After Jenelle's IFSP, we really started to notice Jenelle's seizures increasing, and her activity and development decreasing. It was then that I created my "own nightmare" between myself and Jenelle's Neurologist Dr. Phillips. Remember that love-hate relationship I was describing?... well, as Jenelle's seizures increased with the Pheno wean, I emailed him to see if we could increase her Vigabatrin. At the same time, I also emailed UCLA. Dr. Phillips told me not to increase the Vigabatrin but to increase the Pheno if the seizures were out of control. UCLA told me Jenelle could increase her Vigabatrin by another 1000 mg and that she was really only taking half the amount she could be taking. They suggested we keep up with the wean, and give the Vigabatrin a chance, but increase it slowly. So, I emailed Dr. Phillips with their advice... and of course after the fact I realize how that may have been insulting to him in that I double checked his advice. In return, Dr. Phillips sent me a "lengthy" email suggesting I make any changes in her drugs slowly, and that no seizures should be expected or accepted. We didn't email again until our next appointment, meanwhile I made the decision to increase the Vigabatrin - which in turn decreased the amount of seizures we were seeing. Well, I may have hurt his ego, but I think I made the right choice. It was just so annoying to see her seizures increase and know there was little we could do.

A few days later, our Jack (almost 3) woke one morning with a fever of 103. My first instinct was, "Oh God, please don't get Jenelle sick!" Poor Jack was home for a week with a very high fever. I felt OK and that I wasn't getting sick too until Friday, December 19 - ironically, the day of our next appointment with Dr. Phillips for Jenelle. When he asked how her seizures were doing, and I told him they were better, he smiled and said, "You increased the Vigabatrin didn't you?" That was all we said about that. He felt we should stay the course and once she was completely off the Phenobarbital, we'd give Vigabatrin another month to see if it helped. All three of us (Brett, Dr. Phillips and me) feel our next step will be the Ketogenic diet. That may begin as early as February. I told him that UCLA had a 4 month waiting list for the Keto diet, then Dr. Phillips informed us that he used to work with the best Keto team on the West Coast from Oakland Children's Hospital. The Oakland team was actually trained at Johns Hopkins in Baltimore - where the diet originated. Therefore, we may start sooner with Dr. Phillips here at CHOC. Our next appointment with Dr. Phillips is at the beginning of March.

Jenelle has been asked to participate in the NYU Epilepsy Center study on Infantile Spasms. They got my email from one of my internet support groups and asked if we'd be willing to send her records. The study is not a drug study, and only requires a copy of all Jenelle's treatment records - therefore we won't be heading to New York anytime soon. They will be comparing the two drugs Jenelle has been using, and the overall quality of development on each drug. Let me tell you, I have a ton to say about ACTH - yuck!

And then, just as things couldn't get worse, Jenelle started to have a fever of 102 on December 23. Knowing that a high fever could trigger seizures, I emailed Dr. Phillips to tell him she may have the flu, and asked if we needed to look for anything. He called me back and told me to get her to the ER immediately, as the flu and her seizure disorder was potentially fatal or could lead to a permanent seizure state. Did I mention I was deathly ill with the flu? So, Brett took Jenelle to the ER, where she tested positive for the Influenza Virus that had been going around and was placed on Tamiflu. She hasn't had a fever since and it appears we caught it in time.

So, 2003 will not go out without a real fight, but in a way, I'm glad we're all getting the sickies over so we can start a fresh new year! We hope your holidays were great and that your New Year is fantastic!

We met with Regional Center today to discuss the plan for the next 6 months for Jenelle (her IFSP, individual family service plan.) The meeting included Jenelle's Occupational Therapist, her therapist's Department Head, our Regional Center caseworker and the head of the Early Intervention Program at Regional Center. It was great to have a meeting of the minds, especially minds that have so much knowledge about Jenelle's issues and needs. While I had mentioned I wanted to find an "infant development" program where Jenelle could be "in center" for therapy, they helped us realize it might be too big a step for Jenelle at this time. We are going to increase her therapy at a one on one level in the home, and may try to schedule an additional session that can be "in center" so we can see how she responds to that environment. It's tough because we need to set challenging goals, but at the same time we must take baby steps in order to reach them. If we feel Jenelle is ready for an "in center" program sooner than 6 months, we can re-visit her plan and make adjustments accordingly.

After the meeting this morning, I took Jenelle to the lab for more tests. Poor little baby had to give urine and blood again. She did very well and didn't even cry or scream out when they poked her arm for the blood. Not sure if that is good, or if it means she is just too used to all the poking and prodding! One test is going to be sent to Baylor University for reading, and we most likely will not have the results for another 6 to 8 weeks.

I'm starting to have a love-hate relationship with Jenelle's Neurologist, and I'm never going to send another update saying Jenelle hasn't had a seizure, because I definitely jinx it! Jenelle continues to have more seizures and remains on a lower dose of Pheno and the same Vigabatrin dosage. Brett and I think that the Vigabatrin is probably helping her spasms, but not the myoclonic type seizures that we are seeing.

The day before Thanksgiving just before 5:00p.m., Dr. Phillips called Brett to tell him some of Jenelle's metabolic tests were back and he wanted to run some new tests. We couldn't get to the office in time to pick up the lab work, so he said he'd just mail it. Of course, I didn't take the call and had tons more questions - which I knew I'd worry about over Thanksgiving! I spoke to Dr. Phillips today and he said that one of Jenelle's tests from he last hospital stay came back indicating an elevation in the level of some hormone. Apparently this was different from a test previously run a month earlier, so he wanted to re-test her. Fortunately, the test is only urine, but the results could take 8 weeks. When we talked, I explained that Jenelle was still having seizures, and he said to increase her Phenobarb. Because we want Jenelle off the Phenobarb so we can give the Vigabatrin a chance, I choose to ignore this instruction. Jenelle's seizures remain constant, and are not increasing, so we'll stay the course. We see Dr. Phillips again on December 19.

