Sickle Cell Anemia
Patient Presentation
D.C. is a five year old African American male that presents to the pediatric
emergency room with chest pain. D.C. exhibits tachycardia (HR of112 bpm),
tachypnea (Respiratory rate of 30 breaths/minute), shortness of breath, and
an oral temperature of 100.3 degrees Fahrenheit.
D.C.’s labs are as follows Hgb 8 g/dL Hct 28%, WBC 11,000/mm3. D.C. also
tested negative for ANA (Antinuclear antibody). D.C. was tested for possible
infection so urine and sputum cultures were done. D.C.’s labs showed
positive sputum cultures for Streptococcus pneumoniae. D.C. had an abnormal
chest X-ray as a result of the infection.
Diagnosis
D.C. was found to be in acute chest syndrome secondary to sickle cell
anemia. Acute chest syndrome is caused by a vaso-occlusive crisis or
infection which results in sickling of red blood cells in the small blood
vessels of the lungs which leads to occlusion, stasis, and anemia. Acute
chest syndrome is a life threatening condition that requires rapid medical
management. A vaso-occlusive crisis is when tissue ischemia occurs as a
result of sickling red blood cells which leads to pain in the involved
areas. D.C.’s father was a sickle cell carrier and his mother was also a
carrier for sickle cell. D.C. was diagnosed with sickle cell anemia before
age one.
D.C. underwent a splenectomy at the age of four. The splenectomy was
necessary because the spleen became congested and engorged with sickled
cells. This congestion and engorgement of the spleen caused sequestration
crisis (or pooling of large amounts of blood) in the spleen and then a
splenectomy was done. Patients who have a splenectomy are at increased risk
for infection because the spleen is a secondary lymphoid organ that protects
against infection. Repeated episodes of acute chest syndrome may cause
restrictive lung disease and pulmonary hypertension for patients with sickle
cell anemia.
Sickle cell anemia is one of a group of diseases known as hemoglobinopathies
in which adult hemoglobin (Hgb A) is partially or completely replaced by
abnormal sickle cell hemoglobin (Hgb S). Sickle cell disease refers to a
group of hereditary disorders which are related to the presence of Hgb S,
these include sickle cell anemia, sickle cell C disease, and sickle
thalassemia. Sickle cell disease is the most common genetic blood disease in
the U.S. Sickle cell anemia is the most common form of sickle cell disease
found in African Americans in the United States.
Treatment
D.C. was placed on oxygen and was given intravenous fluids and oral
hydration to increase fluid volume. D.C. was later admitted to the pediatric
unit and was a started on a course of clindamycin to treat the Streptococcus
pneumoniae. D.C. was also given Percocet® to reduce pain secondary to the
vaso-occlusive crisis. D.C.’s parents were given education on how to avoid
infection by giving him annual vaccines and avoiding contact with sick
individuals due to D.C.’s high susceptibility for infection. His parents
were also educated on how to promote frequent rest periods for their son
during physical activities such as running or playing sports.
Case created by Deborah Cleveland,
2010.