Trip to UCLA for Jenelle

Today was our 6 month check up with Dr. Shields at UCLA for Jenelle. It was a good visit. We spoke with Dr. Shields about Jenelle's new seizures of late, and he feels they may be do to yet another growth spurt. Jenelle has gained 3 pounds, and 2 inches since he saw her last September. Dr. Shields decided to increase Jenelle's Felbatol as we have a lot of room left for an increase of that drug. Brett and I agreed with that since Jenelle has done so beautifully on Felbatol for the last 3 years or so. If after the increase, we continue to see the same seizures, we'll let Dr. Shields know and we'll go from there.

Dr. Shields was thrilled to hear about Jenelle's first word ("Mum") and agreed that we were going to see some differences in Jenelle as she reacts to what's been going on with my cancer treatment. He feels my absence in December definitely was a part in getting Jenelle to verbalize the word "Mum". Dr. Shields wants to check Jenelle's blood work again to make sure her levels are OK, and will see us again in 6 months.

As for me and the start of Round 3, no word yet on whether or not next week is approved. I spoke to UCLA this morning, and they asked me to contact my HMO to help get the authorization approved. I made a couple of phone calls, but still no word. Brett and I were thinking if I can't get approved for Monday, we may have to go ahead with a hospital admission for me to get the chemo, in order to keep everything on schedule for me to be out of the hospital for Jack's First Communion. Isn't it weird how insurance companies work? It's easier to get admitted, clearly something more expensive, than it is to authorize an outpatient procedure. It boggles the mind. In the mean time, we are working on a driving schedule to get me to UCLA each day next week in hopes that everything is approved.

The fundraiser on Sunday was very successful and many thanks goes out to my good friend Erin for all her hard work in planning the event. Brett and I attended the event and it was good to see so many of our friends and family. In all honesty, it was a little overwhelming at times. We are so very fortunate to have such a wonderful support group around us. Thanks again to everyone involved.

That is all for now. I will update when I know more. Thanks again for the continued thoughts and prayers. I will keep you posted.

Routine update from trip to UCLA

As mentioned below, earlier this week we passed the 5 year "anniversary" of the day we got the news that Jenelle had epilepsy. I wish I could have told myself back then, that today we would be seizure free, as that might have made the journey a little easier. Today Jenelle had a follow up visit with Dr. Shields at UCLA, and when they asked the date of her last seizure, I really had to think long and hard about it. Jenelle's seizure control has been exceptional, and the last time I saw her have one was maybe a month or more ago. (And I'm knocking on wood as I type that, so you should knock on wood as you read it!)

After celebrating Jenelle's new achievements, and talking UCLA football with Brett, Dr. Shields discussed some of the new behavior issues we are seeing in Jenelle. Specifically, pulling out her hair, the biting, the crying and some small signs of possible early onset of puberty. The behavior stuff is easy to fix, and we are increasing her Risperdol to match her weight increase (Jenelle is now a whooping 48 pounds!) Jenelle ran out of Risperdol a few days ago, so I didn't re-fill it as I was waiting for this appointment. I honestly didn't think it was helping until last night I found a note in her backpack from her teacher asking if we had changed any medicines. Her teacher noted that she did not see the "self control" she had seen previously in Jenelle. That advice is enough for me to agree to give it a longer try.

As for the puberty issue, we have been told that children on seizure meds can often have an early onset, anywhere between age 6 to age 9. Jenelle will be 6 in October, so Dr. Shields thinks a referral to an endocrinologist is a good idea. We aren't anywhere near discussing something like "The Ashley Treatment" (where you can surgically remove female organs to avoid puberty), and Dr. Shields said there are some advantages to consider with early onset. He told us that research has shown for every year you avoid puberty, you guarantee at least 2 inches of growth in height. So if Jenelle were to enter puberty early, it might help keep her small. Again, we are no where near making any decisions about that, but we agree it would be best to start watching it closely now.

Everything else is going great and we will go back to see Dr. Shields in March of next year. Last week, Jenelle started school in the same class as last year with the same teacher. She seems to be happier back on her normal schedule. As for an update on the molars, one has finally cut through and another is giving us some problems. Now that we know what to look for, we've been helping her chew to get them to cut easier. So far, she seems to be taking it all in stride.

