Things continue to remain stable for Jenelle these days (knocking on wood)- thanks for the continued prayers! I spoke to Dr. Shields from UCLA yesterday regarding the improvement we've seen with the "max" dose of Topamax. Just a reminder, since reaching our max dose of Topamax, Jenelle's seizures have decreased in frequency and length - now we only get 2 or 3 a day lasting mere seconds instead of minutes. We have been concerned however because Jenelle's development has not improved much since gaining better seizure control. The sad reality is, as Dr. Shields delicately explained to me yesterday, that even with total seizure control, Jenelle may never improve developmentally. There still is hope that isn't the case - last week Jenelle was diagnosed with a "raging" ear infection, all the while she is still trying to bring in two new molars! He is hopefully that we can still give her development more time. Usually sickness like an ear infection, or teething will bring on more seizures, but we've maintained relative control so that is hopeful.

Dr. Shields decided that we've seen the best we are going to see with Topamax, however before moving onto a new drug, he wanted to wean her off one of her current drugs first. She is on three pretty powerful drugs as it is, so before adding a fourth, we'd decided to get rid of Klonopin first (a drug with valium components in it.) We started to wean Klonopin last night, and she will be off of it within a week. After missing last night's dose, Jenelle decided to wake up and make noise at 1:00 in the morning until 2:30 last night- not sure why, but maybe since Klonopin makes you sleepy, she was showing us how awake she was without it! I wish I had skipped the morning dose instead! Anyway, like sickness and teething, a drug wean can also bring on unwanted seizures, so I'm keeping my fingers crossed we can get through this week quietly. We will be off Klonopin completely by our next appointment with Dr. Shields, so we will then discuss a new drugs and maybe the diet at that time.

Jenelle was finally fitted for her stander and pediatric wheelchair. We are still waiting on insurance approval for the wheelchair, but it should be done by the end of this week. We will also be getting a "bath seat" which will be helpful since I usually just lay her flat in a tub of water 1 inch deep - maybe that is why her ear is getting infected so easily? Who knows! The stander will help her balance and bone density. Once we get the stander, we will fit her legs for supports (AFOs) and buy her first pair of shoes! High top Stride Rites - YAY! I’m sure they'll last a while since it may be a while for her to use them properly. The Pediatric Wheelchair is like a stroller and will be gratefully appreciated. Jenelle is almost sliding out of our current stroller, and I feel it doesn't support her well anymore.

On a final note - Jenelle's hair is getting even longer and thicker, but remains curly! She looks like a Q-pie doll! I have film waiting to be developed and will send new photos as soon as possible. Jack is doing well, and we hope will be fully potty trained by the end of this week. He loves his Nemo underwear! I will continue to keep you all posted. Please keep us in your thoughts and prayers that we don't have any unwanted seizures during this drug wean! Thanks again!

Well, its always nice to hear the good news for a change, and I am happy to report that Jenelle has been doing really well lately (knock on wood!) Since hitting the "max dose" of her Topamax, Jenelle's seizures have decreased in frequency and length. She was having around 10 or more a day, and instead is now having 2 or 3 a day, and these last just a few seconds as opposed to half a minute or more! Her last grand mal (knock on wood) was May 26! We're not sure if this "progress" is promising enough to stick with Topamax or move onto the Lamictal, so we are waiting to hear from Dr. Shields for his expert opinion! ;) As far as I'm concerned, if this is the best control we can get - it is wonderful!

Jenelle's first day back at the Blind Children's Learning Center was wonderful. My Mother in Law picked her up from school that day and said, "she is a completely different baby!" She was much more stimulated and vocal - in fact Marianne said she babbled the whole time in the car on the way home and until she fell asleep for her afternoon nap! And, the best news is that Jenelle didn't have a single seizure while in school that day! It was as if she knew where she was and was on her best behavior, and was obviously so very happy to be back!

Jenelle is also vocalizing much more these days, and finally said "Mum ma" a few times last weekend. This is wonderful progress for her as most children master that sound around the age of 8 to 10 months or more. It wasn't really directed to me, but it was a beautiful sound none the less. She should have said "da" first as most babies master that before "ma" - so that will be our next goal. Her hair is finally growing more and has lots of curl to it too! Finally we can get a pony tail on the tippy top of her head! It is very cute.

A few weeks ago, we drove to San Diego to meet another family from Arizona that has a daughter like Jenelle. It was so nice to meet someone who knows exactly what you are going through, and who has similar experience. Jenelle and their daughter are two months apart (their daughter being older) and literally have identical symptoms. I say they are long lost twins because they look identical as well!

Overall things are stable and we are seeing lots of improvement. Thank you for keeping us in your prayers. We appreciate the support!

