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Intervention helps children with sickle cell disease complete MRI tests without sedation
Date:8/31/2012

ther medical procedures. September is National Sickle Cell Awareness Month. This study highlights the varied efforts underway at St. Jude to improve the health and quality of life for children and adolescents with sickle cell disease.

The paper's senior author, Jane Hankins, M.D., an associate member of the St. Jude Department of Hematology, said that avoiding sedation reduces the risk to the patients, particularly those with sickle cell disease.

Sickle cell disease is caused by a mutation in the gene for assembling hemoglobin. Hemoglobin is the protein red blood cells use to carry oxygen throughout the body. While red blood cells are normally pliable discs, the inherited mistake results in red blood cells becoming brittle and assuming a banana shape that can trigger intense pain and lead to organ damage. Hankins said the disease also makes anesthesia riskier.

General anesthesia can lead to a drop in temperature, blood oxygen levels and dehydration. In patients with sickle cell disease, dehydration can result in a painful and dangerous buildup of sickled cells. Patients can wind up hospitalized, sometimes in the intensive care unit, for treatment of severe pain or acute chest syndrome, a pneumonia-like illness, Hankins said. To reduce that risk, a sickle cell patient scheduled for anesthesia is hospitalized overnight to receive extra fluids and possibly a blood transfusion.

The combination of factors creates inconvenience and added risk for the patient. It also adds to health care costs. While no family ever pays St. Jude for the care their child receives, controlling costs remains important to the hospital. The program could also be adopted by other health care institutions worldwide to help manage cost.

The intervention is now routinely offered to all St. Jude patients undergoing MRIs. Cejda said the program has been used to help children as young as age 4 complete scans without sedation. "This preparation program o
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Contact: Summer Freeman
summer.freeman@stjude.org
901-595-3061
St. Jude Children's Research Hospital
Source:Eurekalert  

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