Sickle Cell Disease

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Sickle Cell Disease Overview
BloodCenter of Wisconsin works with patients who have sickle cell disease, along with their physicians, to help diagnose and treat this life-threatening condition. Sickle cell disease is a genetic disease inherited from both parents, and it affects approximately one of every 400 African-American newborns in the United States. It is caused by an abnormal type of hemoglobin (blood protein) that creates red blood cells that have an abnormal “sickle” shape instead of a normal disc shape. Sickle cells carry less oxygen and can interrupt healthy blood flow, eventually leading to tissue and organ damage.
 
Sickle cell disease can cause premature death. Patients who have the most severe form of sickle cell disease have an average life expectancy of only 42 years (men) and 48 years (women). At some point, most sickle cell patients experience painful episodes in their legs, arms, chest or back, most often felt in their bones.
 
Some sickle cell patients can require repeated blood transfusions and hospital stays as part of their treatment. The drug hydroxyurea has been shown to help extend the lives of some sickle cell patients, but more research is needed to improve clinical care, and new therapies are required to improve the lives of these patients. BloodCenter of Wisconsin conducts clinical research, blood donor matching, transfusion and other therapies, and community outreach to help improve the lives of sickle cell patients.

BloodCenter provides expertise and works to improve care for sickle cell patients in several ways, such as:
  • Clinical research to better diagnose, treat and improve the lives of sickle cell patients
  • Management and transfusion support for chronic transfusion-dependent patients, including red cell exchange for patients with sickle cell disease
  • Finding and managing donors, as sickle cell disease presents special challenges in matching donor blood types
  • Community outreach to raise awareness of the disease, to provide information to help support those who have it, and to encourage blood donorship

Service Approach
In collaboration with local partner hospitals, BloodCenter of Wisconsin contributes to the operation of sickle cell clinics that provide clinical research and comprehensive patient care for adults and children with sickle cell disease.

Adult Sickle Cell Disease Clinic at Froedtert Hospital
Opened in September 2011, this clinic provides comprehensive management of adult patients with sickle cell disease including hydroxyurea and transfusion management, iron chelation therapy, pain management, preventative treatment and social services. The clinic is staffed Monday through Friday and provides an outpatient infusion for management of acute pain episodes.

Sickle Cell Center at Children’s Hospital of Wisconsin
This center provides a comprehensive care model to roughly 400 children with sickle cell disease annually. It offers inpatient and outpatient care at Children’s Hospital of Wisconsin. Health care providers and scientists from seven Medical College of Wisconsin departments participate in research activities. Basic, translational and outcomes research are conducted by faculty and staff who also work with partners in the community to provide outreach communication and education to the public.

Research
BloodCenter conducts a variety of clinical research in sickle cell disease for both adult and pediatric patient care.

Adult clinical research areas include:
  • Regadenoson in Sickle Cell Anemia
  • Fibrocytes in the Pathogenesis of Sickle Cell Lung Disease
  • Lymphocyte Activation in Sickle Cell Lung Disease
  • Evaluation of microvascular blood flow, using imaging techniques
Pediatric research areas include:
  • Basic Science
  • Cell free hemoglobin, lipid oxidation and nitric oxide in SCD
  • Mechanisms of vascular injury in murine heritable hemolytic anemia
  • Mechanisms of Vaso-occlusion in SCD: Role of Coagulation Pathway
  • Erythrocyte adenine nucleotide metabolism
  • Red Blood Cell Adhesion in SCD
  • HDL Dysfunction and Vascular Inflammation in SCD
  • Translational Science
  • Asthma and Nocturnal Hypoxemia in Sickle Cell Anemia
  • Cysteinyl Leukotrienes Receptor Inhibitors: A target for decreasing SCD-related morbidity
  • Pain in mouse model of SCD and hemolytic anemia
  • Pain outcomes in human SCD
  • Intravenous Magnesium for acute painful crisis
  • Patient Outcomes Project
  • Quality of life in children with SCD
  • Improvement of communication process and outcomes after newborn genetic screening
  • Reducing hospital readmissions for children with SCD
  • Emergency department utilization
  • Wisconsin Newborn Screening Program
  • Sickle Cell Scholar
  • Summer-for-Sickle-Cell-Science program