Emergency Department Management of Patients With Sickle Cell Disease

Table of Contents

 

About This Issue

With increased global migration, patients with sickle cell disease (SCD) can be found in every community and every ED. SCD can cause painful and life-threatening complications, and emergency clinicians must be ready to diagnose and treat these complications. In this issue, you will learn:

How to quickly manage painful vaso-occlusive crises with opioids, based on the patient’s home regimen and pain reporting

How many opioid doses to give in the ED, and why giving additional doses can reduce admissions, premature discharge, and readmission

The reasons why patients with SCD should not be given IV fluids and supplemental oxygen routinely

Recognizing splenic sequestration in adults as well as children

The laboratory and imaging studies that are critical in identifying anemia, infection, sepsis, and acute intra-abdominal complications

What the evidence is for nonopioid pain management strategies: NSAIDs, ketamine, acetaminophen, and nonpharmacologic interventions

How to manage priapism, a complication experienced by 32% to 42% of patients with SCD

Acute chest syndrome: diagnostic criteria, severity index, and treatment strategies

Exchange transfusion or simple transfusion: when they are recommended and when they are not

1. About This Issue
2. Abstract
3. Case Presentations
4. Introduction
5. Critical Appraisal of the Literature
6. Etiology and Pathophysiology
   1. Sickle Cell Disease
   2. Vaso-Occlusive Crisis
      1. Triggers of Vaso-Occlusive Crisis
      2. Association of Corticosteroid Use and Development of Vaso-Occlusive Crisis
   3. Splenic Sequestration
   4. Acute Chest Syndrome
   5. Priapism
   6. Stroke
   7. Infectious Complications
7. Differential Diagnosis
8. Prehospital Care
9. Emergency Department Evaluation
   1. History of Present Illness
   2. Physical Examination
10. Diagnostic Studies
    1. Laboratory Studies
    2. Imaging Studies
11. Treatment
    1. Pain Control
       1. Disparities in Analgesia Administration
       2. Opioid Pain Management
          1. Choice of Opioid and Route of Administration
          2. Opioid Dosing
          3. Patient-Controlled Analgesia
          4. Opioid Side Effects
       3. Nonopioid Pain Management
          1. Acetaminophen
          2. Nonsteroidal Anti-Inflammatory Drugs
          3. Ketamine
          4. Nonpharmacologic Interventions for Pain
          5. Intravenous Crystalloid Fluids
    2. Treatment for Priapism
    3. Treatment for Acute Chest Syndrome
    4. Treatment for Osteomyelitis
    5. Treatment for Fever of Unknown Origin
    6. Exchange Transfusion and Simple Transfusion
       1. Transfusion for Acute Chest Syndrome
       2. Transfusion for Suspected Ischemic Stroke
       3. Transfusion for Priapism
12. Special Populations
    1. Pregnant Patients
    2. Immigrant and Refugee Patients
13. Controversies and Cutting Edge
    1. Selectin-Based Treatments
    2. Arginine Treatment
    3. Other Treatments
14. Disposition
15. Risk Management Pitfalls for Managing Emergency Department Patients With Sickle Cell Disease
16. 5 Things That Will Change Your Practice
17. Summary
18. Time- and Cost-Effective Strategies
19. Case Conclusions
20. Clinical Pathway for Emergency Department Management of Patients With Sickle Cell Disease
21. Tables and Figures
22. References

Abstract

Sickle cell disease is a chronic hematologic disease that affects over 100,000 people in the United States. Many of these patients will present to the emergency department seeking treatment for an acute complication. Vaso-occlusive crisis, the most common recurring complication, can be difficult to manage because of the stigma patients face surrounding management of their pain. Patients with sickle cell disease presenting with conditions such as pain, infection, respiratory distress, stroke, or priapism must be given special consideration, as management can differ from that of the general population. This review evaluates the current guidelines and literature on acute complications related to sickle cell disease to dispel misconceptions about seemingly harmless interventions and provide clarification on those that are more controversial. Novel treatments that may have future impact on the management of patients with sickle cell disease are also reviewed.

Case Presentations

An 18-year-old woman with sickle cell disease presents with bilateral hip and low back pain...

