THE VIRGINIAN-PILOT
Copyright (c) 1996, Landmark Communications, Inc.
DATE: Saturday, September 14, 1996 TAG: 9609140221
SECTION: FRONT PAGE: A1 EDITION: FINAL
SERIES: Sickle Cell Anemia
SOURCE: BY DEBRA GORDON, STAFF WRITER
LENGTH: 316 lines
Christopher Smith has had sickle cell disease for all of his 17 years. A year ago, complications from the disease sapped his will to live. Then his doctor suggested that an experimental bone marrow transplant might give him a chance at a normal life. It took Christopher's mother a year to agree to the transplant, which is so risky it could kill him. <
For 17 years, Christopher Smith has battled the monster that lives in his blood.
Seventeen years of doctors' appointments, hospital stays, blood transfusions and medications, all trying to tame his sickle cell disease.
The disease, also called sickle cell anemia, affects one in every 400 African Americans - 2,400 in Virginia. His parents had unknowingly bequeathed it to him, along with high cheekbones, liquid brown eyes and slight build, in their genes.
The monster then spawned its own offspring throughout his body: stroke, heart disease, asthma, gallstones, depression.
Seventeen years of living with the disease had turned a witty, fun-loving, extremely bright child into one who hid his gifts, becoming a sullen, distrustful teen-ager.
A year and a half ago, tired of monthly transfusions, of almost daily needles, of handfuls of pills, tired of the pain, Christopher Smith decided to quit.
For three weeks, he stopped taking the drugs that kept his disease in check.
To his parents, doctor and therapists, he was committing a slow suicide.
Then Christopher's doctor suggested one last shot - a bone marrow transplant.
It's a procedure used to treat terminal cancer patients. In recent years, it has been tried on 22 sickle-cell sufferers nationwide, with mixed results. Sixteen were cured, two died and the treatment failed in four.
For children with terminal cancer, the decision to have a bone-marrow transplant is relatively clear: Try it or die.
For children like Christopher, who could live another 30 years or more with sickle cell disease, the decision is more complicated:
Try it and you may die.
But there is the chance of wiping out the disease, of a normal life.
Christopher made up his mind almost immediately. Go for it.
On Wednesday, doctors at St. Jude's Hospital in Memphis will launch medicine's most lethal weapon against Christopher's disease. They'll infuse bone marrow from Christina, his 10-year-old sister, into his vein, making him the first person from Hampton Roads to undergo this dangerous cure.
Then they will wait to see if it works. If the spongy red marrow from a little girl can finally vanquish the monster. The monster is born
Norma and Roosevelt Smith, Christopher's parents, grew up as best friends in Queens, New York.
They met when he was 9 and she was 7. By the time Norma was 15, she told a friend: I'm going to spend the rest of my life with Roosevelt.
At 17, Roosevelt enlisted in the Navy. That's when a routine blood test showed that he carried the sickle cell trait.
One in 12 African Americans carry the genetic marker for sickle cell disease. If two carriers marry, they have a one-in-four chance of having a child with the disease.
Norma's mother assured her daughter she didn't have the trait, but, like most members of her generation, Norma had never been tested. It wasn't until a prenatal blood test when she was 16 and four months pregnant with Christopher that she learned she was a carrier.
Maybe, she wrote Roosevelt - who was out at sea - the baby won't get it.
But Christopher was only three months old when she read the letter from the New York City Public Health Department: He had sickle cell.
The disease causes normally round red blood cells to deform into a sickle shape. The cells clog tiny blood vessels, cutting off the blood supply and causing organ damage and bouts of severe pain.
It would be another year, however, before her baby felt the fury of the disease. That's when the first pain crisis hit.
Called ``foot-and-hand disease,'' it resulted when sickled blood cells backed up in the joints of Christopher's limbs, causing severe swelling. The pain was so intense the toddler stopped walking, spending days just lying on blankets between his parents.
Norma, only 17, had to crush codeine pills into Christopher's applesauce.
It was a terrible time. Every ear infection, every slight fever, meant a hospital stay. The most common cause of death in sickle-cell children is from bacterial diseases like septicemia or meningitis.
To prevent infection, Christoper took daily doses of penicillin. To increase the amount of oxygen and normal cells in his blood, he had several transfusions.
Then his heart failed when he was 6.
Christopher learned to protect himself against infection, refusing to eat from anyone else's plate or drink after anyone else.
He learned to handle the pain, often refusing narcotics.
Few of his friends even knew that this fun-loving, basketball-playing kid had a life-threatening disease.
When he was 8, the family moved from New York City to Virginia, where the cleaner air was easier on his asthma.
Christopher hated the move. He went from a straight-A, second-grade student in New York, so bright that teachers wanted to skip him to the fourth grade, to one who tested at a first-grade level.
Norma argued with the teachers, and they agreed to put him in the second grade. But Christopher just sat in class. He didn't bring pencils or paper. He wouldn't open his books.
He had begun his downward slide.
