Kawasaki Disease Nursing Care Management: Study Guide

Kawasaki disease is a generalized vasculitis of small to medium arteries, and the part that kills is the coronary arteries. Your job on the floor is to recognize it fast, get IVIG in within the first 10 days, watch the heart, and prevent coronary artery aneurysms. It is an acute febrile illness, most often in boys younger than 5 years, also called mucocutaneous lymph node syndrome or infantile periarteritis nodosa. Prognosis is good with treatment, but a small percentage die from coronary artery aneurysm (CAA).

Pathophysiology

Despite the loud mucocutaneous findings, treat this as systemic vasculitis hitting small to medium arteries. Most cases follow infection and cluster in late winter and early spring.

Early on, endothelial cells and the vascular media become edematous while the internal elastic lamina stays intact. Roughly 7-9 days after fever starts, neutrophils flood in, then CD8+ (cytotoxic) lymphocytes and immunoglobulin A (IgA) producing plasma cells. These cells release cytokines (tumor necrosis factor, vascular endothelial growth factor, monocyte chemotactic and activating factor), interleukins, and matrix metalloproteinases that attack endothelium, fragment the internal elastic lamina, and damage the vessel. Inflammation involves all 3 layers of the vessel.

Over weeks to months, fibroblasts and monocytes replace the active inflammatory cells and fibrous connective tissue forms in the wall. The intima proliferates and thickens, and the vessel narrows or occludes from stenosis or thrombus. The most dangerous window is when the serum platelet count climbs progressively. That is when the risk of death peaks.

Statistics and Incidences

Epidemics hit in late winter and spring at 2 to 3 year intervals.

About 3,000 children are hospitalized with Kawasaki disease each year in the United States. Annual race-specific incidence per 100,000 children younger than 5 years runs 32.5 cases for Americans of Asian and Pacific Island descent, 16.9 for non-Hispanic African Americans, 11.1 for Hispanics, and 9.1 for whites. Outside the US it is most common in Japan, Taiwan, and Korea.

Prevalence rose from 1967 to the mid-1980s and leveled at 5000-6000 cases per year. Japan reports the highest incidence, 10 to 20 times higher than Western countries, with 5000-6000 cases each year; the 2000 incidence was 134.2 cases per 100,000 children younger than 5 years. White populations outside the US track the US rate: 11.3-14.7 per 100,000 children younger than 5 years in Canada, 3.6 in Australia. From 1999-2000 the United Kingdom rate was 8.1 cases per 100,000 children. Ontario has the highest rate outside Asia at 26.2 cases per 100,000 population younger than 5 years.

It occurs in all ethnic groups but is most common in Asian children, especially those of Japanese descent. It is slightly more common in males, with a male-to-female ratio of 1.3-1.83:1 depending on the country. About 85-90% of cases occur in children younger than 5 years, and 90-95% in children younger than 10 years.

Causes

The cause is unknown but the pattern points to an infectious agent.

Genetic factors matter. Siblings of affected children have a 10-20 times higher chance of developing Kawasaki disease than the general population, and Japanese children whose parents had Kawasaki disease tend to get a more severe form and recur more often.

The infectious pattern shows up in the epidemics clustering in late winter and spring at 3 year intervals, their wavelike geographic spread, the self-limited course, and the characteristic fever, adenopathy, and eye signs.

Clinical Manifestations

The presentation moves through three stages: acute, subacute, and convalescent.

Acute febrile stage. Abrupt fever lasting about 7-14 days, typically high-spiking and remittent with peaks of 102-104°F (39-40°C) or higher. Along with fever, expect irritability, nonexudative bilateral conjunctivitis (90%), anterior uveitis (70%), perianal erythema (70%), erythema and edema of the hands and feet that impedes walking, strawberry tongue and lip fissures, hepatic/renal/GI dysfunction, myocarditis and pericarditis, and lymphadenopathy (75%), usually a single enlarged nonsuppurative cervical node about 1.5 cm.

Subacute stage. Starts when fever breaks and runs to week 4-6. Hallmarks are desquamation of the digits, thrombocytosis (platelet count may exceed 1 million/μL), and coronary aneurysms. Risk of sudden death is highest here.

Convalescent stage. Clinical signs resolve, usually within 3 months of presentation, as acute phase reactants (ESR, C-reactive protein) and other labs return to baseline. Deep transverse grooves across the nails (Beau lines) may appear 1-2 months after fever onset.

Physical Examination

No single test and no single finding is pathognomonic, so diagnosis rests on a constellation of findings.

Peripheral extremities show initial reddening or edema of the palms and soles, then membranous desquamation of the finger and toe tips or transverse grooves across the nails (Beau lines). The rash is polymorphous and nonvesicular, usually generalized but sometimes limited to the groin or lower extremities. Oropharyngeal changes include erythema, fissuring, and crusting of the lips, strawberry tongue, and diffuse mucosal injection. Conjunctivitis is bilateral, nonexudative, painless, and bulbar. Lymphadenopathy is acute, nonpurulent, cervical, with a node greater than 1.5 cm, usually unilateral.