Five years ago, we had no idea what the road ahead would be for our Jenelle. The same is true today, but with the love, support, prayers and positive thoughts from all of you we know we will make it through. Thank you so much for everything.

As always, I'll keep you posted.

MRI Results

I got an e-mail yesterday from Dr. Shields' assistant about Jenelle's MRI results from April 16. Not surprisingly, the results were "normal" (whatever that means!) His assistant said there were some "sinus issues" noted, but that it was nothing very serious. I've arranged for UCLA to send the films and the report to our metabolic doctor (Dr. Chang at CHOC) as he originally requested these tests be done. We should meet with Dr. Chang in the next month or two so we can discuss the MRI/MR Spectroscopy in detail.

Speaking of "normal" - How's that for timing with Blogging Against Disablism Day? ;)

Jenelle continues to be battling an increase in seizures recently. It's hard to watch this happen as the seizures leave her so very tired. Last Sunday, Jenelle actually took a nap in the middle of the day (after having a rather long seizure that morning) which is very unusual as she rarely naps. All the usual suspects like illness and teeth have been ruled out. Dr. Shields increased her Felbatol a week ago, and since doing that the seizures has decreased a bit, but we're still seeing more than usual. He and I will speak again next week, and determine where to go from there. Please keep her in your thoughts that they don't get too out of hand until we can get control again of these nasty seizures.

Thanks again for the continued thoughts and prayers. I'll keep you posted.

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.

Brett just spoke with the medical director of Jenelle's insurance medical group, and all of the authorizations for UCLA have been approved! We are scheduled to go to UCLA on Wednesday, April 28, 2004 for her MRI, Video EEG and PET scan. According to UCLA, it will take about 3 months till they do the surgery if they determine she is a candidate. We estimate we'll be at UCLA for 4 or 5 days, depending on how long the tests take. Jenelle must be admitted because they plan to take her off her seizure meds "cold turkey" in order to induce seizures. She needs to be monitored so she doesn't go into status.

Please keep us in your prayers during that time. Thank you so much for your support!

We met with Dr. Shields at UCLA yesterday to update him on Jenelle. He is going have us come back up in the next two weeks to run some more tests. He wants his own 24 hour Video EEG, another MRI and a PET scan, which is a type of scan used to show activity in the brain - Jenelle hasn't had one yet. We brought him up to date on Jenelle's seizures and her developmental status since our last visit in September, and he really zoned in on the fact that Jenelle favors her left side (something she has done since she was only days old.) He said that is probably a very big clue that there is probably something wrong with the left hemisphere of her brain.

He mentioned that surgery to remove part or half of her brain is now an option, and one that has a very short window of opportunity. They prefer to do this surgery between ages 18 months and 2 years of age so there is a better developmental outcome. I reminded him that last time we saw him, he said surgery wasn't an option, and he agreed that was true for Infantile Spasms, but says that she has probably grown past that now. He said that UCLA is the leader in the country right now for performing a new surgery on patients with generalized seizures (seizures that come from all parts of the brain) and that they have been very successful. He wants to run the tests first to determine if she is a good candidate, and also said we still can't rule out the Ketogenic Diet or a Vagal Nerve Stimulator (VNS) which is like a pace maker for the brain - a device that short circuits the seizures before they happen. The problem with the Diet is that UCLA has a very, very long waiting list, and he also agrees somewhat with Dr. Phillips in that she may not be metabolically stable to start it. He said we would probably never be able to pinpoint an exact metabolic problem with Jenelle. He thought she was pretty delayed developmentally, but that most likely her abnormal EEG (the Hyppsarythmia pattern) is the cause of her delays. Once we can give her some normal brain waves, she may start to progress. Continuing on her drugs may also be an option.

