The Race against Time: Living with your sibling’s life-shortening disease

                       

Katie Rupel, age 13, was in 4^th grade when her older brother, Matt, was diagnosed with Friedreich’s Ataxia, a life-shortening disease that causes progressive damage to the nerves and muscles, resulting in symptoms ranging from severe muscle weakness (ataxia) and speech problems to aggressive curvature of the spine (scoliosis) to serious heart problems (hypertrophic cardiomyopathy).  Early on, one doctor had told them that this was a very bad disease and that Matt would die if he had it, so Katie was terrified for her brother.  “My mom took me to Burger King after science camp and told me there,” Katie says. “It was really scary.”

 

Because FA is a genetic disease, Katie was tested for it, too.  She found out that she was a carrier but that she did not have FA herself. Still, being a sibling to someone with such a dangerous disease has changed Katie. “A lot of people don’t get that it affects me, too. I’m not living like, ‘Oh my God, he’s going to die tomorrow.’ I know that’s not going to happen; I know it. But I feel like I had to grow up fast.” At an early age, Katie had to deal with issues that most of her friends don’t have to deal with. For instance, it took her awhile to stop blaming God for her brother’s disease. And she struggled with feeling left out. “We’ve always been a really tight family, but, for awhile, I felt a little bit out of the picture. Everyone was paying attention to Matt. I felt selfish for feeling like that, but that’s just how I felt.” Because she’s more physically able, she’s expected to do more around the house, too. “I understand Matt can’t do all those things.”

 

She knows it’s a lot harder for her brother. “He feels like he gets too much attention, and I don’t think he likes that; he’s never been one to love attention; he’s always wise-cracking. And sometimes he feels our mom is a bit over protective.” Katie had to learn early not to always jump to her brother’s aid. “He’s really brave. A lot of people pity him and feel bad for him, but he doesn’t want that.” She’s learned not to hover, to treat Matt like anyone else. “My friends sometimes think I’m horrible when we hear Matt fall on the other side of the house, and I say, ‘He’s OK,’ but that’s one thing I understand; that’s what he wants.”

 

Katie has a small set of close friends who help her to deal with her brother’s illness. “Matt’s friends really help me, too, because they love him so much.” One of the hardest times for Katie was when Matt went through major back surgery to deal with his scoliosis and kyphosis.  “It was horrible,” she says. Katie is a very talented vocalist, and at the time of Matt’s operation, she was attending a summer camp called, “Jazz Goes to College.” Because she was so worried, it was hard for her to make it through each day. “There was this one Billy Joel song (Goodnight, My Angel), and it would make me think of Matt, and I would cry every time I sang it. I finally had to tell my teacher what was going on.” She laughs. “Then when Matt came out of the hospital he was so grumpy.” Katie and a friend had decorated his room, and being a typical brother on pain medication he didn’t show the appreciation she would have liked. “He told us to take that crap down.” Since Katie went through major kidney surgery when she was young, she could empathize with how he felt, so she didn’t hold his reaction against him.

Next year, Katie will be a freshman in high school, and her big brother will be a junior.  She is very passionate about her future. “I want to do everything. I want to travel everywhere. I want to go to Costa Rica and do photography for National Geographic. And I want to write, and I want to sing, and I want to make music.”  She gets it that she carries the FA gene, and she does think about what that means for her future and any children she might have (FA is inherited recessively so both parents would need to be carriers, and then there is a 1 in 4 chance of a child getting FA), but she’s not hiding from life because of it. “It’s a long time from now.” She believes there will be a cure well before then.

 

Katie hates what her brother is going through. She can see that his walking is getting worse, and she can’t help but get mad when strangers make unkind comments about Matt’s gait. She knows that he has to take too many pills, and they all taste disgusting (she’s tasted them).  “It’s scary, and it’s hard, and you just want it to go away, but it’s good to hate it, because it makes you want to do things about it.” She helps her parents with their FAITH (Friedreich’s Ataxia in the Heart) Fundraiser every year. And she knows that her family also has grown through their experience. “I think we appreciate life more,” she says, “more than other families. Matt could get cured and then die the next day in an accident. Life happens; there’s no stopping that.”

 

Before Matt was diagnosed with Friedreich’s Ataxia, Katie says, “I was so lucky, and I didn’t know it. If there’s one piece of advice I could give to the entire world, it’s to appreciate who you have when you have them, because things change so fast, you don’t even see it coming.” She smiles. “If I can show my friends that then, at least, I’ve done something good out of all this.”

 

Next week: The community’s story—Living with a friend’s life-shortening disease…