Belly Laughs

For the first time this weekend, we heard Matthew have some belly laughs. He would giggle before, but now we're LAUGHING. It's not all the time, but Matthew is starting to respond to sounds. I sneezed the other day and he started to laugh. I also belched on another occasion. He found this funny, too. We haven't quite unlocked the secret to get him to laugh, but at least we're getting some good, real belly laughs. He typically starts to giggle to himself and then we interact with him to get him to continue.

Happy Matt

This is something we haven't see in awhile!! He was responding to me when I was talking and saying his name.

Last week's Neuro Appt...

We went to Biloxi the other day, so I haven't been keeping up with Matthew's progress. So here we go.

Last Monday I took Matthew to see his previous Ped. Neuro here in Pensacola....You know, the one that was treating Matthew for Epilepsy, not Infantile Spasms? Because I'm so non confrontational, WE decided to give him ONE more chance. He's so nice. But, as some of my friends have said, "do you want someone nice or someone who gets the stuff done". He was glad that Matthew had started on the ACTH. He was glad that Matthew had an opportunity to go to Children's Hospital in Chicago. He even thanked me warmly for attending the appointment that day.

I had some questions on this medication. I mean, when a medication costs $70,000 + a month for treatment, you don't get it at Walgreen's---so I can't just ask the local pharmacist. Dr. Coker in Chicago explained to me that children sometimes need two rounds of treatment. Each treatment is 6 weeks. I asked Dr. W (Dr. out here) how and when will we know when he needs another round, if he does. Dr. W. warmly spoke to me with his Latin accent and said that he wasn't sure if we started right when the first round was done or if we wait a few days/weeks. RED FLAG #1.

I continued to ask questions without getting answers I understood. I discussed with him that Dr. Coker said that Matthew's blood pressure needs to be closely monitored. I asked if I could swing by once a week to have it taken by the nursing staff. He said we could work something out. I'm near his office twice a week for physical therapy, so it's not a big deal for me. Plus, it's my son, I'll do whatever I need to do for him. By the end of the appointment, Dr. W says, "So you'll follow up with your pediatrician so they can graph everything?" I had NO idea what he was talking about. I thought we said we would follow up there for blood pressure...and now what is this stuff about graphing? And what are we graphing? When I asked for clarification, he basically used his hand to show me what a mapped out graph looks like. Yah, I get that, but WHAT exactly are we graphing? Weight? BP? I don't know. RED FLAG #2.

I told Dr. W about Matthew still rolling his eyes. I know they are seizures and Dr. Coker explained that "normally" once on ACTH there are NO seizures. He basically told me, "He's better though". That's what he's been saying for the past 5 months!!

When I checked out after my appointment, I softly said to the receptionist, "Um, what do I need to do to switch doctors?" I explained I have a hard time understanding him....but that's only half. I just don't feel comfortable with his experience with ACTH. The lady took my name/number and said she'd call me.

5 days later, I still hadn't received a call. I decided to call the RN at the office....I left her a message and within 3 hours she called me back. I was fully anticipating that I would have to fly to Chicago and follow up with Dr. Coker there. She said she talked to Dr. R and I can see him Nov 10th.

My regular pediatrician had been coordinating with Dr. Coker's office in Chicago. They agreed to see Matthew as a walk-in for BP checks. Dr. Coker's office basically said all they need to do is BP and check the site of his injections to make sure they're not infected. That's it. That's the great mystery of the "Graph".

I'm going to see how it goes with Dr. R. He is the main doc at this practice, he's been there for about 20 years, so I feel good with him.

Taking the good with the bad

With this medication (ACTH) we can have several side effects. One of the effects can be sleep pattern changes. He is up until about 1 AM and sometime later. He's up at 6, maybe 7 if I'm lucky. He sometimes takes naps, but we usually have busy schedules....this has been this weeks:
*Monday~ drive 60 miles each way to get his helmet adjusted
*Tuesday~ physical therapy/dropping grammy Forni off at airport
*Wednesday~ I drove to four pharmacy's trying to get his medication & in home therapy
*Thursday~ physical therapy & lab draw at hospital
*Friday~ pediatrician appointment
Those are just Matthew related things...I worked in between that, unpacked, etc.
We went a week without a seizure. Yesterday we were instructed to decrease his Keppra (ONE of his anti-seizure med) from 2.0 mL to .5 mL. He has been having seizures since last night. It's really frustrating because I thought we were on the right track and then we have a set back like this. I am going back to his 2.0 mL and hopefully we'll be better. Oh and hopefully I'll get to go to sleep soon. But, to see Matthew improving, it's worth it.

