FEATURES • Winter 2001

Michael R. DeBaun, assistant professor of pediatrics and of biostatistics, started the Sickle Cell Disease Medical Treatment and Education Center at the School of Medicine and St. Louis Children's Hospital in 1997. With a multidisciplinary team—physicians, nurse practitioners, research assistants, a nurse, and a social worker—the center cares for children with sickle cell disease and researches ways to improve their quality of life.

By Diane Duke Williams

A few months after her first birthday, Karra Thompson came down with a fever. Although she recently had taken her first steps, she limped when she tried to walk. Her fever spiked dangerously the next day, and her mother, Venus Kent, rushed her to St. Louis Children's Hospital.

Twenty-year-old Kent, who also had a 1-month-old son, waited alone that night on the ninth floor of St. Louis Children's Hospital, wondering what was wrong with her daughter and if she would survive. The next day, doctors delivered the news: Thompson had sickle cell disease. "I didn't know what it was," Kent says.

"I didn't know what I was headed for. It didn't sink in that my life wasn't like other young mothers' anymore because my daughter was chronically ill."

For most of her life, Thompson, now a ninth-grader at Central Visual and Performing Arts High School, has had excruciating, throbbing pain in her arms and legs. About once a month, they hurt so much that she lands in the hospital—sometimes for as long as a week. She's missed countless days of school.

"When I'm sick, I can't walk, I can't go outside. I really don't even eat a lot," Thompson says.

She has been teased by other children, who sometimes wonder if her disease is contagious or why she has to attend school in a wheelchair. And she has been limited socially. "I really don't have a lot of friends because I'm always sick," says Thompson, who dreams of becoming a gospel singer.

Thompson recently has had fewer painful episodes because of a new medication, but last year, she had to have her right hip joint replaced. She underwent surgery in 1999 because a bone in her right arm was infected.

"This is definitely hard," Kent says. "Especially when there is nothing you can do except pray a lot and say soothing words."

Thompson and about 500 other children in St. Louis battle sickle cell disease, a genetic blood disorder a child can inherit when both parents carry the sickle cell gene. The African-American community is greatly affected. In the United States, one in every 400 African-American newborns has the disease.

 

 

Ebonie Bailey (left), age 9, and Capria Rogers, age 11, enjoy swimming at Camp Crescent, a summer camp for area children with sickle cell disease that DeBaun started two years ago.

The disorder causes some red blood cells to become sickle shaped and clog small blood vessels, obstructing blood flow to vital organs. Children with the disease—who can only expect to live until their mid-40s—often experience sudden, severe joint pain; have progressive lung, kidney, and liver damage; and are at an increased risk of having a stroke.

But children with sickle cell disease have a formidable ally: Michael R. DeBaun, assistant professor of pediatrics and of biostatistics at the School of Medicine. Since joining Washington University in 1996, DeBaun has been on a mission to help these children.

"I saw a group of kids who were up against the odds, and no one was really championing their cause," DeBaun says.

 

"Michael DeBaun is terrific to work with," says Desiree White, assistant professor of psychology in Arts & Sciences. "He is always on the move and always looking for opportunities to try to help these kids."

 

In 1997, DeBaun started the Sickle Cell Disease Medical Treatment and Education Center at the School of Medicine and St. Louis Children's Hospital. The center has a multidisciplinary team that includes a hematologist, a neurologist, a neuroradiologist, and a neuropsychologist. It also is the only site in this region that combines research, patient care, and education.

As part of the Sickle Cell Center, DeBaun also started an area summer camp (the only one of its kind in the St. Louis area) for children with sickle cell disease and helped establish a program with the American Red Cross Missouri-Illinois area (for which the organization awarded him a 2001 National Diversity Outreach Award) to increase the number of African-American blood donors—children who've suffered a stroke may require monthly blood transfusions.

Before their 14th birthday, 20 percent of children with this disease will suffer a silent stroke—one with no visible signs. The strokes occur in a section of the brain that controls cognitive skills, so they have problems with attention, language, memory, and problem solving. The telltale sign they have had a silent stroke is that they start failing in school.

In the past, many of these kids were not identified; after failing a grade, they got discouraged and dropped out of school.

"Typically, these kids have fallen between the cracks," DeBaun says.

DeBaun's clinical research focuses on this group of children. He is trying to best identify children who have had silent strokes, the risk factors for strokes, and the best way to help affected children catch up at school.

"It was not a well-recognized problem in the medical community," says Michael J. Noetzel, associate professor of neurology and pediatrics, and the neurologist at the Sickle Cell Center. "Michael has brought this problem to the forefront not only in the greater St. Louis area but also nationally."

