Local Interest

"Once upon a time there lived a perfectly normal family until one day in January 2001, when Haley Vincent woke up with a sore throat and fever." - from Cheryl Vincent's journal entry.

Since then, life has become far from normal for Bruce and Cheryl Vincent and their family.

• • •

Today seems to be a good day for Haley, a talkative, intelligent 10-year-old whose last three years have been consumed with two life-threatening illnesses. She is as quick to jump into the conversation as she is to bounce up and down on the couch.

She remembers clearly most events surrounding her illness - and volunteers that she much prefers the intensive care unit at Children's Healthcare of Atlanta to the one at Duke University Medical Center. She mentions that she has spent Mother's Day, Father's Day, her brother Nick's birthday and Memorial Day hospitalized in Atlanta.

She can name all 14 of her medications and explain what each does, and she giggles when it's revealed that she's allowed to roller skate inside the house to ease her joint pain. Her three siblings - Nick, 12; Logan, 8; and 4-year-old Kendall - don't have that privilege. "They call me ‘the special one' because I get my way a lot," she said.

Like most children with a sore throat and fever, Haley stayed home from school that winter day nearly three years ago. But Bruce, a physician with Mountain Region Family Practice, discovered that his daughter, who was 7 at the time, had an enlarged spleen. Lab worked indicated that Haley had liver abnormalities, and she was admitted to the pediatric intensive care unit at Holston Valley Medical Center. "She had pneumonia, and they tried IV antibiotics, but she got worse and worse and worse," Cheryl said. Haley also developed respiratory failure.

At that point, medical staff decided that Haley should be transferred to a facility better equipped to handle a critically ill child. Cheryl, a former critical care unit nurse, remembers reading the transfer order: "Critically ill child with multiple disease processes." "Basically they had no idea what was wrong with her," she said.

Haley was transferred to Duke University Medical Center. Cheryl wrote an online journal entry (http://caringbridge.org/ga/haley/) about that trip: "We left around midnight on a Thursday, and that trip remains one of the most surreal experiences of our journey. Here I was on a bitter cold, yet beautifully clear January night first flying, then riding in a screaming ambulance through dark, deserted streets, with some man I had never met before keeping my daughter alive by forcing air into her lungs with an ambu bag. I remember thinking, through my panic and fear, that I would never be the same person again. Some experiences are so profound; you know in an instant they have changed you forever."

Because Haley had bone marrow suppression, a deficient immune system and an enlarged liver and spleen, doctors at Duke suspected she had leukemia. "But then they couldn't find cancer cells, so they said she had pre-leukemia," Cheryl said, "then they said she didn't have leukemia. We really thought we were out of the woods."

The IV steroids used to treat her pneumonia probably masked her liver problems, Cheryl said. Haley was sent home, but her parents' instincts told them things still weren't right. In March 2001, Haley went into liver failure. "She was yellow," Cheryl recalled. "It was obviously liver problems." A liver biopsy led to Haley's first diagnosis: autoimmune hepatitis (AIH). Autoimmune hepatitis, a condition in which a person's own immune system attacks the liver, causing inflammation and liver cell death, usually occurs in women between the ages of 15 and 40.

Haley responded well to steroids used to treat AIH, but developed gallbladder problems. She was sent to Cincinnati Children's Hospital. Serial liver biopsies were done, and Haley's liver crumbled as the hepatologist extracted a piece with a needle. "Your daughter cannot live her whole life with that liver," he told the Vincents. "This was the first time transplant was mentioned," Cheryl said.

The Vincents began exploring their transplant options, and found the Children's Liver Transplant Program at Children's Healthcare of Atlanta. In that program, Haley's liver began responding to treatment. The next year, 2002, was fairly uneventful for Haley, except for some pretty significant joint pain. Her pain was treated with strong narcotics, but Haley had to leave third grade at Jefferson Elementary School and had to stop all extracurricular activities.

In early 2003, she began running fevers - 104 degrees F. to 105 degrees F. every day for 30 days. She returned to Atlanta for "fever of unknown origin," Cheryl said. Once again, doctors scared the Vincents with talk of cancer, this time lymphoma. But the bone marrow biopsy came back normal. Still, Haley's symptoms - fevers, swollen lymph nodes and odd rashes - remained a mystery to the doctors. Eventually, Haley received her second diagnosis: systemic lupus erythematous (SLE), another autoimmune disorder which can affect multiple areas of the body.

Dr. Rene Romero, assistant professor of pediatrics at Emory University, came from the University of Miami in 1991 to join the Children's Healthcare of Atlanta's Children's Liver Transplant Program as medical director and chief of pediatric hepatology. He's seen some pretty weird stuff in his career, but Haley, he said, is more like one in a million.

