Every child with lissencephaly is at risk for every type of seizure out there and the vast majority are plagued with uncontrollable and unyielding seizure activity beginning at an incredibly young age. For Madeline, her battle with the dreaded Seizure Monster (the moniker bestowed upon intractable epilepsy in our circles) began at the ripe old age of 6 1/2 months. For the first few days of her seizure onset, her tiny, sweet baby brain was absolutely ravaged. When she was not sleeping, she was seizing. She spent 8 days in the hospital as our neurology team tried to find the best combination of medications to make Madeline stable enough to send home.
The Seizure Monster is always lurking. He exists in every abnormal EEG. Mads’ May EEG indicated she has both focal and generalized seizure activity. Unfortunately, this is par for the course for her brain malformation. For reference, here is Madeline’s brain:
Here is a “normal” brain for a 13 month old:
Madeline’s infantile spasm diagnosis was confirmed around 7 months of age when she was spasming over 100 times a day. This allowed the Seizure Monster to steal her skills and revert her back to essentially a newborn developmentally and cognitively. She no longer made eye contact. She no longer grasped toys. She no longer reached for anything. She no longer responded to voices or to touch. She did not smile, laugh, or coo.
Here she is at 6 months old prior to her first seizure, a happy, joyful, exuberant little girl:
Here she is at 7 months and 8 months, totally withdrawn from her surrounding; a hollow shell of her former self:
The Seizure Monster is a catastrophically dangerous thing. Luckily, a combination of steroids and Sabril allowed Madeline to go 371 days without a single seizure. Here she is at 20 months, a beautiful, vivacious, curious near-toddler:
Madeline was able to rebuild many neural pathways that were destroyed by the Seizure Monster’s early onslaught on her fragile brain. However, we learned, and are continuing to learn, that some of the early damage is still having a lasting effect on her. For example, Madeline’s speech is incredibly delayed. This is not uncommon for her condition. Yet, her uncontrollable infantile spasms did nothing to help this delay. We also learned recently through a hearing test that Madeline is hard of hearing. She cannot hear lower registries of sound. Obviously, this has a profound effect on speech. I have been dueling with Madeline’s birth-to-three provider for months now to get her speech services. I am literally having to make the choice between helping my daughter learn to walk or learn to talk. No parent should have to make this choice. The school district will not provide Madeline speech therapy services at this time because she lacks the attention span they deem necessary to make the sessions worthwhile. They did provide us one session this summer through a special grant program. The therapist encouraged us to mimic Mads, which hopefully, in turn, will encourage her to mimic us. She is quite good at mimicking actions. But, manipulation of words and sounds is incredibly difficult for her. “Dada,” though, she has down pat.
Another significant area of delay for our sweet girl is balance and coordination. I think this has more to do with her her underlying malformation than the Seizure Monster, but persistent seizure activity can cause dizziness and imbalance. Here’s a video of a little girl who has a seizure during therapy. Before the seizure is really over, she wants to get right back up and start playing again. In fact, she doesn’t want to be touched at all. She and Mads have this in common. :) But, you can notice how one seizure can dramatically impact one’s balance and coordination. Imagine having dozens or hundreds in a matter of days.
Madeline has been working really hard in her own therapies. In PT, we are trying to find the best combination of walker/gait trainer and new orthotics for M. She has outgrown her first set of orthotics and needs a new pair for ankle/foot support. The debate is do we get more restrictive custom-fit orthotics that go to the knee that lock her feet into a 90 degree angle? (These make crawling difficult, but walking easier.) Or, do we just increase the size of her current dAFOs because they have served us well? The million dollar question is, which ones do we get for her? Hopefully, we’ll make the decision soon.
Each day, I am simply amazed by how much progress this little warrior has made in her short life. It is astounding. And she has done it all with the best disposition and most positive outlook. It makes my heart burst with pride.