Pediatric emergency medicine trisk 552

TABLE 93.5
TREATMENT OF ANEMIA
Cause

Treatment

Iron deficiency

Iron supplementation and increase dietary intake

Vitamin B12 and folic
acid deficiency
Lead poisoning

Cobalamin and folate supplements

Hemolytic anemia

Supportive care with iron and folate supplementation and
cessation of possible causative medications; autoimmune:
steroids or intravenous immunoglobulin (IVIG),
plasmapheresis; transfusion if clinically unstable
Treat underlying disease process

Anemia of chronic
disease
Aplastic anemia

Remove child from site of exposure; intestinal irrigation if
radiographs demonstrate retained lead particles; chelation
therapy if severe; iron and folate supplementation

Bone marrow transplant; supportive care with transfusion

Thalassemia

Chronic transfusions, iron chelation for prevention of iron
overload and close monitoring for end-organ effects of
iron toxicity; bone marrow transplant

Transient
erythroblastopenia of
childhood
Diamond–Blackfan
anemia

Supportive, self-resolving; transfusion only in the case of
clinical decompensation

Gastrointestinal losses

Supportive, acid blocker; endoscopy to identify source of
bleeding; octreotide infusion and transfusion if severe

Menstrual losses

Supportive, hormonal (e.g., estrogen) therapy, tranexamic
acid, and transfusion if severe

Steroids; if unresponsive, bone marrow transplant;
supportive care with transfusions

CLINICAL PEARLS AND PITFALLS


Sickle cell patients require aggressive pain control when they present with a
vasoocclusive episode. Management includes IV fluids to improve vascular
flow, nonsteroidal anti-inflammatory drugs (NSAIDs), and administration of
narcotics as well as other analgesics.
Acute chest syndrome presents as fever, respiratory symptoms (increased
work of breathing, hypoxia, or chest pain), and a new infiltrate on CXR. Acute
chest syndrome can evolve rapidly to become life threatening.
Patients with sickle cell disease have a 300-fold higher risk of stroke
compared with other children. New neurologic findings should prompt
emergent hematology consultation, plans for exchange transfusion, and head
imaging.
Patients with sickle cell disease are functionally asplenic and at increased
risk of severe bacterial infections. Fever >101.5°F/38.5°C necessitates blood
culture, other cultures as clinically indicated, and empiric coverage with a
broad-spectrum antibiotic such as ceftriaxone.
Splenic sequestration results from pooling of blood within the spleen and can
lead to hypovolemic shock necessitating transfusion. Most presentations are
in children <3 years old; however, later presentations can occur in patients
with HbSC or Hb sickle β-thalassemia.

Clinical Considerations
Clinical Recognition
Patients with sickle cell disease require prompt evaluation for complications including
stroke, vasoocclusive episodes, acute chest syndrome, splenic sequestration, infection,
and priapism. Consider the diagnosis of sickle cell disease in genetically susceptible
children with unexplained pain or swelling (especially of the hands or feet), pneumonia,

meningitis, sepsis, neurologic abnormalities, splenomegaly, or anemia. Recognition of
sickle cell disease is key to appropriate intervention. Hemoglobin electrophoresis
provides definitive diagnosis of sickling disorders, although it is not usually performed
in the ED.
Triage
Immediately evaluate any sickle cell patient with fever, concern for stroke, acute chest
syndrome, splenic sequestration, or priapism.

Vasoocclusive Pain Episodes
Initial Assessment/H&P
Vasoocclusive pain episodes are responsible for nearly 90% of all emergency
department visits and 70% of hospitalizations related to sickle cell disease. Pain may be


severe and debilitating for patients. Many patients live their entire lives with some
degree of baseline pain. Cold exposure and dehydration trigger increased sickling. Joint
pain presents a diagnostic challenge because vasoocclusive pain and the symptoms of
trauma and infection (septic arthritis and osteomyelitis) present similarly and the
physical examination findings, laboratory tests, and imaging features are often
nonspecific. For a given patient, pain that is typical and/or at a recurrent location
supports a vasoocclusive etiology. Dactylitis (infarction of the metacarpals, metatarsals,
and phalanges) usually results in swelling of the hands and feet and is a common
presentation in children 6 months to 2 years of age. Pain usually resolves after several
days, but swelling may persist for 1 to 2 weeks.
Management/Diagnostic Testing
No definitive test exists for diagnosing a vasoocclusive episode. A pathway for the
management
of
sickle
cell

disease
and
pain
is
avaliable
at
.
Hydration and prompt pain management are key ( Table 93.6 ). Improving intravascular
volume with a normal saline bolus of 10 to 20 mL/kg followed by fluids at 1 to 1.5
times maintenance, depending on patient’s hydration status, can help improve distal
blood flow. Overly aggressive fluid management should be avoided due to the risk of
hemodilution in the setting of anemia or cardiac strain in a patient with known or
suspected underlying cardiac dysfunction. Concurrently, initiate a pain control regimen
with the use of NSAIDs, such as ketorolac or ibuprofen, acetaminophen, and narcotics.
Frequent and appropriately dosed narcotics are essential, keeping in mind that patients
with regular home use of oral narcotics may have a degree of tolerance. Timely and
efficient use of pain control early in the crisis decreases hospital admission rates and
length of stay. Patients can frequently identify the medications that best treat their pain.
In some circumstances, oral, intranasal, or intramuscular administration of opiates is
appropriate when IV access is not quickly available. Consider patient/nurse-controlled
analgesia (PCAs) and admission for those patients with inadequate analgesia after 2 to 3
doses of parental narcotics. Transdermal and subcutaneous administration of opioids can
provide adjunctive therapy. Hesitancy in using parenteral narcotics may result in
inadequate pain relief, mounting anxiety, and a loss of trust between physician and
patient. This is a particularly common occurrence when the patient has repeated visits to
the ED and physicians are suspicious of the stated degree of discomfort. Monitoring
patients’ self-reported pain scales at regular intervals will help determine the
effectiveness of pain management.
Consider alternative diagnoses when pain fails to respond to appropriate treatment. If
septic arthritis is strongly suspected, arthrocentesis may be required. Gram stain and

culture are important in guiding management since the cell count and differential may
be similar in septic arthritis and infarction. Bone biopsy may help diagnose
osteomyelitis. Staphylococcus aureus is the most common cause of osteomyelitis;


however, Salmonella and other gram-negative organisms (including Escherichia coli )
are more common in sickle cell patients than the general population.
Patient disposition depends on initial response to therapy. Consider discharge home
on a standing NSAID and narcotic regimen for those patients who have significant
improvement or complete resolution of pain. Instruct patients to decrease frequency of
pain medication use over 48 to 72 hours in consultation with hematologist or PCP.

Visceral Pain Crises and Hepatobiliary Disease
Initial Assessment/H&P
Infarction of abdominal organs may produce symptoms similar to other nonhematologic
disease processes. In the child with sickle cell disease and right upper quadrant
abdominal pain, the differential diagnosis includes liver infarction from sickling in the
vascular beds, cholelithiasis secondary to calculi due to chronic hemolysis, biliary
obstruction, as well as diseases not strictly related to underlying sickle cell disease
including hepatitis from infectious causes, pancreatitis, and peptic ulcer disease.
Common surgical problems such as appendicitis may be difficult to assess and
laboratory values may not differentiate visceral vasoocclusive crises from other
problems.