General info on Infantile Spasms

What is it like?
Here's a typical story: "At first I thought Chris was just having the little body jerks when he was moved or startled, like my other children had when they were infants. But then I knew something was wrong. The jerks became more violent, and his tiny body was thrust forward and his arms flew apart. They only lasted a few seconds but started to occur in groups lasting a few minutes. It was so hard to see such a young baby having these things."
Infantile spasms (also called West syndrome because it was first described by Dr. William James West, in the 1840s) consist of a sudden jerk followed by stiffening. Often the arms are flung out as the knees are pulled up and the body bends forward ("jackknife seizures"). Less often, the head can be thrown back as the body and legs stiffen in a straight-out position, or movements can be more subtle and limited to the neck or other body parts. Each seizure lasts only a second or two but they usually occur close together in a series. Sometimes the spasms are mistaken for colic, but the cramps of colic do not occur in a series.
Infantile spasms are most common just after waking up and rarely occur during sleep.
Who gets it?
Infantile spasms begin between 3 and 12 months of age and usually stop by the age of 2 to 4 years. They are uncommon, affecting only one baby out of a few thousand. About 60% of the affected infants have some brain disorder or brain injury before the seizures begin, but the others have had no apparent injury and have been developing normally. There is no evidence that family history, the baby's sex, or factors such as immunizations are related to infantile spasms.
Tell me more
When a baby with infantile spasms has an EEG, the doctor usually will see an unusual pattern called hypsarrhythmia (HIP-sa-RITH-me-ah) when the seizures are not occurring. This chaotic, high-voltage pattern is often helpful in confirming the diagnosis.
Babies with infantile spasms seem to stop developing and may lose skills that they had already mastered, such as sitting, rolling over, or babbling.
How is it treated?
Steroid therapy (adrenocorticotropic hormone [ACTH] or prednisone) is the primary treatment for infantile spasms. Some experts recommend trying a seizure medicine such as Sabril (vigabatrin, not available in the United States), Depakote (valproate), or Topamax (topiramate) before steroid therapy. In countries where it is available, Sabril is often used as the initial therapy because it is relatively safe (especially for short-term use) and effective. It is especially effective for children with tuberous sclerosis (a disorder associated with abnormalities involving the brain, skin, heart, and other parts of the body). Sabril is associated with damage to the retina of the eye and should be used with caution in children.
What's the outlook?
Most children with infantile spasms are mentally retarded later in life. Those whose spasms are related to an underlying developmental brain disorder or injury have a higher likelihood of moderate to severe retardation. The outlook is brighter for those who were developing normally before the spasms started: 10 to 20% will have normal mental function and some others may be only mildly impaired. Some children with infantile spasms develop autism. Many doctors believe that the quicker the seizures are controlled, the better the results will be.
When the spasms stop, many children later develop other kinds of epilepsy. About one-fifth of children who have had infantile spasms will have the Lennox-Gastaut syndrome.

Home Sweet Home

We survived our 20 hour back to Florida. There were only a few minor mishaps....oh, driving with the gas cap off-dangling from car. Oh, and there was the incident where I put Matthew's bananas on the roof of the car and we drove off. I have a nice banana blob on my rear window now.

We have not seen any seizures in almost a week now. We are coming upon new obstacles. From what I had heard, he would be "normal" in a few days. "Normal" is a subjective term. He is cooing A LOT! He is also awake A LOT. I have been up until 230 AM almost every night and then he's up at 6. They say the meds can do this. He's fussy, but he also popped a SECOND tooth in 5 days. We don't know if the tooth is what's causing the fussy or if the medication.

It's amazing having our little guy back, but we're also readjusting. He is left dominate, he does not use his right arm much. We are working on this in physical therapy. He has figured out how to put the back of his hand in his mouth. (left hand). For most 9 mo old, this is old habit, but it's new for him.

Being your child's advocate

The post below explains his catheter situation, but I learned something from it. My mother-in-law noticed that he was uncomfortable with his leg a certain way. We assumed it was due to the catheter and/or tape from it. Not thinking anything of it, we left it. A few hours later we realized how infected and sore it was.

Later that night, Matthew was still awake. It was past midnight..in the 1 o'clock hour. He hadn't slept much. But, who would with their catheter screwed up, right? At this point, his cath was out, but I'm sure he was still uncomfortable. Well, he had a seizure. I buzzed the RN and we were with him during the seizure. It was a "medium" seizure for Matthew. He had more severe ones earlier that day and the RN received an order for Adivan for that 1 seizure. Knowing this, the night RN talked to the doctor on the floor and got an order for Diazapam (AKA Valium). She kept saying, "this will help stop the seizure and help him sleep". I told her once he fell asleep, he'd be fine and he was probably going to fall asleep soon. She still brought the med in and started hooking him up to monitors. I started to get a feeling we shouldn't take this med. She put the monitor on that measures his heart and respiration.

It had been about a half hour by now, she was almost ready to give him the med. He was sound asleep and not seizing anymore. I said something like, "I don't think he needs that, can we hold off". I felt guilty because she spent a half hour setting up, getting the medication and even had to have another RN come in to help with the monitor. She said we could hang onto it that way if he has another seizure we would have it near by. Oh, and while she was talking about the med, she kept saying, "This will help him sleep". That didn't sit right. If we want something to help him sleep, I'd prefer a more gentle medication....like Benadryl.

Matthew slept the WHOLE night. In fact, he pretty much slept for 24 hours straight. He'd wake up occasionally, but fall back to sleep within an hour. His new ACTH med was/is kicking his butt. Obviously he didn't need an intense medication to help him sleep.

Go with your gut. Who cares if you upset someone.