• As you enter the room, the patient appears to be resting comfortably on a stretcher and is scrolling through her cell phone. Her vital signs are within normal limits and her physical examination is grossly unremarkable.
• The patient reports that her pain started after a recent upper respiratory infection and has been worsening progressively for 3 days. She now rates it a 10 out of 10 in severity. She is on chronic opioid therapy with hydromorphone and has been taking oral acetaminophen, ibuprofen, and oxycodone for breakthrough pain around the clock, with minimal relief. She says this pain feels similar to prior pain crises.
• The nurse has already placed a peripheral IV and sent laboratory tests. The CBC returns with a hemoglobin of 10.2 mg/dL, which is consistent with the patient’s baseline.
• You wonder whether this patient is having a true pain crisis and what pain medication would be best to provide. You also wonder whether “drug seeking” is a concern…

A 42-year-old woman with sickle cell disease presents with fever, chest pain, and shortness of breath...

• On arrival, the patient appears to be in acute respiratory distress. She is coughing, and reports severe pain in her chest.
• The patient is febrile to 39°C, tachypneic to 40 breaths/min, and hypoxic to 86% on room air. Her heart rate is 90 beats/min and her blood pressure is 110/70 mm Hg. Lung examination is notable for crackles and decreased air movement at the bilateral bases.
• A STAT portable chest x-ray shows bilateral lobar infiltrates. She is placed on oxygen via a nonrebreather mask. Her oxygen saturation improves to 94%, although she remains dyspneic.
• You wonder whether this patient needs expanded antibiotic coverage and whether there is a role for a blood transfusion…

How would you manage these patients? Subscribe for evidence-based best practices and to discover the outcomes.

Clinical Pathway for Emergency Department Patients With Opioid Use Disorder

Subscribe to access the complete Clinical Pathway to guide your clinical decision making.

Tables and Figures

Subscribe for full access to all Tables and Figures.

Key References

Following are the most informative references cited in this paper, as determined by the authors.

3. * Brandow AM, Carroll CP, Creary S, et al. American Society of Hematology 2020 guidelines for sickle cell disease: management of acute and chronic pain. Blood Adv. 2020;4(12):2656-2701. (ASH guidelines) DOI: 10.1182/bloodadvances.2020001851

4. * National Heart, Lung, and Blood Institute, Buchanan GR, Yaw BP, co-chairs. Evidence-based management of sickle cell disease: expert panel report, 2014. Accessed July 11, 2024. (NHLBI Expert Panel report)

5. * Freiermuth C, Kavanagh P, Bailey L, et al. Sickle cell. 2023. Accessed July 11, 2024. Available at: https://poctools.acep.org/POCTool/SickleCell/df0c5789-695b-4ceb-8fde-9f3db269a26d/ (Online point-of-care tool)

23. * Walter O, Cougoul P, Maquet J, et al. Risk of vaso-occlusive episode after exposure to corticosteroids in patients with sickle cell disease. Blood. 2022;139(26):3771-3777. (Case-control; 5151 patients) DOI: 10.1182/blood.2021014473

33. * Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med. 2000;342(25):1855-1865. (Retrospective; 671 episodes of ACS) DOI: 10.1056/NEJM200006223422502

42. * Ohene-Frempong K, Weiner SJ, Sleeper LA, et al. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood. 1998;91(1):288-294. (Prospective; 4082 patients) DOI: 10.1182/blood.V91.1.288

56. * Rineer S, Walsh PS, Smart LR, et al. Risk of bacteremia in febrile children and young adults with sickle cell disease in a multicenter emergency department cohort. JAMA Netw Open. 2023;6(6):e2318904. (Retrospective; 1118 patients) DOI: 10.1001/jamanetworkopen.2023.18904

68. * Zempsky WT. Treatment of sickle cell pain: fostering trust and justice. JAMA. 2009;302(22):2479-2480. (Commentary) DOI: 10.1001/jama.2009.1811

73. * Elmariah H, Garrett ME, De Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell disease cohort. Am J Hematol. 2014;89(5):530-535. (Prospective; 542 patients) DOI: 10.1002/ajh.23683

85. * Bellet PS, Kalinyak KA, Shukla R, et al. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med. 1995;333(11):699-703. (Randomized controlled trial; 29 patients) DOI: 10.1056/NEJM199509143331104

106. *Morris CR, Kuypers FA, Lavrisha L, et al. A randomized, placebo-controlled trial of arginine therapy for the treatment of children with sickle cell disease hospitalized with vaso-occlusive pain episodes. Haematologica. 2013;98(9):1375-1382. (Randomized controlled trial; 38 patients) DOI: 10.3324/haematol.2013.086637

Subscribe to get the full list of 110 references and see how the authors distilled all of the evidence into a concise, clinically relevant, practical resource.

Keywords: sickle cell, vaso-occlusion, VOC, acute chest, priapism, splenic, transfusion, sepsis, opioid