Rapid access to higher-level care, including parenteral analgesia, is important if outpatient management is desired. In addition, escalation to a higher level of care should occur if there is hypoxia (or another reason to suspect acute chest syndrome [ACS; discussed in a bit]) or dehydration that requires parenteral therapy. Use of nondrug therapy, such as heat, should be encouraged. The care team should work with the patient’s school or employer to negotiate time away through the federal Family Medical Leave Act of 1993, or other means, because a pain crisis is not a planned event.
Fever. Because of the risk of serious infection as a consequence of functional asplenia, fever is particularly worrisome in patients with SCA and problematic in patients with SCD. The increased risk begins as the physiologic level of Hb F declines beginning at age 2 months.
ACS, characterized by a combination of respiratory symptoms or new infiltrates, often manifests initially with fever, and can progress rapidly to death if treatment is delayed. The initial signs and symptoms may be subtle; suspicion should remain high in any patient with respiratory symptoms who is newly hypoxic, even those who do not have fever. Osteomyelitis, another febrile illness, is also potentially life threatening if not treated promptly.
All patients with SCD whose body temperature is > 101.3 °F should be evaluated with appropriate clinical laboratory testing (complete blood count; inflammatory markers, such as C-reactive protein; basic chemistry parameters; and other tests as indicated, including serum lactate and urine culture), blood culture, and chest radiography. Empiric parenteral antibiotics are required until the patient is known to be nonbacteremic, regardless of vaccination status. Outpatient follow-up and even outpatient management with ceftriaxone can be offered in select circumstances (eg, if the patient so desires or is nontoxic, and if close follow-up can be assured).14 If ACS is a possibility based on symptoms or radiographic findings, outpatient management is not an option.
Anemia. Patients with SCA, and some with SCD, have an Hb level that is chronically, sometimes critically, low. A baseline Hb level should be established for a patient with SCD and then monitored periodically. A drop in the Hb level > 2 g/dL from baseline (or an initial Hb level of 6 g/dL if the baseline is unknown) constitutes acute anemia. Patients in whom anemia has been diagnosed should be emergently evaluated for acute splenic sequestration, an aplastic episode, a delayed hemolytic transfusion reaction, ACS, or infection, and should be treated appropriately.
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