Cook Childrens: Day 1

Monday morning we get to Cook's Children's Hospital at 7:45a. Go straight to registration, then immediately taken to the EMU (Epilepsy Monitoring Unit) of the hospital. It's the 4th floor of the neurology wing.

We get in our room, about the size of a smaller guest bedroom with a small table, 2 chairs, 1 recliner, 1 crib (cage) and a sofa. There is a bathroom that is almost as big as the room itself. A TV mounted on the wall, a built in closet and a space near the door for the nurses.

Let me just say that the first day is very overwhelming for the baby and the parents.

First the nurse comes in, introduces herself and gives a quick run by of the day and week's events to come. Then about 15 minutes later the nurse practitioner, ours was Jericho, comes in and asks a lot of questions that we've probably answered 20 different times now so why not 21 times? Like, was she full term? Yes. When did the seizures start? About 2 weeks old. Diagnosed at 6 weeks. What type of seizures? Usually tonic-clonic, Keppra makes them just tonic seizures though. What medicines has she tried? and so on...

Rayleigh starts drifting off to sleep after Jericho leaves. I buzz the nurse in and let her know that Rayleigh is falling asleep and will most likely have her seizure (normally one a day, at naptime) and ask her what we should do since Rayleigh doesn't the EEG hooked on yet. She said to go ahead and let her sleep and just take the chance because she's sure that Rayleigh will have a seizure sometime during the 3-day EEG. Sure enough she has a seizure. Michael pages the nurse to let her know and she makes note of it.

She then brings in these various sheets. One of which we have to check the times for when she eats, what she ate, and how much and then check the time for each diaper change. They weigh the diapers to see the difference of how much goes in vs. how much comes out. Another sheet was for us to describe each seizure as we see it so they can compare to what the EEG and video shows.

Next Maria comes in to put on the EEG leads. Maria is the Video EEG technician along with another technician that stays in the control room watching the videos, they do up to 6 at a time. Rayleigh has had 2 EEGs before but this one is totally different since she has to have the leads on for 3 days instead of 30 minutes. They use the conducting cream, the lead and then a small piece of gauze drenched in a watery type of super glue and use a cold air blowing tool to apply it. It is very cold and very uncomfortable. Maria gets 5 of the 25 leads on while Rayleigh is still asleep but then she wakes up screaming and so mad that I'm almost in tears holding her down and letting them do this. But knowing that this will give them answers keeps me pushing through and staying strong for my little Bug.  

Not long after Maria leaves Rayleigh starts falling back asleep... then the nurse comes back in with the paramedic, Mike. Mike checks her vitals and then starts the IV. This is standard for the EMU of CCH. They do an IV so if a child has a seizure longer than 2-5 minutes (depending on the child) they will use a rescue anticonvulsant to stop the seizure. Inserting the IV woke Rayleigh again. Screaming, sad, tired. Every time she falls asleep she gets a rude awakening. This kept up for most of the day.

We met one of the epileptologists for about 1 minute. He was pretty much just letting us know that first we need to confirm that she is actually having seizures before we go into the details of anything else. He also let us know that he wouldn't be our epileptologist but he was the only one there that day.

Dr. Coleman warned us that we wouldn't get much face time with the epileptologists while we were there and he was spot on.

Rayleigh was so out of her element that night. They took her Phenobarbital level down from 12ml a night to 6ml so that they would have a better chance at catching seizure activity. After the medicine we fed her and she normally drifts right off after eating. Well, not here. She would scream anytime we lay her in the crib cage thing. She was only comforted by one of us rocking her or laying on the couch/bed with me.

We co-sleep at home for nighttime so I thought maybe I could just lay with her until she falls asleep and then move her into the crib but she just wouldn't sleep. You could tell on her face that she knew this wasn't home.

Around 11:30p after several minutes of rocking with Daddy we finally got her fed again and off to sleep and in her crib.

Well, then at 2:30a she woke with a seizure. This is definitely a change due to the medicine because she never wakes up in the middle of the night with one anymore. There is a red button to push on the VEEG pack when she starts a seizure so we press that and then the microphone is on in the room and the nurses come in and start describing loudly what she is doing, "arms tensed and pulled into body" "hands clutched" "legs tensed" "face reddened" "labored breathing" "coming out of it" "myoclonic twitching" "shes done."

Then Rayleigh begins crying, waking up in a strange place with several people in her face. I pick her up and quietly soothe her on the couch on the other side of the room to let her know that Mommy and Daddy are still here and that everything is OK.

It took a long time for her to go back to sleep but she eventually did so we put her back in the crib and tried to get a couple more hours of sleep on the couch bed before day 2 started.



