THE VIRGINIAN-PILOT
Copyright (c) 1996, Landmark Communications, Inc.
DATE: Thursday, August 8, 1996 TAG: 9608080379
SECTION: FRONT PAGE: A1 EDITION: FINAL
SOURCE: BY DEBRA GORDON, STAFF WRITER
DATELINE: VIRGINIA BEACH LENGTH: 138 lines
Christopher Smith doesn't look sick. His well-muscled arms can pump a basketball as well as any other 17-year-old's.
But in his veins lurks a disease that gave the Virginia Beach youth a stroke at age 11. To survive he must undergo blood transfusions every month and keep a needle in his arms seven hours a day, five days a week.
He has sickle cell anemia, a genetic disorder in which normally round red blood cells are deformed into a sickle shape. The cells clog tiny blood vessels, cutting off the blood supply and causing organ damage and bouts of severe pain. The disease affects about 80,000 people, 2,400 in Virginia, most of them African Americans. Until recently, there was no cure.
In three weeks, Chris will undergo a bone marrow transplant, a highly risky procedure that puts him at the cutting edge of what medical science offers as a cure for this disease.
In doing so, he will become the first person from Hampton Roads, possibly from Virginia, to use the dangerous procedure to cure sickle cell anemia.
Today, the New England Journal of Medicine publishes the first major study heralding bone marrow transplants as a cure for sickle cell anemia. Evidence has been building for more than a decade that transplants can reverse the disease, and the new study, based on 22 children in the United States and abroad, offers proof.
Bone marrow makes blood cells. The treatment cures the disease by replacing the victim's unhealthy bone marrow, which produces the sickle-shaped blood cells, with healthy bone marrow that will produce healthy blood cells.
But while the study found that three-quarters of victims are cured, it also noted that 10 percent die from the treatment itself. In the study, 16 children were cured, two died and the treatment failed in four.
Those are odds Roosevelt and Norma Smith are willing to take. Without the marrow transplant, they said Wednesday, their son is on a path of self-destruction toward an almost certain early death.
For 17 years, Christopher's sickle cell anemia has ruled the Smith household.
Although pain is often a problem with sickle cell - some describe it as a hammer beating on their bones - it is fever that is life-threatening, because kids with sickle cell have trouble fighting off infection.
Chronic respiratory infections plagued Christopher throughout his childhood. He was hospitalized four or five times a year - whenever he ran a temperature of 101.
His parents finally moved from New York City to Virginia Beach, hoping that a gentler climate and cleaner air would reduce his illnesses.
It worked for a while. Until, while hospitalized for another chest infection when he was 11, Christopher had a stroke.
Eight to 10 percent of children with sickle cell anemia have strokes by the time they're in their teens. Each has a 70 percent chance of having another.
To minimize the chance of another stroke, Christopher began receiving weekly blood transfusions. Eventually, they were reduced to once every five weeks.
But the transfusions caused iron to build up in his blood. To rid his body of the excess iron, Christopher had to keep a needle under his skin seven hours a day, five days a week, as part of a painful treatment called ``chelation.''
It is children like Christopher, who have severe complications from their sickle cell anemia, that today's article recommends for the bone marrow transplant. The study estimates that only about 6 percent of sickle cell victims meet that criteria.
That's because the cure carries its owns risks - including early death. Christopher's own marrow, which makes blood cells, has to be destroyed by chemotherapy before the transplant. So the slightest infection before the transplant is effective could kill him, because his body will be unable to make disease-fighting white blood cells.
Then there is the risk that the transplanted tissue might view Christopher's body as foreign and attack it - a common, often fatal, complication. Even if Christopher survives the transplant, he faces an increased risk of sterility and cancer later in life.
All of which creates a difficult ethical dilemma for doctors like William Owen, the Norfolk hematologist who has treated Christopher for years.
It was Owen who, a year ago, recommended that the Smiths consider a marrow transplant for Christopher.
He has only suggested the treatment to one other of the more than 300 children he sees in the sickle cell clinic he directs at Children's Hospital of The King's Daughters. That child couldn't find a donor.
But Owen thought the procedure was worth the risk for Christopher, he said, because the disease was ruining the boy's life.
``He was not living like a normal child and adolescent, because of the disease,'' Owen said.
Christopher was depressed, withdrawn and doing poorly in school.
Without the transplant, he faced a life of blood transfusions and chelation therapy, along with the risk of severe organ damage if he couldn't control his iron levels.
But his parents feared that once he reached adulthood, Christopher would refuse the transfusions, quit the chelation and stop taking his medication.
The bone marrow transplant, they felt, was their last chance to save his life.
On April 18, Christopher's 17th birthday, the Smiths decided to try the transplant.
First, they had to find a donor. The New England Journal study found that only an estimated 18 percent of sickle cell patients have a suitable marrow donor - usually a sibling. Luckily, Christopher has three younger siblings. His 10-year-old sister Christine was a perfect match.
So on Aug. 28, Christopher and his mother will travel to St. Jude Children's Research Hospital in Memphis, Tenn.
There, doctors will harvest some of Christopher's diseased bone marrow. If he rejects his sister's marrow, they will give him back his own - although that would also give him back the disease.
On Sept. 18, doctors will transplant Christine's marrow. If the procedure works, it will be another four months before Christopher can go home; and then eight more months before he can go to school.
Most insurance companies, including the Smiths', won't pay for the transplant because it's considered experimental for treating sickle cell anemia. So St. Jude's is covering the entire $150,000 cost of the procedure, including living expenses for Norma and Christopher.
Roosevelt will stay in Virginia Beach, working nights at his job at Stihl Inc., while caring for the three younger children.
For Christopher, the transplant means the possibility of a life without needles and pain. A normal life.
A life he's confident will come.
``I feel everything will work out,'' he said Wednesday. ``And that we're going to go for the gold.'' ILLUSTRATION: Color photos
BILL TIERNAN/The Virginian-Pilot
THE DISEASE: A genetic disorder in which red blood cells are
deformed into a sickle shape. The disease cuts off blood supply,
causing organ damage and bouts of severe pain. About 80,000 people,
2,400 in Virginia, have the disease. Most are African Americans.
THE CURE: Bone marrow transplant is a cure for many with a severe
form of the disease. It is a risky procedure, and about 10 percent
of recipients will die from it.
THE PATIENT: Christopher Smith, left, of Virginia Beach hopes a
transplant of his sister Christina's bone marrow can cure his sickle
cell anemia.
Photos
Sickle cell, above, and normal cell.
KEYWORDS: SICKLE CELL ANEMIA DISEASES by CNB