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Sickle Cell Disease

Sickle Cell Disease. Sickle Cell Disease. Group of genetic disorders characterized by: 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)

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Sickle Cell Disease

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  1. Sickle Cell Disease

  2. Sickle Cell Disease • Group of genetic disorders characterized by: • 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) • Also occurs in descendents of Mediterranean, Indian, Asian, and Caribbean cultures

  3. Sickle Cell Syndromes • Sickle Cell Trait (HbS gene) • Sickle Cell Anemia (HbS/HbS) • Hgb SC Disease • Sickle Beta-Thalassemia

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

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

  6. CSA: Complications • CNS complications • Cardio-pulmonary complications • Acute Chest Syndrome • Chronic lung disease

  7. SCA: Complications • Hepatobiliary • Spleen atrophies • Gallstones • Liver failure • Genitourinary • Priapism • Delayed puberty • Amenorrhea • Infertility

  8. SCA: Complications • Musculoskeletal • Acute painful crisis • Avascular necrosis • Immunological • Susceptible to infection

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

  10. SCD: Medical Pain Management • Vigorous hydration • Warmth • Rest • Analgesic medications • Demerol • Morphine • Nubain

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

  12. SCD: Pain Management • Biofeedback • Relaxation training • PMR • Deep breathing • Autogenics • Imagery-based techniques

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

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

  15. SCD: Psychological Issues • Social Competence • Peer relationships • Family relationships

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

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

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