Jenelle had her ABR Hearing today to determine if she has any hearing loss, and to determine whether the connection from the sound to her brain is functioning. The results were normal for both ears.

Just prior to her sedation, Jenelle had a "new" seizure that I've never seen before. She was shaking, trembling, and it lasted for about 5 seconds - the longest seizure to date. It may have been the closest thing I've seen to a "grand mal" in her seizure behavior. Dr. Phillips was not overly concerned, but told us to keep an eye out for them. The good news is that Jenelle has been on a "lower" dose of Phenobarbital and a higher dose of Vigabatrin for a week and a half, and we haven't seen a seizure since Sunday. Not sure what the "new" seizure was, but we're glad we haven't seen anymore.

Jenelle had another EEG yesterday. While it was being done, I went behind the technician to look at the monitor. Most of the waves I saw were much better than before, and much less chaotic. I only saw a little bit though, but I was hopeful. I emailed Dr. Phillips today to remind him that her EEG was done, and I told him what I saw. He emailed me back and said I was right! Jenelle's EEG is somewhat improved, but still the same in some areas. The only "new" thing we've done since her last EEG is add Vigabatrin, so hopefully it is working somewhat. Great news... improved is definitely a step in the right direction!

Well, I jinxed it with our last update. Jenelle went 5 days without a seizure, then about 2 days after updating the diary, they came back. First they were few and far between, but then they returned with a vengeance and she is now having as many as 12 to 17 a day (that we can visually see… she could be having ones we don't see.) Dr. Phillips was on vacation last week and we couldn't contact him until our appointment yesterday. Prior to that appointment, I called UCLA to get their thoughts on whether or not the new drug was working, and they recommended we wean her slowly off Phenobarbital, because it is likely that it is working against the Vigabatrin.

We saw Dr. Phillips yesterday, and it wasn't the most uplifting appointment we've had. Dr. Phillips agreed to try to wean her off Phenobarbital, and the scheduled wean will take 6 weeks. We will slowly increase the Vigabatrin in the meantime to see if it makes a difference. He felt, however, that most likely the Vigabatrin isn't working at all. Although Jenelle's EEGs show the typical Infantile Spasm patterns, Jenelle has not acted like the "typical" Infantile Spasm patient, nor is she responding to the Infantile Spasm treatments. The majority of her seizures are described as "myoclonic" which means they look like jerks, startles or head drops. He felt that in addition to her Infantile Spasms, she has a "Myoclonic Seizure Disorder" (basically 2 different seizure disorders at the same time) so we need to start trying to treat that type of seizure, which may mean a new drug. He mentioned trying ACTH again and we thought maybe had her confused with a different patient, but he said that sometimes kids respond to a second treatment as they could vary the dosing differently to where it might be beneficial. We reminded him that she became catatonic on ACTH, and he said we should think about it, but wait a little longer to give the Vigabatrin more of a chance. He also said we may consider another drug called Topomax, and told me to get Dr. Freeman's book about the Ketogenic Diet to help us decide whether or not we want to go that route. The Ketogenic Diet is a very intense diet similar to Atkins, and is not recommended for children under a year, but it has had marvelous results in some Epilepsy patients.

So, we're pretty much doing all we can to find the right drug to stop her seizures. The ultimate goal is to stop them completely, not suppress them. UCLA told me that children are the most difficult Epilepsy patients in that once you find the right dosage and the right drug, they grow and then they throw it all out the window, thus starting the process again of finding the right drug. We're in for a long fight. Also, he didn't have the results in front of him, but he thought that all of her metabolic tests from the spinal tap we did in September were normal.

Two wees ago, Jenelle had some Physical Therapy and Occupational Therapy evaluations, and another initial evaluation for California Children's Services. Jenelle has some issues that could start to indicate Autism, specifically her tactical defensiveness and sensory issues. I asked Dr. Phillips if he was considering that for Jenelle, even as her "underlying" diagnosis, and he said no. He felt she had some Autistic lik tendencies, but it was still too early to diagnose, and it was too early to determine which of those Autistic like tendencies are not just related to the seizures. Fifty percent of kids with Infantile Spasms end up being Autistic, so its not something we've ruled out. Dr. Phillips feels that Jenelle has had something wrong all along, but we just can't diagnose it, which is a good thing, and then the seizures on top of that. He said the kids with the "unknown" diagnosis usually have the best outcome, so we should be happy that her's is undiagnosed.

Prior to our appointment with Dr. Phillips, Jenelle had Physical Therapy. I asked her therapist why she still wasn't sitting up. She said that Jenelle has the strength to sit up, but she has a problem with her "vestibular" system, which controls your equilibrium. Her brain does not compute what she is supposed to do with her muscles when she is placed in the sitting position. This can improve with therapy. Jenelle is having her ABR hearing test this Friday, which is a test that measures her brain's response to sound to see if the brain is computing what it hears. This test may give us some ideas as to why she is having the vestibular issues as well.

The great news is that everyone agrees that Jenelle is looking better than they've ever seen her. She is still improving on her motor skills, still trying desperately to crawl (she loves that swimming motion) and she is starting to chew her foods, so I'm introducing diced peaches and pears as well as cheerios into her diet. She is making eye contact and is more interactive and playful with us. The middle of the night waking have gone away for now (and now I've probably jinxed that too), but she still has difficult getting to sleep, which I would assume would be due to the Vigabatrin. She started back to daycare on November 12, and I went back to work.

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.