Sickle cell crisis

Background

(A) normal red blood cells flowing freely through a blood vessel. (B) abnormal, sickled red blood cells sticking at the branching point in a blood vessel. Cross-section shows long polymerized sickle haemoglobin (HbS) strands stretching and distorting the cell.

Precipitating Factors

Clinical Features

Vaso-Occlusive Crisis[1]

See Vaso-occlusive pain crisis

Bony infarction

  • More debilitating and refractory pain than past episodes
  • Localized bone tenderness, elevated WBC

Dactylitis

  • Tender, swollen hands/feet
  • May have low-grade fever
  • Occurs in <2yr old, extremely rare >5yr old
  • Radiographs will show soft tissue swelling in the acute phase. Recurrent episodes can lead to a mottled appearance of hand and feet small bones on radiography. [2]

Avascular necrosis of femoral head

[3]

  • Due to occlusion of blood vessel lumens, which causes microfractures, leading to collapse of cancellous bone and the articular surface
  • Occurs in 30% of patients by age 30yr, prevalence increases with age
  • Patients present with afebrile, inguinal pain with weight-bearing but can be asymptomatic
    • Decreased ROM and pain with ambulation or passive ROM
  • Other common sites of involvement are the humeral head and the area above the knee

Respiratory distress and chest pain

Abdominal pain

Infection

  • Across all ages, infection is leading cause of death
  • Children aged 6mo to 3yr at greatest risk for sepsis
  • Parvovirus B19
    • Can cause several different syndromes:
      • Erythema infectiosum ("slapped cheeks" rash)
      • Gloves and socks syndrome
        • Well-demarcated, painful, erythema of hands and feet
          • Evolves into petechiae, purpura, vesicles, skin sloughing
      • Arthropathy - symmetric or asymmetric, knees and ankles
      • Aplastic crisis
        • Reticulocyte count drops 5d post-exposure, followed by hemoglobin drop
        • Can cause serious anemia which lasts for 2wk

Musculoskeletal infection

  • Patients with SCD have increased rates of bone and joint infection
    • Difficult to distinguish from bony infarcts
      • High fever is more typical of infection
      • Limited range of motion is much more typical of infection
      • Left shift is unique to infection
      • ESR is unreliable
      • May require bone scan or MRI to definitely distinguish infection from infarct

Splenic sequestration

  • Major cause of mortality in <5yr old
  • Labs: hemoglobin drop, no change in bili, normal to increased retic count

Neurologic disease

  • CVA is 250x more common in children with SCD
    • 10% of children suffer clinically overt stroke
    • 20% found to have silent CVA on imaging
  • Increased rate of cerebral aneurysm and ICH

GU

  • Priapism
    • Occurs in 25% by age 20
  • Papillary necrosis

Differential Diagnosis

Sickle cell crisis

Evaluation

Algorithm for the Evaluation and Management of Sickle Cell Crises

Work-up

Based on clinical presentation, but may include:

  • CBC (assess for significant anemia)
  • Reticulocyte count (<0.5% suggests aplastic crisis - rare in adults), calculating reticulocyte index
    • Laboratory value is a percentage, and absolute count corrects for level of anemia
    • Absolute retic count = % retics x (pt Hct / normal Hct)
    • Retic index = absolute retic count / maturation factor[4]
      • ≥35%, maturation factor 1.0
      • 25 to <35%, maturation factor 1.5
      • 20 to <25%, maturation factor 2.0
      • <20%, maturation factor 2.5
    • Reticulocyte index ≥2 means adequate response
    • Reticulocyte index <2 suggests hypoproliferation
  • Metabolic panel, lipase (if abdominal pain)
  • CXR (if cough, shortness of breath, or fever)
  • ECG
  • VBG
  • Urinalysis
  • Pregnancy test
  • CT brain (if symptoms of CVA)
    • Note that if patient is anemic, CT non-contrast is unreliable in ruling out hemorrhagic stroke, subarachnoid hemorrhage[5]
    • Will need CTA, MRI, and/or MRA

Evaluation

  • Generally a clinical diagnosis
  • Certain syndromes require imaging/labs for confirmation (see below)

Management

  • Only use supplemental oxygen for patients who are hypoxic (<92%)
  • Reserve IVF bolus for patients who are hypovolemic
    • Over hydration may cause atelectasis which may precipitate Acute Chest Syndrome and hyperCl acidosis which could lead to further sickling \
    • Make sure to use hypotonic fluids: 1/2NS, D5-1/2NS

