Kawasaki Disease or Incomplete Kawasaki Disease Clinical Pathway — Emergency Department and Inpatient

Intravenous Immunoglobulin (IVIG)

  • A multidisciplinary team of consultants with the primary team will discuss initiation of treatment and specific treatment interventions. Consults
  • These discussions will be expedited in the setting of critical illness
  • Monitor Clinical Response
    • Resolution of fever, other clinical features and improving markers of inflammation
  • Lack of clinical response: Further recommendations per Rheumatology and DIRT

Discuss Risks of Transfusion

  • Occasional risks include: fever and allergic reactions due to the formation of antibodies
  • Less common risks include: infections with viruses such as hepatitis, and fluid overload
  • Risk of contracting infectious diseases from IVIG is extremely rare, IVIG is considered to be one of the safest blood products
  • Written consent is no longer required for blood derived therapeutics such as IVIG
Dose
  • 2 gram/kg Gamunex
  • IVIG should be dosed based on IBW for patients meeting the following criteria:
    • Patients age 2-19 years with actual body weight > IBW by 20%
    • Adults > 19 years with BMI > 30 kg/m2
Rate Considerations
  • Rate of infusion should be low at the beginning and increased gradually based on patient’s tolerance (osmolality and sugar content of preparation affect tolerability)
  • Monitor for symptoms of fluid overload (Respiratory distress, heart failure) Consider continuing low rated and possible diuretic use for patients with evidence of significant myocardial dysfunction.
  • Administration of Large Volume IV Medications > 60 ml that Require Rate Titration Job Aid
Pretreatment Considerations
  • Administer antihistamines and acetaminophen prior to IVIG.
  • Depending on length of treatment, consider additional doses.
Reactions
  • Reactions may be due to the active component (IgG) or “impurity of commercial preparations” (stabilizing agents).
  • Classified as immediate (during transfusion) or delayed (after infusion).
  • Mild/Mod reactions
    • Pause infusion and call FLOC. Consider slowing infusion rate.
      • Flushing
      • Chills
      • Myalgia
      • Mild fever ( > 1° C increase in temperature)
      • Headache
      • Hypertension
      • Tachycardia
      • Rash
      • Nausea/vomiting/abdominal pain
  • Severe reactions
    • Stop infusion, discuss with Immunology
    • Anaphylaxis Pathway
    • Anaphylactoid reaction (IgA deficient patients or those with anti-IgA antibodies): hives, respiratory distress, hypotension, massive urticaria, wheezing
  • Delayed reactions
    • Hyperproteinemia
    • Hyponatremia
    • Noncardiogenic pulmonary edema (transfusion-related acute lung injury)
    • Hemolytic anemia (due to passive transfer of anti-A and anti-B)
      • Risk factors include patient blood group (A, B, or AB), a high cumulative dose of IVIG, and concomitant inflammation. Patients would be Coombs positive or have signs of hemolysis.
    • Aseptic meningitis
    • Thrombosis such as PE/stroke
    • Renal dysfunction/acute renal failure
Post-infusion Considerations
  • IVIG will elevate ESR, so it should not be rechecked post-infusion.
  • If patient requires 2 or more doses of IVIG, it is prudent to check for hemolytic anemia. Check hemolysis labs (CBC, retic, LDH, LFTs) 12-24 hours after repeat dose of IVIG.
  • Consult Hematology for any patient with severe anemia or hemolytic anemia.
  • Fever is typical within first 24 hours post completion of IVIG and should not prompt retreatment.
Special Considerations
  • Ig-A deficient patients or antibodies against IgA:
    • Discuss with Immunology prior to administration.
  • Risk of thrombosis:
    • Risk factors: advanced age, prolonged immobilization, hypercoagulable conditions, h/o thrombosis, use of estrogens, indwelling central line, hyperviscosity, cardiovascular risk factors
    • Administer at minimum dose and infusion rate, ensure adequate hydration, monitor for signs/symptoms of thrombosis and assess blood viscosity in patients at risk of hyperviscosity
  • Avoid live vaccines (e.g. MMR, varicella)
Related Procedure/Policy/Job Aids
  • Formulary: IVIG
  • Transfusion. 2015 Jul: Suppl 2:S90-4. Intravenous immunoglobulin-related hemolysis in patients treated for Kawasaki disease. Luban NL, Wong EC, Henrich Lobo R, Pary P, Duke S
  • Asia Pac Allergy. 2013;3:249-256. Adverse events of intravenous immunoglobulin infusion: a ten-year retrospective study. Palabrica Frances Rose R, Kwong Shirley L., and Padua, Florecita R.
  • Clin Exp Immunol. 1994; 97 (Suppl 1): 79-83. Side-effects of intravenous immune globulins. Duhem C, Dicato A, and Ries F.