Wednesday, July 30, 2008

One more down.

Emmi is loosing another electrode. It is "high." Not too high to turn it off, but getting there. Progressing. Inching up each time. It looks like she will eventually loose it. There is already a short circuit between two electrodes. Another one was high at the last appointment, and had to be shut off. And? The full array is not inserted on that side. Although, there has been some confusion about that so far. Apparently that information was not passed on from the last audiologist. We are having to go back to the surgery notes. But I know what the doctor told me. We all remember. You can only loose five. Five and we go back in for surgery. It will have to be removed. She will have to be re-implanted. The one good thing is that her current audiologist has a new way of handling short circuits. Instead of shutting both off, he turns only one off. Then he boost the power to the other one. This whole problem is apparently rare. Her audiologist did say we were lucky to have Med-El. With the other brands when this happens failure is rapid and complete. It either works or doesn't he explained to me. I am sure there is a more technical explanation for that. But that is what I got. The whole thing makes me sick to think about. Not the surgery so much. But that part where I will have to make her go a whole month without her beloved left CI. It is her dominant side.

Saturday, July 26, 2008

One way or another. Or somewhere between the two.

There is great debate. To sign or not to sign. And then, which method? ASL? SEE? Emmi's first doctor and audiology team pretty much threatened my life if I taught Emmi to sign. They insisted it would inhibit her language development if she relied on sign. It was ridiculous to assume I would not teach her any sign. There was six months between the time we found out she was profoundly deaf and her first surgery. I was supposed to not talk to my almost two year old for six months? Besides it went against everything I knew. Statistic after statistic linked early language development with children who were taught to sign. They brushed it off, claiming that was for children with normal hearing. I, being me, ignored them, taught Emmi some basics signs, and prayed she would not give me away during any of our appointments. After moving to Houston, the experience was different.

Her new team seemed indifferent to my decision. Emmi attended more private speech therapy and school therapy than most of the children they saw. I was pushing the oral skills. I was doing my job. They gave little thought to whether or not I signed with Emmi. After Emmi's verbal skills picked up, the signing dropped off a bit. I let it be her decision. The basics stuck around. I assumed there would be a time when Emmi remembered very little sign. I assumed wrong.

Emmi has always remained drawn to sign. When I thought at first that it would eventually loose its hold on her as she gained oral language, I soon realized there were large gaps when the implants where ineffective. Bathing. Swimming. Periods of ear infections and equipment malfunctions. Playing in the rain. While Emmi has started talking in full sentences, she has also had a peak in interest in sign again. I can only conclude that she has figured out the joy and benefit of communication, and she is anxious to explore all the different means.

Thursday, July 17, 2008

More explainations....

Many of you will have questions. I am open to answering what I can, so ask away. I will update with another history that will fill in more blanks. But for now I will answer the questions as they are asked....

The brain damage was caused by the metabolic disorder. Basically it goes like this. Protein is not broken down properly causing a buildup of something called methylmalonic acid. In layman's terms, it poisons her. During periods of illness or stress, it is worse. While her immune system functions normally, your body, in it's natural healing process, breaks down proteins in the muscles. Thereby making even a common cold dangerous. She tolerates illness much better now, but when she was younger a cold was deadly. It would set off a chain reaction that ended with her glucose levels falling into the 20's. That is near death. That is the point at which they assume the brain damage was caused. So far it seems to have effected the area that controls key reflexes. Emmi had no startled reflex as a baby. This translates into having none of the reflexes necessary for balance. This portion of the brain is responsible for all of your major reflexes, breathing, etc. So we are extremely lucky that she has only minimal damage. At this point, we don't know the extent of the damage. We know she has a normal IQ, but we also know there appears to be some early signs or learning disabilities. So time will tell just how bad it is....

Friday, July 11, 2008

History, Part I

I knew when I started this blog that I wanted to include a post on Emmi's history. There would be new readers. They would be confused by the sudden leap into year four. In my other writings, I have alluded to Emmi's health issues. But I have left the details out. Three times I have started this post. Three times I have deleted it. It is easy to chronicle four years worth of medical history. I can simply list the facts. I know the dates of tests. I know blood toxin levels. I know the surgeries and stitches and kidney infections and spinal taps and skin biopsies. I have it memorized. But that is not what I intended to do. I didn't want to just list facts. The facts don't tell you how it felt to sit in the doctor's office the day we received Emmi's diagnosis. That I can still recall the color of his shirt. The time on the clock. The smell of the room. It is a memory so clear, I am positive it will never fade. The facts don't tell you that despite test after test and illness after illness that Emmi laughs more than any child I know. That she has a soft voice and a quite, breathy, giggle. That she loves to run on her toes with her arms over her head like a ballerina. They won't tell you that despite being half her sister's size, she will be the first to pinch, bite, or kick. She is feisty. She is hardheaded. It is what we need her to be. She would not survive if she were any different. She has no choice but to be a fighter, and, yet, she does it with such glee. The way she does everything. Grinning. It is how she gets away with being naughty. With a grin and a giggle. None of that comes across in the facts. And there is so much to share. So many details that it becomes bogged down. But they are needed. The facts. Event, and then the emotions. They go hand in hand. I will try little by little to fill in the facts, starting with Emmi's hearing loss.

