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1993.04.27 :  AHCPR Guidelines -- Sickle Cell Disease

Contacts: 301/227-8364 
Harriett Bennett X 174
Rose Findley X 188
Bob Isquith X 173
April 27, 1993

       A federally sponsored panel today issued new guidelines
for diagnosis and treatment of sickle cell disease, an inherited
disorder that makes children susceptible to infections and other
potentially fatal complications.   
       The guidelines include a recommendation for universal
screening of newborns, protective doses of penicillin to ward off
infection and follow-up medical care.  Universal screening is
currently performed in 34 states and jurisdictions, and targeted
screening is done in others.  
       Sickle cell disease is marked by crescent-shaped red blood
cells.  The most common form, sickle cell anemia, affects one out
of 375 African-Americans, causing jaundice and abdominal,
muscular and joint pain, skin ulcerations and gallstones.
       But the disease also occurs in people whose ancestors came
from areas such as the Mediterranean basin, Arabian Peninsula,
the Caribbean and Central and South America.  
       The new guidelines are part of a series of medical
advisories being developed by independent expert panels, with the
support of the Agency for Health Care Policy and Research, to
help improve the effectiveness of medical care. 
       The guidelines call for screening of all newborn babies
for sickle cell disease.  Panel co-chair Jeanne Smith, M.D.,
associate professor of clinical medicine at Columbia University
and director of the Comprehensive Sickle Cell Center at Harlem
Hospital, said screening programs targeted to a specific racial
or ethnic group will not identify all infants with sickle cell
disease. "Because of mixing of the gene pool, it's not always
possible to be certain of an individual's racial or ethnic
background by physical appearance, surname or self-report," Dr.
Smith said.
       "Universal screening is the best and most reliable method
of casting the broadest possible net to identify affected
infants,"
Dr. Smith said, "and it ensures that all babies benefit equally
from state and federal funding for neonatal screening." 
       The test can be done in conjunction with other neonatal
screening tests that are also performed universally for
conditions such as phenylketonuria -- a relatively rare disease
that causes mental retardation -- and hypothyroidism, a thyroid
condition. 
       The guidelines also call for infants whose initial test
reveals sickle cell anemia to be given prophylactic penicillin
within two months of birth, even if the diagnosis has not been
confirmed.  
       "Administration of penicillin can significantly reduce the
number and severity of infections and pneumonia-related deaths," 
said the panel's other co-chair, Thomas Kinney, M.D., professor
of pediatrics at Duke University Medical Center in Durham, N.C.  
     
       The guidelines also recommend improved genetic counseling
for couples with the sickle cell trait, which increases the risk
of offspring being born with sickle cell anemia.  Eight percent
of all African-Americans -- 2.5 million individuals -- carry the
trait; when both parents have it, their offspring have a one in
four chance of being born with sickle cell anemia.
       Furthermore, the guidelines call on screening laboratories
to relay test results promptly to the infant's physician and
hospital of birth.  
       The infant's physician should promptly establish a
definitive diagnosis and the parents should be taught about the
disease, routine child care (including body temperature
measurement, and signs of life-threatening complications, such as
fever, unusual sleepiness or irritability, rapid respiration, or
vomiting), and how to reach medical help quickly.   
       The guidelines also emphasize the importance of regular
visits to assess the child's medical status, administration of 
age-appropriate immunizations, and the provision of other infant-
specific health services.  
       The 13-member private-sector panel of physicians, nurses, 
social workers and consumer representatives reviewed more than 
7,000 studies before writing the guidelines.  The documents then
underwent rigorous review by other experts in sickle cell
disease. 

       According to AHCPR Administrator J. Jarrett Clinton, M.D.,
the agency, which is a part of the Public Health Service within
HHS, will distribute the guidelines widely to physicians, nurses,
hospitals, consumer groups and others. 

       Sickle Cell Disease:  Screening, Diagnosis, Management,
and Counseling in Newborns and Infants, a quick reference guide
for clinicians and a guide for parents are available without
charge by sending a post card or letter to: Sickle Cell Disease,
P.O. Box 8547, Silver Spring, Md. 20907, or calling 1-800-358-
9295. 
     
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