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Tuesday • January 31, 1993
"PHE J^A I FAI ION
The Battalion • Page 9
Doctors discover first effective
treatment for sickle cell anemia
BETHESDA, Md. (AP) — A can
cer drug has proved to be the first
effective treatment for sickle cell
anemia, a disabling blood disorder
affecting 72,000 black Americans.
The drug hydroxyurea reduced the
excruciating attacks of sickle cell so
dramatically that the National Insti
tutes of Health ended drug trials four
months early, and on Monday noti
fied 5,000 doctors of the treatment.
“Patients must understand hy
droxyurea is a treatment, not a
cure,” cautioned Dr. Samuel
Charache of Johns Hopkins Univer
sity, who led the study funded by
the National Heart, Lung and Blood
Institute. And the drug, which car
ries some risks of its own, is not for
all sickle cell sufferers.
Still, “it’s very exciting,” said
Ralph Sutton of the Sickle Cell Dis
ease Association of America. “This
means a significant improvement in
the quality of life for people with
sickle cell disease.”
Sickle cell anemia, an inherited
disease common among people with
ancestors from Africa, the Middle
East and the Mediterranean, causes
hemoglobin inside red blood cells to
clump together. That changes the
normally round cells into a sickle
shape that can’t squeeze through
tiny blood vessels.
Patients, whose tissue doesn’t get
adequate blood, suffer pain so se
vere it frequently requires long hos
pitalization. The disease eventually
causes organ damage, and patients
frequently live only into their 40s.
About 8 percent of black Ameri
cans carry the gene. There is no cure,
only treatment for pain, and until
now, no way to prevent symptoms.
In the study released Monday,
doctors tested 299 adults with se
vere sickle cell beginning in 1992.
Half took hydroxyurea every day,
the others a placebo. Hydroxyurea
reduced by 50 percent patients’ pain
episodes, hospitalizations, need for
blood transfusions and cases of a
life-threatening complication called
acute chest syndrome.
Ruthie Abney, 40, of Washington
once endured weeklong hospitaliza
tions at least eight times a year.
Since beginning the drug in 1992,
she has suffered only six pain
crises, none severe enough to re
quire hospitalization. “I can’t begin
to relate to people what it means to
be pain-free and live a normal life
like anyone else,” she said Monday.
Hydroxyurea appears to work by
stimulating the body to produce a
type of hemoglobin found in fetuses,
a kind that resists sickle cells’
clumping. “It’s like the chaperone at
a dance, keeps the molecules from
getting too close together,”
Charache explained.
After birth, fetal hemoglobin’s
gene becomes dormant and an adult
type susceptible to sickle cells’ sticki
ness forms. Monday’s study found
the cells of hydroxyurea takers con
tained 20 percent fetal hemoglobin —
enough to battle the disease.
Hydroxyurea is already on the
market as a cancer drug, and doc
tors can legally prescribe any drug
for any purpose.
Nuclear accidents, radioactive
pollution plagues Russian village
MUSLIUMOVO, Russia (AP) — The
shallow creek runs beneath an abandoned
mill. Cows wander knee-deep in the water.
In the summer, it is where the village’s chil
dren swim.
This pastoral scene is deceptive, howev
er. The Techa River is radioactive and has
been for almost half a century.
The nearby Mayak nuclear complex, also
known as Chelaybinsk-65, began dumping
raw nuclear waste into the Ural Mountains
river in 1949, when it built the Soviet
Union’s first reactor to produce plutonium
for atomic bombs.
By the mid-1950s, radiation at the top-
secret plant affected 124,000 people living
along the Techa, which flows through a
pretty forest and lake region.
About 20 villages around Musliumovo,
with their 8,000 to 9,000 residents, were
evacuated because radiation levels were
considered too dangerous.
Musliumovo was not, even though radia
tion in the village often exceeded that at the
evacuated sites. Many villagers suspect they
were left behind as human guinea pigs.
“For 40 years, they’ve been checking how a
living being can survive in a radiation zone,”
said Valentina Kaidaneyeva, a teacher.
“A lot of professors studying us must be
dead by now, but we are still alive,” she told
a visiting group of foreign scientists, politi
cians and reporters this fall.
Officials are at a loss to explain why
Musliumovo, 930 miles east of Moscow, was
not relocated.
“I don’t think it was done on purpose, but
probably because the village was too big
and too expensive to evacuate,” said Mira
Kosenko, an expert on radiation medicine
from Chelyabinsk, the regional capital.
Whatever the case, thousands of people
remained in Musliumovo, using the river
water for their households and letting cattle
graze in contaminated fields, unaware of
the poison creeping into their bones.
The former Soviet Union zealously
guarded its nuclear secrets, and public
health hardly mattered. So the villagers
were not told anything about strontium-90
and cesium-137. Instead, they were told to
keep out of the river because it was diirty-
For them, the Techa was a source of life.
If they fell sick, medical personnel were un
der orders to keep silent about radiation,
Kosenko said.
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