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

Question 1

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?

  •  Start intravenous (IV) pain medications, bronchodilators,
    and oxygen
  •  Start IV pain medications, antihistamine, nasal steroid, bronchodilators,
    and oxygen
  •  Start IV pain medications, broad-spectrum antibiotics, bronchodilators,
    and oxygen; transfuse
  •  Start IV pain medications, bronchodilators, and oxygen; check chest
    radiograph; transfuse
  •  Start IV pain medications, broad-spectrum antibiotics, bronchodilators,
    and oxygen; check chest radiograph; transfuse 2 units of packed red
    blood cells; recheck chest radiograph

Updated 11/09/2010 • jdw | Copyright ©2014 Turner White Communications