Monday, June 23, 2008


Oxygen tubes were strewn across the top of their lips and clear tubing clutched in their hands. Occasionally, a hiss would erupt from an oxygen unit. Here and there, the pulse of oxygen being delivered could be heard as a pshshhhhhhhh kind of noise.

I had been there before. Their gulping breaths in and out, pursed lips, laugh lines in all the right places, and wide smiles greeted me. It was what I had waited for yet overwhelming at the same time.

I was alone but yet among relatives of the same family. My Alpha-1 family.

As I walked throughout the conference hall, I began to calm myself with subtle inhales and exhales as I encountered familiar faces as well as new faces. Some were babies and children. Some were young adults; some were middle-aged. All were in attendance to learn, grow, relate, and feel less alone.

I was there to learn more, but I wore a smile on my face like a mask. I really don't think anyone noticed though. All were too busy enjoying each other. I wondered if I wasn't the only one feeling like tears were going to down pour from within at any moment.

I was uneasy there. Denial keeps me safe inside of the world of being at arms length. I don't walk the earth in the body of an Alpha. My children do though, and that knowledge profoundly affects me when it bubbles like a volcano letting a subtle burp burst through the cool crust of lava. It is always there smoldering in my thoughts.

This annual education conference can be both an inspiration and realization. Speeches about treatments and cures inspire this mother's heart yet they grab reality and shove it squarely in my face. Here Jen! Here! See! Your children have a life threatening genetic disorder. Stop living like they are free from potential liver and lung disease! As I screamed on the inside, I smiled and clapped and cheered the idea of a cure. It will come someday, hopefully soon.

My conflicting emotions startle me. I hold Alphas in my heart. They are the bravest group of people I've ever encountered. They deal with failing lungs, sick livers, skin ailments, and bodies failing to keep up with their ferocious spirits. All of this in the prime of their lives. Lives often interupted by the need to take a break to catch their breath. Lives with yellow skin or bellys full of ascites fluid.

It is life though. My brain keeps reminding me of that, but my heart keeps wanting to run from the reality of my daughters' Alpha-1. I remain firmly half way between acceptance and denial.

A kind doctor invites my children into his one of a kind pediatric Alpha-1 practice. I want to hug him. Tears well up, but my defenses shield him from my happy yet sad tears. Conflicted, I know that Grace and Meghan will have access to leading research and potential treatments. At the very least, they along with their Alpha-1 will be entirely understood there with yet another life blessing in a kind, for-a-cure hepatologist and biochemist. A man, who has lost so much himself, but still there giving and giving to our small community of children with Alpha-1. He gathers Gracie and Meghan into his circle of hope. I embrace that hope like a life preserver.

I ride the elevator and head for my hotel room. Upon shutting the door, I break down. I'm simply overwhelmed with his compassion and understanding. I feel honored for my girls. They really do deserve the very best in life, and today, God's blessings erupt like lava and shower down on them. I want to shout the news so all will know, but I place my smile mask back on my face. I reemerge from my room and make my way to the memorial service for Alphas who we've lost, tissue in hand.

There in that room though, I can let it out. I listen to the brave stories of Alphas who now breathe free and live their eternal lives among the angels. I tell the story of my cousin, Butch, who left his Alpha life last year. I recount his dimples, bright eyes, and mile wide smile. I hope he hears me. I hope he knows that my daughters will have a different outcome. I know it deep down in my heart. Angels whisper it to me everyday. Some days I hear their guiding words. Other days I don't.

A lovely lady hugs me close, and tells me that she feels like she knows me a little better after what I've said. She tells me that my children will be fine and that I must believe. I say that I believe but it is tough listening to the stories of Alphas who had believing parents just like me.

Another couple with salt and pepper hair get up to speak. Their daughter has recently passed, and they've come to the conference to understand Alpha-1. Their two other adult children have it too. I think to myself that they are like me. Their parental love seeps from within them. They don't understand why she is gone and why their other children may leave this earth before them. It isn't right. I sob along with them. I am just like them still hoping and praying and desperate for a cure.

The weekend conference comes to a subtle end. I learned. I laughed. I cried. I smiled. I lived. I soaked in the love all around me all with the knowledge that we need a cure for all the Alphas. Long live the Alphas!


mollsrb said...

thanks for your comment. i originally found your blog by searching for alpha-one.
you see my aunt recently passed away from complications of alpha-one and my mother and uncle both have been diagnosed as well. the funny thing is... my grandparents also attended the conference this weekend, in order to better understand. i think they may be the couple with "salt and pepper hair" you speak about.
it's so amazing the way this world works. God bless.

Childlife said...

What a beautiful, touching post, Jen. I have to believe along with you that it will be different for your beautiful girls -- and that it will be different for other families like yours because of the awareness you are raising about Alpha-1. Thank you for courageously sharing your heart -- it is a beautiful thing to witness.