The ghosts of what could have been and what will never be. And even the ghosts of what may come. Out of the three children born to my parents I was the only one who lived to see adulthood. My brother and sister were struck down by what we called the family curse. In more scientific terms it was a genetic defect called methylmalonic aciduria. 

Methylmalonic aciduria is an inherited disorder which results in an enzyme deficiency and the body’s inability to process certain proteins and fats or lipids properly. The result is a toxic buildup of a substance called methylmalonic acid in the blood. The diet is very restrictive. My little brother especially loved Cheetos. He was allowed just a few every now and then while watching other children pull out greedy handfuls from the bag. His short life was more about what he couldn’t have than what he could.

My sister, Lesley, died at six months. I was just a toddler then and unfortunately don’t remember her. My brother, Vince, died at four. I was 10 and so have lots of memories of him… including his death.

I was the one who was asked to go check on him while he was sleeping. He did not respond to my voice, and I noticed his lips were blue. I ran to my mother. She came quickly, gathered him up in her arms and stroking his face, kept repeating over and over, “He’s just a sleep. Wake up Vince. Wake up.”

At that moment my life changed forever.

Any death of a sibling is horrific and lifechanging. But there seems to be a special cruelty to genetic diseases for survivors. It crawls into our minds and takes over our futures. My parents were told that there was a one out of four chance with each pregnancy that they would have a child stricken with the disease. They learned this information after having three children and discovering that they both carried the gene that caused methylmalonic aciduria.

However, the genetic dice had rolled in my favor.

But by being spared, much would be expected of me, I convinced myself. And to make matters worse I was born into a culture which thrilled to the idea of baby boys who would carry on the family name. My father was an only son. His name would end with him because I was born healthy and my brother was not.

Even as a little girl I remember pondering this predicament. One day, helping my father haul wood, I turned to him and asked, “Do you ever wish I was a boy?” He replied, “Not today I don’t.”

In my little girl’s mind that meant I was earning my worth, filling in for the brother who would never be there. It was a powerful and somewhat devastating realization.

And I am not alone. The other night I was watching a documentary on Bill Nye the Science Guy. He is currently on a campaign to educate and sound the alarm bell on climate change. He also talked about his personal life. I had no idea that like me he was one of three, a brother and a sister, and his family has their own curse. Both his brother and sister suffer from Ataxia, a neurological disorder. Although his brother and sister are alive, unlike my siblings, he is still riddled with guilt for being the lucky one, the one to escape the horrors of their disease.

Nye, who has no children of his own, made a curious statement that spoke powerfully to me. He questioned whether his commitment to saving the planet and justifying his worth was a result of his being spared the disease that afflicted his siblings.

I don’t feel that I have the responsibility of the fate of the planet on my shoulders. But I have spent my life attempting to earn my worth, to justify my existence, much as Nye seems to have done.

The message was sent early, and it was clear. Person after person would pat me on the head the day of my brother’s funeral, saying of my parents, “You are the light of their world. You are all they have now.”

So, I became an overachiever. I stuffed down my own grief and sense of loss and set about living not only for one life, but three. Early on, I discovered I was a good writer. And before I could drive, I was working for the local newspaper and United Press International soon after.

I won awards. Associated Press wanted to offer me a full-time job my freshman year of college. I was good at what I did. Overly good.

But, for me, never good enough.

And the presence of methylmalonic aciduria was never far from my mind. I grew up with it, spitting out that big bad word even as a little girl. I could quote you facts, such as, my brother being one of only 12 documented cases in the world with methylmalonic aciduria at the time of his birth.

Imagine as a little girl being told by doctors that when you grow up and meet a man you want to marry you should keep in mind that he as well as you might be a carrier. If genetic testing was available by then you would need to pursue it. And if it was found that both of you were carriers, what questions would you then be forced to confront?

My dating process could be awkward. I inquired about ancestry, geographic area and family last names. I wanted so badly to try to eliminate any possibility of crossing genetic lines with the wrong person. I attempted to work it into the conversation as naturally as possible. No easy task. I recall a moment of panic when I discovered some far-off relative of my new husband’s had a name that matched a far-off relative of my own. Luckily it was by marriage.

I did decide to have a child though. We couldn’t test then to see if my husband or myself was a carrier. So, there could be a risk. But I was determined. A year before attempting to become pregnant, I lost 70 pounds, changed my diet, excluding caffeine, and exercised every day like a mad woman. Once pregnant I stayed positive, going into a Zen-like state of mind as much as possible and listening to the soothing sounds of James Taylor constantly. I couldn’t control the curse, but I could put up the best defenses against it within my grasp.

My son was born healthy. And, I knew he would be my only child. Today, that baby boy is a tall, handsome red head (a pleasant genetic surprise for a change). He is a grown man who works as a producer and is a drummer for a rock band.

And as he dates and meets potential partners, the family curse raises its ugly head once again. Of course, I can’t sit down with any of the young ladies he might be seeing and give them the quiz – ancestry, geography, family names. But I take comfort in the fact that medical technology has come so far that not only may he know the chances of having such a child but will have better treatment options available as well. I remain hopeful.

As I have seen my own child grow and thrive, I have come to accept that it is okay for me to be here. To have survived the roll of the genetic dice. With each passing generation my family is putting up the good fight against the family curse.

So, while watching Bill Nye talk about his worth and his mission and being a survivor, I very much wanted to put my arm around him and say, “We’re here. We’re healthy. And that’s okay.”

Because it is.

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