Then, at age 11, came the stroke.
About 10 percent of ``sickler'' children have strokes, caused when the sickled cells block blood flow to the brain. Christopher's stroke came on the heels of a lung infection, also related to his disease.
The two together brought the 11-year-old within hours of dying.
But Christopher was a fighter. He pulled through with no lasting physical damage from the stroke.
But his near brush with death changed him. While recovering in the hospital, he refused to eat or talk to anyone.
Already far behind in school, his performance worsened. His social worker tried to get him into a gifted program, but his grades and attitude were roadblocks.
``I don't think he ever fit in,'' said Mitzi Stallings, the social worker at Children's Hospital of The King's Daughters. ``And the response of his teachers has not been one that has lifted him up. So he just says, `Oh, well. Whatever I do is not going to work anyway.' ''
By age 16, he was still in the ninth grade, two years behind. Giving up on the dream
Ever since he was small, Christopher had dreamed of becoming a veterinarian. At one point, his animal menagerie consisted of a dog, a ferret, a parakeet, a hampster, rats and an iguana. When the ferret broke the parakeet's wing, Christopher nursed it back to health, slicing off tiny bits of his own penicillin pills and feeding them to the sick bird.
Christopher dreamed big. He didn't want to be just an ordinary dog-and-cat vet. He wanted to be a herpetologist - specializing in reptiles.
Then he figured out how long it would take. The extra training, coupled with the years he'd already lost in school, meant he'd be 42 by the time he started practicing.
He knew he wouldn't live past 50.
When the dream died, so did Christopher's will to live. He withdrew even more. He was failing ninth grade again. His answer to most questions became: ``Why do you want to know?'' It was as if his life were so unimportant it just wasn't worth talking about.
He was so certain he was going to die, he began to view the treatments and medications as a waste of time.
Just before his 16th birthday, he told his mother he'd conducted his own test.
I stopped taking the medicine, Mommy, he said.
Norma checked and discovered dozens of pills in his room. She started crying. He's going to die, she thought. He's killing himself. A dangerous experiment
A few weeks later, Christopher's doctor recommended the bone-marrow transplant. The complications of his disease put Christopher into that tiny 6 percent subset of sickle-cell sufferers who qualified for the transplant.
To reduce the risk of another stroke, he underwent monthly blood exchanges, in which his damaged red cells were filtered out of his blood and replaced with healthy ones. It wasn't a permanent fix because the cells only lived a short time. By the end of the month, Christopher's bone marrow would replace the healthy cells with sickled ones and the cycle would begin again.
Eventually, doctors knew, Chris' body would not tolerate the exchanges, and his risk of stroke would dramatically increase.
But the transplant is equally risky.
It involves killing off Christopher's own marrow - the substance inside the bones that makes blood cells - with massive doses of chemotherapy. Then, the marrow will be replaced with a donor's.
It takes a month for the new marrow to produce an immune system. During this time, the slightest infection could be deadly. The drugs could also cause another stroke; they will almost certainly make him very, very sick.
Then there's the risk of graft-versus-host-disease, which occurs when the new immune system attacks the rest of the body, damaging the skin, liver and gastrointestinal tract.
There was another major hurdle Christopher had to overcome - transplants on sickle-cell sufferers are only done if a perfect sibling marrow match is found.
In that, Christopher was lucky. Norma and Roosevelt had three other children. They all carry the sickle cell trait - but none has the disease.
Two of his siblings - Christina and one of his brothers - matched. Doctors decided to use Christina because, since she is a girl, they'd know sooner if her marrow worked because they'd see her unique X-chromosome in his blood cells.
Christopher had named Christina himself when she was born, calling her ``his baby,'' and becoming fiercely protective of her.
Now it would be her turn to protect him. A mother's dilemma
News of the transplant broke through Christopher's shell. He felt ``excited and happy.'' Here, finally, was something that gave him the promise of a normal life, as opposed to the temporary measures of transfusions and pills.
Christopher and his father had decided they wanted the treatment. But Norma wouldn't discuss it.
She, more than either of them, understood the risks. She read medical journals the way others might read supermarket tabloids.
She'd spent hundreds of hours sitting in the cancer clinic at Children's Hospital, listening to parents talk about their kids' bone-marrow transplants. Those parents had no other option. She did.
How could she take her child, who had no risk of immediate death, and deliberately put him at risk of dying?
Every month when she returned from Christopher's transfusions, her husband asked: ``Did you tell the doctor? Did you tell him yes?''
And she lied: ``Oh, I forgot.''
Then it was April 17. The day before Christopher's 17th birthday, almost exactly one year since his doctor, William Owen of Children's Hospital, had recommended the procedure.
Christopher was worse. He'd stopped the treatments that kept iron from building up in his liver and kidneys. Soon, the buildup would begin causing irreversible damage. Norma had to dole out his pills to him every day, unable to trust that he'd take them on his own. Sometimes he'd disappear, overnight or for several days, with no medication.
