St. Jude Children’s Research Hospital:
Sickle cell disease research and treatment
Sickle cell disease is an inherited blood disorder affecting red blood cells. Sickled red blood cells block small blood vessels and delay the delivery of oxygen to tissues and organs, which causes anemia, painful events, organ damage, and can to lead to death in some cases.
The disease affects 1 in 350 African Americans, as well as some persons of Hispanic, Mediterranean, and Indian descent.
St. Jude began studying sickle cell disease shortly after opening in 1962 and remains a leader in research and treatment of the disease.
St. Jude has one of the largest and most active sickle cell disease programs in the nation. The hospital treats approximately 800 children with sickle cell disease per year, most of whom actively participate in clinical research trials.
Doctors at St. Jude were the first to cure sickle cell disease through bone marrow transplantation. While it is difficult to find a donor with a perfect bone marrow match, this procedure still offers the only option for a cure for the most severe cases of sickle cell disease.
A national study led by St. Jude researchers showed an inexpensive drug reduced episodes of severe pain and a pneumonia-like illness in infants and toddlers with sickle cell anemia. The drug, hydroxyurea, also cut hospitalizations and eased other symptoms of the disease in very young patients. Hydoxyurea was already used to treat adults with the inherited blood disorder.
In 2008, the National Heart, Lung and Blood Institute named St. Jude as one of 12 institutions nationwide to participate in the Basic and Translational Research Program on sickle cell disease.
St. Jude researchers have published several hundred journal articles about sickle cell disease during the past 25 years, and St. Jude is one of the largest publishers of educational literature for sickle cell disease written for parents, children, educators and healthcare professionals.
Since 2008, St. Jude has provided more than 3,000 sickle cell trait screenings in the Memphis area through the Know Your Sickle Cell Status program. This program targets African-American teenagers and young adults at risk for having sickle cell disease by teaming up with a network of public schools, the NAACP Youth Council and faith-based and community groups in the Memphis area.
Through the STARR program St. Jude helps public school teachers to meet the special needs of students with sickle cell disease.
The St. Jude-Methodist Sickle Cell Disease Transition Clinic works tohelp teens make a successful transition from the pediatric to the adult setting for treatment of sickle cell disease when they reach adulthood. This program has the potential to become a national model for programs encouraging teens with sickle cell disease to continue their treatment as adults.