Anemia

Vaso-occlusive pain crisis

  • Analgesia
  • Hydration
    • Controversial
      • Dehydration promotes sickling
      • IVF bolus may lead to atelectasis, pulmonary edema, and acute chest syndrome. Hyperchloremic metabolic acidosis due to normal saline may further precipitate sickling. [8] [9] [10]
      • There are no randomized controlled trials that have assessed the safety and efficacy of different routes, types or quantities of fluid.[11]
  • O2 if hypoxia; otherwise may inhibit erythropoiesis

Acute Chest Syndrome

  • O2; titrate to pulse oximetry >92%
  • Incentive Spirometer
  • Hydration: Oral hydration preferred, IV hydration with hypotonic fluid if patient unable to tolerate PO
  • Analgesia: pulmonary toilet is important but avoid excessive sedation
  • Bronchodilators
  • Antibiotics: 3rd generation cephalosporin + macrolide
  • Transfusion (leucocyte depleted); consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
    • O2 Sat <92% on room air
    • hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit
  • Exchange transfusion; consider for:
    • Progression of acute chest syndrome despite simple transfusion
    • Severe hypoxemia
    • Multi-lobar disease
    • Previous history of severe acute chest syndrome or cardiopulmonary disease

Priapism

Neurologic disease

Splenic sequestration

Fever

  • Any fever of ≥38.5C requires empiric antibiotics (regardless of well appearance or any localized infection found)
  • Ceftriaxone 50mg/kg (maximum single dose 2g)

Disposition

Consider admission to the hospital

Consider discharge

  • Pain is under control and patient can take oral fluids and medications
  • Ensure appropriate oral analgesics are available
  • Provide home care instructions
  • Ensure resource for follow-up


Medication Dosing

Pain Management

See Also

References

  1. ↑ Lovett P. et al. Sickle cell disease in the emergency department. Emerg Med Clin North Am. 2014 Aug;32(3):629-47
  2. ↑ George, A., DeBaun, M., & DeBaun, M. (2021, April 28). Acute and chronic bone complications of sickle cell disease. Retrieved April 20, 2022, from https://www.uptodate.com/contents/acute-and-chronic-bone-complications-of-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=110699&source=see_link
  3. ↑ George, A., DeBaun, M., & DeBaun, M. (2021, April 28). Acute and chronic bone complications of sickle cell disease. Retrieved April 20, 2022, from https://www.uptodate.com/contents/acute-and-chronic-bone-complications-of-sickle-cell-disease?search=sickle%20cell%20disease%20children&topicRef=110699&source=see_link
  4. ↑ Poorana PP and Subhashree AR. Role of Absolute Reticulocyte Count in Evaluation of Pancytopenia-A Hospital Based Study. J Clin Diagn Res. 2014 Aug; 8(8): FC01–FC03.
  5. ↑ Lyon M et al. Approach to the diagnosis and treatment of acute subarachnoid hemorrhage in a patient with sickle cell disease. Am J Emerg Med. 2015 Mar;33(3):481.e3-4.
  6. ↑ *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
  7. ↑ *Lyness, D., & Motov, S. (2016, July 7). CERTA Opioid Alternatives and Analgesics. Retrieved July 25, 2016, from http://www.propofology.com/infographs/certa-opioid-alternatives-as-analgesics
  8. ↑ Glassberg, J., Evidence-based management of sickle cell disease in the emergency department.Emerg Med Pract, 2011. 13(8): p. 1-20
  9. ↑ Carden, M.A., et al., Variations in pediatric emergency medicine physician practices for intravenous fluid management in children with sickle cell disease and vaso-occlusive pain: A single institution experience.Pediatr Blood Cancer, 2018. 65(1)
  10. ↑ Carden, M.A., et al., Normal Saline Bolus Use in Pediatric Emergency Departments is Associated with Worse Pain Control in Children with Sickle Cell Anemia and Vaso-occlusive Pain.Am J Hematol, 2019.
  11. ↑ Okomo U, Meremikwu MM. Fluid replacement therapy for acute episodes of pain in people with sickle cell disease. Cochrane Database of Systematic Reviews 2015, Issue 3. Art. No.: CD005406. DOI: 10.1002/14651858.CD005406.pub4
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