From: Arts and Lifestyle | Health
Tuesday, September 12, 2000
Sickle Cell - Pains and Gains
Racial prejudice complicates
some
patients' lives, but there is new
hope
By AMANDA GARDNER
Daily News Staff Writer
Ivor Pannell came home from the
hospital one day with a prescription for
an opioid medication, Percoset, to relieve
the mind-numbing pain caused by his sickle
cell anemia.
He called the pharmacy closest to his
apartment in Queens and asked if they had
the drug and if they accepted Medicaid. The
answer to both questions was "yes." When
Pannell got to the drugstore a few minutes
later, however, the pharmacist claimed he
did not have the medicine.
Ivor Pannell
"But I just called," Pannell
objected.
"We just ran out," the
pharmacist told him.
Pannell called a second
pharmacy from a pay phone.
Again, he was told that the
store had the drug and that
they accepted Medicaid. When
Pannell walked up to the counter, the
pharmacist claimed they did not accept
Medicaid, despite a sign in the window
stating the opposite.
Pannell went to 11 different pharmacies in a
half-mile-square area of Astoria that
morning, and was refused at every one of
them. He returned home exhausted, in pain
and without any medication to help him.
When his wife came home from work that
night, she took the prescription to the first
pharmacy her husband had visited and had
it filled right away. Deborah Pannell is
white. Her husband is black.
"Drugs and race are linked in their minds,"
says Ivor, who now runs a sickle cell
advocacy group called SCARE (Sickle Cell
Advocates for Research and Empowerment)
from his home in the Bronx, where he now
lives. "They can respond negatively to the
combination of a black face, an opioid
prescription and Medicaid."
But these three characteristics are common
among people who have sickle cell anemia,
an inherited disease for which there is no
known cure. Most of the 72,000 Americans
afflicted are black and Hispanic, but the
disease - for which September has been
designated national awareness month -
also affects people of Arab, Greek, Maltese,
Italian, Sardinian, Turkish and Indian
descent, mostly in other parts of the world.
Though Pannell's story is extreme, it is not
an isolated one. Taeshea Martin, 26, a
student at John Jay College, also has sickle
cell and recounts visits to emergency rooms
when doctors would give her only two pills
rather than a whole prescription.
People with sickle cell disease inherit a gene
(or genes) that causes some of their red
blood cells to become sickle-shaped. These
cells then get stuck in blood vessels, cutting
off oxygen to different organs and tissues,
which can lead to a number of
complications, including infections, stroke
and organ damage.
In recent years, medical researchers have
made some promising breakthroughs in
understanding and treating this debilitating
condition. Bone marrow transplants from
siblings without the disease have erased all
traces of sickle cell in a very small number
of children, but most of the advances treat
the complications, not the condition.
Penicillin given to children during the first
few years of life, for example, has
substantially reduced the number of deaths
from pneumococcal infections. Regular
blood transfusions decrease the incidence of
strokes (which occur primarily in young
children), and new technology enables
doctors to identify individuals at high risk
for stroke.
The hallmark of sickle cell
anemia, though, is periodic
episodes of debilitating pain
like the one that sent Ivor
Pannell in search of Percoset.
Usually referred to as crises,
these episodes occur when
blood flow is cut off from an
area of the body, and can last
for an evening or for as long as
two or three weeks. In the
mid-'90s, an anti-cancer drug
called hydroxyurea was found
to significantly reduce the
frequency of crises. But once a crisis erupts,
there are few options. Very mild episodes
can be handled with non-opioids alone or in
combination with certain opioids (like
codeine), but severe crises may require
treatment with morphine-equivalent opioids
and even hospitalization.
Predicting when episodes will occur is
difficult, and patients differ greatly in how
their disease manifests itself. "It's very
variable. Some patients are very sick, some
are not," says Dr. Sergio Piomelli, director of
pediatric hematology and director of the
sickle cell center at Children's Hospital of
New York, Columbia Presbyterian Medical
Center. "Some patients come here literally
every week for a year and then nothing
more."
From Bad to Much Worse
There is no "typical" sickle cell patient. Paige
and Walter Culbreth are siblings who both
have the disease. Walter, 15, is on
hydroxyurea to help control his pain;
already, he has been hospitalized seven
times, missed a lot of school and has had to
have blood transfusions. Paige, 11, on the
other hand, has been hospitalized only
twice, Motrin is often enough to manage her
pain and she has not missed as much school.