"The presence of both (AIH and SLE) definitely complicates her management," he said. The prevalence of AIH, Romero said, is about two in 100,000, but that number varies from country to country. "As far as age, it's more common in adults, but it does occur in childhood, most often late school age, teenage years," he said. "It's very unusual under age 3 or 4."

AIH is chronic and progressive, and doctors believe a combination of environmental and genetic factors are responsible for bringing about the disease. "Possibly a virus triggers the initial irritation of the liver, and something about the individual's immune system - either it isn't regulated normally, or it responds in an unusual way - sets off the continued irritation," Romero said. Only about 20 percent of patients with systemic lupus erythematous are younger than 16, Romero said. "She's an usual patient in that she has manifestations of this illness," he said. "Usually you have one or the other. It's very uncommon to have both. It'd be like one in a million."

The word "systemic" means that SLE can affect many parts of the body. "It can affect joints, the linings of the organs, there can be skin changes, rashes, and it can affect the blood counts," Romero said. "Both are treated in some ways similarly; you have to suppress their overactive immune system to try to keep their inflammation away. We've used steroids - prednisone - but then we've also had to use other stronger medications used in organ transplantation."

After the hospitalization in which SLE was diagnosed, Haley developed a strange new symptom - her eyelids swelled. A CT scan indicated fluid around her heart and both lungs. She began sleeping between 14 and 18 hours a day. In May 2003, she was sent back to Atlanta, where she stayed for six weeks. There she endured cardiac drains, a bout of shingles that caused her bladder to shut down, a dose of a chemotherapy drug that caused her liver to shut down, and an episode of hepatic encephalopathy. Hepatic encephalopathy is brain and nervous system damage that occurs as a complication of liver disorders. It's characterized by various neurologic symptoms including changes in reflexes, changes in consciousness, and behavior changes that can range from mild to severe.

In the summer of 2002, Haley embarked on a songwriting journey. "We were walking in the mall and my joints were hurting and I said, ‘I've got a dirty rotten liver.' Mom said I should write a song," she explained. Thus was born "Dirty Rotten Liver Blues," which Haley wrote and then recorded with help from Dan Robinson.

"I got a dirty rotten liver

Take it out, and eat it for dinner

I got a dirty rotten liver

When get a new one, it'll be a winner

But right now, I got the dirty rotten liver blues

The blues The blues"

The song is dedicated to children and their families affected by pediatric liver disease. "It has touched people. It really has become a ministry to us because of our liver support kids," Cheryl said. "The song is a funny song. It's us poking fun at a terrible thing. It makes people cry. I guess that's OK. It touches people the way they need to be touched."

Haley recently went back into the studio to record her second song, "Billy Rubin is My Friend." Bilirubin is a byproduct that the liver gets rid of. If the liver isn't functioning, the bilirubin level rises. Bilirubin is what causes the skin to turn yellow. The Vincents don't charge for the CDs, and most of all, want to use them to raise awareness of pediatric liver disease.

"We have other things we fund-raise with," Cheryl said. Because the Vincents don't have prescription drug coverage, they pay out of pocket for Haley's medications. That bill can run between $700 and $1,000 a month. One local radio station proclaimed Haley Vincent Day in November, and played "Dirty Rotten Liver Blues" and interviewed Haley and her mom. The first 98 listeners who donated $10 had the CD delivered to them. From 8 to 10 a.m., Jan. 24, Applebee's will hold a pancake breakfast. Tickets will be available at the door for $5. At 6:30 p.m., Feb. 20, Christ Fellowship Church will host a spaghetti dinner, followed at 7:30 p.m. by a silent auction. Auction items will include a used G4 Apple computer and a baseball autographed by an Atlanta Brave. Other fund-raisers are being planned for March and April, and T-shirts and Love That Smell candles are continually sold to help offset expenses. An account has been set up at SunTrust Bank on Fort Henry Drive. Contributions can be sent to: Haley Vincent's Liver Fund, c/o Sun Trust Bank, 2060 Fort Henry Drive, Kingsport, Tenn., 37664.

For now, Haley's name doesn't appear on the United Network for Organ Sharing (UNOS) transplant list. She's not sick enough. "She does have cirrhosis, which means there is significant scarring," Romero said. "She has enough liver cells remaining to do most of the functions of the liver normally, still. She can clear jaundice from her body so her bilirubin is normal, she can make clotting proteins normally and she can make other proteins we measure normally still. Because those functions are not impaired yet, she doesn't meet criteria to need a transplant yet. "There is a risk to transplant. At every given point that you access a patient, you have to decide where the risk is greater. Is it riskier to do a transplant, or riskier to continue the therapy you are doing?" Romero said. "Right now her current therapy is adequate to keep her alive for the foreseeable future."

