• 14 YEAR OLD BLACK MALE WITH
SICKLE CELL DISEASE ADMITTED TO
THE ED WITH AN ACUTE PAIN EPISODE
UNDERSTANDING SICKLE CELL DISEASE
• INHERITED
• RACE
• AUTOSOMAL RECESSIVE
• RBC’S CHANGE SHAPE INTO A
CRESCENT OR SICKLE
• SICKLE CELL NAMES
• DEFECTIVE HEMOGLOBIN
• 4 PEPTIDE CHAINS
• AUTOSOMAL RECESSIVE
• NEED BOTH COPIES OF
MUTATION TO HAVE
DISEASE
• ONE COPY OF MUTATION
IS A CARRIER
DEFECTIVE HEMOGLOBIN
DEOXYGENATION
• INFANTS AGE 6-12 MONTHS
• ABNORMAL HBS PRESENT IN RBC’S
• TRIGGERS FOR SICKLING
• PERSISTENT HYPOXEMIA
• CORRECT HYPOXEMIA OR SICKLE CELLS CLOG VESSELS
CASCADE OF EVENTS
Triggers erythropoiesis
vaso-occlusion of microcirculation
Decreased blood ph
Increased osmolality/dehydration
Low temperatures
VASO-OCCLUSION
CLINICAL PRESENTATION CAN INCLUDE:
1.
2.
3.
4.
5.
6.
SWELLING IN HANDS/FEET
PAIN IN JOINTS
AVASCULAR NECROSIS
PRIAPISM
SEVERE ABDOMINAL PAIN
CEREBRAL INFARCTS
APLASTIC CRISIS
• APLASTIC CRISIS
• TRANSIENT CESSATION OF RBC PRODUCTION
• ACUTE ANEMIA
• DUE TO VIRAL INFECTION
• HEMOLYSIS CONTINUES
• SEVERE DROP IN HGB & RETICULOCYTE COUNTS
SEQUESTRATION CRISIS
• BLOOD BACKS UP IN LIVER & SPLEEN
• SPLENIC SEQUESTRATION *LIFE-THREATENING*
• YOUNG CHILDREN ONLY
• HYDRATION & BLOOD TRANSFUSION
• RECURRENT SEQUESTRATION
• INCREASED RISK FOR INFECTION
ACUTE CHEST SYNDROME
New pulmonary infiltrate
Chest pain
Increased temp >101.3
Increased respirations, wheezing, cough
Sickled cells attach to endothelium
Poor prognosis
IDENTIFICATION OF SICKLE CELL DISEASE
Newborn blood spot screen
Blood smear
Protein electrophoresis
Chorionic villus sampling
Amniotic fluid analysis
TREATMENT & MAINTENANCE
Oxygen
Iv fluids
rbc
transfusions
Aggressive
management
of fever
Antibiotics
Pain
management
SUPPORTIVE CARE
TEACHING AND SUPPORT ORGANIZATIONS
AVOIDANCE OF RISK FACTORS
VACCINATIONS
OTHER TREATMENTS
• HYDROXYUREA
• FOLIC ACID
• LAPAROSCOPIC SPLENECTOMY
• STEM CELL THERAPY