The doctor decided to try one more time.
``It's been a year,'' he said gently. ``If we're going to do something, we've got to get moving on it.''
And just like that Norma knew.
``We're going to do it.'' The leave-taking
``Come on, Christopher, get moving!'' Norma came as close to shouting at her oldest son as she ever did, and even then her voice was barely raised.
Open suitcases and piles of clothes lay on the living room floor of the Smiths' Virginia Beach home. It was 8 a.m. , Sept. 3, and Christopher, Norma and Christina were supposed to be on a plane to Memphis in an hour and a half.
Norma had been up all night, sleepless with worry and the pressing need to finish painting furniture for her younger sons' room. It was the last thing she'd be able to do for Ashlee,7, and Aaron, 4, for a while.
For although St. Jude's Hospital agreed to pay for the entire, $150,000 procedure after the Smiths' insurance company denied the still-experimental treatment, the financially strapped family couldn't afford for Roosevelt and the other Smith children to spend more than a week in Memphis. Norma, who'd never spent more than a week away from her kids, wouldn't see them for months.
Today was the first day of school, but only Ashlee was loading a book bag. Christina wasn't going to start until she returned from Memphis, sometime in late September.
Aaron, who'd always been home with Norma, would start nursery school next week - without her.
And it seemed everything had gone wrong, starting with a mix-up in the reservations.
Now, the tickets were supposedly waiting at the airport, but she couldn't get Christopher moving. He was using his favorite weapon against her. The more she begged him to speed up, the slower his movements became.
``Finish so we can leave!'' she pushed at him. He just looked at her, then slowly lifted a shirt into the suitcase.
Norma lit a cigarette and surveyed the mess in the living room. She checked her watch. Forget it, she thought. We are just not going to make this flight.
When they missed a second flight after arriving at the airport that afternoon because of a ticket mix-up, Norma was frustrated.
``The devil is out working,'' she said, as the family left the airport for home.
By 7 p.m., they were back. Tears in Norma's eyes as she and Roosevelt held hands, saying goodbye. Christopher hid behind his tough-guy mask, shrugging off the attention and emotion.
``See you,'' he joked to Christina, as she got in line to board the plane while he remained seated in the airport.
``Come on, Chris,'' Norma called again.
And with a sly smile and his customary slow amble, Christopher Smith moved into line, ready for his own final face-off with the monster. MEMO: Coming next week: Christopher Smith gets his bone marrow
transplant.
GETTING TESTED
The only way to know if you carry the gene for sickle cell disease is
with a blood test. There are no symptoms.
Forty-two states, including Virginia, automatically test all newborns
for the disease and for the genetic trait. But those programs are
relatively new - Virginia's program began in 1989 - so most people
don't know if they carry the gene.
Even though sickle cell disease is primarily found in the
African-American community - one in 400 blacks has the disease, one in
12 carries the trait - officials say everyone, particularly those of
Mediterranean descent, should be tested.
If two people who have the recessive gene for sickle cell marry, they
have a one-in-four chance of having a child with the disease.
In the early 1970s, the federal government began a large genetic
screening program for sickle cell.
But the program was unsuccessful, according to the results of a 1990
study published in the medical journal ``Science,'' mainly due to lack
of education and counseling. The end result, the authors noted, was that
few people were screened.
But the pendulum may be swinging the other way, health officials say.
The Sickle Cell Disease Association of America, based in Los Angeles,
has made awareness and education its primary goal, said a spokesman, and
is trying to reach potential carriers through the media, the Internet
and other communication methods.
In Hampton Roads, the Society for the Aid of Sickle Cell Anemia
offers free testing, counseling and support.
Today, the society is sponsoring a sickle cell walk-a-thon at Norfolk
State University. The event includes a picnic and children's activities,
and participants will be able to sign up for testing.
The society is asking for a $50 donation from all participants, which
includes the picnic and a T-shirt.
For more information about testing, contact the society at 624-9225.
The national sickle cell association may be reached at
1-800-421-8453. ILLUSTRATION: BILL TIERNAN color photos, The Virginian-Pilot
Christopher Smith, 17, center, and Norma Smith, of Virginia Beach,
at right in white shirt, are surrounded by their family and friends
as their former priest, the Rev. John Boddie, blesses them before
their journey to Memphis.
On Wednesday, Christopher, above, will have a bone marrow
transplant that may cure his sickle cell disease. The donor will be
his sister Christina, 10, below.
BILL TIERNAN photos, The Virginian-Pilot
After six years of monthly blood exchanges, Christopher finishes
what he hopes will be his last one at Children's Hospital of The
King's Daughters in Norfolk in late August.
Roosevelt Smith, left, sits with his son Christopher, right. When
Christopher's dream of becoming a veterinarian died, so did his will
to live. But when he learned that the experimental bone marrow
transplant might give him another chance, he wanted to grab it.
Chart
Source: Mayo Clinic Family Health Book
About Sickle Cell Disease
For complete information see microfilm by CNB