Haley, who turned 10 on Nov. 5, is homeschooled, and physically, she has good days and bad days. Her liver is functioning OK and her lupus is under fair control, but she's had no height growth since 2001, and she has a cataract in one eye. When her joints hurt bad enough, she's allowed to break out the roller skates to get around the house. She rarely complains about the pain, Cheryl said, or even tells anyone she's in pain. "Children are so amazing," Cheryl said. "They are so adaptable. It's amazing what they will adjust to. ... That doesn't mean she won't get down. Sometimes she gets scared."

Haley's good nature, Romero said, is a reflection of her parents. "When I first heard ‘daughter of a nurse and a doctor,' I cringed. I thought, ‘Oh my God,' but they are the most wonderful parents you could imagine," he said. "They have good heads on their shoulders and they still treat her in so many ways like a normal child - with normal discipline and normal expectations of behavior that permits Haley to flower like she does."

The Make-A-Wish Foundation, which grants the wishes of children with life-threatening medical conditions, is working on Haley's wish - to visit New York City and see "The Lion King" on Broadway. Her original wish - to have TLC's "Trading Spaces" crew redo her bedroom - fell through. In the end, though, Haley will get both wishes, the original courtesy of her parents, who have kicked her out of her current Tweetie Bird-themed bedroom and are redoing it themselves. Haley will get her first peek at her new room on Christmas morning. She knows this much about her new room: it will feature a mural by local artist Suzanne Justis, and it better have a lava lamp.

For more information about Haley or to request a CD, e-mail paw1@chartertn.net or bandcvincent@aol.com, or visit her Web site at www.caringbridge.org/ga/haley/.

Date Published: December 20, 2003

Reprinted with permission from Kingsport Times-News

HONAKER – Mally Gent has asked thousands of people to consider giving the gift of life.

The teen has made organ donation her official platform during her reign as the 2004 Miss Virginia Teen USA. It’s a topic that hits close to home.

"Seven years ago my father received a kidney transplant," she said. "After his kidney transplant, he lives life every day to the fullest. He inspires me because he’s the most caring and loving person."

Six members of her extended family have received kidney transplants over the years after suffering from chronic illness.

"It’s a topic that is close to my heart," said Mally, a Honaker High School senior. "I’ve personally contacted more than 5,000 people about becoming organ donors. I try to incorporate organ donation awareness at every appearance I do."

Mally has worked closely with Mountain Regional Donor Services, which promotes organ donation awareness. April was a busy month for Mally. She made some local television appearances to address the topic because April was National Donate Life Month.

"The doctors had told us that my father was going to die," Mally said. "I’m just glad that I’ve got the chance to give something back."

Mally has been giving to her community for years through volunteer projects. She has volunteered for the American Heart Association and the American Cancer Society, and she has participated in the White Christmas program for underprivileged children in Russell County.

Her community service was noticed by the Virginia General Assembly this session. The House of Delegates and the Senate approved a joint resolution commending her for her community work. Gov. Mark Warner signed the resolution.

"My delegate, Jackie Stump, presented me to the House of Delegates," Mally said. "Senator Phillip Puckett presented me to the Senate, then I got to meet with Attorney General Jerry Kilgore."

"If we can stop violence when the kids are younger, maybe we can stop it for good," she said.

The visit to the state capital has Mally exploring a new career goal. The teen once planned to become a television journalist, but seeing state government work up close has her eyeing political science when she goes to college.

Mally will compete in the Miss Teen USA pageant on Aug. 6, possibly in California. In the meantime, she plans to attend Southwest Virginia Community College as she completes her reign.

She also is excited about the possibility of winning the national pageant, which comes with a free Trump Towers apartment in New York for a year.

"I think I’m going to be prepared," she said. "Everything happens for a reason. If it’s God’s plan, then he’ll let it be."

"My family is so excited," she said. "I could not have done this except for my family. My school has also been very supportive."

Pageants were not in Mally’s plans until relatively recently. She said she was a tomboy who enjoyed nothing better than sports.

"I started pageants a year and a half ago," she said. "I had always said I would never be a cheerleader or be in a pageant, but within a year I was a cheerleader and was in my first pageant."

"I Rollerblade all the time," she said. "I will take a broom and push the snow out of the way just to Rollerblade."

Mally also is a firm believer in self-esteem. She and Kristi Glakas, the reigning Miss Virginia USA, will host a seminar next month when Honaker High celebrates the end of the rigorous Standards of Learning exams. The seminar will address approaching beauty from within, she said.

"I’m really big on self-esteem and self-confidence," Mally said. "I think young women should be confident."

Organ donors are nation's unsung heroes
Wednesday, January 28, 2004

By TERESA HICKS
Times-News