March 27, 2013, was the very first time Madeline was administered a seizure medication. Within five minutes of being hooked up to an overnight EEG, the neurologist gave the order to start Keppra right away. Our six-month-old daughter literally had seizure activity off the charts. Statistically, if a person with seizures responds well to the first anti-seizure medication they are given, they have a 50% chance of their seizures being completely controlled in their lifetime. If it takes 2 seizure medications, then the percentage drops to a 10% chance of having complete control of their seizures. If it takes 3 medications, then the percentage of complete control drops to 3%. It would seem logical that more medications would equal more control. Sadly, that is not the nature of the condition. My sweet Madeline has been on 5 different seizure medications (Keppra, Zonegran, ACTH, phenobarbital, and Sabril) in her short life, which basically renders her chances of complete control to ZERO. If you count her “rescue medication” of Diastat (a medication that we must administer rectally if Madeline ever has a seizure lasting more than 3 minutes or has 3 back-to-back clusters, or if she ever stops breathing), she has been on 6 different seizure medications. Unfortunately, there is a finite number of seizure medications out there. I believe our neurologist said there are around 30 different anti-seizure medications. Though, some are not suitable for children; some have adverse reactions with other types of medication; some only work for specific types of seizures; and ALMOST ALL OF THEM come with at least one (if not several) undesirable side effect(s). This is our life.
Towards the end of May 2014, we received the results of Madeline’s 6th EEG. Yep, 6! I have pictures from them all.
We learned that the hypsarrhythmia is totally gone. This is a great thing because the hypsarrhythmia causes infantile spasms—the horrible, nasty, dreaded seizures that cause developmental regression and permanent brain damage. It is actually clinically described as being “catastrophic childhood epilepsy” and it is gone! Yes, it can come back, but we will deal with that if and when we have to. For now, the horrendous hyps monster is gone! However, no good news comes without a tinge of bad news. Madeline has two types of seizure activity on her EEG—focal (also known as “partial’”) and generalized. In the simplest of terms, focal or partial seizures can be a simple jerk of an arm or leg, lip smacking, etc. It’s a seizure that happens in just one part of her brain. Generalized seizures happen all over the brain and the two most common forms are an “absence” seizure (which Madeline has had) where she stares out into space for a while (she actually loses consciousness during these seizures) and the grand-mal all over body convulsion. When the neurologist gave us this news, I was devastated. I had not been saddened by Madeline’s condition in many, many months. I felt absolutely helpless all over again. No matter how many medical interventions we provide; no matter how many services we engage; no matter how many therapies; no matter how many hopes and prayers, kisses and hugs, laughs and tears; Madeline’s brain was built differently; it communicates differently and it ALWAYS will. The abnormal activity will ALWAYS be there. It is a battle of controlling the seizures and not in eradicating them because there is no cure for epilepsy. There is no cure for lissencephaly. Her neuro explained the EEG “blips” like this: Yes, they do indicate seizure (or abnormal) activity; however, just because there is a fault line does not mean there is going to be an earthquake (i.e., seizure). Madeline is still 11 1/2 months seizure free and going strong!
We started Keppra immediately after leaving our meeting with our neurologist. We decided on this medication because it is typically a frontline treatment for focal and generalized seizures and it has minimal impact with other medications and the organs in the body. Little did we know at the time that Madeline was in the 3-5% of children who would have “an absolutely rotten behavioral reaction” to the medication. Our foray into Keppra and the accompanying “Keppra Rage” was short-lived and we did a fast-paced wean after being on the medication for 11 days. In short, Madeline tried to repeatedly ram her head through the floor in frustration. The Keppra zapped all of the B12 vitamins in her body. Adding a B vitamin supplement to her diet only caused the Keppra to zap her vitamins stores even faster. She is now back on Zonegran. It has caused her to be sleepy and has diminished her appetite. Yet, these side effects are much favorable to a forehead pockmarked in bruises and a screaming, red-faced baby.
Even though, Mads has seizure activity on her EEG, she has still been progressing in her therapies. We are so excited to get her fitted for a gait trainer this summer! Her PT has picked out the Rifton Pacer Gait Trainer. We start her private PT (because the school year is out) the first week of July. I cannot wait to see Mads in action!
Her OT is still going really well. Lots of swinging, hand-over-hand desensitization, and working on going from standing to kneeling and back again.
I have been trying to make a concerted effort to gauge how receptive Madeline is to language. So what if she has never said a true word in her life and she cannot verbalize that she’s hungry/thirsty/tired, etc. I believe, and have been collecting evidence, that she understands language. For example, every night, after dinner I look at her and proclaim, “Bathtime!” and she gets very excited and starts squirming her way up towards the stairs, where she knows her bathtub is. Another example, look at this video, Madeline is banging a hairbrush on the t.v. stand (she loves making noise—all sorts of noise all of the time). I correct her when she bangs the brush on the t.v. She stops doing it. Then, later, as she wants to bang the brush on the t.v., she looks in my direction to see if I’m watching her, then I tell her no again and she stops. This, to me, proves that there is so much intelligence and understanding in this little girl. This, to me, also explains the headbanging. She is receptive to language and the world she lives in, but she cannot reciprocate communication the way we do, which leads to frustration on her part.
Here’s another example of Madeline interacting with her environment. She is mimicking behavior she sees socially. Then, she starts to headbang at one point in the video, after being corrected, she tries to “backtalk” if you will. It pains me to see her fight so hard with expression in the traditional sense. On a positive note, how amazing is that belly laugh?!
I wish I had a magic wand and could undo all the hardships befallen my sweet Mads. I wish I could protect her from all future trials and obstacles. If I could, I would trade places with her in an instant. Though, I gotta tell ya, it takes one special super duper tough little cookie to smile through it all. It warms my heart to no end to call this little fighter “mine.”