Perfect Form....

Tuesday, October 20, 2009

MLD otherwise known as Metachromatic Leukodystrophy

Or the genetic disease that took my daughter's life.

These are my girls. On the left is Laramie, my Mommom is in the middle, and Cheyenne is on the right. They were about 4 and 5 years old in this picture. Cheyenne is now 17.

Metachromatic Leukodystrophy is a genetic disease that affects the myelin on your nerves. Basically it's when the enzyme called Arasulfatase A decides to call it quits, and no longer keeps the myelin on your nerves.  This results in losing your ability to function, you can't walk anymore, talk anymore, or eat anymore. It causes white matter changes in your brain too. It sucks.

As a parent, you get to watch your once normal child slowly waste away and die and there's nothing you can do about it. Nothing. Do I sound a little bit mad?

I am.

I'm more mad now then when we were going through it. Maybe not really mad, just, oh I don't know. It's the helpless feeling I hated the most. There was nothing we could do to stop it.

We tried. We did 2 bone marrow transplants on her. The first one was a cord-blood transplant. After we killed all her good bone marrow with chemo and radiation, we put non related umbilical cord blood into Lara. Her body knew better, we didn't fool it. She grew her own bone marrow back. We then used her brother for a bone marrow donor. She liked that better and kept it.

But you see, it wasn't enough. Nothing was enough to stop the beast.

The bone marrow transplant was the only way to break through the blood-brain barrier to at least keep her brain from getting fried from her disease.

There's always a catch. You have a whole year until it reaches your brain, so until then, you decline at an alarming rate. Once it all reaches your brain, it slows the disease, but doesn't stop it.

This picture breaks my heart.  Grammy made her a princess cake for her birthday. She never really got to eat any of it. She couldn't eat anymore without aspirating.

Here she is with Daddy on the 4 wheeler. There were happy times, but, funny, I can't remember those as much as I remember the times I was impatient with her.  I don't even know what I'm getting at here, just that I have to get this off my chest.

If I had to do it all  over again, I would have not done the bone marrow transplants, I would have taken that Make a Wish vacation and taken all the kids. We have never done that, taken all the kids on a vacation. Our oldest is now 21, and moved out. If we had the opportunity, I guess we could still take them on a vacation, it just wouldn't be Disney world, it would be out west, I think they'd like that alot better now that they're older. But there's something missing, their sister.

Laramie did have a "wish" fulfilled from Dream Come True in the Lehigh Valley. She got a computer with a touch screen that she used as long as she could. In the end, they ended up paying the balance of her funeral costs. They just did it without letting us know. I am forever greatful to them for that.

This is not all of them, we're missing this guy......

He's now 9 yrs. old.

On October15th , 2005, Laramie went into the Lehigh Valley Hospital with a fever, and an upper respitory infection. In one day, she was put into the PICU and placed on a ventilator because her lungs were totally filled.

She had pneumonia. She was up there for a month.

Meanwhile, unknown to me, my Mommom was getting sick. Mommom died on November 12, 2005.

It was then that I knew Laramie was going to die. Even though she was getting better, and was scheduled to be taken off the ventilator, I knew in my heart Mommom went  first to welcome Lara into Heaven. Mommom knew I would feel better knowing she was up there with Lara. I miss her. She was so special.

Laramie was taken off the ventilator because her lungs appeared to be healed. I knew better.
On November 18th, we called family and friends and told them to come and say good bye. I remember her siblings laying on the bed with her watching The Lion King and getting her to laugh. She loved them soooo much and was happiest when they were around. We kept them there as late as we could, kissed them goodbye (our oldest could drive), gave them money for pizza and sent them home.

Laramie Jane Moyer passed away on November 19, 2005.

She fell asleep and never woke up.

Every day, every single day, I feel like I'm missing something. I may have had 8 children, and I may have 7 left. Don't think I'm not grateful for that, but in my heart, there will always be 8, and no one can take that way from me.

It hurts so much.

1 comment:

pastorjim said...

Dear Keena:

Thanks SO MUCH for sharing your feelings about Laramie this way. You & your family are very dear to me....Please feel free to e-mail me as much as you like.

In Christ's Love,
Pastor Jim