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From: NIH news releases and news items [mailto:[log in to unmask]] On
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Sent: Tuesday, May 11, 2010 4:38 PM
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Subject: NEW STUDY SUGGESTS SICKLE CELL DISEASE MAY AFFECT BRAIN FUNCTION IN
ADULTS

U.S. Department of Health and Human Services 
NATIONAL INSTITUTES OF HEALTH NIH News 
National Heart, Lung, and Blood Institute (NHLBI)
<http://www.nhlbi.nih.gov/> 
For Immediate Release: Tuesday, May 11, 2010   

CONTACT: NHLBI Communications Office, 301-496-4236, <e-mail:
[log in to unmask]>

NEW STUDY SUGGESTS SICKLE CELL DISEASE MAY AFFECT BRAIN FUNCTION IN ADULTS
Research to Preserve Cognitive Abilities is Under Way

Sickle cell disease may affect brain function in adults who have few or mild
complications of the inherited blood disease, according to results of the
first study to examine cognitive functioning in adults with sickle cell
disease. The multicenter study, funded by the National Heart, Lung, and
Blood Institute (NHLBI), part of the National Institutes of Health, compared
brain function scores and imaging tests in adult patients with few sickle
cell complications with results in similar adults who did not have the blood
disease. 

Researchers report that the brain function scores in sickle cell patients
were, on average, in the normal range. However, twice as many patients as
healthy adults (33 percent versus 15 percent) scored below normal levels.
Those who were more likely to score lower were older and had the lowest
levels of hemoglobin, the protein in red blood cells that carries oxygen in
the blood, compared to sickle cell participants who scored higher. Findings
from brain magnetic resonance imaging scans did not explain differences in
scores.

Researchers at 12 sites within the NHLBI-supported Comprehensive Sickle Cell
Centers conducted the study. Their results are published in the May 12 issue
of the Journal of the American Medical Association. An editorial accompanies
the article. 

"This study suggests that some adult patients who have sickle cell disease
may develop cognitive problems, such as having difficulty organizing their
thoughts, making decisions, or learning, even if they do not have severe
complications such as stroke related to sickle cell disease," said NHLBI
Acting Director Susan B. Shurin, M.D. "Such challenges can tremendously
affect a patient's quality of life, and we need to address these concerns as
part of an overall approach to effectively managing sickle cell disease."

Researchers tested cognitive functioning of 149 adult sickle cell disease
patients (between the ages of 19 and 55) and compared them to 47 healthy
study participants of similar age and education levels from the same
communities. All of the participants were African-American. 

More sickle cell disease patients scored lower on measures such as
intellectual ability, short-term memory, processing speed, and attention,
than participants in the healthy group. The sickle cell disease participants
did not have a history of end-organ failure, stroke, high blood pressure, or
other conditions that might otherwise affect brain function.

"We need to study whether existing therapies, such as blood transfusions,
can help maintain brain function, or perhaps even reverse any loss of
function," noted Elliott P. Vichinsky, M.D., of the Children's Hospital &
Research Center Oakland, principal investigator of the study and the lead
author of the paper. "These effects were found in patients who have
clinically mild sickle cell disease, which raises the question of whether
therapies should be given to all patients to help prevent these problems
from developing."

Researchers involved in this study are recruiting patients with sickle cell
disease into a clinical trial to determine whether blood transfusions may
help preserve cognitive function. Participants will receive transfusions
every three or four weeks for six months as part of the clinical study.
Information about this study can be found at <www.clinicaltrials.gov>,
search for NCT00850018.

Sickle cell disease affects about 70,000 Americans. At one time, many
children died from the disease, but new therapies have enabled sickle cell
disease patients to live well into middle age or beyond. As more people with
sickle cell disease are living into adulthood, health care providers are
uncovering previously unrecognized complications. 

Studies of brain function in children who have sickle cell disease have
suggested that some children with the disease, even if they have not
suffered a stroke, have experienced silent brain injury. Others without
obvious changes on brain scans may have some level of cognitive dysfunction
that seems to worsen with age.  Stroke is a common complication of sickle
cell disease, and can lead to learning disabilities, lasting brain damage,
long-term disability, paralysis, or death.

Sickle cell disease involves an altered gene that produces abnormal
hemoglobin. Red blood cells with sickle hemoglobin that have too little
oxygen become C-shaped in addition to becoming stiff and sticky. These
crescent-shaped cells can clump to block blood flow, causing severe pain and
potential organ damage. In the United States, the disease mainly affects
those of African descent, but it is also found in other ethnic groups,
including those of Hispanic and Middle Eastern descent.  

To speak with an NHLBI spokesperson, please contact the NHLBI Communications
Office at (301)496-4236. To speak with Dr. Vichinsky, please contact Erin
Goldsmith at 510-428-3367 or email <e-mail:[log in to unmask]>. 

Part of the National Institutes of Health, the National Heart, Lung, and
Blood Institute (NHLBI) plans, conducts, and supports research related to
the causes, prevention, diagnosis, and treatment of heart, blood vessel,
lung, and blood diseases; and sleep disorders. The Institute also
administers national health education campaigns on women and heart disease,
healthy weight for children, and other topics. NHLBI press releases and
other materials are available online at <www.nhlbi.nih.gov>.  

The National Institutes of Health (NIH) -- The Nation's Medical Research
Agency -- includes 27 Institutes and Centers and is a component of the U.S.
Department of Health and Human Services. It is the primary federal agency
for conducting and supporting basic, clinical and translational medical
research, and it investigates the causes, treatments, and cures for both
common and rare diseases. For more information about NIH and its programs,
visit <www.nih.gov>.
-------------

RESOURCES:
Sickle Cell Disease:
<http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhatIs.html> 
Clinical Trials: <http://www.clinicaltrials.gov>
  
##

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