Friday, July 28, 2006

Video of Jenelle's movements

Thought I'd share a short video clip of Jenelle's "movements" that we showed to Dr. Shields. Realize that this is just a short clip, and that Jenelle does this all day, non-stop.

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The video clip was taken at Daddy's basketball game last weekend (hence the background noise) and is just a brief example of the constant wiggling and moving she does. This was in an open area - imagine how she hurts herself in a room full of furniture.

Wednesday, July 26, 2006

Disciplining Jenelle - Day One!

After my last update, I got a lot of email and comments from many who were concerned as to "how" we were going to be disciplining Jenelle. Many could not understand why it was necessary. Believe me, we realize she has no ability to communicate with us (which could be most of our problem) and that she is mentally retarded. If anyone knows Jenelle well - it would be us! Trust me, we are going to approach this delicately.

On the drive home from UCLA on Monday, Brett and I decided that when Jenelle started crying for no apparent reason (like she usually does in the evenings) that we would put her in her bed for a "time out" each time. This would provide a safe way to keep her by herself. When placing her in her bed, we would explain that she is getting a "time out" for crying (or throwing a tantrum or whatever fits.)

Tuesday night when I got home from work, Jack and Brett were playing a game, and there was no Jenelle to be found. I looked at Brett and said, "Where is Jenelle?" and he smiled and said, "In time out!" When I found her in her room, she was sitting quietly in her bed and looked up at me and smiled. I brought her back out to the living room and she was perfectly happy until we started eating dinner (a favorite time for her to cry out.) As she started crying, I made sure she was OK (checking her diaper, etc.) and then took her to her room for time out. I explained that she was getting a "time out" for throwing a tantrum. After 5 minutes, I retrieved her from the room, and the rest of the evening was fine.

So, the first day went well and to me, it sounds like she is getting the idea. And as cruel as it sounds (if she were a typical child) it has been less stress on us as well.

The saddest thing happened after I put her to bed Tuesday night. We went through our usual routine; dressing, setting up her IV pump, turn out the lights and turn on the music, etc. I was on the computer for a few minutes, intending to check in on her to see if she was asleep, and all of a sudden, I heard her crying. I ran into her room, and she was face down in bed just sobbing. I picked her up and couldn't figure out what was wrong, so I said, "Jenelle, you aren't in "time out" - it's night night time!"... And you know what, she looked at me and smiled a little. I gave her a hug and put her back to bed, reassuring her that is was time to go to sleep. I left again and 5 minutes later she was out!

I'm not sure why she was crying in bed, but it broke my heart a little to think that she could have thought she was in time out without needing it! Also, it makes me thing that she truly understands us more than we know. So, rewarding her good behavior is easy, but I think this "time out" thing will work as long as we talk her through it! I think she has the capability to understand and hopefully "time out" will soon be something we don't need.

Wednesday night was much better. There were no episodes of "crying", but Jenelle did move a lot. As I put her to bed, I reassured her that she had been a good girl this evening, and that it was time for sleep. I think she knows the difference.

Tuesday, July 25, 2006

Update from our trip to UCLA!

Yesterday we made the trip to UCLA to visit Dr. Shields and Dr. Martinez in the Genetics Department. I don’t know why, but I always look forward to and enjoy these trips. Walking on campus and driving through Westwood; it just makes you feel like a young college student again (and no, I did not go to UCLA.) I also feel like the smartest minds in the world are working on my child, and that they truly want to help us. It could have been a little cooler yesterday, but overall our trip was great.

We saw Dr. Shields first and that visit was mostly uneventful. He is very happy with her seizure control and said it is “better than he ever imagined we could achieve.” He was also impressed with her developmental progress and felt it was due to the maintained seizure control along with coming off Topamax. I think I tend to agree. As I’ve mentioned, I’ve been worried that Jenelle may be showing signs of having a movement disorder. In the last 4 to 6 months (and more so since coming off Topamax) Jenelle’s movements have been out of control. She hurts herself; she injures us and is difficult to control. I brought some video clips of Jenelle’s movements for Dr. Shields to view. He is convinced that her movements are 100% behavioral, and not movements that are consistent with a specific movement disorder, nor do they look like seizures.

While it is a relief to hear that, it doesn’t help us much. Dr. Shields said that if we want to stop the movements then we need to discipline her. “Reward her good behavior” and “discipline her bad behavior” he said. I jokingly responded, “Give her more time outs?” to which he replied, “Exactly!” While it may sound like I’m joking, in all seriousness, we need to find a way “discipline” Jenelle so that she can learn to control herself. Not an easy task for anyone with a typical 3 year old child, let alone one with special needs. There are medications we could use to “drug” Jenelle and keep her from moving so much, but Dr. Shields feels they would also inhibit her development, and that is the most crucial thing at this time. Instead, he recommends we increase her Tranxene to add one pill in the mornings. The only time Jenelle is somewhat controllable is just after waking while she is still drugged from her Tranxene (valium), so we’ll add one pill during the day and see how it works. If she is too tired, we may go back down to our original dose. Our next appointment with Dr. Shields is in February.