Seizures and Earthquakes

I'm sure this analogy has been made before, but living with a child with Epilepsy is like living in Southern California and waiting for the next earthquake. Even the EEG monitor and Earthquake Seismograph make similar "wavy" motions when the event happens. As a California native, I've learned that you can't live your life being afraid for the next earthquake anymore than you can live in fear of the next seizure. Still, the feelings and anticipation are very similar for me. We had an earthquake last week that was centered somewhere off the coast near San Diego. I felt it from my 13th floor office in Newport Beach, and believe me is was a fun ride as we swayed back and forth and jiggled up and down. The earthquake itself was minor with no major damage, so it literally was fun… but it got me thinking about past earthquakes, and how scary they really can be. Is it going to be a mild 20 second seizures, or a 7 minute grand mal? Will we continue with what we were doing, or will our activity stop so we can call 911 and go to emergency? Like earthquakes, you just never know what to expect with a seizure.

This is just an update with the latest on the kids. I've been keeping some things out of email lately until issues were finally resolved, but our family has been on quite a roller coaster since the beginning of May. Upon our return from the hospital at UCLA for her testing in late April, we were asked to find a new daycare for Jack. I think he got to a crisis point where he really needed us to understand that he was afraid, and not getting enough attention. As I mentioned, he really didn't take our leaving for the hospital well, and cried hysterically like he'd never cried before. Later he was acting out by hitting and biting other kids. He was definitely trying to tell us something. Jack started a new daycare with less kids the following week. The change in daycare has been good for him and he is much more himself these days. You can't really blame it as Jack being spoiled or having unusual tantrums - he is going through more than most 3 year olds and handling it as well as can be expected. Overall, he's a great kid and is now having a good summer.

Shortly after Jack switched daycare, the Blind Children's Learning Center asked us to remove Jenelle from their "in center" program because they felt her seizures were "uncontrolled" and that she was "medically unstable" for in center therapy. We were devastated because we were not only forced to find new daycare on short notice, the act of removing her from therapy so abruptly because of her seizure status was a harsh reminder of the new "reality" we must face having a daughter with Epilepsy. Jenelle seizure status was really no different than it was before we went to UCLA, but upon hearing about her 7 minute grand mal and her new Diastat prescription, the school was concerned. We asked the Epilepsy Foundation and Regional Center to work with us and the school and eventually worked out the issues with a couple of meetings and an educational seminar on seizures provided by the Epilepsy Foundation. Jenelle started back to the "in center" Infant Development Program today and we are grateful that she is back to the wonderful therapy that was benefiting her so much. During this time, we kept Jenelle at home using our respite nursing and/or using various babysitters (Grandma and Aunt Nicki). Her therapy continued (PT and OT in the home) but it was not the same amount of hours as before and we definitely saw the difference, as did Dr. Shields. Regional Center really came through for us and now Jenelle will be getting 20 hours of in home nursing daycare, so we will have a nurse staying with her at home. At this time we decided to separate Jack & Jenelle in the daycare setting to make Jack's environment more stable, but now that Jenelle is back at school, and with their summer schedule, she will start in the afternoons at his daycare next week.

Some have told me that I probably shouldn't have told Jenelle's school so much about her seizures and her new diagnosis, but I feel I would rather have people know everything (even the scary stuff) for Jenelle's safety and benefit. It would not be in Jenelle's future interest if we did not advocate for Epilepsy and start educating the public now so they can understand her issues and accept them. I realize some parents feel they need to be private about their child's seizures, but I feel we are doing more for Jenelle by bringing them out in the open than if we were to keep them private. So as you can see, the past two months have been a real ride of ups and downs as we've been scrambling for daycare and managing to get through these difficulties day by day. We seem to have survived.

Jenelle is relatively stable at this time. She's been on the max dose of Topamax for a week or more now, and is fighting constipation and is still having daily mild seizures. She usually has a grand mal every two or three weeks, but all this is pretty "stable" for us… meaning at least we aren't in the hospital. She is really responding well vocally, and her vocabulary includes "ah boo", "ah pffft", and "uh oh". Jack has affectionately nick named her "Boo" and she is starting to acknowledge that name as well as her own. I'm trying to slip in "mum" these days and she seems to be hearing me. She is also holding up her head well, and lifting up her body while on her tummy. Its wonderful to see her do so well, though we did have a set back with the decrease in therapy.

