Delayed Recognition and Management of Sickle Cell Disease Results in Crisis and Stroke

Description

The omission of prescribed medication and delayed treatment of vaso-occlusive complications in a patient with sickle cell disease led to a sickle cell crisis and thrombotic stroke.

Key Lessons

• Failure to review the patient record and obtain collateral information can lead to inadequate patient assessment.
• Breakdowns in communication between care providers pose significant risks to patient care.

Clinical sequence

A patient of Middle Eastern descent with sickle cell disease (SCD) and a history of vaso-occlusive disease (VOD) was admitted to the hospital with abdominal pain and hematemesis. The patient had a baseline hemoglobin of 12 with no history of blood transfusions.

During admission, the patient developed a high fever and hypotension. An oncologist evaluated the patient and ordered laboratory testing, a blood type and screen, and an anticoagulant for VOD prophylaxis. The oncologist also ordered a chest X-ray & brain CT scan, both of which were reported as normal.

The oncologist reviewed the lab results, noting a low white blood cell count and low hemoglobin levels. The patient was found to have had splenomegaly and also tested positive for influenza. The oncologist then ordered additional blood work to confirm sickle cell anemia before transfusion, but was unaware of the patient’s baseline hemoglobin and did not contact the patient’s primary care provider (PCP). A repeat chest X-ray revealed pneumonia, and the infectious disease team initiated treatment for influenza and pneumonia.

The oncologist documented improvement in the patient’s condition and after testing confirmed sickle cell anemia, an outpatient follow-up appointment was scheduled. Later that day, however, the patient reported worsening joint pain that was not relieved by pain medication. Due to concern for a sickle cell crisis, the infectious disease team ordered additional labs, including a complete blood count. The physician assistant (PA) documented the crisis but continued pain management only. That evening, the patient’s white blood cell (WBC) count increased to 35.

The infectious disease physician did not recommend any changes to the patient’s care plan and attributed the increase in WBCs to oral steroids. It is unclear if the oncologist was informed of the lab results. The patient’s pain continued to worsen while in sickle cell crisis.

The oncologist then discovered the patient’s nurse had not administered the anticoagulant order. The oncologist then re-entered orders for the anticoagulant, an antimetabolite, and a blood transfusion. Following the transfusion, the patient experienced a seizure and was transferred to the intensive care unit (ICU). The patient continued to deteriorate, and a brain CT showed thrombotic strokes and signs of liver failure. The patient was transferred to another hospital and underwent a red blood cell exchange transfusion. The patient suffered long-term effects from the stroke, including sensory, cognitive, and motor deficits; they now require full-time care at home.

Allegation

The patient alleged improper management of their medical treatment course.

Disposition

This case was settled in the high range (> $1M).

Clinical Analysis

The care team failed to adequately evaluate the patient’s worsening condition and consider complications of an evolving sickle cell crisis. There were deficiencies in patient assessment and monitoring, including a failure to appreciate and reconcile significant signs, symptoms, laboratory abnormalities, and diagnostic findings that suggested clinical deterioration. The case also involved a failure to administer medication as ordered, concerns regarding the selection and management of therapy, and insufficient communication among providers regarding the patient’s condition and abnormal clinical findings, resulting in missed opportunities to escalate care.

Discussion Questions

• How does your organization approach the management of patients presenting with a sickle cell crisis?
• Does your organization have a comprehensive plan for the evaluation and treatment of sickle cell disease?
• What processes are used to obtain and verify a patient’s prior medical history, including previous pain management plans or crisis patterns?
• What is the process for acknowledging and administering medication orders?
• How are changes in a patient’s clinical condition communicated across the care team in real time?

This is a fictitious case that illustrates commonly encountered issues and is for educational purposes only. Any resemblance to real persons, living or dead, is purely coincidental.