LOS ANGELES, Jan. 23, 2006 (PRIMEZONE) -- Childrens Hospital Los Angeles, working in conjunction with the Sickle Cell Disease Foundation of California, was host to a day-long symposium entitled, Sickle Cell Family Education Day: Living with Sickle Cell Disease, at The Saban Research Institute of Childrens Hospital Los Angeles last weekend.
More than 20 presenters, many from the Childrens Center for Cancer and Blood Diseases at Childrens Hospital Los Angeles, one of the nation's largest pediatric hematology/oncology programs, provided an overview of sickle cell disease, targeted toward individuals and their families affected by the disease.
Information about support, coping, transition and school-related issues, was provided in separate breakout sessions during the morning and afternoon sessions for specific age groups and special interest groups.
Sickle cell disease is a hereditary disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent. More than 70,000 Americans have sickle cell disease.
It occurs when a person inherits two abnormal genes (one from each parent) that cause their red blood cells to change shape. Instead of being flexible and round, these cells are more rigid and curved in the shape of the farm tool known as a "...sickle..." which is where the disease gets its name. The shape is similar to a crescent moon.
The most common types of sickle cell disease: Sickle Cell Anemia, Sickle-hemoglobin C Disease, Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.
"Sickle cell anemia is a particularly devastating disorder with serious complications that start in childhood and become crippling in adults," said Thomas D. Coates, M.D., director of the Red Cell Defects Program at Childrens Hospital Los Angeles. "Significant advances have been made in clinical management and the promise of gene therapy puts a cure on the horizon."
Sickle cell is a complicated disease to manage, according to Dr. Coates, who is also professor of pediatrics and pathology at the Keck School of Medicine of the University of Southern California. Children with the disease have a 15-percent chance of having a stroke before the age of 20. Many die in their 20s and 30s due to some type of organ failure. Others must live with chronic pain, the need for regular blood transfusions, anemia or heart and lung problems. Sickle cell disease can cause damage to most organs, including the brain, lungs, spleen, kidneys and liver. Young children, in particular, can easily develop life-threatening complications due to certain bacterial infections.
Since there is no readily available cure, those afflicted with sickle cell diseases must focus on proper health maintenance strategies, such as recognizing the early signs of life-threatening complications and seeking prompt treatment. For example, blocked blood vessels can cause acute painful episodes referred to as a "sickle cell crisis." These episodes marked by severe pain in arms, legs, chest or abdomen, which occur in almost all sickle cell patients at some point in their lives, can last for hours or days. The "crisis" can be severe enough to require hospitalization for pain control and intravenous fluids. Repeated crisis can cause damage to the kidneys, lungs, bones, eyes and central nervous system.
"Our goal is not only to keep these kids alive, but healthy, with a good quality of life and to make complications much less damaging," Dr. Coates said, adding that the Sickle Cell team at Childrens Hospital is committed to preventing the crippling complications of the disorder so children and adults will be in good clinical condition, and able to take advantage of the cure when it becomes available.
The morning keynote addresses were delivered by Thomas Hofstra, M.D., clinical director of Hematology at Childrens Hospital Los Angeles ("Overview of Sickle Cell Disease"); Debbie Harris, R.N., M.N., PNP, an advanced nurse practitioner in the Sickle Cell Disease Program in the Division of Hematology/Oncology at Childrens Hospital ("Pain Management"); and Yutaka Niihara, M.D., the head of the adult sickle cell program at Harbor UCLA Medical Center ("L. Glutamine").
During her presentation, Ms. Harris quoted 12-year-old patient Meme Umoh from Los Angeles, who said of the pain he experiences with sickle cell disease, "...It can hurt really bad, lots of pressure, it's hard to say, it's different, and it just hurts soooo much."
The afternoon keynote addresses were delivered by John Wood, M.D., Ph.D., director of Cardiovascular MRI for The Heart Institute at Childrens Hospital Los Angeles ("Pulmonary Hypertension"), and Dr. Coates ("Searching for a Better Treatment and Potential Cure").
Dr. Coates concluded his presentation by comparing the great strides made in leukemia research to what is possible for sickle cell disease. "The cure rate for childhood leukemia in 1955 was zero, but approaches 90 percent in common kinds of childhood leukemia in 2006. This amazing success was achieved because all the children's cancer centers, led by physicians at Childrens Hospital Los Angeles, banded together in 1955 to do multi-center clinical research on the treatment of childhood leukemia.
"This same process started only a few years ago in sickle cell disease," he said. "The only way we will conquer this disease is if all the sickle cell centers cooperate, and sickle cell patients volunteer to take part in research trials."
Closing the symposium was a lively patient/family panel discussion entitled, "Infant Through Adulthood," which was moderated by Dawn Canada, LCSW, a clinical social worker and team leader with the Division of Hematology/Oncology at Childrens Hospital.
Childrens Hospital Los Angeles annually treats 400 children and adolescents who have sickle cell disease. More than 100 of those patients must undergo monthly blood transfusions for the remainder of their lives.
Individuals who are interested in donating blood or platelets at Childrens Hospital Los Angeles should make an appointment by contacting the Blood Donor Center at (323) 669-2441. The Blood Donor Center is open for blood donations, Tuesday through Friday, from 8 a.m. to 5 p.m., and Saturday, from 8 a.m. to 1 p.m. (by appointment only).
Founded in 1901, Childrens Hospital Los Angeles has been treating the most seriously ill and injured children in Los Angeles for more than a century, and it is acknowledged throughout the United States and around the world for its leadership in pediatric and adolescent health. Childrens Hospital is one of America's premier teaching hospitals, affiliated with the Keck School of Medicine of the University of Southern California for more than 73 years. It is a national leader in pediatric research.
Childrens Hospital Los Angeles treats more than 58,000 patients a year in its Emergency Department. It admits more than 11,000 children a year to the hospital, with almost 50-percent of those admissions children under four years of age. There are more than 285,000 visits a year to its 29 outpatient clinics; nearly 5,000 visits at community sites through its Division of Adolescent Medicine. Childrens Hospital Los Angeles is able to offer the optimum in multidisciplinary care, with 33 pediatric subspecialties and dozens of special services for children and families.
Investigators at The Saban Research Institute of Childrens Hospital Los Angeles are working to create a world in which all children are healthy -- a world in which they are no longer threatened by such diseases as cancer, congenital heart defects, diabetes, sickle cell anemia, thalassemia, epilepsy, immune deficiencies and respiratory disorders. They ask basic questions about human biology, find new ways to see inside the body, explore genetic mysteries, develop promising drug treatments and test preventive strategies -- scientific inquiries that benefit both children and adults. The Saban Research Institute is among the largest and most productive pediatric research facilities in the United States, with 100 investigators at work on 251 laboratory studies, clinical trials and community-based research and health services. It is one of the few freestanding research centers in the nation to combine scientific laboratory inquiry with patient clinical care -- dedicated exclusively to children -- and its base of knowledge is widely considered to be among the best in pediatric medicine.
Since 1990, U.S. News & World Report and its panel of board-certified pediatricians have named Childrens Hospital Los Angeles one of the top pediatric facilities in the nation. Child magazine also ranked Childrens Hospital Los Angeles among the Top Ten children's hospitals in America in its February 2005 issue.
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