Rayleigh with gauze wrapped around the EEG leads so she doesn't pick at them and a "no-no" boot cover over the IV.



Ft. Worth

Michael and I always talked about Rayleigh's first trip out of state. We figured it would either be a quick weekend down to Texas for some fun at the beach or amusement parks or to visit my family in Florida. We never thought her first trip would be to a children's hospital.

We knew we had to be at Cook's Childrens Hospital in Ft. Worth, TX by 7:30a to "check-in". Ft. Worth is about 3 1/2 hours from our home. We could have made the drive but we would have been exhausted and stressed out. Thankfully, Michael's parents got us a hotel room in the Hampton Inn just outside of downtown Ft. Worth (where the hospital is located).

This was Bug's first hotel stay. We were so excited to experience another first with her! And it was going to be so great to have a nice relaxing evening in the hotel before the hospital stay.

We made 1 stop for gas and some fast food shortly after getting out of the city and then we had to stop on the side of the highway twice to change diapers.

We arrived to Ft. Worth around 4 in the afternoon and checked in to Hampton Inn.

That reminds me of how much our lives have changed since Rayleigh Bug arrived! First its the diaper changing in the car, something I never thought I'd be doing! And then there's the packing. Michael and I can pack for 5 days in 2 suitcases, including all clothes, books, laptop, everything. The trunk of our SUV was literally full, also had the diaper bag and a bag of snacks in the backseat!! Babies come with sooo much!!

In our hotel room you could tell that Bug knew we weren't at home but she didn't seem uncomfortable. I broke out her Bumbo chair and tray and some of her jarred food to feed her in the room. She's not used to eating this way but she did pretty darn well!

After she ate I went to the gym, literally right next to our room! Michael and Bug played the whole time I was gone and she didn't cry once! She sure does love her daddy-time!

When I came back we figured her tummy was settled from eating so we put on our swimsuits and headed to the indoor swimming pool for some relaxing fun time before settling in for the night.

Back in the room we each showered (I held Bug in the stand up shower while she cried during her whole bathtime!!), gave Bug her medicine and then we snuggled up in the king size pillowtop bed, Bug took a bottle and fell asleep so fast we thought she liked that place better than home!

Michael and I had a quick dinner, watched some tv and followed Bug's lead to sleep.

Woke up at 6am, got ready, loaded the car, all went to the lobby for breakfast and checked-out of the Hampton Inn to go check-in to Cook's Childrens Hospital.

A Referral

We keep Dr. Coleman, her pediatric neurologist, updated on Rayleigh's seizures via e-mail and phone calls in between visits. So we called him while she was taking the Phenobarb+Keppra to let him know that her seizures and frequency hadn't changed. After 2 weeks of trying this combination he called to check in and we decided then to take her off the Keppra.

Dr. Coleman put in a prescription for Rayleigh to start taking Clenazepam. Clenazepam is a wafer tablet that dissolves in her mouth. It is used as an anticonvulsant, also can be used as a muscle relaxer. We have been giving her one tablet a day (at nighttime to reduce fatigue during the day) and she has been taking it very well. No side effects to report. We started the night we talked to Dr. Coleman, so Bug has been taking it for a week and a half now.

Dr. Coleman let us know that he received the results of the chromosomal analysis and the DNA test that we had blood drawn for last month. Both came back normal in regards to anything epileptic or neurological. There was a slight abnormality though. Bug has a gain, or extra material, on the P32.2 band of her DNA. This can lead to different cholesterol issues, but she shows no signs of that now and it's unlikely that this will ever turn into a problem based on its current state.

The biggest piece of information Dr. Coleman told me was that he put in a referral for Bug to go down to Ft. Worth, TX and be examined at the Cook Children's Hospital.

Cook Children's Hospital has a special, top ranked pediatric epilepsy center. One of the best in the country, if not the best!! While there, she will be seen by Dr. Saleem Malik. His page on the Cook Children's Hospital website is impressive. We look forward to meeting him and getting a second opinion from an epileptologist.

Rayleigh Bug will have a 3-day EEG done when we first arrive to Cook. The last day, day 4, she will have another MRI. They may not need to do this after they view the MRI she had done 3 months ago. The chances they will want to do their own is about 50/50. Their MRI machine is more powerful and will see more clearly and thoroughly into the brain. Her brain has matured since her last MRI so we think they'll probably want to do their own test. Other than that, she will have more blood tests done to check metabolic levels for Coleman and a few blood tests of their own. We will also talk with a nutritionist/dietitian and a metabolic geneticist.

As I'm sure you can imagine we are anxious, excited, nervous and ready to get this done. Hopefully we can get some answers out of this trip.

