Part 3: Hemoglobinopathies
Katharine Batt, MD, MSc, and Thomas Reske, MD
Dr. Batt is a fellow in Hematology/Oncology, Mount Sinai Hospital New York, NY, and Dr. Reske is a fellow in Hematology/Oncology, Boston University Medical Center, Boston, MA
A 27-year-old woman with a past medical history significant for hemoglobin S (HbS) disease and mild asthma presents to the emergency department (ED) complaining of severe pain in her hands and arms, similar in nature to prior sickle crises. She states that the pain started suddenly this afternoon and describes it as unremitting and unresponsive to her oral pain medications, prompting her to present to the ED. Other than the pain, she reports being in her usual state of health but with a 3-day flare up of her "allergies," comprising nasal congestion, cough, and occasional headache. On physical examination, her vital signs are blood pressure, 108/75 mm Hg; heart rate, 110 bpm; respiratory rate, 30 breaths/min; oxygen saturation, 92% on room air; and temperature, 37.9ºC. Her complete blood count is significant for a hemoglobin of 7.2 g/dL (baseline is 9.5 g/dL). Physical examination is consistent with a young woman in mild distress, holding her arms and grimacing, with positive exam findings for maxillary sinus tenderness and an occasional wheeze on auscultation. Appropriate diagnosis and treatment of this patient's underlying condition would include which of the following?