Sickle cell: Stopping kids' silent strokes


ST. LOUIS, Mo. – 12-year old Alexis Haynes has come a long way. She has sickle cell disease which is a genetic condition where the body's red blood cells are deformed, clogging up arteries, and causing pain, disability or major stroke, even in kids. At age six, a sudden stroke put her in a coma for a full month.

"The doctors told us that she wouldn't be able to walk, she wouldn't be able to talk, she wouldn't remember us," Kelvin Haynes, Alexis' father said.

Every six weeks, Alexis spends hours getting her blood transfused. New red blood cells replace her sickle-shaped ones. While Alexis' stroke was apparent, experts say one in three children with sickle cell suffer silent strokes.

"These are injury to parts of the brain that don't control speech, they don't control movement in an arm or a leg, so they typically go unnoticed," explained Michael Noetzel, M.D., and Pediatric Neurologist at Washington University School of Medicine, St. Louis.

These kids have a higher risk of memory problems. Many have trouble at school. They're also at much higher risk for having a major stroke.

Noetzel studied one-hundred and 96 children age five and older, who had brain scans that showed evidence of silent strokes. For three years, 99 received monthly transfusions, the rest did not. Researchers found the transfusions reduced the risk of strokes of any kind by 58-percent.

"Now that we have an intervention at hand that could be helpful-there's no reason not to think about screening younger children," Noetzel explained.

Risks from transfusions include infections, reactions to donated blood and buildup of iron in the bloodstream. Researchers are planning longer-term studies to see whether transfusions, in combination with other sickle cell treatment options -- like stem cell transplantation, can help prevent kids from losing cognitive function.

Additional Information:

Sickle cell disease is an inherited blood disorder in which red blood cells contain an abnormal type of hemoglobin, hemoglobin S. When a red blood cell becomes sickle-shaped (crescent), they have a hard time passing through blood vessels; this in turn causes less blood flow to tissues in the body, which can become damaged. Lung tissue damage, pain episodes in the extremities and torso, and stroke can result from this lessened blood flow. Spleen damage, especially in children, can cause overwhelming susceptibility to bacterial infections. The most common types of sickle cell disease are Sickle Cell Anemia (SS) and Sickle-Hemoglobin C Disease (SC).
(Source: http://www.sicklecelldisease.org/index.cfm?page=about-scd)

TREATMENT: There is no cure for sickle cell disease. Early diagnosis of sickle cell disease can be maintained through vaccination against pneumococcus bacteria, folic acid supplements, or penicillin prophylaxis. Antibiotics, intravenous fluids and pain management can all be a part of a comprehensive program of care to manage complications and symptoms.  For sickle cell anemia, bone marrow transplants are the closest treatment to a cure, however with very serious risks. Bone marrow treatment is aimed at symptom relief and avoiding major crises.
(Sources: http://www.sicklecelldisease.org/index.cfm?page=about-scd, http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/treatment/con-20019348)

TREATING SIDE EFFECTS: Doctors can find out when a child has a high risk of stroke by using a special non-invasive ultrasound machine, called a transcranial Doppler (TCD). It can be used on children as young as two years of age to assess the probability of stroke, which can be lessened with blood transfusions. The TCD examines blood circulation within the brain with sound waves that reflect movement of blood cells within blood vessels. A radiologist will then calculate their speed. Blood transfusions replace the abnormal red blood cells, and reduce recurrent pain, but at the risk of developing an iron overload. Treatments are available to remove any excess iron from the body, in turn avoiding organ damage in areas such as the heart, liver pancreas, and other endocrine organs.
(Sources: http://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/basics/treatment/con-20019348, http://www.ncbi.nlm.nih.gov/pubmed/9647873, https://my.clevelandclinic.org/health/diagnostics/hic-abdominal-renal-ultrasound/hic-ultrasonography-test-transcranial-doppler)