Quick Update

Way to go UTES!

I had to get that in before Rob reads this.

It has been one week since we started the ACTH therapy so I thought I would let you know how it's been going so far. He does pretty well with getting the shots even though the medication is extremely thick and takes a while to inject it into his thigh.

Last Saturday (day 1) was pretty bad. His seizures were getting more frequent, more intense and lasting longer. As the week progressed he was still having more seizures but he also was extremely stiff. He would wake up in the morning with his arms fisted and straight up towards the ceiling. His head also took a lot of pressure in order to turn it to the middle and he would scream when he did therapy. Wednesday I increased his Baclofen (muscle relaxer) to the limit the doctor had allowed me and he has improved some. By Friday I think the seizures have improved some although he has started to get more angry. His blood pressure has remained low and I haven't seen any water retention :) He gets his blood checked in one more week to check for liver and kidney problems.

Update Finally!!!

I haven't forgotten about blogging but Charlie has been confusing me for the last couple weeks. He hasn't been progressing very well but nothing specific enough to put into print. His effort, connection, agitation, and stiffness varied day to day and even hour to hour. Something wasn't right but it was so variable I had a hard time describing it.

3 weeks ago Charlie started jerking around 10pm. This was consistent. He falls asleep at 8 and then would wake up sometime between 10 and 11 and starts jerking. I've written before about how this time we felt Charlie connected with us but now he was waking up and do funny movements. Typially he is throwing his arms up and to the side, his head to the left (opposite than the normal positioning) and pursing his lips for a second then relax back for a few seconds only to do the jerking again. At first he would do this 15-20 times then stop and fall back asleep. In between the jerks he would move like normal and his eyes even would follow movement. This pattern continued mostly every night so the Dr. I work for let me take the old EEG machine home to do an EEG. I didn't start until 10:00p so Britton wouldn't mess up the electrodes and ended up recording until 2a. I recorded a long time so I could make sure to get the jerking on record. It looked like he was having seizure ..... even for the 2-3 hours before he was jerking. SO the next morning I called the neurologist who had seen us in the hospital. After getting a little bit of the run around from the neurologists office we tried Phenobarbital from 17.5 to 20. We did this for a week and it didn't seem to help. He then started doing this jerking occasionally throughout the day, usually after he wakes up and his chin started twitching. We drew his blood and he was as high as he can go on Phenobarbitol medication without causing problems. Dr. Filloux worked Char in early Wednesday morning and he spent over an hour with us getting everything figured out. I showed him the seizures I had videoed and the EEG I had recorded. I figured he would say that Charlie was having breakthrough seizures and that we would try changing his medication. It was somewhat true...He believes that Charlie has Infantile Spasms. These usually occur in children under the age of 1 and are a type of seizure that typically happens upon awakening from sleep. They jerk and then relax several times. Read here or here for more info. The video below is what I showed the Dr.. I'm not sure how to cut the video so it's sort of a lot of the same thing.


I've read that there is a decline in the children's progression due to the infantile spasms. The therapists and I had been noticing this over the past couple weeks with Charlie. He had shown glimpses of better head control, purposeful movements and tolerance for therapy but all of this had either plateaued or declined recently.

The treatment the Doctor recommended was ACTH which is a hormone that increases the body's production of steroids. It usually stops the infantile spasms if the child is an infant but has a lesser chance the older they get. Dr. Filloux and I decided that we would try this more aggressive therapy despite the side-effects. If it doesn't work then we will try different seizure meds.
Things Dr. Filloux told me about ACTH
* It is an injection that I will need to give him daily for at least 6 weeks (probably more like 8-10) into a muscle.
* It costs $29,000 for a 30 day supply. Where do I sign up to sell hormones for that much profit? I'm so grateful we have insurance!
* We will know in about 2 weeks if it works or not. It is usually all or nothing. If it isn't working we will will try an increased dose once and if it still doesn't work we will taper off and try something else. If it does work then after the treatment the seizures shouldn't return.
* It has similar side-effect to steroids- excessive weight gain, water retention, “moon face” characteristics, Irritability, Anger. Sound fun yet?
* It can affect the kidneys and liver and mess with his blood sugars so he will be monitored every 2 weeks.
* It can increase his blood pressure so he will need to have to monitor it nightly.
* It decreases his immune system- This is what I am most nervous about because we are just about to start cold season. I was just starting to feel comfortable taking both boys with me everywhere. I guess we are back to staying home and limiting sick visitors.

Highlights from appointment with Dr. Such Neibar (rehab dr)
* Results from his hip X-ray show that he has mild hip dysplasia (they come out of socket) so she wants us to put him in his stander more often (3 times a day for up to 1 hr each) to erode the joints.
* Tight ankles and hands- keep stretching them every day and go back to using the leg and arm braces.
* Neck tortacollis- he still can move his head to the left but the fact that he prefers to have it to the right is making his right neck muscles stronger and shorter. We will start using a neck brace to keep his head mid-line and possibly botox his neck muscles in the future.

Britt trying out Char's new sensory therapy (Thanks Suz)
GOOD THIINGS I'VE NOTICED:
A month ago Charlie was discharged from speech therapy because she thought we could do everything she was doing at home. He seems to enjoy baby food. On a good day he can swallow ½ jar of sweet potatoes. We try to practice this everyday. He also likes to suck on pretzel sticks.

Improved vision: He is doing so great with his vision. He is now following moving objects more and more. There are no indications that he recognizes anything that he sees yet. Eyes still prefer to be to the right but now he will occasionally move them to the left. The right eye also still drifts to the right.