Professional Documents
Culture Documents
Epidemiology
Occurs in endemic malarial areas sub-Saharan Africa, parts of India and the Middle
east
High carrier frequency among infants helps protect against malaria
Effects 90,000 people in America, mainly those of African-American decent
44/50 states provide universal neonatal screening for SCD
Clinical Manifestations
Newborns are usually asymptomatic (HbF has no beta globin 2 alpha & 2 gamma chains)
Hemolytic Anemia develops over 2-4 months as HbF is replaced
Splenic damage causes children to become functionally asplenic by the age of 5
FIRST PRESENT WITH HAND-FOOT syndrome (dactylitis)
Painful swelling of hands and feet symmetrically
List of Clinical
Manifestations
Lab Work
Increased reticulocytes
Mild to moderate anemia
Abnormal PFTs
Sickle RBCs
Normal MCV
Severe Anemia target cells,
poikilocytes, hypochromasia, HollyJolly bodies (lack of spleen)
Bone marrow markedly hyperplastic
Diagnosis
Prophylaxis
Educate guardians and patients about the various manifestations of SCD and home
remedies of clinical manifestations
Stay hydrated and NSAIDs for pain/inflammation
Immunizations Pneumococal (13 & 23), meningococcal, H. influenza type B are highly
recommended
Live attenuated vaccine for influenza is contraindicated
Treatment
Acute, painful episodes are the most common reason for individuals with SCD to seek
medical attention
Pain can be associated with ischemia and tissue inflammation, includes Acute Chest
Syndrome, sickle cell crisis and dactylitis
Administration of Fluids and Opioids followed with potential precipitating condition
management.
At home oral fluids and NSAIDs with mild opiates (acetaminophen with codeine)
ED Analgesic Opioids and hydration with treatment of precipitating condition (infection?
Ischemia?)
Review
Presentation infant with symmetric hand-foot swelling, pain crisis family from sub-Saharan
Africa
Clinical Manifestations
Vasooclusive infarcts, strokes, hemolytic anemia, acute chest syndrome
Infections Aplastic crisis (B19), encapsulated bacteria (sepsis), osteomyelitis (salmonella)