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)
 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

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
Clinical manifestations do NOT appear
until after ~ 6 months
 Vaso-occlusive pain crises

CSA: Complications

CNS complications

Cardio-pulmonary complications
Acute Chest Syndrome
 Chronic lung disease

SCA: Complications

Hepatobiliary
Spleen atrophies
 Gallstones
 Liver failure


Genitourinary
Priapism
 Delayed puberty
 Amenorrhea
 Infertility

SCA: Complications

Musculoskeletal
Acute painful crisis
 Avascular necrosis


Immunological

Susceptible to infection
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
Vigorous hydration
 Warmth
 Rest
 Analgesic medications

Demerol
 Morphine
 Nubain

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

Biofeedback

Relaxation training
PMR
 Deep breathing
 Autogenics
 Imagery-based techniques

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 Issues

Emotional and behavioral competence of
children
Self-concept
 Externalizing behavior problems
 Internalizing behavior problems


*links between depression/anxiety and pain
perception
SCD: Psychological Issues

Social Competence

Peer relationships

Family relationships
SCD: Coping Strategies

Related to:
Adjustment (e.g., anxiety)
 Activity level
 Health care services
 Distress in episode

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