Seven months ago, when we were first introduced to seizures and Jenelle's diagnosis, I read all about surgical solutions I wanted to vomit. I couldn't imagine making such a decision as to agree to let doctors remove half your child's brain. However, now that we're on our 5th drug and all the difficulties we've encountered in the past few months, I realize it may be her best option if it looks like something that she could benefit from. He said before making that decision, he would have us meet with families that have done the surgery, as well as other counselors that will help us make that decision. I got a message last night from a parent that had the surgery done for her child, and she say it is "frightening but awesome" at the same time. This Mother told me that upon waking from surgery, it was very apparent that her child was already doing better and controlling both sides of the body very well. In fact, the side of the brain they removed had not been working, so the "good" side of his brain had already started to compensate and was already controlling the other side of the body. They said the PET scan made their decision very obvious in that they knew the one side of the brain wasn't working. Her son is walking, talking and living a normal life. If we must choose surgery, I hope our tests are just as obvious.

So, for now we're just waiting to hear from UCLA as to when we need to come back for testing. He said their hospital was full right now with RSV patients, but it would most likely been in the next two weeks. We need to move quickly, since Jenelle is almost 17 months old and we are quickly approaching the "window of opportunity."

Jenelle started taking Vigabatrin the day she stopped the ACTH (10/28), and things are good! She is definitely "different", but its hard to explain how. The Vigabatrin comes in powder form, and we are to increase the dosage every three days until we don't see any more seizures. Last Thursday, her 4th day of Vigabatrin, and the first day of the increase dose, Jenelle developed a bumpy rash, so our Neurologist told us not to increase the dose until she could tolerate it better. She still has the rash, so he'd like us to keep her out of daycare a little longer to see if it subsides. Also, this will give her immune system a good 2 weeks to come back. She should be going back to daycare sometime next week - we hope, depending on the rash.

Now, the really good news. We haven't seen a seizure since Saturday night, November 1. I've seen a couple of "strange" facial expressions, similar to ones she used to make during seizures, but I'm not positive they are actual seizures. These facial expressions have been too slight and usually accompany her being touched or startled in some way. Dr. Phillips said if we aren't positive it was a seizure, then it probably wasn't.... which is good news! We need to schedule another EEG for next week, and then we will see him again on November 17. If I see another seizure, then we are instructed to increase her dose. She is on 500mg a day at this point.

Now, for the great news. Jenelle is much more alert and attentive since starting Vigabatrin. She has really made efforts to hold her head up, she is using her hands for support when on her tummy (something she's never done) and she is making excellent eye contact, and turns to voices or sounds. It's so hard to describe how she is "different", but it is definitely the "different" we were hoping to see, and we hope it continues and improves. It also appears that she is making efforts to crawl. Jenelle still can not sit up, so I didn't think she was trying to crawl, but her Physical Therapist said it was possible that she may crawl before she sits up. Jack sat up before he crawled, so I didn't think a baby could crawl first. Any improvement would be wonderful, but she has a long way to go before she starts moving around the room.

One of the not so nice things about Vigabatrin is that Jenelle is having bouts of insomnia... much to our dismay. She is usually not falling asleep until midnight or 1:00 am, and often wakes up at 3:00 am to coo and make noises in her crib until 5:30 am! I guess the good side of this, if any, is that at least she isn't "alert and cranky" and we don't have a child who is crying non-stop at 3:00 am. Hopefully, this too shall pass. As for her "one year" progress report... Jenelle is now 25 pounds, and 29 1/2 inches long. The day we started her treatment on September 29, she was only 22 pounds.

We traveled to UCLA to meet with Dr. W. Donald Shields who is in the Pediatric Neurology Department and is the head of UCLA's Epilepsy Program. While we were joking that we were there to check out UCLA as a possible college for Jenelle, we were very anxious to get some answers about her diagnosis of Infantile Spasms. Everyone at UCLA was absolutely wonderful, and we got a lot of straight-forward answers, as well as some choices to consider. Dr. Shields did not "sugar-coat" any of the information and spent lots of time talking with us. We also met his nurse Sue, and she spent a great deal of time with us answering lots of questions. She was absolutely wonderful and so very understanding and supportive. What a great team they were.

