Miracle Stories
Carmen, Age 15
Like in a game of chess, 15-year-old Carmen is always thinking about her next move.
When Carmen was three, she was diagnosed with Juvenile Rheumatoid Arthritis (JRA). About 285,000 children in the United States have juvenile arthritis, a term used to describe any type of arthritis that develops before the age of 16. Of those cases 50,000 are specifically JRA, the most common form.
A child with JRA often has chronic inflammation in the joints which can restrict movement and cause tenderness and pain. Because there is a tendency to avoid pain by not using the affected joints, certain muscles can weaken over time which may result in limited motion or permanent contraction of the joint. As a child grows, there is also a risk of abnormal bone development because the inflamed joints can alter the growth patterns of the bones.
Carmen’s JRA affects her knees, ankles and feet, as well as her hands and wrists. According to her mom, Laurie, Carmen tends to be a bit slow moving and experiences stiffness in the morning, especially in cold, damp winter weather.
“As you can imagine with this disease there are many obstacles physically and many fears and trials,” says her mom. “Carmen has struggled at times when she was in dire pain.”
Distances are difficult for her so Carmen schedules her high school classes close together to minimize walking. She also uses computers and recorders or relies on help from friends so she can avoid taking extensive notes during classes.
Carmen’s treatment has included a series of splints and casts, along with physical and occupational therapy, all of which focus on reducing pain and keeping her muscles flexed and joints “working” so she maintains as much motion as possible.
However, Carmen’s overall care involves more than what is generally considered medical services. A related condition, known as Temporomandibular Joint Dysfunction (TMJ), affects the left side of her jaw so she also needs specialized dental services, which are available through Children’s Hospital as well. “For a child with multiple needs, Children’s Hospital is a place of inclusive treatment,” says Dr. Tom Doyle, director of the hospital’s Pediatric Dental Department.
TMJ
TMJ Dysfunction, which most likely results from JRA, affects the ascending bone that connects the lower jaw to its joint. According to Dr. Doyle, the affected bone is very short and gets smaller toward the back of the airway, making it more difficult to open the mouth and keep it open.
Carmen’s condition has been treated with oral surgery, orthodontics and pediatric dentistry, all of which are part of the Children’s Specialty Services’ Maxillofacial Program at Children’s Hospital. This program serves patients who have a surgically-indicated need for orthodontic or corrective jaw surgery. With Dr. Doyle’s dental services, the program joins plastic surgeons Dr. Austin Mehrhof, Dr. Carroll Petty, and Dr. Isaac Wornom; oral surgeons Dr. Michael Miller and Dr. Greg Zoghby; orthodontist Dr. Steve Lindauer; and prosthodontist Dr. Harlan Shufeldt.
According to Dr. Doyle, a specialized program where physicians and dentists work together is critical for a child with multiple needs: “If you had twins and one had cerebral palsy, would you just go to the pediatrician for the child with cerebral palsy? Probably not. You would seek the services of the different medical specialists for all the issues a child with cerebral palsy would have. It is the same thing with dental dysfunctions that are more than crooked teeth and cavities.”
In Carmen’s case, the issues are skeletal. “Her growth patterns are compromised by JRA, then magnified through growth and impacted on her temporomandibular joints [the joints in her lower jaw],” explains Dr. Doyle.
What this boils down to are problems with Carmen’s bite. More specifically, issues with how her mouth opens and closes and the way her teeth fit together.
With maxillofacial treatment, the goal is to maintain as much use of her jaw as possible as she matures into adulthood and growth patterns stop. If unresolved, the joints’ range of motion can decrease which may limit the mouth’s ability to open and close as well as make lateral movements.
Carmen’s care has included oral surgery to expand her narrow upper palate followed by an orthodontic program involving braces to correct her bite and reduce pressure on the affected joints. She has also had several permanent teeth removed.
According to Dr. Doyle, Carmen’s treatment can be considered a success “both subjectively and objectively.”
“She’s progressed well and responded well to all of our efforts,” he says.
A Different Type of Success
Carmen’s braces were removed in February. “I was waiting so much for that day,” she says, no doubt looking forward to having such a big step in her treatment behind her.
In addition to this success, what Carmen and her mom are most thankful for is the trusting relationship they have established with Children’s Hospital.
Though Carmen has visited doctors and dentists for many years, these frequent visits have not always been easy for her. She vividly remembers one of her first experiences at a dentist’s office. This memory involved having a tooth shaken loose when she and her brother collided heads. “It was a horror,” Carmen says.
Laurie recalls another incident when Carmen fell off her bike and hit her mouth. She took Carmen to a doctor who she says just wasn’t good with children. “Finally, I told him to let her up and we left,” she remembers.
But at this point neither Carmen or her mom are complaining. In fact, Laurie would go so far as to say she welcomes the hour-and-20-minute drive it takes to travel from their home in Hampton, Va., to Children’s Hospital.
“They are so good with kids. They treat children like they are of the utmost importance,” she says.
Laurie appreciates the patience and support shown by Dr. Doyle and his staff. “They know how hard it is for Carmen,” she says. “Sometimes Dr. Doyle will tell her what he’s going to do over the phone before she comes in to kind of prepare her so she’s not so scared.”
For Dr. Doyle, ensuring comfort is simply part of the day to day. “We take time to make sure each patient is comfortable. It’s the whole premise of ‘Tell, Show, Do. I am telling you this, Let me show you what I’m going to do, Now I’m going to do it.’ “
His focus on comfort also has an eye toward the future. “Adult women who are terrified of the dentist don’t take their kids to the dentist until there is an emergency,” he explains. “That’s a negative cycle that we are trying to correct, prevent, rectify, rehabilitate.
People who have confidence in their dentist and their dental teams have confidence in the profession. They manifest that by early appointments with pediatric dentists.”
Long-Term Relationship
Currently, there is no known cure for JRA. Researchers are studying both genetic and environmental factors that may be involved with its development.
For now, Carmen aspires to be a lawyer or a social worker.
“Everybody tells me I’d make a good lawyer,” she laughs, admitting that the comment is a positive way of saying she argues too much.
Despite any limitations related to JRA, Carmen excels in school and has already scored well on pre-SAT tests which she took as a seventh-grader.
She says she loves to read “anything I can get my hands on,” and also enjoys competitive games of chess. When asked how long she has been playing chess, her response is quick, “Playing well or playing at all?” she asks.
Carmen does acknowledge that she’s been faring rather well for the past two years. She knows this because she can sometimes beat her Uncle Craig who taught her how to play when she was eight. She’s also been known to beat her brother’s electronic chess game
Very fond of her family, Carmen is a well-adjusted teen with definite ideas about the world. What bothers her most are people who are narrow-minded. She says she tries not to criticize what others believe, but always likes to point out that there are different ways of looking at things.
As with all his patients, Dr. Doyle has high hopes for Carmen. He has seen her blossom from what he describes as a “timid, introverted patient” into a young adult with confidence, not to mention comfort with dental services.
His hopes for her include someday having a family of her own and bringing her children in early for dental services.
“Pediatric dentistry is a long-term relationship. Orthodontics is two years. I get them for 10 years,” he says.
It is this lasting relationship between the patient, family and doctor that has been crucial for Carmen. “Her family has been extremely supportive and caring,” says Dr. Doyle. “We couldn’t have done it without Carmen’s trust and the family’s help at home.”
And for the Pagan family, being there for Carmen comes easily. “Carmen has never used this illness to say ‘I can’t,’ “ says Laurie. “She has amazing inner strength and an unwavering determination to overcome her fears. Carmen is truly a beautiful person and a genuine inspiration to all who know and love her, especially me.”
