Managing Pain in Sickle Cell Vaso-Occlusive Crises

Vaso-occlusive pain episodes are the most common reason children and adolescents with sickle cell disease present to the Emergency Department. Prompt, protocol-driven management is essential starting with early administration of IV opioids, reassessment at 15–30 minute intervals, and judicious hydration. Understanding the patient’s typical pain pattern, opioid history, and psychosocial context can guide more effective care. This episode walks through the pathophysiology, clinical presentation, pharmacologic strategy, discharge criteria, and complications to watch for helping you provide evidence-based, compassionate care that improves outcomes.

Learning Objectives

1. Describe the pathophysiology of vaso-occlusive crises in children and adolescents with sickle cell disease and how it relates to clinical symptoms.
2. Differentiate uncomplicated vaso-occlusive crises from other acute complications of sickle cell disease such as acute chest syndrome, splenic sequestration, and stroke.
3. Implement evidence-based strategies for early and effective pain management in vaso-occlusive crises, including appropriate use of opioid analgesia, reassessment intervals, and disposition criteria.

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References

1. Kavanagh PL, Fasipe TA, Wun T. Sickle cell disease: a review. JAMA. 2022;328(1):57-68. doi:10.1001/jama.2022.10233
2. Yates AM, Aygun B, Nuss R, Rogers ZR. Health supervision for children and adolescents with sickle cell disease: clinical report. Pediatrics. 2024;154(2):e2024066842. doi:10.1542/peds.2024-066842
3. Bender MA, Carlberg K. Sickle Cell Disease. In: Adam MP, Everman DB, Mirzaa GM, et al, eds. GeneReviews®. University of Washington, Seattle; 1993–2024. Updated February 13, 2025. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1377/
4. 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. doi:10.1182/bloodadvances.2020001851
5. Brandow AM, Carroll CP, Creary SE. Acute vaso-occlusive pain management in sickle cell disease. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. UpToDate. UpToDate; 2024. Accessed July 2025. https://www.uptodate.com
6. Glassberg JA, Strouse JJ. Evaluation of acute pain in sickle cell disease. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. UpToDate. UpToDate; 2024. Accessed July 2025. https://www.uptodate.com
7. DeBaun MR, Quinn CT. Overview of the clinical manifestations of sickle cell disease. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. UpToDate. UpToDate; 2024. Accessed July 2025. https://www.uptodate.com
8. McCavit TL. Overview of preventive outpatient care in sickle cell disease. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. UpToDate. UpToDate; 2024. Accessed July 2025. https://www.uptodate.com

Transcript

Note: This transcript was partially completed with the use of the Descript AI and the Chat GPT 4o AI

Welcome to PEM Currents: The Pediatric Emergency Medicine Podcast. I’m your host, Brad Sobolewski. In this episode, we’re digging into a common but complex emergency department challenge: pain management for vaso-occlusive crises in children and adolescents with sickle cell disease.

These episodes are painful—literally and figuratively. But with thoughtful, evidence-based care, we can make a big difference for our patients.

Overview and Epidemiology

Vaso-occlusive crises, or VOCs, are the most frequent cause of emergency visits and hospitalizations for individuals with sickle cell disease (SCD). They are responsible for more than 70 percent of ED visits among children with SCD and account for substantial healthcare utilization and missed school days.

Most children with homozygous HbSS will experience their first painful episode before the age of 6. Recurrent VOCs are associated with higher risks of chronic pain, opioid use, and diminished quality of life.

Why Do VOCs Happen?

Sickle cell disease is caused by a point mutation in the beta-globin gene, leading to hemoglobin S. Under stress—such as infection, dehydration, or even cold exposure—red blood cells polymerize, sickle, and become rigid. These sickled cells obstruct capillaries and small vessels, leading to local tissue ischemia, inflammation, and pain.

It’s not just about the blockage—the inflammatory cascade, endothelial damage, and cytokine release all contribute to the pain experience.

What Does the Pain Feel Like?

Ask kids and teens with sickle cell disease, and they’ll describe their pain as deep, throbbing, stabbing, or aching. It often feels bone-deep and can be relentless and exhausting. Many say it’s unlike any other pain—they may compare it to being “hit with a bat,” “bone being crushed,” or “something stuck inside my limbs trying to get out.”