First, the "hard" facts... Dr. Shields confirmed that Jenelle's diagnosis is Infantile Spasms. We were not really questioning the diagnosis, but he pointed out two of Jenelle's EEG patterns, and said that unmistakably, those were signs of IS. In a way, it is comforting to have such confirmation and know what we are fighting. Dr. Shields considered Jenelle's history, and felt that her global delays were fairly severe. Because of this, he felt Jenelle has a strong chance of being a part of that "bad" 50% of patients with IS... those with the "bad" prognosis as well. Basically, for Jenelle to be in the "good" 50%, we need to hope that her seizures were there from day one, and that we just didn't recognize them, then this would be the cause of her development delays, and once we stop her seizures, she will develop "normally." He also felt that Jenelle has a 30% or greater chance of being Autistic, and a 30% or greater chance of developing another form of Epilepsy around the age of two called Lennox Gastaut Syndrome (LGS), which is similar to Infantile Spasms, but the prognosis is even more severe. If her seizures had gone unnoticed or untreated, she would definitely develop LGS. So, those are the "hard" facts based on Jenelle's history, from the nation's leading expert in the field. Dr. Shields treats only patients with this diagnosis, so he knows the statistics well.

So, we now have to make a decision between two drugs. One drug is the ACTH steroid. ACTH is FDA approved (and covered by insurance), begins in the hospital, is an injection that must be given twice a day for 6 weeks, has serious, life threatening side effects (including decreased immune system) and has a 50% chance of working. Once treatment begins, we'll know within 2 weeks if it is working, and if it stops her seizures, she is done with the drug in 6 weeks and may never need it again, unless she has a relapse. The side effects are reversible. The other drug is called Vigabatrin (Sabril) which is not FDA approved and must be obtained in either Canada or Mexico. Vigabatrin comes in pill form, and does not have the severe side effects associated with ACTH (including the fact that it will not alter her immune system, and she does not need hospitalization.) Vigabatrin has been in use everywhere except the US for 10 years, and has not been approved by the FDA because there is a report that some patients (1 in 3) experience partial and permanent vision loss (most end up with tunnel vision). When describing this "side effect" of Vigabatrin, Dr. Shields said basically we're choosing between a "mentally retarded brain" for life, or "vision loss", which of the two, the vision loss is acceptable if the brain can function normally. If it works, Jenelle could remain on Vigabatrin for as little as 3 years, or the rest of her life.

Both drugs have about a 50 - 70% success rate, and often times, one drug does not work for a patient, but the other will. So, it is possible that we can choose one drug, but end up needing the other two weeks later. Dr. Shields says he also has patients using both drugs at the same time. It just depends on what works for the individual to stop the seizures - sometimes each person needs a different "mix" for it to work. Dr. Shields agreed with our neurologists (Dr. Phillips) plan for treatment for Jenelle, starting with a B6 treatment, and the low dose of ACTH, if we didn't want to use the Vigabatrin. Dr. Shields also recommended doing a spinal tap to rule out three other rare metabolic disorders that can only be detected in the spinal fluid. He also recommended that we would be more comfortable treating Jenelle at CHOC since it was closer to home, but offered that they were a phone call away if we needed anything in regards to the Vigabatrin, if we go that route.

We spoke to Jenelle's Neurologist, Dr. Phillips on the ride home and again the next day about our options. Dr. Phillips does not want to start with Vigabatrin, and will not provide a prescription for it- he is just not comfortable with the ethical issues concerning his license and we understand. His reasoning for this is that Vigabatrin does not have any long term studies so there is no evidence that Jenelle's seizures could not return at age 5, and so on. ACTH has been used for about 50 years, and there is evidence that if it stops the seizures, it does so permanently. He said if we could get the Vigabatrin, he would help us use it. The plan as it stands today is that Jenelle will be admitted to CHOC on Monday, September 29 for a spinal tap, a video EEG and a B6 Treatment. We need to choose which drug we plan to start by Monday.

Brett and I realize that there is no "wrong" choice here, in that both will help Jenelle, but there are many things to consider with both drugs. We spoke to Dr. Shield's nurse Sue a few times since our appoitnment and she is making arrangements to fax a prescription to Canada for us to get the Vigabatrin - in case that is our choice. It would be so much easier if it were as simple as "it is a tumor and it needs to be removed", but with the choice of two drugs, with completely different and complex things to consider involving each, we really have a tough decision to make this weekend.