Saving Sarah; Little Brother's Cells Give Belleville Girl a Chance For a Normal Life
By Roger Schlueter
Belleville News-Democrat (IL)
March 24, 2002
A child's birthday is supposed to be a happy, carefree time for families. But when her daughter turned 4 in June 1995, Lori Cable had just one wish as little Sarah blew out the candles on her cake.
"I just prayed that she would have another birthday," recalled her mother, distant memories welling up again in her voice.
Her prayer was granted a month later in a way that made St. Louis medical history.
After battling a potentially fatal blood disorder for two years, Sarah Cable underwent the area's first umbilical cord blood transplant. It was an infusion of life-saving stem cells that had been saved and stored from the birth of her baby brother, Shawn, in January 1995.
In record time, Sarah was out of Cardinal Glennon Children's Hospital. Within six months, she was off all medications as her body finally began producing the blood cells it needed to stay healthy.
Nearly seven years later, the Belleville family hardly gives it a second thought. There's just no time amid Sarah's dance classes and volleyball games and a thousand and one other things that keep the energetic 10-year-old hopping. WHAT GRADE IS SHE IN AT WHAT SCHOOL?
"Great --- she's great," Lori Cable said as her daughter looked on with a big smile. "There's no reason why she shouldn't just be normal, healthy and happy. She should have no side effects from any of this."
"We're happy that it's over and everything turned out OK, so we don't really dwell on it that much," said father Danny. "It's just amazing how smoothly a life-threatening disease can go."
Smoothly?
For two years, the Cables took Sarah on weekly trips to St. Louis for blood transfusions. Meanwhile, anxious parents, friends and family scoured the Internet for information and new treatments as they prayed for a cure.
Still, for a disease that can have a high mortality rate, little Sarah astounded even her doctors by her ability to stay healthy.
The first sign of trouble popped up at Sarah's second birthday party in 1993. Always a healthy baby, the toddler appeared tired and "not herself," although the Cables chalked it up to the excitement of the day. When Sarah developed a cough days later, Lori asked her husband to take her to the pediatrician.
The pediatrician told Danny to take Sarah to a local hospital for a blood test. From there, the stunned 25-year-old father was advised to head straight to Children's Hospital in St. Louis. The best guess: leukemia.
After a week of tests and waiting, the Cables learned it wasn't leukemia but something potentially as dangerous: aplastic anemia. Sarah's bone marrow had stopped making the red blood cells that carried oxygen, the white cells that protected her body against infection and the platelets that aided in clotting.
Worse, aplastic anemia can occur in a range of severities, depending on how low those blood cell counts are. The Cables learned that little Sarah had a "very severe" case. The mortality rate for children after a year could be 50 percent or more.
"That just kind of made my heart sink," Lori Cable said. "They kept talking about `quality of life' and we knew that meant `not good.'"
Doctors weren't overly enthusiastic about potential cures. One involved giving her the chemotherapy drug ATG followed by a bone-marrow transplant if a related donor could be found. But the chances of it working were only 50-50. Because Sarah was still healthy, they could not recommend it.
So, week after week, Sarah went to "Jean's house" --- the name she gave Children's Hospital because her main nurse was Jean. Once a week, she would get a platelet transfusion; once a month, they would infuse her with red blood cells.
"It was actually a fun time for her," her mother remembers. "She had a Portacath in her chest so her hands were free to play with the toys. So she didn't feel that she was any different."
Less drastic remedies were tried. In case her body was destroying its own blood cells, doctors gave her a six-month regimen of the immunosuppressive drug cyclosporine without success. Her mother also gave her nightly shots of a substance designed to stimulate the growth of what stem cells she had left. This also seemed to have little effect.
Doctors began hinting at the inevitable. Bone marrow transplants were risky, but a brother or sister might improve the odds by offering a better marrow match. If Lori and Danny were thinking of having a second child, now might be the time.
Suddenly, everything started falling into place. First, Danny Cable discovered a story on the Internet describing the "tremendous" success European doctors were having in the use of umbilical cord blood transplants.
Such blood is rich in stem cells, the precursors of all blood cells. Normally just thrown away with the cord after birth, it is easily recovered with absolutely no discomfort to either mother or infant. It can then be packaged and frozen for use months or years later.
Then, Dr. Donna Wall, the doctor who diagnosed Sarah, left Children's Hospital to start a cord blood bank at Cardinal Glennon Hospital for Children across town. In the spring of 1994, Lori became pregnant with Shawn.
"We asked Dr. Wall, `Should we save his cord blood?'" she said. "We didn't know if we'd use it, but we said we'll do it so we have it if we want it."
By the time Shawn was 6 months old, the Cables got another dose of good news: Shawn's marrow and cells were a perfect match with Sarah's.
"I screamed," Lori Cable remembers of hearing the news. "I remember that day clear as a bell. I was ecstatic."
Still, could they trust Sarah's life to a treatment that had yet to be tried in St. Louis? The Cables visited Indianapolis Children's Hospital, where doctors also recommended a bone-marrow transplant, not cord blood cells.
"But everyone told us, `There's no right answer,'" Lori Cable said. " `Whatever you choose, if it's not the right outcome, don't blame yourself.' They were all very supportive."
Finally, Wall and the Cables reached a happy medium: In addition to the cord blood cells, doctors also would take a small amount of Shawn's bone marrow and infuse it into Sarah, too. To do this, they were hoping to wait until Shawn reached his first birthday, but in July Sarah's blood count numbers suddenly bottomed out. It was then or never.
On July 21, Sarah began undergoing four days of a mild chemotherapy that would kill off the rest of her immune system. After a day of rest, doctors infused Sarah with her brother's cells and marrow from a bag labeled "To Sarah with love, Shawn."
It was time for Lori and Danny to hold their breath again. Defenseless against infection, Sarah was quarantined in the transplant ward. Instead of playing with her dolls (which weren't allowed), Sarah would watch TV, suck her thumb and rub her nose with nurses' latex gloves that came to be a kind of security blanket for her.
"I remember watching `Lady and the Tramp' one time and doing that," Sarah said.
Never losing her spirit, Sarah would be the first one up at the hospital. Her room faced east, so she would announce to whatever tired parent was staying with her, "Sun's up! Time to get up!" However, her parents needed their own security blanket after being told that the transplant would kick in after 10 or 11 days.
"And, it kind of went a day or two beyond that," Danny Cable said. "We were nervous. Then, all of a sudden, when it hit, it hit."
Sarah was back home three weeks after the transplant, more than a week sooner than any other case. The Cables were becoming a normal family again.
"This was the first meal we had together in a month," Lori Cable said as she showed off an album of photographs dedicated to her daughter's transplant. "I hadn't seen that look of contentment on (Shawn's) face for a long time."
For the next three months, Sarah could have no raw fruit or vegetables, no leftovers, none of her favorite McDonald's. Everything had to be individually packaged and cooked very well. Anyone with the slightest sniffle couldn't come near as her immune system restored itself.
Shawn's cells never attacked Sarah's body as sometimes happens after a transplant. Shawn was unfazed by it all, learning to walk soon afterward.
Now as Sarah approaches her 11th birthday, the weekly visits to "Jean's house" have long since ended and the hats that a friend's wife made for her to hide her bald head are buried in a box somewhere. But her ordeal is never quite forgotten.
"I think we're really fortunate to have the hospitals that we have here for children's care," Danny Cable said. "People ask us how you get through something like that with your kids. You don't even think about it. You just do it."