Jack is your typical 3 1/2 year old, and makes us laugh every day. He has his first crush on "Dora the Explorer", and fell in love with a "Dora" electric toothbrush I purchased a few weeks ago. He literally told me, "Mommy, I LOVE her. I LOVE her Mommy!" While the toothbrush was an easy purchase, it was a little more difficult to explain why he couldn't have the "pink" Dora tennis shoes! Jack also has a new love for "pockets" and likes for us to give him coins to put in his "pockets". This has been an excellent tool for teaching him the names of coins, such as quarter, dime, nickel, etc. However, Brett was a little confused the other day when Jack ran to him saying, "Daddy, I want my money back!" We eventually learned that the money was "deposited" into Jack's tummy as Jack decided to swallow a quarter. I guess we'll be looking for the return on that deposit with "interest" in the next week or so. And finally, Jack had the clever idea to fool Mommy the other day into thinking he'd finished all of his dinner. I found his vegetables all hidden neatly under his upside down milk cup! Ah, this age is so fun. Jack really loves his "Baby Boo" and likes to help with her therapy. He is pretty much the only person that can get her to laugh and smile on command! Its wonderful to see!

So, that is our update for now. As I said, things are stable and moving along day by day. We're pretty sure that we'll be trying a new drug for Jenelle (Lamictal) at the end of the month since the Topamax hasn't stopped her seizures. Our next visit with Dr. Shields is in August.

I told everyone that we were seeing Dr. Shields today (Tuesday) when in actuality, our appointment was for the "7th" which was yesterday. I knew it was the 7th, I just thought the 7th was a Tuesday! LOL Don't worry, we didn't miss the appointment!

Brett and I took Jenelle to UCLA yesterday where we met with Dr. Shields and his resident Dr. Shariti to discuss the recent test results. There is not much new to report since we left UCLA in April - Jenelle is still not a surgical candidate. Her EEG was abnormal (obviously) with no focal point to her seizures, the MRI was "normal" and there were no changes from her MRI in July 2003 (which is really good news), and her PET scan found an abnormality regarding her glucose metabolism in the right frontal lobe, but the results were "too inconclusive" to warrant surgery. Basically, our little girl is one huge mystery! I'll discuss the PET results in detail a little later...

Dr. Shields feels that Jenelle is "transitioning" into Lennox Gastaut Syndrome (LGS), but as he says, "the writing is on the wall, but its not carved in stone yet!" When a child has Infantile Spasms that are symptomatic like Jenelle (i.e. the patient has an "underlying" neurological condition, and in Jenelle's case, her "underlying" condition is undiagnosed) they have a greater possibility of transitioning into LGS, which means their seizures will change their presentation. This is something we have seen in Jenelle. Basically, her seizures have changed from "spasm" like seizures to "myoclonic and tonic/clonic" seizures. This could be the LGS, or simply that her brain has grown, and thus her seizures have changed on us. For now, he is calling it LGS; her ultimate "prognosis" with that is yet to be seen and/or determined. Basically, we were told to expect to deal with her seizures for the rest of her life - and honestly, this wasn't a huge surprise.

Dr. Shields feels we have a "few" more drugs to try before attempting the Ketogenic Diet or a VNS implant. He feels that the Vigabatrin has helped control her Infantile Spasms, and wants to continue with this drug a little longer. He also wants to increase her Topomax a third time to the max dosage to give it a full trial before moving onto another drug called Lamictal. We are slowly increasing Topomax now and will give it another month on the max dose. The Klonopin will stay the same as we don't want to make too many changes at once. He was honest with us and said he felt the other drugs we have yet to try may only have a 10% to 15% chance of successfully stopping her seizures. We asked for more details about the LGS prognosis since we've seen such varying information, and he admitted it was quite grim in that most LGS patients are severely mentally retarded and usually wheelchair bound, but in his experience, very few die. In fact, in his 20+ years of experience, he said he has had 2,000 patients with LGS, and only 2 have died. That is a much better statistic than those we've previously seen. A "shorter" life is still a possibility, and we accept that possibility.

He was very concerned with Jenelle's therapy, and felt our main goal at this time was to provide as much therapy and stimulation as possible so that we can help Jenelle reach her full developmental potential. He felt strongly that in a year or two as her brain grows, we may determine a focal point in the brain to her seizures that can be corrected surgically. It is very important that she progress developmentally prior to any surgery as there is always a risk of losing skills after surgery. The more development, the better the outlook after surgery.

About the PET scan results. We had received a copy of Jenelle's medical records prior to our visit yesterday, so we already knew about the focal point in the Right Frontal Lobe. This was concerning because Jenelle consistently favors and uses her left side of her body better than her right side. We assumed the problem was in the left side of her brain. Usually with a problem in the right side of her brain, it would be more likely that she would have problems using the left side of her body - not that it would be her "best" side. I asked Dr. Shields about this result, and he agreed it was very strange. Because the PET scan results and her physical presentation are confusing and/or conflicting, this is a reason not to support surgery at this time. It is quite possible that if the brain damage is to her right frontal lobe, it happened immediately during her development, and her brain has since taught itself to compensate and make different connections in order for her to use her left side so well. Frankly, they cannot explain the results, but it is something we can watch in the coming years as her brain grows. We are scheduled to see Dr. Shields again in August.

