Appeal type: Medical necessity
Decision: Upheld

Diagnosis: Sickle Cell Disease
Treatment: Inpatient Hospital Admission
The insurer denied: Inpatient Hospital Admission
The denial is upheld.

The patient is a female infant with medical history significant for sickle cell disease, acute chest syndrome, prematurity born at 37 weeks' gestation that was taken to the emergency department with same-day history of fever to 101.6 that was treated with acetaminophen. The patient developed cough after presenting to the emergency department. The sister, also with sickle cell disease, was recovering from respiratory symptoms. The patient's vital signs included temperature 38.9, heart rate 168, respiratory rate 36, and 100 percent saturation in room air. Examination was significant for non-toxic appearance, moist mucous membranes, no congestion, clear lungs with no distress, benign abdomen, brisk capillary refill, no rash, and non-focal neurologic exam. Laboratory evaluation was significant for potassium 5.6, carbon dioxide (CO2) 18, total bilirubin 2.3, aspartate aminotransferase (AST) 52, reticulocyte percentage 2.7, white blood cell (WBC) count 5.4, hemoglobin 9.9, 30 percent neutrophils, and 57 percent lymphocytes. Rapid viral screen was negative. Chest x-ray revealed increased perihilar markings and peribronchial cuffing with no focal consolidation. Urinalysis screen was unremarkable. The patient was started on the Fever in Sickle Cell Protocol and admitted because of her age. She was treated with ibuprofen and ceftriaxone prior to admission. Admission orders included ceftriaxone for 48 hours pending culture results (blood culture, urine culture), acetaminophen for fevers, continuation of home medications except penicillin (on hold while on ceftriaxone), and intravenous fluids. Hematology was aware of her presentation. She was stable overnight but spiked a fever 101.1 in the morning of hospital day two. Respiratory pathogen panel was ordered, subsequently reported negative. Cultures were negative pending. 
Fevers responded to antipyretics. The patient was feeding well with good urine output. She was deemed stable for discharge with close outpatient follow-up.

The inpatient hospital admission was not medically necessary.

Sickle cell anemia is a common genetic disease in which hemoglobin in red blood cells is replaced by an abnormal form, hemoglobin S. This causes red blood cells to form sickling shapes that block small blood vessels. In addition, these red blood cells do not survive as long, causing anemia. As a result, multiple complications can occur, including chronic anemia, acute chest syndrome, vaso-occlusive crisis, splenic sequestration, dactylitis, stroke, altered immune function with increased risk of bacterial sepsis, pulmonary hypertension, and renal disease. Fever in patients with sickle cell disease must be taken seriously. These patients are immunocompromised due to abnormal immune function and functional asplenia. Infection with encapsulated bacteria (eg Streptococcus pneumoniae, Staphylococcus aureus, Escherichia coli, Salmonella) can be particularly dangerous. In fact, infection is the leading cause of death in children with sickle cell disease. While pneumococcal vaccination and penicillin prophylaxis have reduced the risk of mortality, they have not eliminated it, and any history of fever must be taken very seriously. 
This female infant, not yet completely vaccinated against encapsulated bacteria (eg Pneumococcus), presented with fevers and negative evaluation except for chest x-ray with signs of viral respiratory illness. Cultures remained negative, viral panel was negative. While it was reasonable to monitor the patient for developing sepsis, acute chest syndrome, and/or vaso-occlusive crisis, she was overall hemodynamically stable with no evidence of impending sepsis or shock. She did not require acute inpatient admission and could have been safely managed at lower level of care.