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.


Allie Bear said...

Wow, I'm so glad you linked to this on your blog. You answered so many questions I had about Emmi but I guess I was too afraid to ask. You never cease to amaze me with how well you and Emmi handle everything that you have been through, simply amazing.

I am Trish Marie said...

An always ask away!! I will answer anything. Oh wait. Not ANYTHING. I forgot who I was talking to for a second!

Anonymous said...

Thanks for sharing this stuff, I know it isn't easy. You guys have really been through it, and the wait and see game is pretty stressful as well. She's a lucky girl with a mom like you.

Anonymous said...

Wow, she really is a fighter! And she's lucky to have family, especially you, that is going above and beyond to do so much that you have every detail memorized! Is the brain damage due to the metabolic disorder, or did she lose oxygen when she was born? I'm just so curious about all of it.

David said...

Thanks for doing this. Heather is so right on, She is so fortunate to have a Mom like you!