Childhood Illness Series: Kawasaki Disease

What is Kawasaki disease?

Kawasaki disease is a rare disease that was discovered in 1967 by Dr. Tomisaku Kawasaki.  Its’ origin is still unknown and researchers are unsure if the pathogen is bacterial or viral.  However, they don’t believe it is contagious or genetic. 

Although Kawasaki disease was discovered decades back, it has recently gained new attention due to COVID-19.  In a “letter to the editor” several providers expressed their concern about the possibility of parents not seeking in-person medical care due to worries that their children may contract COVID-19 in healthcare facilities.  There is growing concern that diseases other than COVID-19, such as Kawasaki disease, could be under-diagnosed and possibly result in delayed treatment due to this phenomenon. 

This letter to the editor also expressed concern that first-line providers may be focused on COVID-19 when children present with a fever.

First line providers who see pediatric patients with fevers lasting longer than 5 days should continue to have a high suspicion for Kawasaki disease.  This is especially important for children less than 1 years old.

Kawasaki disease is the leading cause of acquired heart disease in children and affects the small and medium-sized arteries.  It causes inflammation and weakening in the blood vessels and can cause aneurysms, coronary thrombosis, stenosis, scarring and myocardial infarction.    

What is the cause?

Researchers are still searching for answers about the cause of Kawasaki disease.  For years there has been speculation that that the disease is associated with the use of carpet cleaner or stagnate water, however, researchers are working on “de-bunking” this theory.

Experts speculate that the pathogen is viral or bacterial, however, it does not appear to spread person-to-person and it does not appear to be genetic.

How is Kawasaki disease diagnosed?

There is no diagnostic tool for Kawasaki disease, so it is diagnosed based on a child’s history of signs and symptoms.

Echocardiograms are often ordered to rule out cardiac abnormalities.  Lab work is usually obtained to look for systemic inflammation.

Kawasaki Disease Signs & Symptoms

Kawasaki’s disease occurs in three phases

• ·      Fever/acute phase

• ·      Subacute phase

• ·      Convalescent phase

Acute phase (0 to 19 Days) criteria:

For classic (typical) Kawasaki disease to be diagnosed, the child will have a fever (102 F+ (39 C+) lasting more than 5 days and 4 of the following symptoms:

• Red eyes

• Swollen, dry & red lips and tongue (strawberry tongue)

• Swollen lymph nodes in the neck

• Extremity changes such as swollen, red feet & hands or peeling of the fingers & toes

• A body rash on the trunk and/or genital area

 Atypical Kawasaki disease may be diagnosed with a fever lasting 5 or more days and 2 or 3 of the above symptoms.

The child will also often display decreased appetite & irritability. 

Subacute Phase (12 to 25 Days)

The subacute phase starts as the fever goes away. 

Symptoms often include:

• Arthritis frequently includes multiple joints, most commonly

• Joint pain

• Decrease in fever 

• Dry cracked lips

• Dry or peeling fingers and toes

• Cardiac disease, Inflammation & weakening of coronary arteries, formation of aneurysms

The convalescent phase

The convalescent phase begins when the symptoms have stopped, although blood tests may still show signs of inflammation. 

Kawasaki Disease Treatment

Typical treatment for Kawasaki disease is aspirin & IVIG intravenous gamma globulin. 

Children who are treated within the first 10 days with (IVIG) intravenous gamma globulin and aspirin tend to recover well and not have long lasting effects. 

Less than 5% of children who are treated with IVIG in the acute phase of Kawasaki disease develop coronary artery problems. 

However, 20% of children not treated develop coronary artery disease. 

Kawasaki Disease & Aspirin

The first time I cared for a child with Kawasaki’s disease, one of my first questions was, why are we giving aspirin to a pediatric patient?

My second question was, why so much aspirin?

We generally avoid giving children aspirin because of the possibility of Reyes syndrome.  If a patient develops the flu or chickenpox while taking aspirin, Reyes syndrome (which can cause swelling of the liver and brain) could develop.

There is some debate about whether a high dose is needed in comparison to a low dose of aspirin, however, it is given because the benefits of reducing the risk of heart problems and aneurysms in children with Kawasaki seems to outweigh the risks. 

Family Education: It’s important for families to know that aspirin should not be given to young children unless it is under the supervision of a healthcare provider. 

Summary

Top 10 quick notes: Kawasaki Disease

1.     Kawasaki disease is rare.  It is more common in the late winter and early

spring and usually affects children 6 months to 5 years old.

2.     It causes inflammation of the blood vessels and can lead to aneurysms,

coronary thrombosis, stenosis, scarring & eventually, heart attack. 

3.     Is the leading cause of acquired heart disease in children.

4.     Children with a fever lasting more than 5 days should be evaluated for

Kawasaki disease (diagnosis usually includes a fever for 5 days and 4

of the symptoms)

5.     Kawasaki’s disease occurs in three phases – symptoms include:

• Redness and swelling of the hands and feet (especially the palm and soles)

• Red eyes

• Swollen, dry, & red lips and tongue

• Diffuse red body rash

• Swollen lymph nodes in the neck

• Cardiac abnormalities

• “Strawberry tongue” - may also see bleeding of lips

• Painful extremities

• The skin can become shiny and stretched6.    

• Early treatment (within 10 days) shortens the illness and reduces risk of long-term effects.

7.     Treatment includes Aspirin, IVIG intravenous gamma globulin & comfort.

8.     The incidence is higher in boys than in girls.

9.    The cause is unknown.  There is no evidence of genetic origin or

person-to-person spreading. 

10.  There is growing concern in the medical community that first-line

providers may be focused on COVID-19 when children present with a fever,

it’s important to consider Kawasaki disease in children with a fever for

greater than 5 days. 

References

American Family Physician.  (2015). Diagnosis and Management of Kawasaki Disease. https://www.aafp.org/afp/2015/0315/p365.html

American Heart Association (2019).  Kawasaki Disease. https://www.heart.org/en/health-topics/kawasaki-disease#:~:text=Kawasaki%20disease%20(KD)%2C%20also,vessels%2C%20particularly%20the%20coronary%20arteries.

Kawasaki Kids Foundation.  (2020).  Kawasaki Disease Symptoms.  https://www.kawasakikidsfoundation.org/kawasaki-disease-symptoms/?gclid=CjwKCAiAnIT9BRAmEiwANaoE1a5Tw9npHOiMseC5EUHXPk2QvICpHyDNGl7WXleHaLrFUp5KHv4GLhoCerIQAvD_BwE

MD Calc. (2020).  Kawasaki Disease Diagnostic Criteria.

https://www.mdcalc.com/kawasaki-disease-diagnostic-criteria

Pediatrics: Official Journal for the American Academy of Pediatrics (March 20, 2017). Aspirin Dose and Prevention of Coronary Abnormalities in Kawasaki Disease. https://pediatrics.aappublications.org/content/139/6/e20170098

Sundel, R. (2020, June 29). Kawasaki disease: Clinical Features and Diagnosis. UpToDate. https://www.uptodate.com/contents/kawasaki-disease-clinical-features-and-diagnosis

The National Center for Biotechnology Information advances science. (2020, July 1).  Letter to the Editor: Missed or delayed diagnosis of Kawasaki disease during the 2019 novel coronavirus disease (COVID-19) pandemic https://www.jpeds.com/article/S0022-3476(20)30556-4/fulltext