THE VIRGINIAN-PILOT
Copyright (c) 1996, Landmark Communications, Inc.
DATE: Thursday, September 19, 1996 TAG: 9609190632
SECTION: FRONT PAGE: A1 EDITION: FINAL
SERIES: SICKLE CELL ANEMIA
SOURCE: BY DEBRA GORDON, STAFF WRITER
DATELINE: MEMPHIS LENGTH: 250 lines
On Sept. 3, Norma Smith and her 17-year-old son Christopher traveled from Virginia Beach to St. Jude Children's Research Hospital in Memphis to prepare for a bone marrow transplant to try to cure Christopher's sickle cell disease. Christopher was born with the genetic disease, which causes normally the round, smooth red blood cells to take on a sickle shape and clog tiny capillaries, causing severe pain and organ damage. Christopher qualifies for the risky and experimental transplant, which carries a 10 percent chance of death, because of the severity of his disease. His 10-year-old sister Christina donated her bone marrow for her brother. On Wednesday, Christopher got his transplant and began the long wait to find out whether it will work.
There is no emergency room at St. Jude Children's Research Hospital. No neonatal intensive care unit for babies born too early. Only 54 in-patient beds. St. Jude's has one mission: to treat kids with cancer and other catastrophic diseases.
The children are everywhere in the sprawling glass-and-brick building near the banks of the Mississippi River. Most are bald or sport wispy fringes of hair - the results of intense radiation or chemotherapy drug treatments.
They've come here from 45 states and 47 foreign countries - their travel, lodging and medical care all paid for by St. Jude's - for the experimental treatments the hospital offers. Treatments like bone marrow transplants.
Seventeen-year-old Christopher Smith is the third child to have a transplant at St. Jude's to try to cure his sickle cell disease. The other two, an 11-year-old boy from Florida and a 10-year-old girl from Jamaica, are doing fine, and doctors hope to continue the hospital's perfect record.
But things had gone awry from the day of Christopher's first treatment, on Sept. 5. That morning, doctors harvested about three cups of Christopher's bone marrow and froze it. If his body rejects his sister's marrow, they will re-infuse his frozen marrow so he won't die. But they'll also be giving him back his sickle cell disease.
Doctors also inserted a tube into the vein above Christopher's heart to provide easy access for IV fluids and drugs. This would be the conduit for the bone marrow donated by Christina, his 10-year-old sister, on transplant day.
But the two surgeries together severely stressed Christopher's battered system.
When Norma, Christopher's mother, and Christina saw him after lunch, his face was pale and puffy. His heart was beating so hard and fast that his whole body shook. Christina took one look and ran crying from the room.
Fifteen minutes later, Christopher spiked a fever, and his blood pressure plummeted. A blood transfusion brought his pressure back to normal. But Norma and Christina never made it back to their hotel that night. Instead, they slept near Christopher, Norma in a chair in his room, Christina on a fold-out couch.
Norma prayed.
``Oh Lord,'' she thought, as she watched her sleeping son. ``This is just the beginning.''
Waiting for the transplant: Taking pills and playing video games.
Christopher looked at the 26 small, white pills that nearly filled the clear plastic cup in his hand. Called Busulfan, they were a potent chemotherapy drug designed to kill cancer cells. In Christopher's body, they would wipe out his bone marrow - thus destroying his own immune system and creating room for his sister's marrow.
The pills could also cause an array of side effects: nausea and vomiting, dizziness, blurred vision, seizures, mouth sores, lung and liver damage, possible sterility.
Christopher gulped down the nasty-tasting tablets with some apple juice. Then he waited.
Nothing. And nothing six hours later, when he took another 26. Or six hours after that, with another 26. . . . Maybe, he thought, this won't be so bad.
A week later, however, his optimism had plunged. The boy who spent hours on the basketball court was barely able to complete two miles on the stationary bike in his room. A short game of ring toss with the physical therapist left him winded. Most of his food sat untouched - the nausea had kicked in.
And then there were the mouth treatments.
