Sixteen-year-old Michelle Smith’s above average height and thin profile are a few reasons why she was crowned Miss Teen Maine in March and will compete in an international competition in July, although her appearance comes with a price.
 Six years ago, the Scarborough resident was diagnosed with Marfan syndrome, a type of connective tissue disorder.
“Imagine you have glue in your body holding everything together,” she said. “It’s like my glue is watered down.”
 Smith’s height – 6-foot-1-inch at age 16 and still growing – elongated hands and her flexibility are all common of the syndrome, which can affect tissue in the skeletal system as well as the eyes and heart. Over time, tissue around the heart’s aortic valve become weak and the slightest increase in heart rate can cause it to rupture, as was the case with Broadway musical “R.E.N.T.” writer and composer Jonathan Larson, who died in 2001, Smith said.
“His case was like me times 10,” she said.
Smith said it was difficult to see the many degrees of Marfan syndrome when she was first diagnosed at a hospital in Chicago.
“It was scary seeing people who have it worse than I do, and not knowing where I would lie,” she said.
Doctors concluded Smith’s syndrome was focused in her skeletal system and showed no signs of affecting her heart. Smith doesn’t have to take any medication, but receives an echocardiogram every other year to make sure her heart isn’t threatened. She also gave up sports once she was positively diagnosed, a tough decision for an active child, she said.
“It was scary, everybody telling me I can’t do this and can’t do that. Nobody likes being told what they can’t do, especially a 10-year-old,” she said. “Being 10 and doing cheering, it was one of the worst spots to be. Obviously I was sad.”
While Smith will most likely be near sighted – the tissue in her eyes, much like in her joints, isn’t as strong as it should be – and has pinched nerves and disc problems in her back, she said luckily her diagnosis is not as severe as others she has met, and can live with the back pain.
“I know it,” she said. “It’s all I know, it’s not like I was in an accident.”
Smith began to train herself to lower her heart rate – which can double suddenly for no apparent reason – by “bearing down,” and concentrating on calming the muscle; a skill not all people can do unless they are taught at an early age.
It took Smith a year to “turn around” her diagnosis and convince her mother to let her enter a beauty pageant.
“I’m not going to sit around and not use [Marfan syndrome] to my advantage,” she said.
In 2004, Smith was rated in the top 10 of more than 140 girls to participate in a beauty pageant in Lewiston.
“I had no clue it was going to be that big,” she said. “I figured it might be beginners luck, but what the heck, I’ll keep trying.”
The syndrome not only gave her the looks to turn heads on the runway, but a cause she could devote herself to, she said.
“You can’t win a beauty pageant just on a boardwalk,” she said. “Looking good is just putting the ribbon around the package that’s already wrapped.”
Smith has been featured in Supermodel magazine, the Miss Teen International competition and interviewed by FOX News, and next to nearly every one of her headshots is a statement raising public awareness of Marfan syndrome and its effects.
“Not enough people know about it,” she said. “Using my crown, I can open the door and show people.”
Doctors believe about one in 5,000 people have Marfan’s, and Smith said they estimate only half of those with the syndrome are ever diagnosed.
With her mother’s help, Smith has put together what she calls a “Marfan for dummies” awareness package with diagnosis criteria, common symptoms and information on the National Marfan Foundation to help those who may have the syndrome, or have already been diagnosed, understand that it is possible to live successfully with Marfan syndrome.
Smith said she particularly tries to inform school nurses about the syndrome, and recommends they screen students with a “positive thumb and wrist sign” technique – can someone bend their thumb inside their palm so it protrudes beyond the pinky finger, or touch their thumb and pinky around their wrist – that could identify Marfan syndrome early on.
“It’s non-invasive and kids love to show the weird things they can do,” Smith said.
This year Smith has been selected to speak at the July 10, three-day National Marfan Foundation’s annual conference in Boston, and said she’s excited to connect with other “Marfies” from around the world.
“There’s a whole different level of understanding at the conference,” she said. “I love being different, but I’m still a teen, I want to fit in.”
As part of her involvement with the foundation, Smith said she often converses with other teens diagnosed with the syndrome, which can be particularly devastating for teenage boys heavily involved with sports.
“I tell them it’s not the end of the world,” she said. “You can’t sit around saying, ‘Why me,’ at some point you need to say, ‘Why not me?’”
Smith and her mother are both hopeful she may never require heart surgery – as those whose aortic valves are directly affected by the syndrome often do – but are keeping their eyes on the progress of Losartan, a blood pressure medication that has shown signs of repairing damaged tissue in trials with mice.
“It’s very promising,” Smith’s mother, Blue Smith, said.
For more information on Marfan syndrome or the July 10 national conference taking place at the Westin Copley Place in Boston, Mass., visit the National Marfan Foundation’s Web site www.marfan.org.