Common sites include:

• Long bones (femur, humerus)
• Lower back
• Chest (look out for acute chest syndrome)
• Abdomen
• Hands and feet (especially in younger children—think dactylitis)

Clinical Presentation

History

• Ask about typical pain patterns and how this episode compares to prior ones.
• Look for triggers: dehydration, weather changes, infection, stress.
• Document home medications, including opioid tolerance and response to prior ED treatments.

Physical Exam

• Often nonspecific.
• Localized tenderness, guarding.
• May have fever if infection is present (but fever is not diagnostic of VOC).
• Look for signs of acute chest syndrome: tachypnea, hypoxia, chest pain.

Vitals

• May show tachycardia from pain or dehydration.
• Febrile patients should be evaluated for sepsis or osteomyelitis.

Pain scales

• Use age-appropriate tools: FLACC, Wong-Baker FACES, or numerical rating scales.

Management: Treat Early, Treat Effectively

Pain Medications

• Start early. Do not delay for labs. Aim for analgesia within 30–60 minutes of arrival.
• Mild pain (rare in ED): Acetaminophen or NSAIDs (e.g., ibuprofen, ketorolac).
• Moderate to severe pain:
  • Opioids are first-line.
  • Morphine IV: 0.1 mg/kg (max 10 mg) every 15–30 minutes as needed; consider PCA in admitted patients.
  • Hydromorphone IV: 0.015 mg/kg if morphine does not work or if the patient has used it effectively in the past.
  • Intranasal fentanyl: 1.5–2 mcg/kg as a bridge while waiting for IV access.
• Avoid codeine and meperidine due to poor efficacy and neurotoxicity risks.
• Reassess every 15–30 minutes until pain is controlled, then space doses out.

Adjunctive Therapies

• Hydration:
  • Lactated Ringer’s is associated with shorter hospital stays and lower readmission rates than normal saline.
  • Avoid fluid overload; maintain euvolemia.
• Heat packs for local comfort.
• Distraction techniques, Child Life, music, games, screens.
• Anxiolytics may be considered for severe distress but use cautiously.

Labs and Imaging

• Labs are not always needed if the child looks well and has an uncomplicated VOC. Follow local protocols.
• Consider:
  • CBC and reticulocyte count:
    • A low retic suggests aplastic crisis (often parvovirus B19).
    • A high retic is appropriate in VOC, showing marrow response.
  • Compare hemoglobin to baseline.
  • BMP for renal function.
  • LFTs or lipase if right upper quadrant pain.
  • Chest x-ray if chest symptoms present.
  • Blood cultures if febrile.

Oxygen

• Only indicated if the patient is hypoxic.

Transfusion

• Routine transfusion is not indicated for uncomplicated VOC.
• May be used in complications such as acute chest syndrome, stroke, or symptomatic anemia.

Disposition: Discharge vs. Admission

Discharge if:

• Pain is improved and manageable on oral medications.
• Tolerating oral intake.
• No concern for complications.
• Reliable follow-up and support available.

Admit if:

• Persistent severe pain despite multiple IV doses.
• Need for frequent parenteral opioids.
• Acute chest syndrome, sepsis, or other complications.
• Poor outpatient support or unreliable follow-up.

Complications to Watch For

• Acute chest syndrome: Chest pain, hypoxia, new infiltrate on chest x-ray.
• Splenic sequestration: Rapid hemoglobin drop, splenomegaly, signs of shock.
• Stroke: New neurologic deficits.
• Sepsis: Fever, tachycardia, especially in asplenic patients.
• Avascular necrosis: Recurrent or chronic hip or shoulder pain.
• Chronic pain: Increasing in frequency in adolescents and young adults.

Prevention

Hydroxyurea is the cornerstone of prevention. It increases fetal hemoglobin and reduces the frequency and severity of pain crises. It can be started as early as 9 months of age in children with HbSS or Sβ⁰-thalassemia.

Other preventive strategies include:

• Staying hydrated.
• Avoiding extreme cold exposure.
• Keeping up with vaccines and penicillin prophylaxis.
• Addressing mental health and social stressors.

Take-Home Points

1. Treat pain promptly and aggressively. Do not wait on labs.
2. Use IV opioids for moderate to severe pain and reassess often.
3. Lactated Ringer’s may be preferred for IV hydration, but avoid overload.
4. Labs and imaging should follow clinical appearance and local protocols. Reticulocyte count and hemoglobin trends are key.
5. Disposition should be based on pain control, potential complications, and social support.
6. Prevention matters—hydroxyurea and primary care follow-up reduce crises and admissions.