Kawasaki disease as a case study

With my previous focus on the urgency of preventing medical diagnostic errors in Contemporary Pediatrics commentaries (July 2018; August 2018), the article in the October 2018 issue authored by Anne Rowley, MD, professor of Pediatrics (Infectious Diseases) and Microbiology-Immunology at Northwestern University Feinberg School of Medicine, titled “Kawasaki disease: AHA statement and recommendations” provides valuable insights for the care of children with a suspicion of Kawasaki disease (KD) which, when applied to clinical practice, may prevent medical diagnostics errors.

Thus, for many reasons, Dr. Rowley’s article is a must read for all pediatric healthcare providers. Failure of a timely and accurate diagnosis of KD may result in adverse outcomes for children, including coronary artery abnormalities, dilatation, and aneurysms. Dr. Rowley provides an overview of KD based on the latest 2017 American Heart Association’s (AHA) statement and recommendations for diagnosis and treatment of children who present with prolonged, unexplained fever. Throughout the article, Tables and Figures highlight critical information for the diagnosis and treatment of KD and represent the current best-available evidence. Further studies, both prospective and retrospective, are needed to further clarify diagnostic criteria and treatment options for children of different nationalities because the prevalence and severity of the disease vary based on ethnicity-in particular, for Asian/Japanese children who are aged younger than 5 years.

AHA on incomplete Kawasaki disease

The 2017 AHA statement provides an algorithm for the diagnosis of Incomplete KD (previously termed atypical KD) for any infant aged younger than 1 year with an unexplained fever for 7 days, or in infants with a fever for 5 days and presenting with only 2 or 3 of the principle clinical features of KD. In addition, there are recommendations for laboratory testing to aide in the diagnosis, which may help avoid a medical diagnostic error.

The essential history

The value of a comprehensive history cannot be overemphasized. Parents should be asked specific details related to fever onset, use of antipyretic medications, dosage, and the effect on the infant or child’s fever. Some parents may be hesitant to report how long the infant was at home with a fever, while other parents may not have a means of measuring the temperature of an infant at home and merely “take a guess” concerning the height of the fever.

Dr. Hallas’ Practice Pearls

Parents also should be asked whether the infant or child displayed any of the 5 principle clinical criteria of KD that may have spontaneously resolved. These factors must be considered when making a diagnostic decision about an infant or child presenting with an unexplained fever and fewer than the 5 principle clinical criteria of KD.

Recovery and immunization administration

Another consideration for care of the infant and child after recovery from KD is the administration of immunizations. For an infant aged younger than 12 months who has had a diagnosis of KD or Incomplete KD and received intravenous immunoglobulin (IVIG), the live vaccines, such as measles and varicella-containing vaccines, should not be administered until 11 months after receiving IVIG.¹

Likewise, a child who received the first set of measles and varicella vaccines at 12 months of age and has not received the 4- or 5-year-old doses should not receive the vaccines until 11 months after receipt of the IVIG. The rationale for delaying the vaccines for 11 months postreceipt of IVIG for all children who need the live vaccines is attributed to a possible interference with the development of adequate immune response by the child (AAP). However, readers are referred to the Red Book: 2018 Report of the Committee on Infectious Diseases (AAP)¹ for further details for administration of vaccines to children who are at risk for exposure to measles or varicella and for those children on prolonged aspirin therapy. Infants and children recovering from KD may continue to receive inactivated vaccines per the Centers for Disease Control and Prevention (CDC)² and Advisory Committee on Immunization Practices (ACIP) vaccine schedules.³

Quality diagnostic reasoning

Nurse practitioners and all healthcare providers must remain acutely aware of the diagnostic criteria for KD and Incomplete KD and vigilant when an infant or child presents with a prolonged unexplained fever. Let’s remove the diagnosis of KD from the list of diseases that have an increased likelihood of a medical diagnostic error by applying the scientific evidence throughout the diagnostic and treatment processes. 

References:

1. American Academy of Pediatrics. Section 3: Summary of infectious diseases. In: Kimberlin DW, Brady MT, Jackson MA, Long SS, eds. Red Book: 2018 Report of the Committee on Infectious Diseases. 31st ed. Elk Grove Village, IL: American Academy of Pediatrics; 2018:494-500.

2. Centers for Disease Control and Prevention. Immunization schedules. Available at: https://www.cdc.gov/vaccines/schedules/. Updated February 6, 2018. Accessed October 20, 2018.

3. Advisory Committee on Immunization Practices. Immunization schedules. Available at: https://www.cdc.gov/vaccines/acip/index.html. Updated October 11, 2018. Accessed October 20, 2018.