NR 328 EDAPT NURSING CARE SICKLE CELL ANEMIA|2025 Update with complete solution
NURSING CARE: SICKLE CELL ANEMIA
Sickle cell anemia is the homozygous and most common form of sickle cell disease. Sickle cell anemia is an
inherited, autosomal-recessive genetic disorder that affects the red blood cells (RBCs), which become acutely
sickle-shaped, occluding small blood vessels (blocking blood flow) causing pain and decreased function. The
child must receive the autosomal-recessive gene from both parents in order to have sickle cell anemia. If a
child has sickle cell trait, then the child inherited the gene from one parent but is asymptomatic.
Sickle Cell Genetics
A newly-married couple, who are both carriers of the sickle cell trait, ask what the risk is of their children
having sickle cell anemia. What is the best response by the nurse?
Sickle cell anemia is an autosomal recessive condition requiring the gene from both parents. When both
parents are carriers, each pregnancy presents with a 25% chance that a child will be born with the disease and
a 50% chance that the child will have the sickle cell trait.
Risk Factors
A nurse is teaching the parents of a child with sickle cell disease about factors that predispose the child to a
sickle cell crisis. The nurse determines that the teaching was successful when the parents identify what as a
factor?
The body’s response to infection can trigger a sickle cell crisis. Avoid infection by practicing good hand hygiene
and avoiding individuals who are ill. Receive early treatment for infections as fever can trigger a crisis.
Sickle Cell Disease Manifestation
In a child diagnosed with sickle cell disease, which clinical manifestations would the nurse most likely see?
Select all that apply.
Clinical manifestations of sickle cell disease are:
• acute pain due to blocked blood vessels and tissue ischemia in the:
• extremities, especially the hands, feet, and joints because of the smaller capillaries and reduced
blood flow to those areas
• abdomen
• chest
• liver - jaundice and hepatic coma
• kidney - hematuria and impaired function
• brain - stroke
• genitalia - painful erection (priapism)
• shortness of breath, fatigue
• pallor, pale mucous membranes
• jaundice
• hands and feet cool to the touch
• dizziness
• headache
Sickle Cell Anemia
Sickle cell anemia is a congenital hemolytic disease resulting from a defective hemoglobin molecule (Hgb S)
that causes red blood cells (RBCs) to become sickle-shaped. Sickle cell anemia is a chronic illness with distress
, resulting from blocked and inadequate circulation and tissue/organ damage that causes pain over time, organ
failure, and death.
Pathophysiology
The pathology of sickle cell anemia is that the RBCs become rigid, rough, and elongated, forming a crescent or
sickle shape. This process is called sickling, which can produce hemolysis (cell destruction). The altered cells
accumulate in capillaries and smaller blood vessels, making the blood more viscous. Normal circulation is
impaired, causing pain, tissue ischemia and infarctions, and swelling.
Risk Factors
Sickle cell anemia is an autosomal recessive condition requiring genes from both parents. When both parents
are carriers, each pregnancy presents a 25% chance that a child will be born with the disease and a 50%
chance that the child will have the sickle cell trait.
Sickle cell anemia primarily affects African-Americans, but may also be seen in those of Mediterranean,
Indian, Middle Eastern or Hispanic descent.
Sickle Cell Crisis
Children with sickle cell disease have three types of problems or crisis episodes. A crisis episode can be the
result of:
Vaso-Occlusive Crisis
• This is the most common, non-life-threatening crisis related to blocked blood flow (ischemia) of tissues
and bone from sickling.
• Blood stasis causes ischemia and infarction. Seriousness of sequalae depends upon the site of the
occlusive crisis.
• Acute (related to dehydration and decreased oxygen) vaso-occlusive crisis:
o Severe pain, usually in bones, joints, and abdomen but can be located anywhere on the body
(e.g. low back pain)
o Hematuria
o Obstructive jaundice
o Visual disturbances
o Tissue engorgement
Sequestration
• This is a potentially life-threatening crisis where there is excessive pooling of blood primarily in the
spleen (splenomegaly) and sometimes in the liver (hepatomegaly) resulting in lethargy, pallor, and
hypovolemia which can progress to shock.
• Hypovolemic shock signs include irritability, tachycardia, pallor, decreased urinary output, tachypnea,
cool extremities, thready pulse, and hypotension.
Aplastic Crisis
• This results from an extreme drop/reduction in red blood cells (RBCs; bone marrow suppression– often
a vital trigger) leading to anemia, pallor, lethargy, sleepiness, and dyspnea.
