It’s a tradition for Dr. Ira Taub and patient Sam Jimenez to get their picture taken together.
Sam Jimenez was born prematurely at 32 weeks’ gestation, weighing 6 pounds and 12 ounces. He grew significantly, and at age 6, his pediatrician referred him to an orthopedic specialist for bone problems often connected to Marfan syndrome.
The genetic disorder affects the body’s connective tissue, which holds cells, tissues and organs together – and helps the body grow and develop. People with Marfan syndrome are usually tall and thin with long fingers, toes, arms and legs. Marfan syndrome can also affect the heart, blood vessels, skeleton and eyes.
Diagnosis and surgery for Sam
“The orthopedist was astute enough to say there’s only one thing this could be. Sam had Marfan syndrome and referred him to me,” said Akron Children’s Pediatric Cardiologist Ira Taub, MD. “As soon as Sam walked into my office, I knew he had it. I confirmed it with an echocardiogram – because Marfan is a dangerous disease.”
The aorta is the biggest blood vessel in the body, and it transports blood from your heart to the rest of your body. An aorta for a 6-year-old like Sam should be around 2.5 centimeters – but his was 7 centimeters. “It looked like a giant water balloon,” Dr. Taub recalled. “When you have an aorta like that, it could rupture very easily.”
Sam’s mom, Zaida Cintron, got a call on the way home from the appointment with Dr. Taub. “They told us Sam’s aorta was buckling, and he needed aortic root replacement surgery. We needed to report the next day for presurgical testing, and he was having surgery the day after that,” she said. “They told us Sam wasn’t allowed to run or do any physical activity. We were freaking out because we had just been riding roller coasters at Cedar Point, and Sam had been playing all kinds of sports. It was a lot to process in a short amount of time.”
Sam was a good candidate for the David procedure. This is open-heart surgery to replace the diseased aortic root with a tube of synthetic material, while sparing the aortic valve. By keeping the aortic valve, patients avoid having to take blood-thinning medicine for the rest of their lives.
Sam’s surgery took more than 8 hours, and he recovered in the hospital for a few days. “He wanted to go home so badly,” Zaida said. “He’s a stubborn kid, and thankfully, he recuperated nicely.”
Sam’s family surrounded him with love after his heart surgery.
How Sam manages his condition
Sam takes multiple medicines to control his blood pressure and manage other aspects of his condition. “He’s been really compliant with his meds, and he’s and done everything I’ve told him to do,” Dr. Taub said. “He’s done superbly since his surgery.”
Although his heart issue has been repaired, Sam has other health challenges because of Marfan syndrome. He is in the early stages of scoliosis, and he complains of back and feet pain when he hits a growth spurt. He currently wears glasses in addition to contacts for vision problems, and he has crowded teeth.
Today, Sam is 6’10” tall and a sophomore at Heights High School in Cleveland Heights. He’s often mistaken for someone older. “Students who don’t know him think he’s flunked seven times, or they think he’s a teacher,” Zaida said. “It can be overwhelming when shopping at stores with people commenting about his size and asking if he plays college sports.”
Sam’s shoe size varies from 18 to 20, depending on the brand. “Name-brand shoes in his size can be difficult to find, and they are costly,” Zaida shared. “In this day and age, kids don’t want anything cheap. Sneakers in his size are $250, and he goes through about 5 pairs a year between growth spurts and wear and tear.”
‘It’s gratifying that Sam is alive’
Dr. Taub applauds Sam for having a positive attitude and constant smile, despite the daily challenges. “Our world isn’t built for people with Sam’s body type. Think about having to duck when you walk through a doorway or trying to get in and out of a car,” Dr. Taub said. “But Sam has taken a bad situation and rolled with it. It’s gratifying that Sam is alive because his heart condition was lethal before the 1970s – and most people didn’t live beyond early adulthood.”
Sam cannot take a hit to the chest, so he avoids sports. He started working out, after consulting Dr. Taub, and he enjoys other activities. “Sam is big into music and loves to sing karaoke,” Zaida shared. “He plays video games, hangs out with his friends and is the No. 1 fan of Shemar Moore and the ‘S.W.A.T.’ show.”
Sam wanted to be a pilot, but the vision issues have forced him to think about a different career. He’s in the engineering program at school, and he’s considering being a pharmacist or pharmaceutical representative. Regardless of the path Sam chooses, his future looks bright. “We have a good system in place here at Akron Children’s,” Dr. Taub said. “As long as we stay in touch with patients who have Marfan syndrome – and they tell us if something doesn’t feel right – we can take care of them at every stage of their lives.”
Learn more about the Akron Children’s Heart Center.
