Sixteen years ago Erika came into our lives in the usual way that babies do. And that is where this story both begins and ends. Erika’s life with Tyrosinemia 1 began. Her life as a normal child ended with her first breath of air. Thus, normal life, if there is any such thing, also ended for Erika’s mother, Patti, who is my sister.
The majority of us live day to day dealing with the average stresses in life: how to pay the bills, how to go to work in a job that affords us the lifestyle for which we strive, etc… Most of us get married and have children. Sometimes we get divorced. Sometimes we deal with personal loss and deaths, but somehow most of us manage to survive.
But my sister Patti had an extra challenge because Erika was born with a rare debilitating disorder and it was predicted that her life would be short. Patti’s coping mechanism now had to encompass caring for a child with a disease little understood in this world and she had to embrace the notion that her lovely daughter may not live long. As a family we offered Patti and Erika what support we could, but like everyone else we had our own lives to live, our own problems to solve, our own tragedies and triumphs.
Erika is now a sixteen-year-old, and I have only been partially involved in her life. It would be correct to say that I let sixteen years go by with my head stuck in the sand hoping her problems would correct themselves. I had my own life to live. I mistakenly assumed that my sister had a handle on things. But Erika’s problems became even more unique because few children with Tyrosenimea 1 live this long. Nobody had much knowledge about how the limitations of this illness (and medications used to mitigate it) would affect the life of a teenager. Being a teen is rough anyway, but the peer pressure for one with limitations can be devastating. None of us knew how to cope, or how to help Erika, especially my sister Patti.
It wasn’t like I didn’t know most of what was going on. My sister would call me from time to time and tell me about the latest crisis. Or when she was at her wits end with Erika, she would call for me to come and fix the situation. My way of fixing things was to give Erika my don’t you dare cross me glare but this only lasted a few weeks before Erika would again succumb to peer pressure and go back to doing things that were unhealthy for her.
To answer the constant nudging I was receiving from God to step in and help with this family situation, I finally decided that in order to fully understand what my sister was living with I had to walk in her shoes for a while. Patti was on the verge of giving up. The stresses of parenting a teen with extra needs was becoming more of a burden than she could cope with. I needed to reach out and I needed to do it immediately. In order to help Erika I knew I had to get inside her head.
Although I knew the problems my sister was having with Erika were getting worse, I wasn’t ready to get in the middle of things. Even though I could see that my sister was just short of giving up I still couldn’t make myself reach out and try to help. I wasn’t certain that I could.
June 15, 2011, I got a telephone call.
Erika’s monthly blood work revealed levels of protein that were off the charts. With Tyrosinemia 1 the body is unable to metabolize protein, therefore a strict diet is necessary to keep the disease in check. The doctor gave my sister an ultimatum: “Either Erika’s weight comes down and the blood work returns to acceptable levels or he would report abuse to state officials.”
Abuse is a harsh word when you work full time and have a willful teen that just wants to fit in with her friends. My sister wasn’t available to watch Erika twenty-four hours a day, seven days a week to insure that what Erika did was right. This panicked phone call was the catalyst for me. I had to step in and offer whatever assistance I could, and I had to do it immediately. I could no longer ignore that little nagging voice in my ear, because now God was screaming at the top of His lungs, DO SOMETHING NOW!
I knew if I didn’t try and help, then it might be too late. I knew my family needed to be uplifted and I knew from where it had to come. Yes, the usual questions ran through my mind. How I was going to continue working and living the life style I had created for myself and still care for a teenager? But I put all that aside in order to do what was right. My sister needed relief and my niece needed guidance and motivation. Erika need to learn to begin to take care for herself.
Thus, Erika has come to stay with me for the next four weeks. Our journey together begins now. Our goals are to fight the effects of Tyrosinemia 1, to understand the intricacies of the diet associated with such a malady, and to still let Erika be the teenager she has become. This will be a huge challenge. I continue to pray for the strength I need to do what I can to be of assistance to my niece, to my sister, but also to all those who suffer from this disease.