Sickle Cell Disease Sickle Cell Disease l Group

Sickle Cell Disease

Sickle Cell Disease l Group of genetic disorders characterized by: l l Hemolytic anemia - not enough red blood cells in the blood Vasculopathy - disorder of blood vessels In US, predominantly with African-Americans (i. e. , 1 in 400) l Also occurs in descendents of Mediterranean, Indian, Asian, and Caribbean cultures l

Sickle Cell Syndromes l Sickle Cell Trait (Hb. S gene) l Sickle Cell Anemia (Hb. S/Hb. S) l Hgb SC Disease l Sickle Beta-Thalassemia

SCA: Impact on Red Blood Cells l With low oxygen levels, the shape of red cells becomes distorted l They appear elongated and sickle shaped l Red blood cells become very fragile, short lived, and can clog blood vessels

SCA: Complications l Clinical manifestations do NOT appear until after ~ 6 months l Vaso-occlusive pain crises

CSA: Complications l CNS complications l Cardio-pulmonary complications Acute Chest Syndrome l Chronic lung disease l

SCA: Complications l Hepatobiliary Spleen atrophies l Gallstones l Liver failure l l Genitourinary Priapism l Delayed puberty l Amenorrhea l Infertility l

SCA: Complications l Musculoskeletal Acute painful crisis l Avascular necrosis l l Immunological l Susceptible to infection

SCD: Transfusion Therapy l Used to treat certain complications: Splenic sequestration l Aplastic crisis l Priapism l Stroke l l Used to prevent complications Repeated strokes l Surgery-related problems l

SCD: Medical Pain Management l Vigorous hydration l Warmth l Rest l Analgesic medications Demerol l Morphine l Nubain l

SCD: Psychological Issues l Consequences of repeated pain crises Compromises daily routines l Repeated hospitalizations l Side effects of narcotic analgesics l Development of “dependent” behaviors l Apprehension, anxiety, depression l

SCD: Pain Management l Biofeedback l Relaxation training PMR l Deep breathing l Autogenics l Imagery-based techniques l

SCD: Pain Management l Pain behavior contracts Hydration l Deep breathing l Out of bed activities l Dosage/frequency of pain medications l l Patient pump) controlled anesthesia (PCA

SCD: Psychological Issues l Emotional and behavioral competence of children Self-concept l Externalizing behavior problems l Internalizing behavior problems l l *links between depression/anxiety and pain perception

SCD: Psychological Issues l Social Competence l Peer relationships l Family relationships

SCD: Coping Strategies l Related to: Adjustment (e. g. , anxiety) l Activity level l Health care services l Distress in episode l

SCD: Now and the Future l Longevity and Quality of Life Has improved over past 20 years l Prophylactic penicillin l Newer antibiotics l Judicious use of blood transfusions l l Bone marrow transplant as “cure” l New drugs to decrease pain episodes l Genetic manipulation