One minute, 8-year-old Katie Daniel's main concern is whether Erica, her American Girl doll, should wear the purple dress or a white one. The next, she sounds like an adult as she explains how a mass growing in her neck and jaw could make it hard for her to breathe.
The rising third-grader at Swift Creek Elementary School in Chesterfield County talked to us in early May as she was getting ready to undergo surgery to have the mass removed at St. Luke's-Roosevelt Hospital Center in New York City — her sixth surgery. Katie, who has an older brother and younger sister, was born with cystic lymphatic malformation, which causes benign masses or lesions to form generally in the head and neck area.
"I really don't want to go through the surgery, but I don't like to hurt," she said matter-of-factly while sipping one of her favorite drinks, English breakfast tea, during an interview at a Richmond coffee shop.
Coping with Katie's health problems is hard for her parents. "It's tough. I don't like to see Katie cry," says her father, John Daniel, who is an IT programmer. Katie's pain has been intense sometimes, or dull and achy at other times, says her mother, Allison Daniel. She adds, "While I would move heaven and earth to stop this from happening, I cannot help but be proud of how she is handling it."
But Katie doesn't dwell on her health problems. She made all A's in second grade except for a B in physical education. "School can be fun," she says. "I like social studies." She also likes to play outside with friends on a trampoline, and she describes a recent accomplishment: "I did a front flip and landed on my feet for the first time."
Still, much of her life has revolved around doctor's offices and hospitals. In addition to her surgeries, she has had 15 IVs and multiple MRIs, her mom says. "MRI machines are cool," Katie says. "They sound like a bird tweeting."
Her condition was diagnosed at age 2, when a mass popped out on the back of her neck. An MRI showed the mass was also inside her throat and jaw.
There are two categories of cystic lymphatic malformation: macrocystic, which is one large or several large cysts, and microcystic, which are multiple small cysts. Katie has both, says Dr. David H. Darrow, Katie's physician at Children's Hospital of The King's Daughters in Norfolk. Some sources estimate the incidence of cystic lymphatic malformation at one per 6,000 live births. Darrow's practice sees a few dozen children each year with the problem. Katie's case is fairly extensive, which makes it harder to treat, says Darrow, a professor of otolaryngology and pediatrics at Eastern Virginia Medical School. When he first saw Katie in 2006, her lesions went behind the back of her throat and around the base of her tongue. Doctors don't like to operate in those areas because they are difficult to reach and any nerve damage could affect speech, eating and swallowing.
But Katie was able to participate in a clinical trial of a drug used in Europe and Japan to shrink macrocystic malformations. The U.S. Food and Drug Administration has not yet approved the drug, known as OK-432, which is injected into a patient's lesions.
The injections eliminated Katie's larger cysts, but the microcystic malformations are so small that you would have to inject hundreds to get rid of them, Darrow says. The microcysts swell from time to time and can cause pain. A common cold, for example, can trigger a flare-up. If the cysts swell around the airway, it can close.
"This is difficult to cure," Darrow says. "It's more a question of lifetime management in most cases." If all of the cysts cannot be removed, they tend to come back.
Katie's May 25 surgery was performed at St. Luke's-Roosevelt because that hospital is equipped with cutting-edge nerve monitoring equipment. The surgery was a success, Allison Daniel says. "They got it all. An upper nerve had grown all around the growth." However, Katie is scheduled to return to the New York hospital Aug. 12 for a procedure to remove a mass from under a main salivary gland.
"We are hoping that it will take only one surgery, although the surgeon's office is saying it will probably take several," her mother says, adding that Katie still experiences pain, but it's less intense than before her May surgery. "So while a big piece of this is behind us, we are not out of the woods yet."