Kawasaki Disease

- also called mucocutaneous lymph node syndrome
- an inflammatory condition that affects small- and medium-sized arteries throughout the body, including coronary arteries
- also a febrile, multi-system disorder that also affects lymph nodes, skin, and mucous membranes of the mouth, nose, and throat
- occurs almost exclusively in children ages 2 to 5, and commonly affects boys than girls
- also common among children of Japanese or Korean descent
- no exact cause of Kawasaki disease has been identifiedand it doesn't appear to be hereditary


Assessment Findings

A.) Acute phase

- high fever--> does not respond to antipyretics
- enlarged cervical lymph nodes
- conjuctivitis--> inflamed mucous membranes of the eyes; without discharge
- 'strawberry' tongue
- red, cracked lips
- lethargy or irritability
- abdominal pain, anorexia, diarrhea
- red and swollen hands and feet
- rash--> often in diaper area and trunk
- swollen and reddened joints
- diagnostic tests: elevated WBC count, elevated ESR


B.) Subacute phase (about 10 days after onset)

- possible formation of coronary aneurysms--> very dangerous and can lead to death; requires sequential echocardiograms
- skin desquamation in palms and soles of the feet
- elevated platelet count--> increases clotting and necrosis of cells not receiving adequate blood supply, esp. in the fingertips


C.) Convalescent phase

- signs and symptoms go away unless complications develop



NOTE: to be diagnosed with kawasaki disease, a child must manifest fever and four of the following symptoms:

1.fever of more than 5 days
2.bilateral congestion of conjuctiva
3.red and dry pharynx, red and cracked lips, 'strawberry' tongue
4.peripheral edema, peripheral erythema, and desquamation of hands and soles
5.rash in trunk
6.swollen cervical lymph nodes



Medical Management

a.) aspirin--> reduces inflammation and prevents clot-formation; a dose of 100mg/kg/day may be required; not to be given if child develops flu or chickenpox during treatment (may cause Reye's syndrome)

b.) dipyridamole--> may be given with aspirin; increases coronary vasodilatation and decreases platelet accumulation

c.) IV gamma globulin--> decreases immune response; given instead of steroids ( may increase aneurysm formation)



Surgical Management

- If the patient develops coronary artery disease, the following procedures may be required:

a.) coronary artery angioplasty--> opening of the arteries that have narrowed

b.) stent replacement--> implanting a device to re-open a blocked artery

c.) coronary artery bypass graft--> involves rerouting the blood around a diseased coronary artery by grafting a section of blood vessel from the leg, chest or arm to use as the alternate route



Nursing Management

a.) always observe patient for signs of heart failure such as tachycardia, dyspnea, rales, and edema
b.) evaluate peripheral tissue perfusion
c.) monitor for chest pain and ECG changes
d.) administer medications as ordered
e.) observe for signs of aspirin toxicity (tinnitus, nausea, vomiting, headache, blurred vision)
f.) provide addition comfort measures such as rocking and holding
g.) protect lips from cracking and drying; apply lip balm
h.) ensure adequate fluid intake to maintain hydration
i.) protect edematous extremities from pressure
j.) offer soft, non-irritating foods such as gelatin
k.) provide oral care with the use of padded toungue blades or soft toothbrush
l.)provide health teachings (to parents ) regarding the importance of echocardiogram every 1-2 years to screen patient for heart problems



Prognosis

- Full recovery can be expected with early recognition and treatment. Unfortunately, 2% of patients with Kawasaki Disease die from complications of coronary blood vessel inflammation.


Prevention

Kawasaki disease is not preventable.