After spending time with Dr. Shields, we met with Dr. Martinez in Genetics. He reviewed the results from our last visit last January and confirmed that everything was within normal limits, including the test for typical Rhett Syndrome. We were surprised to learn that since our last visit with Dr. Martinez, a new test for a different “gene” has been discovered to be the cause for “a-typical” Rhett syndrome. Dr. Martinez wants to re-test Jenelle for a mutation of that specific gene that was recently discovered. After mentioning our test for Rhett last time, I received many emails asking “which gene” we were testing. So for those of you who pay attention to specifics – Jenelle was previously tested for a mutation in the MECP2 gene; and her results were normal. This new test will test for mutation in the STK9 gene, and the results will take 8 weeks. Of course, we’re waiting for approval from insurance for this test, so it may be a few months before we get an answer. Amazing how science is constantly changing these days – and imagine where we’d be if they hadn’t mapped that human genome! ;) Also according to Dr. Martinez, we have ruled out some other possible genetic causes including PKU (Phenylketonuria), SCAD (short chain acyl-coa Dehydrogenase Deficiency), mitochondrial disease and Praeder Willi Syndrome.

Jenelle has been doing well with the exception of some increased seizures over the weekend due to the heat (110 degrees on Saturday!) She demonstrated a new skill this past weekend in that she put her lips around a straw. I had fluid in the straw and was putting lemonade in her mouth, and she enjoyed it. Hopefully she’ll learn to suck from a straw soon!

Thank you for the continued prayers – I’ll keep you posted!

Monday, July 24, 2006

My horoscope for today...

Capricorn: The last piece of the puzzle arrives; you can enjoy a new completeness to your life.

I find this very interesting... Seeing as we are driving to UCLA today to see Dr. Shields and to talk with the Genetics department. Hmmm.

Friday, July 21, 2006

Big Brother Jack has learned new skills!

Last Sunday we were busy painting the ceiling in the kitchen (notice the cover on the counters in back of the photo) and it came time to feed Jenelle. I told Brett it was time to teach Jack how to feed Jenelle and he agreed. Jack was very excited about it as well. See Jack holding the syringe (bolus) for Jenelle's g-tube feeding? Jack was so proud of himself and only spilled once when Jenelle accidentally hit the syringe (which is something she does frequently.) He even learned how to "clamp off" the tube!

Not sure why he felt it necessary to wear his swim goggles. Sometimes he like to wear them and pretend he is Spartacus (a cartoon character on Lazytown)! Someday soon, Jack will be big enough and strong enough to lift and carry Jenelle as well. And someday, only Jack and Brett will be the only ones able to lift Jenelle. My back is already not dealing well with her 30 pounds.

What a different life Jack will have, huh?

Monday, July 17, 2006

Just a little laugh...

Yesterday, Jenelle woke at 5:30... Laughing her butt off! At first, I heard it on the monitor - her sweet giggle. Then belly laughs and hearty laughs. So, I got up to check on her - I thought maybe the cat had snuck into her room or something. She was rolling around in her bed by herself- simply laughing to no end.

Makes me wonder if she was dreaming? Of if an Angel was tickling her toes? Who knows, but after I left, she kept up the giggles for 10 more minutes, then slept in until 8:30! That's my girl!

Thursday, July 06, 2006

Jenelle's "post" 4th of July update!

Just a little post 4th of July update on our girl. We had a wonderful, long 4th of July weekend as Jenelle was out of school until today. We took the opportunity to get out of town and go to the Curran Cabin at Lake Arrowhead. Lake Arrowhead had a boat parade on the 2nd and their 4th of July fireworks the night before on the 3rd. The weather was beautiful, however, Jenelle did not seem to do well in the higher altitude. We're not really sure why she was acting so "differently" and can only assume it was the new environment as she does not seem to be getting sick. Her unusual behavior included some noticeable seizures, and a very, very quiet demeanor. Normally, Jenelle is constantly moving and cannot be kept still. However, during our time in Lake Arrowhead, she didn't wiggle, giggle or kick like she usually does - which did provide a bit of respite for us, but had us concerned as well. She took 3 naps in one day, and seemed to cry a lot in the evenings - most of which calmed down after a bit of Tylenol. She enjoyed looking at the lake and playing at the park, and didn't mind the fireworks one bit!

We were home on the actual holiday and Jenelle was the center of attention with our neighbors at our neighborhood block party. Many have noticed her improved tone and many commented that she simply looks like a little girl these days. Although the firework show was cut short by the Orange County Fire Authority (fireworks are illegal in Tustin) Jack and Jenelle both enjoyed the firework show in the middle of the street and Jack told me that "I make the best water balloons" as we enjoyed playing in the water balloon war with the other neighborhood kids.

I took Jenelle to school this morning, and she seems to be back to her usual wiggly self. We are looking forward to our next appointment with Dr. Shields on the 24th as we have lots to discuss.
Here is a photo of Jenelle "sitting pretty" at the Lake Arrowhead Village playground, with the lake in the background.

And here is our girl with "fireworks" in her hair!

We hope you had a safe and happy 4th of July. I'll keep you posted!