``Come on man, come on. There you go. Take it,'' his father said. Roosevelt Smith had tried teasing. Now he was going to try threats to get his son to swish the pale yellow liquid around his mouth.
``Don't make me get your mother. You know she'll squeeze your mouth open,'' he said to his son.
The Nystatin suspension was to minimize the mouth sores Christopher would develop after Wednesday's transplant, when his nonexistent immune system could no longer protect him against previously harmless germs. For the clear liquid dripping into the catheter in Christopher's chest was doing its job - destroying his bone marrow, leaving his body defenseless against a host of bacteria, viruses and parasites.
To protect him against infection, all visitors had to scrub to the elbows before entering his room. He couldn't even have ice in his ginger ale because of the mold ice machines harbored.
If he wanted to leave his room, he had to don a large, pink-and-plastic face mask, which the teen-ager, always concerned about looking cool, refused to do. So he spent his days sitting in a chair next to his bed, playing endless games of Nintendo in the oversized, peach-colored hospital room.
Roosevelt had arrived at 5:30 a.m. the day before, after a grueling 15-hour drive from Virginia Beach with 4-year-old Aaron and 7-year-old Ashlee, siblings of Christopher and Christina. They'd stay a week, possibly longer, depending on how Christopher did after the transplant.
Now the kids were bouncing off the walls in the tiny ``parent'' room next door, where a small television blared out endless cartoons and the floor was sticky with spilled soda.
Roosevelt was relieved to have the family together. The past two weeks without Norma had been exhausting. He worked nights, leaving the boys with a friend, then picking them up at 7:30 a.m., just in time to get them ready for school. He was averaging two to three hours of sleep a day.
Norma hadn't fared much better in Memphis. Deep circles rimmed her eyes after nights spent snatching an hour or two of sleep in the chair in Christopher's room.
And she was worried about Christina. The normally bubbly girl had grown quiet as transplant day approached. Instead of playing Monopoly or video games with Christopher, as she'd done earlier in the week, she now spent most of her time staring at the TV.
Around their son, however, Roosevelt and Norma followed the pattern they'd perfected in 17 years of coping with a chronically ill child: Never let him see you sweat.
They joked, teased and chattered. About the pickles on Christopher's dinner tray - which were supposed to help with nausea. About the congealed gravy Norma had ordered for his wild rice and chicken-fried steak - a meal she knew was going straight into the garbage.
But they were serious about making Chris take his medicine.
``Where is that stuff?'' Norma demanded.
``Still in his hand,'' Roosevelt said.
Twenty-five minutes later, Christopher finally swished the medicine in his mouth, then moved toward the bathroom, tugging his IV pole behind him. His father heard the water running for a few minutes and sighed. Christopher was rinsing the medicine's residue out of his mouth, nullifying its protective benefits.
When Roosevelt told Norma a few minutes later, she punched the elevator button angrily. ``This makes me real tired. Real tired.''
Wednesday, Sept. 18: Transplant Day
The night before the transplant, Christopher had a quiet talk with Christina, as his mother listened.
The harvest, he told her, would hurt. He'd felt like he'd been beat up after doctors took his bone marrow.
Chris, don't tell her that, his mother said.
``I don't want anyone lying to her,'' he said. ``It's going to hurt, and she should know that.''
He'd also told her how to breathe during the anesthesia, so she wouldn't throw up. Deep breaths in and blow it out, he said.
The next morning, as the nurse prepped her for surgery, Christina kept her eyes glued to the television, watching the movie ``Jumanji.''
``You kind of have to look at what you're doing for Chris, right?'' asked the nurse, Simone Shirley. ``You still want to do it for him, don't you?''
``Yeah,'' Christina answered, her eyes on the TV.
By 10:30 a.m., Christina was in the operating room, lying on her stomach, deeply asleep from anesthesia. ``She's all yours,'' said one of the nurses.
Two doctors, hematologists John Cunningham and Asim Belgaumi, stood on either side of the blue-draped Christina. Almost simultaneously, they each pushed hollow needles about the thickness of a coffee stirrer, into the area just above her buttocks. They pushed down hard on their needles, forcefully twisting them into the bone of her ``iliac crest,'' the cavity of the rear hip bone where the richest supply of marrow is found.
