Kawasaki Disease in the Pediatric Patient

Kawasaki disease is a condition affecting the blood vessels, mainly in children between 1-8 years of age, with the most prevalence occurring in those five and younger. You’re most likely to see it in boys, especially those of Japanese or Pacific Island descent, though it can certainly appear in boys and girls of any ethnic background.

Kawasaki causes severe inflammation of the vessels (vasculitis), namely the coronary arteries, which can be quite severe and cause permanent heart damage if left untreated. The heart damage can take the form of myocarditis, endocarditis, pericarditis, arrhythmias, heart failure and coronary artery aneurysms. Children who develop aneurysms larger than 8mm in diameter have the poorest outcomes, while ischemic heart disease can develop leading to the child needing a heart transplant.

Its cause isn’t fully understood, but some evidence shows it is thought to be an immunologic reaction to a toxic, antigenic or infectious substance. Because of the range of symptoms, Kawasaki disease can sometimes be mistaken for Stevens-Johnson syndrome, juvenile rheumatoid arthritis, scarlet fever and even measles. The good news is, once identified it’s very treatable and most kids recover just fine. If treatment is delayed and cardiac involvement occurs, some children can develop heart disease later in life. So, the best course of action is to identify and treat it early. Using the Straight A Nursing LATTE method, we’ll go through the key nursing assessments and interventions for this disease. Ready? 

L: How will the patient LOOK?

Kids with Kawasaki disease typically exhibit a common symptom of high fever and bright red skin. 

Symptoms often present in a predictable pattern: 

• Fever that lasts about 5 days with possible irritability, fatigue and abdominal pain.
• Bulbar conjuctiva tends to appear after one to two days of fever.
• Next, the child typically develops a erythematous macular rash that is mainly concentrated in the perineal area but can also be over the trunk. The child’s mouth may now be very red, along red and swollen palms of the hands and soles of the feet.
• Around the 10th day of the disease, the skin will begin flaking off leaving normal-appearing skin exposed.
• Approximately 50% of patients will have swollen cervical lymph nodes, and the disease is also sometimes referred to as mucocutaneous lymph node syndrome for this reason.
• Other symptoms include joint pain, ear pain, inflammation of the eye, vomiting and diarrhea. 
• More serious complications can also occur, such as hepatitis, aseptic meningitis and hydrops of the gallbladder.
• It is estimated that approximately 20% of children will develop cardiac complications 1-4 weeks after symptom onset or even later in life. There is also a small risk of the child developing Reye’s Syndrome (which is related to the medication used to treat Kawasaki).

A: How do you ASSESS the patient?

Assessing children is a whole different process than assessing an adult. Children that are frightened or in pain often don’t cooperate, and many children are simply too young to tell you what’s bothering them. As you approach your assessment of the pediatric patient, the first thing to notice is how alert they are. A child that is lethargic or not responding to the environment is worrisome. Also, if the child is listless and lets you do your assessment without responding to you or struggling in any way, this is also a sign that something is possibly very wrong. 

A child with Kawasaki disease is likely to be very irritable and possibly also in pain, so getting a full assessment is going to be tricky. Some key things to assess are: 

• Temperature; children with Kawasaki disease often have a fever above 102 degrees
• Skin; look for reddened, swollen and even peeling areas
• EKG: the disease can affect the vessels of the heart causing dysrhythmias, so it’s always a good idea to have the child on a continuous monitor
• BP and Heart rate: expect tachycardia if the patient is irritable, possible hypotension in cases of severe dysrhythmia. 

T: What TESTS will be ordered?

• EKG and possibly an echocardiogram to evaluate if the disease has caused any damage to the child’s heart.
• CBC will show elevated WBCs (leukocytosis) and possibly elevated platelets (thrombocytosis) and normocytic anemia.
• Inflammatory markers (ESR or CRP will be elevated)
• BNP is elevated when the heart is under stress, so it could be elevated
• ANA and rheumatoid factor are used to exclude autoimmune diseases such as lupus or rheumatoid arthritis as the cause for the rash and joint pain.
• Serum aspirin level may be necessary if the child is taking it for more than two weeks.

T: What TREATMENTS will be provided?

Kawasaki disease is treated with high doses of immune globulin (IVIG) and aspirin. The IVIG is used to help prevent the coronary arteries from getting affected while the aspirin is used to manage inflammation, help prevent coronary artery abnormalities and lower fever. If the coronary arteries develop aneurysms, the patient may also need anticoagulants. 

E: How will you EDUCATE the family?

It is important that the family understand that the children with cardiac involvement will need continued evaluation of their heart through periodic ECG and echocardiograms. Also, if the child receives IVIG therapy, he could have a suppressed response to vaccines so adjustments may need to be made in the child’s vaccination schedule (sources indicate that live viral vaccines should be delayed for 11 months after the IVIG therapy). Also, children over 6 months should receive the inactivated flu vaccine.

The family should also be informed that the aspirin therapy can cause a complication known as Reyes Syndrome, which can cause brain and liver damage.

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I hope that helps you take care of these little patients in clinical (or on your exams).

The key takeaways for Kawasaki are: 

• Disease that causes vasculitis that can affect the coronary arteries
• Symptoms include fever, bright red hands/feet/mouth and peeling skin, swollen lymph nodes
• Treatment is aspirin and IVIG
• Risk for Reyes Syndrome with aspirin therapy

Get this on audio in Podcast Episode 106

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References

American Heart Association. (n.d.). Kawasaki Disease. Www.Heart.Org. Retrieved April 29, 2020, from https://www.heart.org/en/health-topics/kawasaki-disease

Deglin, J. H., & Vallerand, A. H. (2007). Davis’s drug guide for nurses(11th ed.). F. A. Davis Company.

Freeman, A. F., & Shulman, S. T. (2006). Kawasaki Disease: Summary of the American Heart Association Guidelines. American Family Physician,74(7), 1141–1148.

Mayo Clinic. (n.d.). Kawasaki disease—Symptoms and causes. Mayo Clinic. Retrieved from https://www.mayoclinic.org/diseases-conditions/kawasaki-disease/symptoms-causes/syc-20354598

Morikawa Yoshihiko, Miura Masaru, Sakakibara Hiroshi, & null null. (2015). Abstract 135: Effectiveness Of Live Vaccines Following Intravenous Immune Globulin Therapy During The Convalescent Phase Of Kawasaki Disease Using The Schedule Recommended In Japan. Circulation, 131(suppl_2), A135. https://doi.org/10.1161/circ.131.suppl_2.135