Sickle Cell and Acute Chest Syndrome — Acute Chest Treatment — Clinical Pathway: Inpatient

Sickle Cell and Acute Chest Syndrome Clinical Pathway — Inpatient

Acute Chest Treatment

Goals of Treatment

  • Treat for infection
  • Minimize pain
  • Treat hypoxia
  • Consider transfusion to improve Hgb oxygen-carrying capacity

Respiratory Treatment

Evidence substantiates that Incentive Spirometry (IS) reduces hospital LOS in patients with ACS. IS use also decreases the development of ACS in patients who are admitted with VOC. Incidence of asthma and airway hyperresponsiveness is known to be increased in patients with SCD compared to those without SCD. Asthma in pediatric age group is associated with increased risk for ACS development. Use of albuterol will help in patients who have co-existent asthma and those with underlying AHR.

Consultation Considerations

  • Consider Pulmonary consultation for all patients with ACS
  • Obtain Pulmonary consult (inpatient or outpatient) for patients with more than 1 episode of ACS
  • ICU consult

Interventions

Monitoring SpO2
Cardiorespiratory
  • Continuous pulse oximetry
  • Continuous cardiorespiratory monitoring
  • Assess blood pressure every 4 hrs
Respiratory Supplemental O2 Maintain SpO2 ≥ 92%
Incentive Spirometry
  • Every 2 hrs while awake
  • Pt performs 10 times every 2 hrs while awake; at least once in 24 hr period with RN/RT
Airway Clearance
  • Acapella: 4 times per day while awake as tolerated
  • IPV: 4 times per day while awake as tolerated if patient is unable to tolerate Acapella
Albuterol
  • Use for known asthma diagnosis or history of albuterol responsiveness
  • Wheezing on clinical exam
  • 2 puffs every 4 hrs for 24 hrs, then reassess
    • Multidisciplinary team (RN/RT, Resident, Attending)
  • Continuous Albuterol, consider ICU Admission
IV Fluids Treat dehydration
  • Goal: Euvolemia
    • Aim for IV and PO at 2/3 maintenance once intravascular volume is restored
Additional Labs
  • Type and Cross ABG
  • Consider CMP
  • Consider ABG if
    • SpO2 < 90%
    • Requires FiO2 > 35%
    • Clinical evidence of distress
  • Monitor renal, hepatic function in ill patients
Antibiotics Ampicillin, IV
  • Infant, Child, Adult
  • 50 mg/kg/dose every 6 hrs (max: 2,000 mg/dose)
Azithromycin, PO and IV
  • Child ≥ 3 mos
  • 10 mg/kg/dose (max 500 mg/dose) on day 1 then
  • 5 mg/kg/dose (max 250 mg/dose) once daily, days 2-5
  • Adult
    • 500 mg on day 1 then
      250 mg once daily, days 2-5
Steroids
  • Prednisone/Prednisolone, PO
  • Discuss with Hematology before initiation
  • Infant, Child, Adult
    • 1-2 mg/kg/day, (max 60 mg/day); duration per provider discretion
    • Follow steroid course with a taper, using below taper as a guide:
      • 0.6 mg/kg/day x2
      • 0.3 mg/kg/day x2
      • 0.2 mg/kg/day x2
      • 0.1 mg/kg/day x2, then discontinue
Transfusion Simple Transfusion
  • Indications
    • Decrease in hemoglobin 1-2 g/dL from baseline
    • Hypoxemia
    • High cardiac output (tachycardia)
    • Symptoms (lightheadedness, easy fatigability)
  • Consult Hematology when considering transfusion for hemoglobin > 9.5 g/dL
  • Recommendations
    • Infants and children: 10-15 ml/kg packed RBCs
    • Adults: 10-15 ml/kg packed RBCs rounded to full units on individual basis
Exchange Transfusion/Erythrocytapheresis
  • Indications
    • Supplemental oxygen FiO2 > 35%
    • Impending/existing respiratory failure
    • Preferred option over simple transfusion for patients whose baseline hgb is high (i.e., patients with SCD-SC and SCD-S beta + thalassemia) to avoid issues with hyperviscosity
  • Goals
    • SCD-SS, SCD-S beta 0 thalassemia and S beta + thalassemia: Hgb S < 30%
    • SCD-SC: Hgb S (+C) < 30%