Erin Kiernan Tries to Face Each Day With a Positive Attitude

The Baltimore Sun

August 31, 2003 Sunday

BYLINE: Gerald P. Merrell

Karen Kiernan strolled into the first-grade classroom for a routine conference with her younger daughter's teacher, confident, with the approaching holidays on
her mind.

"I expected to hear that she's a great student," Kiernan recalls, "but the teacher began, 'You know the librarian noticed that Erin moves around a lot.  Something just doesn't seem right. She's leaning a little off-balance ... and she seems to have a little bit of trouble with her speech.' "

The family wasn't alarmed. After all, Erin was only 6, and many her age are human forms of Raggedy Ann dolls, awkward and still in search of poise and equilibrium.

But that brief meeting, in November 1995, was the beginning of an incomprehensible journey that altered the entire family's most fundamental assumptions, hopes and dreams.

After nine months of tests, which included torturous electrical shocks and muscle and nerve biopsies, specialists at the Kennedy Krieger Institute in Baltimore performed a DNA test and discovered that Erin suffers from Friedreich's Ataxia, an incurable disease that strikes only one in 50,000 people.

The disease, which can be passed on only when both parents have the gene, causes deterioration to the spinal cord, nerves and the cerebellum, the part of the brain responsible for movement. Heart disease is common, and about one-third of the victims develop diabetes.

Each member of the Kiernan family reacted differently to the dreadful diagnosis. The elder daughter, Megan, now 17, withdrew into herself, both jealous of the attention showered on her sister and suffering from something like survivor's guilt. Today, she still prefers not to discuss her sister's illness.

Erin, now 14, refused to acknowledge the illness until she was forced to a year later when there were visible signs that the disease was progressing. "I don't think I've totally accepted it yet," she says, "but I'm getting there."

Karen Kiernan put on her own blinders. "I didn't want an answer. I didn't want to know what the diagnosis was unless there was a pill she could take and everything would be fine," she recalls. "This was bad stuff and I did not want to hear it. There's nothing you can do for it; it's just going to get worse. So, my way of dealing with that was (that) I kind of shut down for a few months. It was difficult for me to talk about it because when I did, I would cry. I tried to ignore it. If you ignore it, it's not there. And that's how I handled it."

Patrick Kiernan says the diagnosis was "devastating --it's like your whole life passes before your eyes. Everything gets called into question."

But, he adds: "I didn't have the luxury of falling apart. I had to look at the practical aspects of making our lives more livable: What do we do about cars, what do we do about the house, what do we do about bathrooms, what do we do about travel, what do we do about saving for college? How do we make sure that we manage our other daughter so that she's not negatively impacted?"

Early signs were there.

How Erin and her family have coped and changed and how they were assisted by the Muscular Dystrophy Association will be profiled on the Jerry Lewis Muscular Dystrophy As-sociation Telethon. The telethon begins tonight and will be broadcast on WNUV (Channel 54), beginning at 10 o'clock.

As they reflect, the Kiernans say there were early signs that Erin was different, but they didn't put much stock in them.

When Erin was in kindergarten, her mother was a classroom volunteer helper.

"I noticed when they played music and the teacher would say hop on the left foot, hop on the right foot,
everybody else was doing it but her," Karen Kiernan recalls. "Erin was having a hard time hopping on one foot. But she was 5 years old and, I thought, so what, she'll catch up. It's a developmental thing. We never put much substance in that."

Several months later, while she was undergoing testing, Erin suddenly was unable to ride her bicycle.

A neurologist declared that Erin's problems were developmental and "that she'll be fine."

Patrick Kiernan was incensed. " 'You don't understand. She was riding a bike; now she's not riding a bike. That's a problem,' " he recalls telling the neurologist.

The diagnosis, delivered on Oct. 7, 1996, required immediate changes in lifestyle for the family.

At the time, they lived in a three-story Colonial in Damas-cus. But the Kiernans realized that Erin would be limited to the ground floor. "I didn't think that was right that she was cut off from two-thirds of the house," Patrick Kiernan says, so the family moved to a home in Mount Airy.

That home required extensive remodeling, though, to accommodate Erin. The family has spent about $50,000 making bathrooms accessible, installing a roll-in shower and grab bars throughout the house, and enlarging her bedroom so she can maneuver in her wheelchair, and building special closets. A $17,000 lift has been installed so Erin can go downstairs, and $45,000 was spent to purchase and convert a van to accommodate her.

"Those (costs) look big," Patrick Kiernan says, "but the real hard part is ... the extra five hundred for a beach wheelchair, the extra money it takes to fly on an airplane, because you have to go direct because you have to minimize the number of times you're handling the wheelchair situation."

Remarkably upbeat.

Today, seven years after the diagnosis, the disease has progressed to the point where Erin requires a walker in the house and a wheelchair outside. Soon, ramps will have to be built as Erin is forced to use the wheelchair in the house. Her speech is slightly slurred and will worsen.

The Kiernans are still adjusting, and although there are moments of despair, the family seems remarkably upbeat.

"I do get depressed," Erin says, "but you can go two ways: you either go down and just be depressed all the time and think that no one cares about your life, or you can be upbeat and more positive about it. I don't want to be depressed all my life."

"I think about the thousands of people who have it a whole hell of a lot worse than me," adds her father. "There's always someone who's going to be worse off --not just people who are disabled, but people in all walks of life. Everybody's got challenges, and not small ones. It's what you do with it that defines your character."

There are still difficult moments, of course. A painful subject for Erin is her treatment by some students. "That's a hard topic," she says, declining to say more.

Her father, though, gently adds insight: "With kids her age, it's all about being the same, and they don't value the differences yet."

Erin has won many friends, though, at the annual camp she and other disabled children attend, through a teen support group that meets monthly, and through the Internet, which keeps her in touch with children, counselors and teachers who share their most intimate thoughts online.

And she has discovered that her sister, Megan, is perhaps her biggest fan. While applying for early acceptance to the University of Delaware, Megan wrote an essay about Erin.

"I did not necessarily get a 'normal' sibling who can run, jump, and play, but instead was given someone equally important," the essay says in part. "Watching Erin maneuver around the house with difficulty has inspired me to work harder ... Erin has taught me to appreciate what I have and my abilities."

"At first," their father says, Megan "was almost a little jealous, I think. Erin started doing a lot of things for MD almost right away, and she was getting a lot of attention."

Without any nudging, though, Megan quietly began involving herself on Erin's behalf. She has raised money for the MDA, and will be a counselor this summer at Erin's camp.

"She really holds it inside," Karen Kiernan says. "She doesn't talk about it much. She's a very subtle type ... in both humor and disposition. But all of a sudden, she's doing more volunteer work. ... She started involving herself, but almost on her own
terms."

As the telethon approaches, the family knows there will be critics, most of whom accuse Jerry Lewis of raising millions of dollars through a campaign of "pity."

They believe the critics are misguided. "The issue is really very clear," Patrick Kiernan says. "These people are raising money to help people with MD. The families that go and participate are not going to be pitied. They're going to appeal to people to help cure all of the 40 neuromuscular diseases that MDA addresses. Nothing more, nothing less. There are people who are going to look for the worst in
anything. What can you say to those people? Since they are not affected, they have no right to determine whether people on that show are pitied, pitiable, or deserving of pity."

For now, the family hopes.

It hopes for a cure, of course, but for more.

"That Erin goes to college and comes out to be whatever she wants to be when she gets older, with an independent life," Karen Kiernan says.

Erin's dreams are simpler: "I don't quite want people to know me for having FA (Friedreich's Ataxia)). I want them to know me for me."