Children with sickle cell disease and silent strokes show some relief with blood transfusion therapy

December 11, 2007

A group of children who have sickle cell disease and who experience silent strokes showed some relief from the silent strokes with blood transfusion therapy, researchers at Washington University School of Medicine in St. Louis have found.

The study's results will appear in a future issue of Pediatric Blood and Cancer but are available for review in its advance online publication.

In a Phase II study of 10 children with sickle cell disease who also had multiple silent strokes, or cerebral infarcts, the majority of families were committed to having their children receive blood transfusions for two years, showing that the therapy was feasible. In addition, the blood transfusion therapy helped to shrink the lesions on the brain caused by the infarcts and eliminated one lesion completely, said Allison A. King, M.D., a pediatric hematologist at St. Louis Children's Hospital and a researcher at Washington University School of Medicine. Lesions are small areas of damaged tissue thought to be due to blockage of small arteries in the brain.

Silent strokes are strokes that don't show the classic symptoms of overt strokes, such as numbness, tingling, headache or slurred speech. Blood transfusion therapy has been shown to be effective in preventing overt strokes in patients with sickle cell disease, but its effectiveness and the willingness of families to participate in long-term treatment to prevent silent strokes had not been tested.

Sickle cell disease is an inherited blood disorder affecting red blood cells that contain hemoglobin, a substance that carries oxygen from the air in the lungs to all parts of the body. In patients with this disease, red blood cells contain an abnormal type of hemoglobin that causes the normally round, flexible red blood cells to become sickle- or crescent-shaped. The sickle cells can't pass through tiny blood vessels, preventing blood from reaching the body's tissues, which can result in tissue and organ damage, pain and stroke.

Sickle cell disease affects about 70,000 people in the United States. It occurs in about 1 of every 500 African-American births and 1 of every 1,000 to 1,400 Hispanic-American births. While there is no cure for the disease, blood transfusions and bone marrow transplants have been shown to be effective treatments by replacing short-lived sickle cells with longer-lived healthy red blood cells, although bone marrow transplants have a 10 percent mortality rate because of the possibility of rejecting the bone marrow, complications such as seizures and a high risk of infection.

In the study, brain lesions in six patients shrank after two years of regular blood transfusions, and no new silent strokes occurred. One patient had a lesion disappear, however, that patient did not continue with further blood transfusions, and the lesion returned at more than three times its original size, suggesting the need for prolonged transfusions. A lesion grew larger in the seventh patient.

Many of the lesions occur in the frontal lobe of the brain, which controls the cognitive function or problem-solving area, or in the occipital lobe, which controls the visual processing center, King said. Neuroradiologists can locate the lesions using magnetic resonance imaging (MRI).

"Because these lesions are usually in the frontal lobe, it is important to do cognitive testing on these children to determine any impairment," King said. "We hope that by preventing further lesions through blood transfusions that we can preserve their ability to think and learn."

A total of 71 percent of the school-aged children in the study were receiving special education services and 57 percent had failed a grade in school. Previous School of Medicine research shows that 80 percent of students with silent strokes perform poorly in school.

King said the results of the trial were encouraging to health-care professionals treating children with sickle cell disease.

"What we found in this trial is that families and children with sickle cell disease and who experience silent strokes are willing to commit to blood transfusion therapy for this condition," King said. "If these children are left untreated, their risk for an overt stroke is very high, but if we use blood transfusion therapy, they may have a lower risk for overt stroke and no evidence of new silent infarcts on an MRI."

Results of this Phase II trial spearheaded a larger, Phase III study that is evaluating 1,800 children in the United States, Canada and Europe. That trial, called the Silent Cerebral Infarct Multi-Center Clinical Trial, is headed by Michael R. DeBaun, M.D., professor of pediatrics at the School of Medicine. Through it, researchers will further determine the effectiveness of blood transfusion therapy to prevent silent strokes in children with sickle cell disease and to prevent further cerebral injury.

