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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 disease2
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
sickle cell syndromes
Sickle Cell Syndromes
  • Sickle Cell Trait (HbS gene)
  • Sickle Cell Anemia (HbS/HbS)
  • Hgb SC Disease
  • Sickle Beta-Thalassemia
sca impact on red blood cells
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
sca complications
SCA: Complications
  • Clinical manifestations do NOT appear until after ~ 6 months
  • Vaso-occlusive pain crises
csa complications
CSA: Complications
  • CNS complications
  • Cardio-pulmonary complications
    • Acute Chest Syndrome
    • Chronic lung disease
sca complications7
SCA: Complications
  • Hepatobiliary
    • Spleen atrophies
    • Gallstones
    • Liver failure
  • Genitourinary
    • Priapism
    • Delayed puberty
    • Amenorrhea
    • Infertility
sca complications8
SCA: Complications
  • Musculoskeletal
    • Acute painful crisis
    • Avascular necrosis
  • Immunological
    • Susceptible to infection
scd transfusion therapy
SCD: Transfusion Therapy
  • Used to treat certain complications:
    • Splenic sequestration
    • Aplastic crisis
    • Priapism
    • Stroke
  • Used to prevent complications
    • Repeated strokes
    • Surgery-related problems
scd medical pain management
SCD: Medical Pain Management
  • Vigorous hydration
  • Warmth
  • Rest
  • Analgesic medications
    • Demerol
    • Morphine
    • Nubain
scd psychological issues
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
scd pain management
SCD: Pain Management
  • Biofeedback
  • Relaxation training
    • PMR
    • Deep breathing
    • Autogenics
    • Imagery-based techniques
scd pain management13
SCD: Pain Management
  • Pain behavior contracts
    • Hydration
    • Deep breathing
    • Out of bed activities
    • Dosage/frequency of pain medications
  • Patient controlled anesthesia (PCA pump)
scd psychological issues14
SCD: Psychological Issues
  • Emotional and behavioral competence of children
    • Self-concept
    • Externalizing behavior problems
    • Internalizing behavior problems
      • *links between depression/anxiety and pain perception
scd psychological issues15
SCD: Psychological Issues
  • Social Competence
    • Peer relationships
    • Family relationships
scd coping strategies
SCD: Coping Strategies
  • Related to:
    • Adjustment (e.g., anxiety)
    • Activity level
    • Health care services
    • Distress in episode
scd now and the future
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