“Hemophilia is absolutely manageable, it’s just a matter of how you choose to live your life.”
-2.jpg)
Vernon resident Ana Marie Presti watches intently as her son Rhett dribbles a basketball in the school gym. She offers words of encouragement when it’s his turn to shoot and winces when he’s narrowly missed by the ball.
This is only the second practice for Rhett’s “Green Team”, but in Ana Marie’s eyes the season is already memorable. Rhett has hemophilia, an inherited disorder in which one of the proteins needed to form blood clots is missing or reduced. Rhett’s disorder is categorized as severe, meaning his body produces virtually no clotting factor. For him, any injury can lead to a bleeding episode, but even a brisk walk can cause a bleed in a joint, or it can happen for no reason at all.
Ana Marie says she and her husband Jeffrey introduced Rhett to sports like basketball and tee ball because they don’t want him to be afraid to try new things. Ana Marie makes sure he understands how this condition can impact his body. “He knows to be careful. He could get a bleed and will need a needle, which he hates. But it doesn’t faze him once treatment is over,” she says.
Rhett receives injections of clotting factor twice a week at home. Having factor present in his body at all times enables him to participate in normal childhood activities. This “prophylaxis therapy” to prevent bleeds has drastically improved patients’ lives since it was introduced about 10 years ago, says Phyllis Kandl, MA, CSW, Program Manager of the Comprehensive Hemophilia Treatment Center at Children’s Hospital of New Jersey (CHoNJ), where Rhett has been treated since infancy.
Ana Marie and Jeffrey have been closely involved with the Hemophilia Treatment Center since Rhett was a baby. Ellen White, RN, BSN, Nurse Coordinator, taught them how to infuse Rhett with clotting factor when he was 19 months old. “I had no comfort level with it, so I decided to go to school to study phlebotomy since it’s a skill I’ll always need,” Ana Marie says. She earned a certificate and now gives Rhett infusions of factor twice a week. “It’s helped a lot. I don’t stress when I have to treat him, and it’s not an issue anymore when we go away for the weekend, or on vacation.”
The Center helps families cope in creative ways. The “You Can Be a Star” program helps kids explore new interests like cooking and music, and patients can attend a special summer camp for kids with hemophilia.
Ana Marie works closely with his school nurse and teacher, and even gave a lesson to his classmates about hemophilia. “With guidance from Phyllis’ and a mother from the center who I’ve connected with, I was able to put together a workshop that had Rhett’s approval,” she says. “The kids are more careful now, and know not to push him.”
“Hemophilia is absolutely manageable, it’s just a matter of how you choose to live your life,” says Ana Marie.