Washington University School of Medicine in St. Louis

---

---

	Understanding Sickle Cell Disease (Understanding Health & Sickness) by Ph.D. Miriam Bloom (Author) Although more is known about sickle cell disease than about any other inherited disease, no cure for it exists. In America alone, about one in 375 who are of African ancestry is born with sickle cell disease. A smaller number of Americans descended from families from the Mediterranean area, the Middle East, and India also are affected. In addition, about eight percent of black Americans who do not suffer from the disease itself carry the gene for it that can be transmitted to their children.Sickle cell disease is of enormous biological, social, and historic importance. It was first described in medical literature almost a century ago. Improvements during the past two decades in our understanding of the disease and in medical care are permitting those afflicted to live longer, more...
	Hope & Destiny: The Patient and Parent's Guide to Sickle Cell Disease and Sickle Cell Trait by Allan F. Platt Jr. P.A.-C. M.M.Sc. (Author), James Eckman M.D. (Author), Lewis Hsu M.D. Ph.D (Author) For the more than 80,000 Americans diagnosed with sickle cell disease, Hope & Destiny provides clear-cut and in-depth information on living with the illness. It offers the latest information on preventing complications, research, and treatment. The knowledge patients and family members need is organized in an easy-to-read format that promotes overall healthy living.
	Sickle Cell Disease - Symptoms, Diagnosis, Treatment and Recent Developments of Sickle Cell Anemia Sickle Cell Disease - Symptoms, Diagnosis, Treatment and Recent Developments of Sickle Cell Anemia For an estimated 70,000 to 100,000 people in the United States, sickle cell disease is a painful, lifelong battle. But advances in sickle cell research are allowing many sickle cell patients to live productively.
	I Only Cry At Night, living with Sickle Cell Disease by P. Allen Jones A heartfelt story about the humanistic side of living in pain while overcoming everything else. P. Allen Jones gives an insightful look into her journey living with Sickle Cell Disease which moves from suffering to success.
	I Only Cry At Night: living with Sickle Cell Disease by P.Allen Jones (Author) I Only Cry at Night, living with Sickle Cell Disease. A personal story about overcoming and living beyond sickle cell disease.
	The Troubled Dream of Genetic Medicine: Ethnicity and Innovation in Tay-Sachs, Cystic Fibrosis, and Sickle Cell Disease by Keith Wailoo (Author), Stephen Pemberton (Author) Why do racial and ethnic controversies become attached, as they often do, to discussions of modern genetics? How do theories about genetic difference become entangled with political debates about cultural and group differences in America? Such issues are a conspicuous part of the histories of three hereditary diseases: Tay-Sachs, commonly identified with Jewish Americans; cystic fibrosis, often labeled a "Caucasian" disease; and sickle cell disease, widely associated with African Americans. In this captivating account, historians Keith Wailoo and Stephen Pemberton reveal how these diseases—fraught with ethnic and racial meanings for many Americans—became objects of biological fascination and crucibles of social debate. Peering behind the headlines of breakthrough treatments and coming...
	ACUTE CHEST SYNDROME IN SICKLE CELL DISEASE: Crucial considerations in adolescents and adults (Postgraduate Medicine) by JTE Multimedia Rapid recognition of acute chest syndrome is essential in patients with sickle cell disease. The condition can be particularly severe in adolescents and adults and often leads to death. In this article, the authors review the challenges of evaluating the syndrome and outline current treatment and supportive care. Yale SH, Nagib N, Guthrie T. Acute chest syndrome in sickle cell disease: crucial considerations in adolescents and adults. Original Publication Date: January 2000
	ADOLESCENTS WITH SICKLE CELL DISEASE: Determinants of Support Group Attendance and Satisfaction.: An article from: Health and Social Work by Joseph Telfair (Author), Marilyn M. Gardner (Author) This digital document is an article from Health and Social Work, published by National Association of Social Workers on February 1, 2000. The length of the article is 4460 words. The page length shown above is based on a typical 300-word page. The article is delivered in HTML format and is available in your Amazon.com Digital Locker immediately after purchase. You can view it with any web browser. Citation Details Title: ADOLESCENTS WITH SICKLE CELL DISEASE: Determinants of Support Group Attendance and Satisfaction. Author: Joseph Telfair Publication: Health and Social Work (Refereed) Date: February 1, 2000 Publisher: National Association of Social Workers Volume: 25 Issue: 1 Page: 43 Distributed by Thomson...
	Uncertain Suffering: Racial Health Care Disparities and Sickle Cell Disease (George Gund Foundation Imprint in African American Studies) by Carolyn Moxley Rouse (Author) On average, black Americans are sicker and die earlier than white Americans. Uncertain Suffering provides a richly nuanced examination of what this fact means for health care in the United States through the lens of sickle cell anemia, a disease that primarily affects blacks. In a wide ranging analysis that moves from individual patient cases to the compassionate yet distanced professionalism of health care specialists to the level of national policy, Carolyn Moxley Rouse uncovers the cultural assumptions that shape the quality and delivery of care for sickle cell patients. She reveals a clinical world fraught with uncertainties over how to treat black patients given resource limitations and ambivalence. Her book is a compelling look at the ways in which the politics of racism, attitudes...
	The clinical features of sickle cell disease (Clinical studies) by Graham R Serjeant (Author)