Keppra's Second Chance

The first medicine Rayleigh tried as an anti-convulsant to control her seizures was Keppra. This was when she was 6 weeks old. Keppra took her number of seizures down from 4-6 a day to 2-3 a day but never decreased the number from there, even with dose increases. After a few weeks on Keppra we added Zonegran. Zonegran is a capsule form medicine generally used to treat partial seizures in combination with other medications. Adding Zonegran didn't change anything so we stopped giving it to Rayleigh after 10 days and proceeded to Phenobarbital. While she was taking Phenobarb we weaned her off Keppra so she was taking just Phenobarb. She went seizure free for about 16 days and then had a breakthrough almost 2 months ago.

We have since been trying increased doses of Phenobarb, taking Phenobarb twice a day rather than just at night and then added Keppra twice a day. Adding the Keppra made no difference in her seizures. She continued to have 2 daily. Once at naptime and once at bedtime.

We were told by Dr. Coleman, her pediatric neurologist, to give the Keppra+Phenobarb combination 2 weeks to see results. During these weeks we noticed Rayleigh eating less and not taking her solid food well at all. At first, we attributed this to a cold she had been getting over but once the cold was out of her system and this eating issue continued we realized it was more likely a side effect of the Keppra.

The 2 weeks came and went and she was still having 2 seizures a day. No improvement with the Keppra+Phenobarb combination.

Second EEG

Rayleigh had her second EEG Tuesday, October 12, 2010. She is 6 months old. The goal was to see if the brain activity has changed when not seizing and if she does seize during the test, where it is coming from.

Rayleigh finishing her EEG

We go to a small, cool, dim room with a bed and one chair. I lay on the bed with Rayleigh while Michael sits in the chair next to us. We quickly soothe Rayleigh to sleep and the EEG technician comes in and begins sticking the receptors to Rayleigh's head. She has a note that she needs to do it quickly because the patient (Rayleigh) seizes within the first 20 minutes of sleep.

We were conflicted. We absolutely do not ever want Rayleigh to have a seizure. But if she was going to that day, during the test would be the time to do it so Dr. Coleman could get a better idea of what is going on.

She had a seizure during the test.

Rayleigh slept for the first part of the test, woke up about 10 minutes into the test and had a 37 second seizure. Stayed awake for a little bit after and then went back to sleep. Once she was back asleep the technician came back in and did the strobe light section of the EEG. Rayleigh did not react to the strobe light at all.

The technician said that the test went very well, got all the information they needed from her asleep, seizure and awake sections. After the EEG we went over to Dr. Coleman's office so he could go over the results with us.

Dr. Coleman said that she is not having focal seizures. Focal seizures is when the brain activity is abnormal on one side of the brain. He thought maybe that is why she used to always turn her head and eyes to the right when she had a seizure, something she no longer does on medicine. He said that the seizure activity happened on both sides in the frontal lobes of her brain.

She is now having Tonic seizures as he explained. Before getting on anti-convulsant medicines she was having tonic-clonic generalized seizures. The Tonic part of a seizure is the initial muscle lock up and tense. Clonic is the twitching or jerking of the tensed muscles.

Dr. Coleman told us that tonic seizures are generally harder to control with anti-convulsant medicine. Tonic seizures can still be grown out of but it is not as likely as the tonic-clonic seizure. Dr. Coleman said that the harder the seizures are to control, the less likely they will grow out of them. This was a huge hit to Michael and I.

New course of action is to try a combination of medicines. Her Phenobarbital + Keppra. Yes, we are going back to Keppra to see if maybe it will work when combined with the current prescription. She is taking Keppra twice a day, 1.4ml each time and then 14ml of Phenobarbital at night. If this combination works we will slowly start weaning the Phenobarbital back so that she is at a lower dose of it along with the Keppra. Keppra is much more concentrated than Phenobarb so you don't take as much of it.

We are supposed to give this combination a couple weeks to see results. So far, it worked well for the first couple days. She went from 2 seizures a day to 1 a day. Yesterday she had 2 seizures though. Today, she has taken 2 naps and has not yet had a seizure with either one. This is a good sign, but can't get too hopeful yet.

If we don't start seeing results in a couple weeks we will either see a metabolic specialist to try and find an underlying cause that can be treated OR we'll see a nutritionist about the Ketogenic Diet.

Right now, worse case scenario is that she continues to have a couple seizures a day, with the medicine controlling all but those couple, for the rest of her life. But we can't think about that now, we still have a lot of options to go through before we get to worse-case scenarios.

And So It Continues

Three weeks ago Rayleigh had a major breakthrough from her Phenobarbital medicine and began having seizures again. They started gradually, 1 seizure every 2 days or so and then got to where we are now at 2 a day.

