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Patient handout

Kawasaki disease

PRODUCTION

1. Your condition

This handout is for kawasaki disease. Your care team identified this based on: fever >=5 days in a child (aha 2017 mccrindle).

Other reasons your team may use this plan: conjunctivitis + rash + lip/oral changes + extremity changes + lymphadenopathy (aha 2017 principal criteria); markedly elevated crp/esr with normocytic anemia + thrombocytosis (aha 2017 supplementary); echocardiographic coronary artery dilation / aneurysm (aha 2017).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
IVIG2 g/kg single dose IV over 10–12 hoursIVsingle doseAHA 2017 — primary intervention; reduces coronary aneurysm risk from ~25% to ~4% if given <10 days; still indicated after day 10 if persistent fever or evidence of inflammation
aspirinHigh-dose 80–100 mg/kg/day PO divided q6h (US/AHA) OR 30–50 mg/kg/day (Japan/Europe)POq6hAHA 2017 — anti-inflammatory dose acutely; controversial whether high vs moderate is better; both effective when paired with IVIG

Plan: Kawasaki disease — IVIG + ASA → escalation for IVIG-resistance → long-term cardiac surveillance

3. Your action plan

Use these zones to know what to do based on how you feel.

GREENRecovering well — no aneurysm or small aneurysm regressing
If you have:
  • Afebrile x 1 week (AHA 2017)
  • Normal/mild coronary changes (z-score <2.5) (AHA 2017)
  • Normal CBC + CRP/ESR trending normal (AHA 2017)
  • Tolerating low-dose ASA (AHA 2017)
  • No new symptoms (AHA 2017)
Do this:
  • Continue low-dose ASA 3-5 mg/kg/day until 6-8 weeks afebrile + normal echo (AHA 2017)
  • Cardiology follow-up at 1-2 weeks + 6-8 weeks (AHA 2017)
  • Avoid live vaccines for 11 months after IVIG (consult vaccine schedule; AAP 2024)
  • Avoid contact with VZV/influenza while on ASA (Reye risk) — switch to clopidogrel if exposed (AHA 2017)
  • Daily activity as tolerated (AHA 2017)
  • Inform pediatrician + cardiologist of any new symptoms (AHA 2017)
YELLOWCaution — coronary aneurysm OR fever recurrence OR medication reaction
If you have:
  • New coronary aneurysm (z-score 2.5-10) (AHA 2017)
  • Recurrent fever after discharge (AHA 2017)
  • Persistent inflammation (CRP/ESR not normalizing) (AHA 2017)
  • Medication side effects (GI bleed on ASA, easy bruising) (AHA 2017)
  • Suspected adverse reaction to IVIG (delayed hemolysis) (AHA 2017)
Do this:
  • Contact pediatric cardiology within 24h (AHA 2017)
  • May need additional anti-thrombotic (clopidogrel if medium aneurysm) (AHA 2017)
  • Recurrent fever then re-evaluate for IVIG resistance OR alternative diagnosis (AHA 2017)
  • Adverse reaction then contact provider for medication change (AHA 2017)
  • Bring child to ED if signs of bleeding or coronary ischemia (chest pain, syncope) (AHA 2017)
Call your provider if:
  • Any aneurysm progression or new aneurysm (AHA 2017)
  • Fever recurrence (AHA 2017)
  • Medication side effects (AHA 2017)
REDEmergency — coronary thrombosis, MI, severe bleeding, KDSS recurrence
If you have:
  • Chest pain in child with prior KD (AHA 2017)
  • Syncope, sudden collapse (AHA 2017)
  • Severe bleeding on antiplatelet/anticoagulant (AHA 2017)
  • New shock syndrome with rash + perfusion changes (AHA 2017)
  • Stroke symptoms (focal neuro deficit) (AHA 2017)
Do this:
  • Call 911 immediately (AHA 2017)
  • Bring all medications + KD records (AHA 2017)
  • Identify as Kawasaki disease patient with coronary aneurysm if applicable (AHA 2017)
  • Pediatric cardiac center transfer if available (AHA 2017)
Call your provider if:
  • Any red zone trigger — go to ED, do not wait (AHA 2017)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Fever ≥5 days + ≥4 of 5 principal criteria
  • Fever ≥5 days + 2–3 principal criteria + supportive labs (CRP ≥3, ESR ≥40, ≥3 supplementary)
  • Persistent fever ≥36 hours after end of first IVIG
  • Kobayashi score ≥5 (or Egami ≥3 / Sano ≥2) at diagnosis (Asian cohort)
  • KD with hypotension, poor perfusion, ventricular dysfunction, or vasoactive requirement(life-threatening)
  • Coronary z-score ≥2.5 at first echo
  • Coronary z-score ≥10 OR absolute diameter ≥8 mm(life-threatening)
  • Suspected MIS-C with KD-like features (post-SARS-CoV-2 exposure 2–6 wk prior)

5. Follow-up

Pediatric cardiology follow-up at 6-8 wk + lifetime if aneurysm per AHA 2017; influenza/VZV vaccination timing relative to IVIG (delay live vaccines 11 months per AAP 2024); discontinue ASA at 6-8 wk if no aneurysm; family counseling on recurrence (~2%)

6. Sources

Guideline: 2017 AHA Scientific Statement on Diagnosis, Treatment, and Long-term Management of Kawasaki Disease + RAISE trial (steroid for high-risk) + Egami/Kobayashi/Sano IVIG-resistance scores

  1. pubmed.ncbi.nlm.nih.gov/28356445
  2. pubmed.ncbi.nlm.nih.gov/30337183