ATLAND Nursing
Pediatric Hematological Disorders
Nursing Study Notes
1. Sickle Cell Anemia
Overview
Sickle cell anemia is an autosomal recessive hereditary form of anemia in which the normal
hemoglobin on the red blood cells (Hb A) mutates to abnormal Hemoglobin (Hb S).
Hb S is very sensitive to oxygen hence low
oxygen levels cause the red blood cells to
become sticky, stiff, and assume a
crescent shape, which obstructs blood flow
resulting in pain.
Sickle cells have a lifespan of about 10
days thus increasing hemolysis which
results in anemia.
Fig 1: Vaso-occlusion.
Risk factors include;
Having parents with sickle cell trait.
African American race.
Sickle cell crisis(es); exacerbation of the sickle cell anemia which includes; aplastic crisis,
splenic sequestration, hyper-hemolytic crisis, and vaso-occlusive crisis. The factors that cause
sickling crisis include;
ATLAND Nursing 1 Pediatric Nursing Study Notes
, Use pneumonic ‘SICKLE’
S Significant blood loss, trauma
I Illness
C Climbing at high altitude
K Keeping continued stress
L Low fluid intake
E Elevated temperature
Assessment
a) General
Hemoglobin of 6-9g/dl indicates chronic anemia.
Growth retardation
Possible delayed sexual maturation
Fever
b) Sickle cell crises
i) Aplastic crisis (caused by increased destruction and diminished production of red blood
cells.
Pallor
Profound anemia
ii) Hyper-hemolytic crisis (accelerated rate of red blood cells destruction)
Anemia
Reticulocytosis
Jaundice
iii) Splenic sequestration (Caused by pooling and clamping of blood in the spleen)
Profound anemia
Hypovolemia
Shock
iv) Vaso-occlusive crisis (caused by clamping of cells in microcirculation and stasis of
blood, ischemia, and infarction.)
Fever
Swelling of hands and feet
Abdominal pain
Priapism
ATLAND Nursing 2 Pediatric Nursing Study Notes
Pediatric Hematological Disorders
Nursing Study Notes
1. Sickle Cell Anemia
Overview
Sickle cell anemia is an autosomal recessive hereditary form of anemia in which the normal
hemoglobin on the red blood cells (Hb A) mutates to abnormal Hemoglobin (Hb S).
Hb S is very sensitive to oxygen hence low
oxygen levels cause the red blood cells to
become sticky, stiff, and assume a
crescent shape, which obstructs blood flow
resulting in pain.
Sickle cells have a lifespan of about 10
days thus increasing hemolysis which
results in anemia.
Fig 1: Vaso-occlusion.
Risk factors include;
Having parents with sickle cell trait.
African American race.
Sickle cell crisis(es); exacerbation of the sickle cell anemia which includes; aplastic crisis,
splenic sequestration, hyper-hemolytic crisis, and vaso-occlusive crisis. The factors that cause
sickling crisis include;
ATLAND Nursing 1 Pediatric Nursing Study Notes
, Use pneumonic ‘SICKLE’
S Significant blood loss, trauma
I Illness
C Climbing at high altitude
K Keeping continued stress
L Low fluid intake
E Elevated temperature
Assessment
a) General
Hemoglobin of 6-9g/dl indicates chronic anemia.
Growth retardation
Possible delayed sexual maturation
Fever
b) Sickle cell crises
i) Aplastic crisis (caused by increased destruction and diminished production of red blood
cells.
Pallor
Profound anemia
ii) Hyper-hemolytic crisis (accelerated rate of red blood cells destruction)
Anemia
Reticulocytosis
Jaundice
iii) Splenic sequestration (Caused by pooling and clamping of blood in the spleen)
Profound anemia
Hypovolemia
Shock
iv) Vaso-occlusive crisis (caused by clamping of cells in microcirculation and stasis of
blood, ischemia, and infarction.)
Fever
Swelling of hands and feet
Abdominal pain
Priapism
ATLAND Nursing 2 Pediatric Nursing Study Notes