``You've hit pay dirt there,'' Cunningham said, as Belgaumi pulled up on the plunger. The syringe filled with about three tablespoons of deep red, viscous fluid - the marrow. In the next hour, the two doctors would fill their syringes about 85 times as they withdrew nearly 20 percent of Christina's bone marrow.
One floor up on the bone marrow unit, Christopher was struggling to complete a battery of psychological tests with the hospital's psychologist Dawn Vinson. The word games and puzzles measured his current emotional and intellectual functioning. They would be repeated in six months, and the scores compared.
It had taken Vinson three days to finish a two-hour series of tests with Christopher, and she was racing the clock to finish the last test before the marrow arrived.
By 11:30 a.m., the harvest was complete. Now the marrow would be filtered of the bone and fat it contained, then processed to double-check for diseases and compatibility.
It arrived on the fourth floor at 2:05 p.m.
``Christopher, I've got your transplant for you,'' called medical technician Caye Western, as she handed the bag of marrow to nurse Jo Beth Sousoulas.
Christopher, drowsy from the Benadryl and steroids he'd been given to prevent an allergic reaction to the marrow, was curled up in the chair near his bed, dozing.
``Here we go,'' Sousoulas said. ``Let me see your bracelet.'' She read off the plastic band Christopher wore on his wrist. ``Christopher Smith, 14327.''
After all the planning and worrying, the transplant itself was almost too simple, comparable to one of the hundreds of blood transfusions Christopher had received over his lifetime. Neither of the doctors was even there; the nurses handled it all.
Sousoulas hung the bag on his IV stand and attached the plastic tubing to the hub in the central line that led into the vein about his heart. The marrow would enter that vein, traveling throughout his body. Eventually, the stem cells, the special cells that made bone marrow, would settle into the middle of his bones.
Sousoulas pushed the clamp open and watched the dark red liquid slowly fill the clear tube. The transplant itself would take 2 1/2 hours, but it was just the beginning.
It will be 14 days before they know if the graft has taken, another 21 days before doctors can tell if it is Christina's marrow or leftover marrow from Christopher making the new blood cells.
If the transplant works, Christopher can leave the hospital in six to eight weeks, going only as far as the Holiday Inn in mid-Memphis that will be his home for another two months. Even if the early signs of success do come, it will be another year before doctors will dare declare that his sickle cell disease is cured.
But on Wednesday, the first step of the journey began. As Sousoulas watched the liquid inch through the tube, she uttered her customary good-luck phrase.
``OK, little cells, find your way home.'' ILLUSTRATION: Color photo
BILL TIERNAN/The Virginian-Pilot
Christopher Smith, 17, drowsy from medications he'd been given to
prevent an allergic reaction to the marrow he would get, was dozing
when nurse Jo Beth Sousoulas arrived with the bag containing his
sister's bone marrow.
BILL TIERNAN photos/The Virginian-Pilot
Christopher Smithage already in a1 cutline[sic] and his sister
Christina, 10, watch TV Tuesday, the day before Christina donated
her bone marrow.
Since his immune system was affected by chemotherapy, Christopher
Smith wears a mask when he leaves his room at St. Jude hospital in
Memphis, Tenn.
Norma and Roosevelt Smith talk with their daughter, Christina, in
St. Jude's Children's Research Hospital moments before surgery to
harvest her bone marrow.
Drs. Asim Belgaumi, left, and John Cunningham harvest bone marrow
from Christina Smith's lower back Wednesday morning. In an hour,
they filled their syringes 85 times from the ``iliac crest,'' the
cavity of the rear hip bone where the richest supply of marrow is
found. In all, about 20 percent of Christina's bone marrow was
harvested.
Graphic
TO SEND A LETTER
Write Christopher Smith c/o St. Jude Children's Research Hospital,
Room 4005, 332 N. Lauderdale St., Memphis TN 38105-3103.
KEYWORDS: DISEASES SICKLE CELL ANEMIA TREATMENT
BONE MARROW TRANSPLANTS by CNB