An Unnecessary Procedure in a Sickle Cell Patient with Acute Chest Syndrome

Gregory Bump, MD, FACP1, Roby Thomas, MD2, Maria Nepa, PA3, Enrico Novelli, MD4, Laura De Castro, MD, MHSc1, 1University of Pittsburgh Medical Center, Pittsburgh, PA; 2University of Pittsburgh Medical Center; 3University of Pittsburgh Medical Center, Adult Sickle Cell Anemia Program; 4University of Pittsburgh Medical Center, Department of Hematology, Pittsburgh, PA

Meeting: Hospital Medicine 2018; April 8-11; Orlando, Fla.

Abstract number: 497

Categories: Adult, Clinical Vignettes, Uncategorized

Keywords: , , , ,

Case Presentation: A 28-year-old woman with sickle cell disease and chronic pain who required inpatient care several times yearly presented with worsening nausea, emesis and uncontrolled pain to the emergency department (ED). Symptoms were typical of prior pain crises. The patient followed regularly with a hematologist undergoing monthly red blood cell exchange transfusions. In the ED, vital signs were normal. She was admitted to the teaching hospitalist service. Initial laboratory studies showed a leukocytosis (WBC: 11.2 X 109/L ), Hemoglobin of 12.0 g/dL and Hematocrit of 35.3%. A chest x-ray was normal. A urine drug screen was positive for marijuana and benzodiazepines.
A sickle cell pain crisis was diagnosed and the patient’s “Pain Care Plan” was initiated, consisting of intravenous (IV) hydromorphone by PCA, IV fluids and anti-emetics. The sickle cell service was consulted.
A few hours after admission, the patient experienced dyspnea and chest tightness. A repeat chest x-ray was again normal. She received ipratropium bromide/albuterol inhaled with relief. The next morning, she had recurrent respiratory symptoms with wheezing and tachycardia to 103 beats per minute. A chest x-ray revealed a “vague left basilar opacity that could represent a developing pneumonia”. A CT scan was ordered and demonstrated “Patchy ground-glass opacities in all lobes, possibly acute chest syndrome.”
The covering hematology fellow recommended initiating emergent red-cell exchange transfusion for acute chest syndrome. Standard instructions for placing a pheresis catheter,calling the blood bank and pheresis nurse were given by the on-call fellow to the medicine team. The medicine team called the critical care medicine (CCM) to place the pheresis catheter. The CCM team inserted the catheter at the bedside. Throughout insertion the patient resisted, stating she received chronic exchange transfusions via an indwelling port. The teams explained the necessity of the pheresis catheter and the patient was convinced to undergo the procedure. A 20-cm right internal jugular double-lumen dialysis catheter was placed too far into the right atrium requiring replacement with a new 16 cm line.
The patient underwent RBC exchange with improvement in her acute chest syndrome. Antibiotics were given. The following morning it was recognized the line placed overnight was unnecessary; her indwelling dual lumen port was used regularly for exchange transfusions. This error was discussed with the patient. The right IJ central line was removed.

Discussion: This case highlights the importance of communication between multiple services, particularly at night, when providers who may not be fully familiar with a patient’s history are directly responsible for the care. Systemic interventions, such as electronic medical records flags and alerts of patients with indwelling ports and structured sign-out procedures may prevent similar incidents in the future.

Conclusions: This case also highlights biases that affect provider judgement. Visceral bias refers to how we feel about certain patients (those frequently admitted or with chronic pain) and how we react to them when they provide us information. There has been much data to support bias towards patients with sickle cell disease that are admitted to the hospital. In this case, the patient provided helpful information that was discounted by multiple team members. Efforts are being made by national institutions to increase awareness in this important issue, as we hope to provide the best care for our patients.

To cite this abstract:

Bump, GM; Thomas, RA; Nepa, M; Novelli, EM; De Castro, LM. An Unnecessary Procedure in a Sickle Cell Patient with Acute Chest Syndrome. Abstract published at Hospital Medicine 2018; April 8-11; Orlando, Fla. Abstract 497. Accessed April 1, 2020.

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