How would you evaluate and manage a pediatric patient who has a painful swelling of the hands and
feet, fatigue, or fussiness? Which diagnostic studies would you recommend for this patient and why?
Sickle cell disease (SCD) is an inherited chronic hemolytic anemia related to the abnormal S
hemoglobin. This disease is characterized by the occurrence of acute complications such as painful vaso-
occlusive crises, acute chest syndrome, cerebrovascular accidents, acute splenic sequestration. Clinical
manifestations vary from patient to patient. In some, the disease can be severe with frequent
hospitalizations, whereas in others it can be moderate. Sickle cell symptoms typically begin to emerge in
the second 6 months of life as the number of Hgb S increases and Hgb F declines. Subsequently, painful,
vaso-occlusive crises occur (Meier & Miller, 2013). Due to the multisystem nature of complications, these
children need prompt, detailed evaluation and intervention (Burns et al., 2016). In an instance a child
presents with Miah’s symptoms, the health care provider should inquire about common childhood
illnesses such as respiratory infections, ear infections, diarrhea, vomiting, dehydration, individual, and
family history. Joint pain and swelling are common manifestations of musculoskeletal and rheumatologic
diseases. Miah’s differential diagnosis could be several benign and severe conditions. Her assessment
needs to differentiate between conditions of varying severity, especially conditions that warrant urgent
medical attention. Diagnostic studies that are essential to developing a correct diagnosis are:
CBC with differential and reticulocyte count (Hollier, 2018)
Peripheral Blood smear (Hollier, 2018)
C-reactive protein (Hollier, 2018)
liver function test (Hollier, 2018)
LDH (Hollier, 2018)
hemoglobin electrophoresis (Hollier, 2018)
blood smear (Hollier, 2018)
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