The seizure will generally happen about 15-20 minutes into sleep. She usually has one for naptime and one at bedtime. Today was weird, she had one when waking up in the morning at 7:30a. She did not have one during naptime today but then had one when she went to bed at 9:30p.

A couple weeks ago on Saturday, Oct 2, we did as Dr. Coleman prescribed and started giving Rayleigh 12ml of Phenobarbital a day. We were to do 6ml morning and night. Each time you change the dose of Phenobarb you have to give it 5-7 days to take effect because it has a long half-life. After 5 days there was absolutely no change. We expected to at least see them slow down if not go away on this dose.

We called Dr. Coleman and spoke with him on the phone for quite a little while. He is ordering a new EEG to see if her brain activity has changed. If so, this will give him more information on the direction we should go in. The EEG is tomorrow.

Also, he had us up her dose to 14ml (7ml twice a day). This seems like a lot to us. We took Rayleigh to get her blood drawn today so Dr. Coleman can check the Phenobarb levels to make sure they are still within the normal 20-40 range.

Dr. Coleman had a couple of points of information that were a bit unsettling:

1.) The harder the seizures are to control, the more likely we are dealing with a longer prognosis of her having seizures.

2.) He has had patients where the best you can do with medicine will still have them having a few seizures a day. It sometimes comes to the point where the parents have to make the decision to either give her a higher dose of medicine than normal range and risk developmental side effects OR keep the dose normal and prevent most of the seizures but not all.

We had to stop by Dr. Coleman's office today to pick up the paper order for her bloodwork to take to the lab. While in there, Dr. Coleman talked to Michael quickly and said that even though she's still having a couple seizures a day and today is day 5 on this new dose he doesn't want to make any changes on her medicine until after the EEG and then he asked us to come to his office right after her EEG because he will immediately have the results and he'd like to go ahead and squeeze us in so we can go over them as quickly as possible.

We know that she's not conscious when she's seizing. For her it's like sleeping and waking up feeling weird, but it is so upsetting to watch this happen to your child and know that there isn't anything more you can do for them. Sometimes we think it scares her and then she's afraid to go back to sleep which is heartbreaking.

We always tell her, "I promise we are working with the doctor to get rid of these!" And we are. We just love her so much and hate that she's going through this. No child deserves this.

Epilepsy brings out a lot of emotions and very little answers.

The Search for Information

Today started off on a bad note as does this post. Michael woke up feeling under the weather so he went to sleep in the other room so he wouldn't get Bug or I sick. Then when we woke Bug up to get ready for her doctor appointment with Dr. Coleman she had a seizure. Doesn't get much worse.

If you don't already know, our pediatric neurologist is Dr. Coleman, his office is located in the Baptist Medical Building in OKC.

Dr. Coleman said that since she is continuing to have seizures but responding to the Phenobarbital he would like to up her dose to 12ml. He wants us to give her 6ml twice daily rather than the full 12ml at night. So tonight we will give her 12ml to make up for the missing lapse that we'll encounter when switching to 6ml twice a day.

We got an explanation for why she had such a major breakthrough while on Phenobarb if he believes this medicine is the right one for her. He said that Phenobarb is an anti-convulsant medication that goes in to fix the root of the problem, often after a patient has been taking it routinely for a long period of time the body may recognize the Phenobarb as a threat and in a sense, attack itself to compensate for the work the Phenobarb has been doing.

He did his routine check of hearing, tracking and visual interest and she passed all with flying colors! We'll see SoonerCare next week to do a developmental assessment on her. Phenobarb has a nasty side effect of slowing brain development and we have noticed it in a few things like reaching and playing with her mirror image.

Dr. Coleman put in the order for her to get a second EEG done in the next couple of weeks. It is normal for a pediatric neurologist to get multiple EEGs because their brains can change, their seizures can change and the brain activity when not in a seizure can change. Rayleigh's last EEG was completely normal which means that her brain acts as any other infant's does when not having a seizure. As much as we do not want our baby to seize, if she does have a seizure during the EEG it helps Dr. Coleman determine what kind of seizure along with where in the brain it is happening.

He also had us go over to the lab and get some blood drawn to do DNA and chromosomal tests to possibly find a reason for her seizures. We'll get the results on the chromosomal analysis within the next couple of weeks and the DNA test will take longer.

The statistics say that 2 out of 3 children with epilepsy outgrow them in their adolescence, Dr. Coleman believes that Rayleigh is still within that statistic because all of her tests are coming back normal and she is responding well to the Phenobarb even with the need to up the dose.

At this point, all we can do is get those seizures under control and do the tests Dr. Coleman wants done to get more answers. Frankly, we hope these DNA and chromosomal analysis come back normal and don't show anything about why she's having seizures so that we're still dealing with something benign.