Sickle Cell Case Study

A.R., an 8 year old male child with SCD, is admitted to the ER complaining of right
knee and lower back pain. His mother gave him Tylenol with Codeine for pain but had
no relief and is having a difficult time getting him to drink fluids. She tells you he was
sick the week prior with a stomach virus and temperature of 101.8º. When asked the last
time he required a transfusion, the mother said about a year ago when he had Fifth
Disease.
Vital signs: 99 – 120- 26- 120/78
Medications: Penicillin, Folic Acid and Hydroxyurea
Immunizations are up to date

1. What is the pathophysiology of SCD?

 Substitution of the amino acid (Valine) for Glutamic acid in the 2 beta chains of
the hgb
 Causes a change in RBC so that when Hgb is deoxygenated, it crystalizes and
change the shape of RBC
o Change from disk shape to a sickle cell shape
 Initially, it can be reversed with O2
 But after episodes, there is a damage to the RBC membrane and it cannot be
change back to the bio-concave shape and hemolysis happens
 = occlusion and damage to the walls of the vessels over time due to the sickled
RBCs
2. What crisis is A.R. experiencing?
 Sickle Cell Vaso-occlusive
o Pain
o Swelling
What are A.R.’s triggers for this crisis?
 Infections
 Cold weather
o Trigger vasoconstriction
 Poor fluid intake
 Temperature
o Fever

3. What other crisis can patients with SCD experience?

 Acute Chest Syndrome
 Infections
 Anemia
 Shortness of Breath, Lightheadedness

What are the signs and symptoms associated with these crises?
 Sequestrations (spleen or liver)- pooling of blood in liver or spleen causing
hepatomegaly/ splenomegaly, decrease in hemoglobin and hematocrit can lead to
shock because of decrease circulating blood volume

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 o Bc RBC is stuck in the organs so there is a decrease in hemoglobin and
hematocrit
o = causing hypovolemic shock
 Aplastic- decrease RBC production causing profound anemia, can be caused by
parvovirus
o Very dangerous when having 5th disease prior
 Hyperhemolytic- increase rate of RBC destruction causing anemia, jaundice and
increase in reticulocytes (immature red blood cells)
o Compensating for the rapid breakdown of red blood cell so there are more
reticulocytes
 Acute Chest Syndrome- chest pain, fever, pneumonia like cough, anemia
 Megaloblastic- excessive neeed for folic acid and vitamin B12
 CVA- stroke
4. How will A.R.’s pain be managed and what are the side effects of pain medications?

5. Why is A.R. on Penicillin and Hydroxyurea?

Are there any immunizations that are contraindicated with SCD and do they need
additional immunizations?

6. Is there a connection between Fifth Disease and the need for a blood transfusion?
What are indications for blood transfusions in patients with SCD?
Why do you administer Desferal while receiving a blood transfusion?

A.R’s mother tells you she is pregnant and asks you the following questions:

7. “Can they test the baby for SCD before he/she is born”
 Chorionic Villus Sampling- check tissue from the placenta for birth defects and
genetic conditions (10-12 weeks of pregnancy)
 Amniocentesis- check amniotic fluid from the amniotic sac around the baby for
birth defects and genetic conditions (15-20 weeks of pregnancy)
“Does the baby get tested for SCD when he/she is born”
 State newborn screening
 Hemoglobin electrophoresis blood test can confirm the diagnosis
o Looks for the sickle cell trait/ gene

8. What are long term complications of SCD?

 Pain
 Anemia
 Splenic Sequestration
o When large amount of sickle cells get trapped in the spleen
 Vision loss
 Infections
 Avascular necrosis- when bones of the major joints cause bone tissue to die
 Blood clots
 Deep Vein Thrombosis
 Pulmonary Embolism

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  Stroke
Is there a cure for SCD?
 Bone marrow transplant
 Gene therapy

9. What should be included in discharge education for A.R.?

 Fluids
 Fever temperature lower
 Infection
 Palpate spleen
o Sign of splenomegaly (sequestration)

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