The goal at this point is to help Jenelle progress in her development as much as possible. We realize that seizures are just going to have to be a part of our lives - as much as we may hate them. We hope to find a medication that will provide some amount of seizure control that will help provide Jenelle the most development at this time. I'll continue to update when I have something new - thank you all for your continued prayers and support!

Just a quick update to let you know how Jenelle is doing. So far, we haven't seen a grand mal since UCLA… then again, the teething has died down a bit, so we're positive that played a part in bringing on the grand mal in the first place. Friday, Jenelle had a follow up visit with her Ophthalmologist Dr. Ching. According to Dr. Ching, Jenelle's eyes and vision are "normal" and she is far sighted. We talked about her failure to track objects and reach for or hold items in front of her. He finally gave her an official diagnosis of "Cortical Blindness". Basically, Cortical Blindness, often referred to as CVI (cortical visual impairment), means that the brain is not telling the eyes how to respond properly to what it sees. With a "normal" brain, Jenelle would properly track things and would perhaps reach for toys and/or other objects. Dr. Ching suggested we talk with Jenelle's Neurologist about how to work with this, and I've heard from parents that some vision therapy may be necessary.

Home from UCLA

We are home from UCLA and our trip was shorter than anticipated. The short story is, they think that Jenelle is not going to be a candidate for surgery. While heartbreaking, we knew this was a strong possibility. We were very impressed with UCLA and their "team" approach. We were admitted on Wednesday morning and Jenelle immediately gave them quite a performance. She started seizing about 2 minutes after they applied the EEG monitor. She had many seizures throughout the course of the day.

Wednesday afternoon we were visited by Dr. Shields and his nurse Sue Yudovin. It was wonderful to see them, and he check out Jenelle and commented that her tone was much improved since he'd last seen her 5 weeks ago. We told them we were already recording seizures and he thought that was great. They told us they would know quicker if surgery wasn't an option, than they would if it were. Later that day, we were visited by the "Team" which included about 8 to 10 med students, doctors, residents, attending, etc. Just like on TV, they swarmed over Jenelle, asked questions and made observations. I just stood in a corner in awe. They told us they already had a lot of seizure information on the EEG, but would continue through the night to see what they could get.

At 3:30 a.m. on Thursday, Jenelle had a grand mal that lasted approximately 7 minutes. This is the first one she has had for over 5 weeks and since starting Klonopin. It was also the longest one she's ever had. We had to use oxygen because her lips were turning blue- if we'd been at home, I'm sure we would have called 911. She seized for another hour after the grand mal and if they weren't recording the information, we'd probably would have medicated her with emergency meds (valium) to stop it. Oh, and all of this seizure activity was happening while Jenelle was still on her full meds. They were only intending to stop her "cold turkey" if she wasn't giving them anything, so like I said, she really performed well. Many other parents we know often spend days in the hospital and never record a single seizure on an EEG. We not only got her regular ones, but a grand mal as well! If anything, I think prayers were answered in that we have no doubt that UCLA got to see all of Jenelle's different seizures, so in essence, they gave us their opinion based on all the information, which in itself is a blessing. Or, as Brett says... "we need to tell everyone to stop praying for seizures because they over did it!" ;)

Overall they got a lot of information from the EEG. She is still having infantile spasms, myoclonics, tonic/clonics as well as EEG patterns that resemble seizures but are not (basically abnormal brain waves). She is not a candidate for surgery because there was no "focal point" to her seizures, and most likely at this point, she is moving into Lennox Gassteau Syndrome (LGS.) As some of you may recall, LGS is the more "devastating" type of Epilepsy, where most end up severely mentally retarded, or have a short life expectancy.
They ran the PET scan on Thursday, but not the MRI as we had a conflict in finding (or rather in keeping) the anestheologist. We have to go back up to do that as an outpatient in the next week or so, and we will return in a month to see Dr. Shields. Even though they are confident from the EEG that surgery is not an option, they want to do the entire work up to see what they can recommend. There are still different drugs we haven't tried, and of course, the Ketogenic diet may still be an option.

Jack took our leaving for "the hospital" very hard this time. When we left him at daycare on Wednesday, he cried hysterically. The night before I told him we had to take Jenelle to the hospital, and he asked, "where am I going to stay?" He's been temperamental, but overall is handling this well. We spent most of today spending special time with just him.

Brett and I are doing OK. We not sure what our next step will be as far as treatment, and we are somewhat disappointed. We truly appreciate all of your prayers and support during this time. You have no idea how much it means to us.

I'll update when we have some new results.

Sorry I haven't updated before now. We are set to go to UCLA for admission at 10:00 a.m. Wednesday April 28, 2004 for testing. Things have been hectic trying to make arrangements for this as well as Brett and I managing work. We will keep you all posted as soon as we have email access, or anything to report. Thank you for keeping us in your thoughts and prayers!