September 14, 1999
Aggressive Pain Control Encouraged in Sickle Cell
By WARREN E. LEARY
CLEVELAND -- Patients with the agonizing
and intractable pain of sickle cell disease
should get better, more consistent relief because
of new treatment guidelines that call for
comprehensive management of the pain, experts
say.
The guidelines, intended for doctors, nurses and
other health professionals who treat sickle cell
disease, call for aggressive use of drugs along
with behavioral, psychological and physical
therapies to relieve patients' suffering.
The recommendations, issued here Friday at the
annual meeting of the Sickle Cell Disease
Association of America, reflect growing attention
to the importance of relieving pain, not just in
sickle cell disease but throughout medicine.
Drafted by an expert panel sponsored by the
American Pain Society, a national organization of
medical specialists, the guidelines are the first in
a series of research-based recommendations for
treating pain in several diseases, including
arthritis and cancer.
But pain is different in sickle cell patients,
because it can start when they are a few months
old and persist, off and on, for life. "A lifetime of
unpredictable, recurrent, intense, and frequently
persistent pain experiences and the
accompanying recurring and necessary use of
opioids make pain related to sickle cell disease
unique among pain syndromes," the guidelines
say. Opioid means narcotic.
Dr. Lennette J. Benjamin, clinical director of the
Comprehensive Sickle Cell Center at Montefiore
Medical Center in the Bronx and a member of
the drafting panel, said, "This is the first national
guideline to treat acute and chronic sickle cell
pain, and we will push to have it adopted by
everyone treating sickle cell patients."
Pain is the primary reason sickle cell patients
seek hospital care, Dr. Benjamin said, adding
that these patients traditionally are not given
enough medicine.
"I see patients every day who have suffered a
lifetime of needless pain simply because their
doctors and others treating them don't
understand or practice the principles of good
pain management," she said. "Unrelieved pain
leads to longer hospitalizations, greater suffering
and complications and more deaths."
Sickle cell disease encompasses a group of
inherited blood disorders that afflict primarily
people with ancestors from Africa, the
Mediterranean and India. In this country, an
estimated 80,000 people, mainly from
African-American, Caribbean and
African-Hispanic backgrounds, have the disease,
and about 8 percent of blacks carry a genetic
tendency for it.
With the disease, the body produces abnormal
forms of hemoglobin, the oxygen-carrying
protein of red blood cells. Sometimes this
hemoglobin forms rigid crystalline structures
that cause the normally pliable blood cells to
stiffen and contort into jagged, sickle shapes.
These cells then block small blood vessels and
cause vascular inflammation that inhibits blood
flow to organs, resulting in cell death or damage.
These episodes can result in bouts of acute pain,
called crises, that often affect the chest, back,
abdomen, joints and extremities, and are
described by patients as being worse than the
anguish of cancer or childbirth. In addition to
crises that can last hours or days, some patients
suffer chronic, but less severe pain that can last
months or longer because of bone or organ
damage.
The pain society's panel, which included experts
in sickle cell disease, pain, medicine, nursing and
psychology as well as a patient representative,
took two years to draft the guidelines, consulting
experts and examining published studies on
sickle pain.
A draft report underwent peer review by 61
experts, and a revision was evaluated at 10
hospitals or medical practices that did not have
sickle cell programs to see if the
recommendations were clear, useful and
practical, even to nonexperts.
Experts say there are a number of barriers to
effective treatment of sickle cell pain: inadequate
knowledge of the condition and of pain
management in general; the variability and
unpredictability of episodes of the disease;
skepticism among some doctors that patients are
as miserable as they say they are, and an
unfounded fear that pain medication will lead to
addiction.
The panel said that pain assessment was a key
part of treatment and that health workers should
rely on patients' reports of discomfort as the
primary source of this evaluation.
The recommendations also call for aggressive
use of painkilling drugs for acute and chronic
episodes, starting with aspirin or acetaminophen
and moving to opioid medicine if needed. The
guidelines include a detailed regimen of doses
for many painkillers, and they call for detailed
monitoring of any adverse side effects. They also
include step-by-step procedures for looking for
underlying causes of pain and moving patients
through varying options for treatment.
Among those options are behavioral approaches,
including relaxation therapy, meditation and
biofeedback exercises; psychological methods
like counseling, support groups and hypnosis,
and physical treatments, including massage,
acupuncture, heat and administering
intravenous fluids to improve blood flow.
The American Pain Society plans to distribute
the guidelines to its members and provide them
to sickle cell treatment centers nationwide.
Others may order copies for $15, not including
shipping, by calling (847) 375-4715 or through
the organization's Web site
www.ampainsoc.org/
Related Articles
Drugs May Ease Sickle Cell Crises (June 22, 1999)
Embryos Can Now Be Analyzed for Sickle Cell Gene (May 12, 1999)
Gene-Altered Mice Are Called First True Sickle Cell Model (Oct. 31, 1997)