If a student starts having problems in school, it is a sign that further tests are needed. DeBaun arranges for the child to have a brain MRI. Robert C. McKinstry III, assistant professor of neuroradiology and the center's neuroradiologist, interprets the MRI to see if a silent stroke has occurred.

If it has, the center's neuropsychologist Desiree A. White, assistant professor of psychology in Arts & Sciences, tests the child's cognitive abilities. "We use a lot of the tests that a clinical neuropsychologist would use to assess any child with brain damage," White says.

 

Desiree A. White (left), assistant professor of psychology in Arts & Sciences, is the Sickle Cell Center's neuropsychologist. White tests children's cognitive abilities to assess possible brain damage resulting from silent strokes. Above, she works with Ebony Lucas, age 10.

 

She sends the child's school a cognitive report that helps teachers know the student's strengths and challenges. "We want to empower the families to solicit the school district to get the best educational resources for children once we know their strengths and weaknesses," DeBaun says. "This is a very important part of what we do. Sixty percent of the children with silent strokes will be detained a grade or need special education. Who ensures they will get what they need?"

DeBaun's colleagues describe him as determined and inquisitive. "Michael DeBaun is terrific to work with," White says. "He is always on the move and always looking for opportunities to try to help these kids."

And his drive inspires those who work with him. "He is consumed by this quest to offer children with sickle cell disease hope, and it's infectious," McKinstry says. "You know he's doing the right thing, and you're willing to make personal sacrifices to help him achieve this goal."

DeBaun also has a close relationship with his patients and their families, and many parents feel as if he cares about more than just their child's medical condition. "He's awesome," says Cynthia Harmon, whose daughter, Alexandria, is one of DeBaun's patients. "He's brought something really special to all of the kids here [at the center]."

 

Dr. DeBaun makes sure that his patients' visits to the Sickle Cell Center are about more than receiving medical treatment. Playing video games are (from left) Diamond Penn, age 15; DeBaun; Derrell Brothers, age 4; and Isaac Cornell, age 7.

 

DeBaun grew up in University City, the son of a McDonnell Douglas employee and an elementary school teacher at Montessori Euclid School. DeBaun's close-knit family gave him a strong sense of self. He also believes his enthusiasm for life began at home.

He attended St. Louis University High School, where he learned an outstanding code of discipline. While in high school, DeBaun decided to become a physician. He was heavily influenced by his pediatrician, Helen Nash, a well-known St. Louis physician who cared for his family. "She was the only powerful figure that I knew," DeBaun says. "I always admired her. She was at the vanguard for children's health issues in St. Louis."

He then attended Howard University as an undergraduate, attended medical school at Stanford University, and returned to St. Louis in 1987 for a pediatric residency, chief residency, and fellowship in pediatric hematology/oncology at the School of Medicine. During his fellowship, DeBaun began studying strokes in children with sickle cell disease.

He then decided to pursue a master's degree in public health from Johns Hopkins University School of Hygiene and Public Health, receiving the degree in 1993, as well as an epidemiology fellowship at the National Cancer Institute for three years.

DeBaun was recruited to the School of Medicine in 1996 as an assistant professor of pediatrics. "Michael DeBaun is a fine clinical scientist, as recognized by his receipt of the very competitive Doris Duke Clinical Scientist Award," says William A. Peck, executive vice chancellor for medical affairs and dean of the School of Medicine. "His work has led to enhanced understanding of the complications of sickle cell disease. He is a great representative of Washington University and a wonderful individual."

DeBaun attributes much of his success to the support he has received from his wife of 15 years (and friend of 20), Sandra Alsup DeBaun. "A large part of my professional accomplishments are a direct reflection of our partnership," he says. "Sandra is an unselfish, silent member of the team. Although I work seven days a week, our home life remains stable for our children, Malcolm (14) and Morgan (11). What's more, her encouragement for me to persevere despite the odds and the obstacles has kept me on course. She is the reason that I can pursue my passion without hesitation."

Parents whose children are treated at the Sickle Cell Center hope researchers will one day find a cure for this devastating disease. In the meantime, having DeBaun care for their children gives them some comfort. "I took Karra to the Sickle Cell Center, and my life has been better for two years," Kent says. "That's because Dr. DeBaun took an interest in Karra, and he still does. Every time I think about him, it's with such warmth and admiration for the things he has done for us. My daughter and I tear up when we talk about him."

Diane Duke Williams is a free-lance writer based in St. Louis.

 

 

 

 

 

Dr. Michael DeBaun is close to his patients and their families. He helps Martisha Wilson feed her 15-month-old son, Aaron Smith IV, during a recent visit to the Sickle Cell Center.