A 20-year-old African American woman with sickle cell disease comes to the emergency department with complaints of severe generalized body pain for 1 day. She has been hospitalized for similar episodes in the past. Her medications include folic acid, hydroxyurea, and hydrocodone as needed for pain.
After initial evaluation, including pertinent laboratory studies and chest x-ray, she is started on 1/2 normal saline at 125 mL/hr and hydromorphone every 3 hours. Over the next 36 hours, the patient complains of worsening chest pain and mild shortness of breath. Her temperature is 38.9 C (102 F) , pulse is 112/min, and respirations are 23/min. Oxygen saturation is 93% on 2 L O₂. Lung examination shows bilateral crackles. Lower-extremity examination shows tenderness with palpation.
Laboratory results are as follows:

She has good urine output. Her chest x-ray on admission (Image 1) and repeat x-ray after 36 hours (Image 2) are shown below.

Which of the following, if instituted on initial presentation, may have helped prevent the clinical worsening that occurred in this patient?

Question

A 20-year-old African American woman with sickle cell disease comes to the emergency department with complaints of severe generalized body pain for 1 day. She has been hospitalized for similar episodes in the past. Her medications include folic acid, hydroxyurea, and hydrocodone as needed for pain.
After initial evaluation, including pertinent laboratory studies and chest x-ray, she is started on 1/2 normal saline at 125 mL/hr and hydromorphone every 3 hours. Over the next 36 hours, the patient complains of worsening chest pain and mild shortness of breath. Her temperature is 38.9 C (102 F) , pulse is 112/min, and respirations are 23/min. Oxygen saturation is 93% on 2 L O₂. Lung examination shows bilateral crackles. Lower-extremity examination shows tenderness with palpation.
Laboratory results are as follows:

She has good urine output. Her chest x-ray on admission (Image 1) and repeat x-ray after 36 hours (Image 2) are shown below.

Which of the following, if instituted on initial presentation, may have helped prevent the clinical worsening that occurred in this patient?

Quizplus · Accepted Answer

This patient, who initially presented with sickle cell pain crisis, now has symptoms suggestive of acute chest syndrome (ACS). ACS is defined as a new infiltrate on chest x-ray plus any of the following: fever; chest pain; or respiratory symptoms such as cough, wheezing, decreased oxygen saturation, tachypnea, or accessory respiratory muscle usage. Important etiologies for ACS are infection, asthma, hypoventilation, and pulmonary vasoocclusion from fat emboli. Negative chest x-rays obtained during initial presentation do not rule out subsequent development of ACS. ACS usually occurs within 48-72 hours of admission for pain crisis. Patients admitted for sickle crisis should be considered to be in the prodromal phases of ACS. Prophylactic measures such as close pulmonary monitoring, cautious hydration, optimal pain control, and incentive spirometry remain essential components of therapy. Treatment of ACS is based on severity. Simple transfusion for mild ACS and exchange transfusion for moderate-to-severe ACS are recommended with a target Hb of 10 g/dL. ACS and pneumonia are difficult to differentiate from each another. Therefore, empiric antibiotics that include coverage for atypical organisms (eg, Chlamydia and Mycoplasma) must be administered. However, prophylactic use of antibiotics to prevent ACS is not recommended (Choice A). Hydroxyurea should also be started for all patients with a history of ACS or at risk of developing it (eg, HbSS). (Choice B) A PCA pump may be helpful for better management of pain, but it does not help prevent ACS. Over-sedation can cause hypoventilation and should be avoided. (Choice D) Routine use of bronchodilators without evidence of asthma/wheezing has not been found useful in preventing ACS. (Choice E) Hypotonic fluids (1/2 normal saline or D51/2 normal saline) are recommended for acute sickle cell crisis. Adequate intravenous fluids are important to reduce sickling, but excess use for prolonged periods can cause pulmonary edema. This patient had received 125 mL/hr for 36 hours with good urine output, which should not lead to pulmonary edema. Educational objective: Incentive spirometry has been shown to reduce the likelihood of developing acute chest syndrome and should be encouraged in all patients presenting with sickle cell pain crisis. Similarly, hydroxyurea should be started in all patients with a history of acute chest syndrome or risk of developing it (eg, HbSS). __________ References: The use of incentive spirometry in pediatric patients with sickle cell disease to reduce the incidence of acute chest syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/21792036) Pulmonary complications of sickle cell disease. (http://www.ncbi.nlm.nih.gov/pubmed/21973051)

A 20-Year-Old African American Woman with Sickle Cell Disease Comes