NURSING CARE: SICKLE CELL ANEMIA
Sickle cell anemia is the homozygous and most common form of sickle cell disease. Sickle cell anemia is an
inherited, autosomal-recessive genetic disorder that affects the red blood cells (RBCs), which become acutely
sickle-shaped, occluding small blood vessels (blocking blood flow) causing pain and decreased function. The
child must receive the autosomal-recessive gene from both parents in order to have sickle cell anemia. If a
child has sickle cell trait, then the child inherited the gene from one parent but is asymptomatic.
Sickle Cell Genetics
A newly-married couple, who are both carriers of the sickle cell trait, ask what the risk is of their children
having sickle cell anemia. What is the best response by the nurse?
Sickle cell anemia is an autosomal recessive condition requiring the gene from both parents. When both
parents are carriers, each pregnancy presents with a 25% chance that a child will be born with the disease and
a 50% chance that the child will have the sickle cell trait.
Risk Factors
A nurse is teaching the parents of a child with sickle cell disease about factors that predispose the child to a
sickle cell crisis. The nurse determines that the teaching was successful when the parents identify what as a
factor?
The body’s response to infection can trigger a sickle cell crisis. Avoid infection by practicing good hand hygiene
and avoiding individuals who are ill. Receive early treatment for infections as fever can trigger a crisis.
Sickle Cell Disease Manifestation
In a child diagnosed with sickle cell disease, which clinical manifestations would the nurse most likely see?
Select all that apply.
Clinical manifestations of sickle cell disease are:
• acute pain due to blocked blood vessels and tissue ischemia in the:
• extremities, especially the hands, feet, and joints because of the smaller capillaries and reduced
blood flow to those areas
• abdomen
• chest
• liver - jaundice and hepatic coma
• kidney - hematuria and impaired function
• brain - stroke
• genitalia - painful erection (priapism)
• shortness of breath, fatigue
• pallor, pale mucous membranes
• jaundice
• hands and feet cool to the touch
• dizziness
• headache
Sickle Cell Anemia
Sickle cell anemia is a congenital hemolytic disease resulting from a defective hemoglobin molecule (Hgb S)
that causes red blood cells (RBCs) to become sickle-shaped. Sickle cell anemia is a chronic illness with distress
, resulting from blocked and inadequate circulation and tissue/organ damage that causes pain over time, organ
failure, and death.
Pathophysiology
The pathology of sickle cell anemia is that the RBCs become rigid, rough, and elongated, forming a crescent or
sickle shape. This process is called sickling, which can produce hemolysis (cell destruction). The altered cells
accumulate in capillaries and smaller blood vessels, making the blood more viscous. Normal circulation is
impaired, causing pain, tissue ischemia and infarctions, and swelling.
Risk Factors
Sickle cell anemia is an autosomal recessive condition requiring genes from both parents. When both parents
are carriers, each pregnancy presents a 25% chance that a child will be born with the disease and a 50%
chance that the child will have the sickle cell trait.
Sickle cell anemia primarily affects African-Americans, but may also be seen in those of Mediterranean,
Indian, Middle Eastern or Hispanic descent.
Sickle Cell Crisis
Children with sickle cell disease have three types of problems or crisis episodes. A crisis episode can be the
result of:
Vaso-Occlusive Crisis
• This is the most common, non-life-threatening crisis related to blocked blood flow (ischemia) of tissues
and bone from sickling.
• Blood stasis causes ischemia and infarction. Seriousness of sequalae depends upon the site of the
occlusive crisis.
• Acute (related to dehydration and decreased oxygen) vaso-occlusive crisis:
o Severe pain, usually in bones, joints, and abdomen but can be located anywhere on the body
(e.g. low back pain)
o Hematuria
o Obstructive jaundice
o Visual disturbances
o Tissue engorgement
Sequestration
• This is a potentially life-threatening crisis where there is excessive pooling of blood primarily in the
spleen (splenomegaly) and sometimes in the liver (hepatomegaly) resulting in lethargy, pallor, and
hypovolemia which can progress to shock.
• Hypovolemic shock signs include irritability, tachycardia, pallor, decreased urinary output, tachypnea,
cool extremities, thready pulse, and hypotension.
Aplastic Crisis
• This results from an extreme drop/reduction in red blood cells (RBCs; bone marrow suppression– often
a vital trigger) leading to anemia, pallor, lethargy, sleepiness, and dyspnea.
