An expert panel has issued new guidelines for managing sickle cell disease, stressing the use of the drug hydroxyurea and transfusions for many with the genetic disorder.
By Kathleen Doheny
HealthDay Reporter
HealthDay Reporter
As many as 100,000 Americans
have sickle cell disease, according to background information with the
guidelines. In sickle cell disease, the body makes sickle-shaped or
crescent-shaped red blood cells. Normal red blood cells are disc-shaped,
like a doughnut without holes, allowing the cells to move easily
through blood vessels.
Sickle cells are stiff and
sticky and tend to block blood flow, leading to organ damage, pain and
increased risk of infection and strokes, among other problems. Black
people are more commonly affected than other people.
The expert panel reviewed more
than 12,000 scientific articles and sifted through the evidence to issue
the new blueprint for care. The new guidelines are published Sept. 10
in the Journal of the American Medical Association.
One of the new recommendations
is to give children oral penicillin daily until age 5. This is a
preventive measure, aiming to reduce the risk of pneumonia and other
infections.
“That has been the standard for a while now,” Yawn said. “Now we are saying ‘This is an absolute necessity.'”
In addition, everyone with
sickle cell disease should be vaccinated against pneumonia, according to
the guidelines. “The children need pneumococcal vaccine as early as
they can possibly get it, around six weeks of age,” Yawn said.
Children from ages 2 to 16
should have an annual exam known as a transcranial Doppler, which
measures blood flow in the brain, the guidelines explain. If it’s
abnormal, long-term transfusion therapy to prevent stroke is
recommended.
When acute complications occur,
opioids (also known as narcotics) are suggested to treat the pain
linked with blood flow blockage. A method of encouraging deep breathing —
incentive spirometry — is also advised for those hospitalized with a
blood flow crisis.
If adults have three or more
severe blood flow crises in a year, treatment with the drug hydroxyurea
is recommended. The drug works in sickle cell by helping to prevent the
formation of sickle-shaped red blood cells. Hydroxyurea can be used in
infants, children and teens, whether or not they currently have
symptoms, according to the guidelines.
One difficulty in developing the guidelines, Yawn said, is that there is “not enough research to answer all the questions.”
People with sickle cell disease
are living longer, she said. A generation ago, many with sickle cell
disease only survived until their 20s or 30s. “We know there are more
living into their 40s, 50s and 60s,” she said.
Dr. Michael DeBaun, professor
of pediatrics and medicine at the Vanderbilt University School of
Medicine, said, “These are really strong recommendations.”
DeBaun wrote an editorial to
accompany the guideline report. “This should now be a road map of how
your child should be cared for,” he said.
The information also applies
for adults with sickle cell disease, said DeBaun, who is also director
of the Vanderbilt–Meharry Sickle Cell Disease Center of Excellence.
He advises patients and parents
to use the new guidelines to have a dialogue with the doctors providing
care. “We expect these recommendations to change over time as more
evidence becomes available,” DeBaun said.
In his own recent study, published in August in the New England Journal of Medicine,
DeBaun and his colleagues reported that monthly blood transfusions
appear to reduce the risk of strokes in children with sickle cell
anemia, the most common form of sickle cell disease.
More information
To learn more about sickle cell disease, visit the U.S. Centers for Disease Control and Prevention.
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