We don't really have an answer as to why. It is this way with most of Emmi's health issues. We think we know. They have made their best educated guess. But they can not tell us 100%, without a doubt what is causing all of this. Due to some extremely high levels of methylmalonic acid, we know Emmi has a protein metabolism disorder. The genetic test revealed she didn't have what they suspected. However, according the the geneticist, that just means she has a form they can't yet test for. They suspect a form of or a disorder similar to a condition called MMA. A generally fatal genetic condition. One that would leaves it mark.

Emmi was born with normal hearing. She passed both a newborn screening test and another test at around six months old when she was hospitalized for testing after some possible seizures. She cooed like normal. She started babbling. By six months old, she was saying something suspiciously like "Mama." It all followed right along with Jill's speech pattern. Although, Jill's first word was "hungry," at six months old. Fitting. Then one day she just stopped. She started making this strange sound. It eventually evolved into what we called the "whale baby" sounds. I cried. But I never suspected hearing loss. We knew Emmi had a small amount of brain damage. She was missing reflexes (such as the startle reflex) that pinpointed the damage. Her neurologist suspected the brain damage might be worse than we thought.

But then, my mom, who watched the girls while I worked, noticed Emmi wasn't really responded to sound consistently. Then there were metal bowls dropped onto a tile floor with no response from Emmi. My aunt and uncle came to my parents' house with their dog while evacuating for a hurricane. My parents' dogs and their dog snapped and barked at each other during one point. It was loud. Everyone startled and jumped. Jill ran scared. Emmi, playing a few feet from them, didn't even flinch. I made an appointment the next week, and learned what we already knew.

Emmi was profoundly deaf. No one could pinpoint exactly when it happened. The metabolic disorder being the culprit. Or really, one of the times when her glucose level plummeted into the 20's and remained uncontrollable even on glucose, all caused by the metabolic disorder. It was a blessing really. It meant that hearing loss was to blame for some of Emmi's odd behavior. It gave us faith that the brain damage was not as bad as we thought. The ENT was confident that because Emmi was born with normal hearing, she would be a good candidate for cochlear implants. The moment he suggested implants, we never looked back.

It required a lot of testing. Not just on Emmi's part. I had to undergo psychological testing, as well. And counselling. It was a decision. We were told Emmi would not be accepted in many deaf communities because of the implants. A fact that made me mad. How do you cut off one of your own, because they choose to implore one tool that could help her function in a society that, let's face it, is made for people with normal hearing? For the most part, we have not seen this. However, I have had comments such as, "you can't consider her deaf anymore. I am deaf. She hears." I bet Emmi would beg to differ. Implants only get you so far. They aren't waterproof. They don't function well in a noisy room. She relies heavily on sign and lipreading. We couldn't get through a bath or the hours of swimming without signing. The malfunction from time to time, plunging her into silence sometimes for days until the repair is made. She has gone through two major surgeries for this. She takes hours a week of therapy. This is not the easy way out. It would have been a lot easier on me and her to learn sign. But I wanted to arm Emmi with everything I could.

So we went forward with the implants. Thirty days after her second birthday, Emmi received her first implant. Eighteen months later, she received the second implant. She sings to the radio. She loves to dance. She talks....barely, due to a deformed soft palate. Well, more accurately, she talks A LOT, we understand a little of it. But she hears. It is amazing really.

Everyone speaks the most about Emmi's hearing loss. Like it is the biggest issue we face with her. When in reality, we deal with a lot more. A metabolic disorder that at any point can strike again with more damage. Kidney problems. A deformed palate that will require surgery. Brain damage that we are still uncovering the extent of. Honestly the hearing loss is a non issue. Sometimes, I tire of going to therapy several days a week. But that is a silly little complaint in the grand scheme of things. The hearing loss is just that. Nothing more. It will mold her. Perhaps she would be different if she didn't have profound hearing loss. But I wouldn't want her to be.

Sunday, July 6, 2008

Here It Comes.

Sometime last year, we started calling Emmi "It."

"It's being naughty again."

No one remembers who started calling her It. It is her pet name. She giggles.

Out With the Old, In With the New.

I have had a caringbridge site for Emmi since she was nine months old. I am horrible at updating it. I thought I might do a little better with a blog. So here we are! Over the next few days, I will get the page set up and add a few blogs with Emmi's history to catch new readers up to speed.