Friday, November 21, 2003

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.

Wednesday, November 19, 2003

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!

Tuesday, November 18, 2003

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.

Wednesday, November 05, 2003

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.