Transcript Sickle Cell Disease

Sickle Cell Disease
Sickle Cell Disease
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Group of genetic disorders characterized by:
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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 Trait (HbS gene)
 Sickle Cell Anemia (HbS/HbS)
 Hgb SC Disease
 Sickle Beta-Thalassemia
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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
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SCA: Complications
Clinical manifestations do NOT appear
until after ~ 6 months
 Vaso-occlusive pain crises
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CSA: Complications
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CNS complications
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Cardio-pulmonary complications
Acute Chest Syndrome
 Chronic lung disease
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SCA: Complications
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Hepatobiliary
Spleen atrophies
 Gallstones
 Liver failure
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Genitourinary
Priapism
 Delayed puberty
 Amenorrhea
 Infertility
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SCA: Complications
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Musculoskeletal
Acute painful crisis
 Avascular necrosis
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Immunological
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Susceptible to infection
SCD: Transfusion Therapy
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Used to treat certain complications:
Splenic sequestration
 Aplastic crisis
 Priapism
 Stroke
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Used to prevent complications
Repeated strokes
 Surgery-related problems
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SCD: Medical Pain Management
Vigorous hydration
 Warmth
 Rest
 Analgesic medications
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Demerol
 Morphine
 Nubain
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SCD: Psychological Issues
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Consequences of repeated pain crises
Compromises daily routines
 Repeated hospitalizations
 Side effects of narcotic analgesics
 Development of “dependent” behaviors
 Apprehension, anxiety, depression
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SCD: Pain Management
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Biofeedback
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Relaxation training
PMR
 Deep breathing
 Autogenics
 Imagery-based techniques
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SCD: Pain Management
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Pain behavior contracts
Hydration
 Deep breathing
 Out of bed activities
 Dosage/frequency of pain medications
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Patient controlled anesthesia (PCA
pump)
SCD: Psychological Issues
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Emotional and behavioral competence of
children
Self-concept
 Externalizing behavior problems
 Internalizing behavior problems
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*links between depression/anxiety and pain
perception
SCD: Psychological Issues
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Social Competence
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Peer relationships
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Family relationships
SCD: Coping Strategies
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Related to:
Adjustment (e.g., anxiety)
 Activity level
 Health care services
 Distress in episode
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SCD: Now and the Future
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Longevity and Quality of Life
Has improved over past 20 years
 Prophylactic penicillin
 Newer antibiotics
 Judicious use of blood transfusions
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Bone marrow transplant as “cure”
 New drugs to decrease pain episodes
 Genetic manipulation
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