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Emergency Guidelines for Sickle Cell Anemia:
General Guidelines

Red Flags in Sickle Cell Disease
Immediate Attention Shall be Given To:

  • Fever of 101oF or higher
  • Pain (pain not relieved by home management)
  • Neurologic symptoms/deficits
  • Respiratory distress/chest pain
  • Recent onset of joint swelling
  • Priapism
  • Splenic sequestration (pallor, lethargy, hypotension, increased size of the spleen)
  1. Patients should be seen promptly.
  2. If the patient is in pain, give analgesics Promptly.
  3. Call patient's hematologist or physician responsible for sickle cell management. Many children carry an ID card which contains their diagnosis, baseline CBC, hematologist's name and telephone number, etc.

Treatment Guidelines

Fever
  1. Obtain CBC and blood culture. Culture other sites, as indicated.
  2. Consider parenteral broad spectrum antibiotic(s) based on patient's age; There is no need to delay for CBC results or urine culture.
  3. Obtain chest X-ray for cough, dyspnea, hypoxemia (including measurement by pulse oximetry), or high fever with no focus of infection.
  4. Admission criteria
    1. ADMIT ANY CHILD OR ADULT WHO APPEARS ILL
    2. Admission recommendations for adults and children
      1. Respiratory distress
      2. Temperature >I 02.2'F with no focus of infection upon examination
      3. WBC or Hgb greatly altered from baseline
      4. Uncertain or unlikely follow-up (e.g. no phone or transportation):
        Relative Indication
    3. Specific admission criteria for children.
      1. Fever 100.4'F or greater
      2. non-compliance with penicillin prophylaxis
      3. age < 12 months
  5. Specific discharge instructions:
    1. Prescribe a long acting antibiotic, such as ceftriaxone, if antibiotics are indicated.
    2. It is suggested that the Sickle Cell physician/pediatrician be contacted PRIOR to discharge.

Painful Vaso-Occlusive Crisis (OR VOC)

  1. Start hydration at 1.5X maintenance immediately. (Unless patient is hypovolemic, recommended fluid is D5 1/4 NS or D5 1/2 NS).
  2. Start analgesics promptly to provide effective relief of pain.
  3. Narcotic addiction concern is not appropriate during VOC.
  4. Recommended analgesics:
    1. Morphine sulfate is the analgesic of choice. Administer 0. 15 mg/kg/dose IV, IM, or SQ. Maximum single IV dose is 15 mg q 2-4 h for unhabituated patients.
    2. Alternative medications
      1. Dilaudid 0.02 mg/kg/dose IM, IV or SQ. Maximum single IV dose is 4 mg q 3-4 h for unhabituated patients.
      2. For Children, consider Ketorolac 10-30 mg IM/IV q 4-6 h. Maximum daily dose 90 mg.
      3. Meperidine (as a last resort) 1.5 mg/kg/dose IM or IV Maximum Single IV dose is 100 mg q 3-4 h for unhabituated patients
    3. Certain patients may require higher individual doses, based on prior history or may benefit from continuous infusion via PCA (Patient Controlled Analgesia).

Acute Splenic Sequestration

  1. Most common in infants and young children. High mortality rate.
  2. Clinical presentation
    1. pallor
    2. lethargy
    3. hypotension
    4. increased splenic size
    1. type and cross match*
    2. CBC
    3. reticulocyte count
  3. While waiting for blood, give isotonic IV fluids to treat hypovolemia.
  4. Suggest initial transfusion of 10 cc/kg of packed red cells.
  5. Do not raise Hgb above baseline, since the spleen will shrink and autotransfusion will occur. This will result in an increase in Hgb, % Hgb S and risk of stroke.

Stroke

  1. Etiology:
    1. adults: cerebral hemorrhage
    2. children: cerebral infarction
  2. Lab Work:
    1. CBC
    2. type and cross match*
    3. arrange for an exchange transfusion
  3. Obtain CT without contrast.
  4. Admit to Hematology; consult neurology and/or neurosurgery.

Acute Chest Syndrome

  1. Monitor 02 saturation by pulse oximetry and/or ABG.
  2. Obtain chest X-ray. Consider admission for all patients with chest pain, even if X-ray is normal.
  3. Type and cross match* for possible exchange or simple transfusion.
  4. Consider broad spectrum antibiotic(s), such as ceftriaxone.

Priapism

  1. Administer intravenous fluids at 1.5-2X maintenance.
  2. Encourage emptying of the bladder; catheterize, if necessary.
  3. If no response, arrange for partial exchange transfusion.*
  4. Admit to Hematology; consult urology.

*All blood to be transfused should be sickle negative.

This document was developed in collaboration with the Sickle Cell Advisory Committee and representatives from various professional organizations for emergency personnel in New Jersey. Funding was made available through a grant from Special Child and Adult Health Services, Family Health Services, New Jersey Department of Health.


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Last Modified: Thursday, 12-Jul-12 15:04:05