Ivor Pannell suffered a paralyzing stroke at
age 16 and yet managed to have a successful
career as a professional dancer (including
break dancing) in his 20s.
Among the so-called high-frequency pain
patients - the small percentage of those
who suffer an inordinate number of severe
crises - there are additional problems.
Many of these patients have been on opioids
around the clock for years, a factor that
complicates pain management. Many people
on short-acting opioids become dependent
and experience withdrawal, which often
involves pain similar to that caused by the
disease. Untreated pain also causes many of
the conditions that precipitate a crisis in the
first place. "The pain can ultimately become
a disease within itself," says Dr. Lennette
Benjamin, clinical director of the
Comprehensive Sickle Cell Center at
Montefiore Medical Center, associate
professor of medicine at Albert Einstein
College of Medicine and chief medical
officer for the Sickle Cell Disease
Association of America.
Benjamin uses two strategies when treating
patients: She administers longer-lasting
opioids so that the patient is not eternally
tied to the peaks and valleys of medication
and withdrawal, and, more significantly, she
has changed the traditional setting for
treatments. Because of the unpredictable
nature of sickle cell (sometimes complicated
by matters of health coverage), many
patients receive care for their crises in
emergency rooms, which means that they
see many different doctors, are prescribed
many different medications and must often
take a backseat to a gun-shot victim or
heart-attack patient.
In 1989, Montefiore started an experimental
day center designed for sickle cell patients in
crisis. Patients are treated within 15 to 20
minutes of arriving, reassessed by staff
members every 30 minutes and treated until
their pain is relieved. The first five years of
the center's existence saw a 40% reduction
in the hospital admission rate of people in
crisis, and a decreased number of emergency
visits as well.
Patients stay an average of 4 1/2 hours, at
which point more than 90% are able to go
home. "If you don't minimize the time that
the person is in pain, it can cause more pain.
The consequences of unrelieved pain can
contribute to the person being hospitalized
unnecessarily for days or even weeks," says
Benjamin.
For Pannell, the results of this approach
have been dramatic. Before Montefiore, he
was hospitalized, on average, every two to
three months. In the last five years,
however, he has only been hospitalized
twice. "I still have pain, but it's not the daily,
oppressive, nonstop pain that it was," says
Pannell, who refers to himself as a sickle cell
"defier," rather than "patient" or "sufferer."
"A lot of defiers believe life will always be
pain," he says. "I let them know you don't
have to wait for a cure, for research to be
done 'someday.' You can be living the quality
of life that you were always dreaming about
if you get proper management."
The Genetics of the Disease
People with sickle cell disease inherit one
diseased gene from each of their parents;
different combinations of genes can cause
different forms of the disease. More than 40
states now test newborns for sickle cell and
expectant parents can choose to receive a
prenatal test.
Although gene therapy is still years away for
this disease, there has been some progress.
One possibility would involve correcting the
defective gene and inserting it into the bone
marrow in the hopes that it would produce
normal hemoglobin. Another possibility
would be to deactivate the defective gene.
One promising note: In 1999, researchers at
New York Weill Cornell Center successfully
used pre-implantation genetic diagnosis
(PGD) to produce healthy twin girls from
parents who had sickle cell genes. Eggs were
fertilized in vitro, then tested for their
genetic status. Healthy embryos were
implanted in the mother.
National Heart, Blood & Lung Institute
(301) 592-8573 www.nhlbi.nih.gov
SCARE (Sickle Cell Advocates for Research
and Empowerment, Inc.), (718) 884-9670,
http://defiers.com
The National Institutes of Health has
funded 10 comprehensive sickle cell centers
around the U.S. Two of them are in New
York City:
Columbia Presbyterian Medical Center
Sickle Cell Services
Department of Pediatric Hematology
Columbia University, Harkness Pavilion
180 Fort Washington, FL 5
New York, NY 10032
(212) 305-2466
Montefiore Medical Center
Sickle Cell Center
111 E. 210th St.
Bronx, NY 10467
(718) 920-7373/4321
From: Arts and Lifestyle | Health