Specific Recommendations for Complications

INFECTIONS Sickle cell patients with infectious symptoms or a temperature greater than 38.3°C (101°F) should be presumed to have a bacterial infection. Basic laboratory studies should be obtained as well as cultures of blood, sputum, and urine as indicated. Radiographs should be obtained. There should be a low threshold for lumbar puncture. In most patients, parenteral antibiotics should be administered for 48 h or until the cultures are finalized. Intravenous cephalosporins such as cefuroxime or ceftriaxone are generally used. Suggested oral or outpatient regimens would be cefprozil or amoxicillin/clavulanate.

Sickle cell patients need to be vaccinated in childhood against S. pneumoniae, H. influenzae, and hepatitis B. Children with SCD generally receive prophylactic daily oral penicillin up to age 6; this practice is not known to be helpful in adults.

PULMONARY CRISIS Sickle cell patients with pulmonary crisis need aggressive management including hospitalization until the diagnosis becomes apparent. Since the differential diagnosis includes pneumonia, empirical antibiotics such as cefuroxime and erythromycin are often given. Oxygen is administered for hypoxemia, and close monitoring is needed for possible decompensation. Pulmonary thromboembolism is also in this differential diagnosis. If significant V/Q mismatch is demonstrated on V/Q scan or |arge vessel occlusion on pulmonary angiography, heparinization is indicated. Patients with significant cardiopulmonary decompensation may be treated with exchange transfusion.

CNS CRISIS Sickle cell patients with signs or symptoms of neurologic compromise need emergent care and close monitoring. After the patient is stabilized, a head CT or MRI should be obtained. If there is no contraindication, a lumbar puncture should be performed to rule out SAH and meningitis. Seizures and cerebral edema are managed conventionally. These patients require admission to an intensive care unit. When there is an acute CNS event, exchange transfusion is used to lower the HbS concentration to less than 30 percent.

LOCALIZED BONE PAIN This finding, especially when accompanied by fever and leukocytosis, raises the possibility of osteomyelitis. Radiographs, bone scan, or MRI may not be immediately helpful or available. The site should be directly aspirated for culture, and the patient started on parenteral antibiotics to cover S. aureus and S. typhimurium, such as fluoroquinolone or third-generation cephalosporin.

PRIAPISM When a patient with sickle cell disease presents with priapism, urologic consultation should be obtained. The patient should receive analgesia and hydration, and the bladder should be emptied by catheterization, if possible. Exchange transfusions may be instituted, but if not successful, surgical intervention will be necessary.

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