing so good then Monday morning she had a grandmal and continued to have drop seizures on Tuesday and Wednesday. We did another 24 hour fast starting Wednesday afternoon. She had another grandmal seizure in the night but woke up great and has been seizure free today. I talked to her doctor at Johns Hopkins and they are going to go over her diet and call me with some changes tomorrow. The fact that she responds so well when we fast her tells them that something needs to be adjusted in her diet to maintain that state. So the roller coaster continues.

2008-04-17T22:39:00.000-07:00

Lauren is doing so good since we fasted her on Monday. (It is so hard to not give your child any food! ) It is spring break for the other kids so we took them up to Salt Lake to a baseball game and to the discovery museum. She had her last drop at about 3pm then she started walking around the museum in a crazy daze -almost delirious. We got her to lay down in the stroller and she slept for a couple of hours. Then like a switch at around 6pm she stopped her weird twitching and constant little seizures and has not had another seizure since then. It is so amazing. She has been so active just like a little 2 year old. Her doctors called Tuesday morning and they are going to keep her calories the same. They took lettuce out of her diet thinking that might of triggered all of the seizures this past week. I gave her 8 grams the night before ( a couple of bites). it is hard for me to get my hopes up because I know how fast things can switch but I am so grateful for every good day.

2008-04-13T21:33:00.000-07:00

Well the roller coaster continues. Lauren had about 6 great days of no seizures, then on Tuesday night she had 3 grandmal seizures throughout the night. Since that night she has continued to get worse with the drops and little jerks. We don't know what has caused this sudden change. We talked to the doctor tonight and we are going to fast her again for 24 hours and then readjust her calories Tuesday morning. They want to fast her again to give a "jump start" to her body. The team of doctors and the dietician are going to meet Tuesday morning and decide if they are going to increase or decrease the calories. I guess this is all part of the fine-tuning the diet.

2008-04-03T20:18:00.000-07:00

Lauren is doing so good. This past week she has improved each day. Yesterday she had only one little head drop and today she had no seizures! She has been herself most of the day these past few days. We never know what tomorrow will bring but today is such a miracle-no seizures and no medications! It is so nice to be able to start doing things as a family again and taking Lauren with us places. We feel so blessed that this diet has started helping Lauren so much. We are so thankful for everyones continued prayers and concern for Lauren.

2008-03-29T22:13:00.000-07:00

Here is a short video of Lauren today. As you can see, she is doing much better than a few days ago.

2008-03-29T20:44:00.000-07:00

Lauren has been doing a lot better these past few days. She kind of came out of her zombie like state Thursday. It could be from the calorie increase they started on Tuesday night? She has not had a grandmal seizure in over a week. The drop and jerking seizures still go up and down but she has been herself a lot more. Today she had a bad afternoon with lots of drops and jerking. I tested her ketone levels and they were low. When ever we have tested them in the past they have been really high (which is what we want). The only thing we could trace it to was giving her a late lunch because of soccer games. Also she gets 25g of lettuce a day for a "free" food- so I brought that to the soccer game so she could snack on that. I think her not eating the lettuce with the fat messed up her ketosis. After lunch and her afternoon snack her levels were back up again and her seizures went back down. Tonight she was talking and playing like her old self again. This whole experience is so amazing! We still take everyday one step at a time but we are enjoying seeing her little personality coming through. It is also nice to see the effects of the medicine leaving. She has been spending long periods of time doing her puzzles these past few days. When she was on the medicine her attention span was very short (even for a two year old!).

2008-03-27T10:56:00.000-07:00

Here is a video of the kind of seizures Lauren is having lately. These little twitches of her hand and eyes go on most of the day. In the morning she is non-responsive and just likes to be held. As the day continues she starts talking and moving more, but she still has the constant little seizures. The "Keto" team at John Hopkins raised her calories by 100 a day-which translated into a little more cream and butter per meal. They tell me it takes 1-3 months to "fine tune" the diet. So it is still a waiting game.

2008-03-23T20:03:00.000-07:00

Sorry it has been a few days since we have posted the latest details of Lauren. The last few days have been similar to the report on Wednesday. Lauren has not been herself and is having more seizures. She had a grand mal seizure on Friday morning about 6am. She has not had as many drop seizures - only a couple a day. Before the diet she was having up to 100 per day. The last couple of days she has been more twitchy and having the little jerk seizures. She has been very dazed and just not there at times. Yesterday morning was really bad but then after her nap she kind of snapped out of it and was more herself. Lauren is doing excellent at eating her food and keeping up with the diet. She has her three meals per day and her two snacks. She will now eat her butter as-is at the start of most meals. Her favorite snack seems to be peanut butter-butter and celery.

Today was Easter and the Easter bunny was kind enough to bring Lauren a cash register and toys rather than candy. He also was stingy with the candy with the other kids and they were all as happy as could be. Being Easter Sunday we all finally made it to church. A little late, and Lauren quite a bit late. We never know how she will wake up. After a day much like Wednesday Tami gave Lauren some more diastat (valium) to break the seizure cycle. She was doing much better tonight. The diastat seems to last about two days and keep the seizures away.

Right now we don't know if the increase in seizures is because Lauren was taken off her medications so quickly or if the diet is not working. We are hoping it is related to the medications because Lauren did have so many seizure free days when she first started on the diet. Only time will tell. Other than that, we take each day a little at a time. Again, our thanks and appreciation go out to all of you that have expressed your concern for Lauren and our family.

2008-03-19T21:59:00.001-07:00

Some days are bad and some days are good. Today was one of the bad days. Lauren was still not herself today and was having lots of little jerk type seizures. She also had another grand mal seizure about 10pm last night ( Tuesday). It lasted under a minute but still is traumatizing. With the grand mal seizure and how Lauren was today, Tami gave her diastat (valium) to stop the seizures. After this medicine she slept and then was much more herself. The diastat seems to break the cycle when it gets bad like this. Lauren is doing very well eating her meals and snacks. We are gradually getting her to take her vitamin and calcium supplements. This has been one positive thing over the last few days. Tami talked to the doctor at Johns Hopkins today and he would like to see how Lauren does the next few days. He might then add back an anti seizure medication to help. This whole process just takes a lot of patience. Thanks again for all your support.