| ABOUT ME |
| I was born on July 17, 2005. Within hours of my birth, my Mommy and Daddy knew something was not quite right with me. I was sent to the nursery that first night for observation by the nurses. By the next morning, they came to tell Mommy and Daddy that I had what they suspected were seizures during the night. After reviewing my CT scan, the pediatrician stopped in to talk to us. She said that it looked as if I had a condition known as hemimegalencephaly (I can't pronounce it either!), commonly referred to as HME for short. She couldn't offer us a whole lot more information, but she was very kind to us all. Since the hospital was not equipped to deal with such a potential diagnosis, they sent me to the neonatal intensive care unit at the James Whitcomb Riley Children's Hospital in Indianapolis, Indiana. It was there that I spent the next 3 weeks of my life and where my diagnosis was confirmed. Specifically, I have left-HME. Hemimegalencephaly translates to "half big brain" in medical terms. My "big" half is the left half of my cerebral cortex. It is a problem of brain development. For whatever reason (some people at Walsh Labs are investigating this "reason" now), the left side of my cortex did not form properly. This is why I have the seizures. The epilepsy that results from HME is intractable in almost all cases. Sometimes it is intractable early on (like in my case); in fewer instances it may be controlled with medications for a while or even a few years. Because the left-side of my cortex is malformed, I have a lot of developmental delays and a hemiparesis of my right-side. I do move my right leg pretty well, but I struggle a bit more with moving my right arm, and especially my right hand and its fingers. With all the seizure activity that goes on in my brain, my brain activity never really reaches a normal baseline. Also, because I am on so many medications for the seizures, I and my brain in general are often pretty sedated. Both of these things make it very hard for the "good" parts of my cortex (i.e my right cortex) to function normally. That's where the hemispherectomy comes in. A hemispherectomy is the removal of one-half of the cerebral cortex. A hemispherectomy can be anatomical or functional or some combination of both. In the anatomical hemispherectomy, the surgeon actually removes that brain tissue of the affected side. In the functional hemispherectomy, the surgeon simply neurologically disconnects the bad side of the cortex from the good side so that is can no longer affect the good side or send signals to the rest of the body. The combinations of the two are variable and ever changing. The main purpose of the hemispherectomy, from a surgical standpoint, is seizure cessation. Seizures cessation is not always achieved for various reasons, but significant reductions in seizures and severity are nearly always achieved. The improvements that are often seen in development (motor, speech, cognitive) post-surgery are mostly a side-effect of seizure cessation or reduction. While developmental prognosis is not the driver for such a radical surgery, it can't be ignored by parents. The theory is that, because the infant and young child's brain is very plastic, a lot of what is lost with removal, or often simply due to the malformation of the HME side in of itself, can be picked up by the normal side (hopefully composed of normal functioning neurons and helper cells). Pretty COOL huh? My doctors at Hopkins think everything I currently do is coming from my good side. That is great! Not everything can transfer though. Vision does not transfer. For that reason children like me have what is called a visual field loss. For me, it is a right field loss. You see, each eye has a right and left field of vision controlled by the respective sides of the cerebral cortex. So, after surgery, I won't have vision in the right field of each eye (version 4 or 5 on the diagram). Sounds scary huh? Well, luckily, children learn to compensate well. Essentially, the only perceived loss should be in my right peripheral vision. That would mean that I will have to turn my head to my right to see anything on that side; it will be my blind side. Funny enough, my vision will likely improve overall from what it may be now. Because of my seizures and my malformed cortex, and even though we know that some visual signals are being received by the brain, I already have what the doctors call cortical vision impairment. This means that, even though my brain may be receiving visual signals, it can not properly interpret what it "sees" or do so consistently and accurately...at least not yet. So, while everyone thinks that I do "see" things, they aren't sure that I "know" what I see. They also are not sure about my left eye, as an exam revealed some physical abnormalities to it. Time will tell them more about that. The other things that are apparent after a hemispherectomy, are a "limp" and gait problem with the opposite leg (that would be right leg for me). That's right, hemispherectomy kids can walk with only half-a-brain! Some learn to do it faster than others for various reasons, but it seems all eventually learn to walk. The other thing that happens has to do with the opposite hand. While most hemispherectomy patients can move the opposite arm at the shoulder and elbow, there is much less gross and fine motor control in the hand of that side. They learn to do things one-handed in a two-handed world. My Mommy and Mamaw Marilyn got to see demonstrations of this first-hand at the Hemispherectomy reunion we attended in Baltimore in July 2006. We met several kids like me (with HME) there, as well as other hemispherectomy children. (You see, hemispherectomies are also performed for other brain problems that result in intractable epilepsy, such as Rasmussen's, cortical dysplasia, Sturge-Weber disease, and stroke). They were all amazing!!! All the older left-sided HME children we met were walking and TALKING post-surgery. That's pretty amazing since language usually comes from the left side of a person's brain. Cognitive abilities post-operatively run the full spectrum. For HME, they spectrum is typically a little shorter and not quite as good as for some of those other conditions. But, I am going to fight for everything I can and my Mommy and Daddy, family and friends will challenge me and provide lots and lots of stimulation to help my brain pick up and retain as much as possible. I think I will do better that anyone can anticipate. I certainly will try hard! I'm looking forward to my re-birthday. Thank you for learning more about me and my condition. |
| ABOUT ME |