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