Assessment and Diagnostic Findings

No specific lab diagnoses Kawasaki disease, but certain abnormalities track the stages.

Urine proteins meprin A and filamin C show promise as biomarkers and were diagnostically superior to ESR or CRP; investigators found more than 190 proteins present only in children with Kawasaki disease, including filamin C (endothelial and myocardial cell injury) and meprin A (immune regulation). On CBC, mild to moderate normochromic anemia appears in the acute stage and the WBC is moderate to high, with 50% of patients showing a WBC greater than 15,000/µL and a left shift. Platelet count is the subacute marker: it rises in the second week and keeps climbing through the third, averaging 700,000/μL with levels as high as 2 million observed. Serum cholesterol, HDL, and apolipoprotein A fall and stay low past clinical resolution.

Echocardiography is the study of choice for coronary artery aneurysms (CAAs), in both full and suspected incomplete cases. MRI, MRA, and ultrafast CT are other noninvasive options for coronary abnormalities. On ECG, tachycardia, prolonged PR interval, ST-T wave changes, and decreased R wave voltage suggest myocarditis; Q waves or ST-T changes suggest myocardial infarction. Cardiac enzymes (CK, CK-MB, cardiac troponin, lactate dehydrogenase [LD-1 >LD-2]) rise during a myocardial infarction.

Medical Management

The goal is to prevent coronary artery disease and relieve symptoms.

Pharmacologic Therapy

Full-dose intravenous immunoglobulin (IVIG) is the mainstay.

IVIG relieves acute inflammation and drops the coronary aneurysm rate from greater than 25% in untreated patients to 1-5% in treated patients; benefit is maximal when given within the first 10 days of illness. Aspirin is synergistic with IVIG and a long-standing part of therapy. Teach families that ibuprofen antagonizes aspirin's irreversible platelet inhibition and should be avoided during therapy, and address the Reye syndrome risk during active influenza or varicella. Corticosteroids are used for IVIG-resistant disease and have been proposed as primary therapy. Roles for pentoxifylline (an anti-inflammatory that inhibits tumor necrosis factor-alpha and may cut aneurysm incidence) and abciximab (a platelet glycoprotein IIb/IIIa receptor inhibitor used with standard therapy in giant aneurysms) are not yet settled. Anticoagulants such as warfarin and low molecular weight heparin are used for large aneurysms with high thrombosis risk, targeting an INR of 2-2.5.

Nursing Management

Nursing Assessment

Assess the child in every phase. In the acute febrile phase the child looks severely ill and irritable, with high spiking fever for 5 or more days, bilateral conjunctival injection, oropharyngeal erythema, strawberry tongue or red dry lips, erythema and edema of the hands and feet, periungual desquamation, an erythematous generalized rash, and cervical lymphadenopathy greater than 0.6 inch (1.5 cm). In the subacute phase the acute symptoms subside and temperature normalizes, but the child stays irritable and anorectic. In the convalescent phase, check the new diagnostic results to establish disease status.

Nursing Diagnosis

Major diagnoses include chronic pain related to myocardial or pericardial inflammation, risk for decreased cardiac output related to pericardial fluid accumulation, activity intolerance related to myocardial inflammation and degeneration, impaired skin integrity related to the inflammatory process and altered circulation and edema, and impaired oral mucous membrane related to inflammation, dehydration, and mouth breathing.

Nursing Care Planning and Goals

The family understands that Kawasaki disease inflames and swells blood vessels, that this can involve the vessels of the heart, and that this is why the child is monitored closely. They understand the need for cardiorespiratory monitoring and possibly oximetry, the medications used to control inflammation, and the need for echocardiogram and possibly EKG during admission and as an outpatient to follow cardiac status. Family and child demonstrate coping strategies, accept child life referral, and follow the plan at home for compliance.

Nursing Interventions

Monitor pain level and the child's response to analgesia. Run cardiac monitoring: take vital signs as conditions dictate, assess for myocarditis (tachycardia, gallop rhythm, chest pain), and watch for heart failure. Monitor intake and output closely and track hydration by skin turgor, weight, urine output, specific gravity, and presence of tears. Plan rest and activity: allow uninterrupted rest, let the child move freely under supervision, provide soft toys and quiet play that uses hands and fingers, and keep the environment calm with diversional activities. Provide oral care: offer cool liquids (ice chips and ice pops), progress to soft bland foods, give mouth care every 1 to 4 hours with special mouth swabs, and use a soft toothbrush only after healing.

Evaluation

Goals are met when the child's symptoms and overall condition improve, there is no fever for at least 18 hours before discharge, the echocardiogram is complete, the cardiologist has seen the child, the physician has been contacted and discharge instructions and followup plans are finalized, the child has a confirmed appointment with the physician within 48 hours of discharge, and cardiology followup appointments are scheduled.

Documentation Guidelines

Document the duration of the problem and specific contributing factors; perception of pain, effect on lifestyle, and expectations of management; baseline and subsequent hemodynamic patterns, heart and breath sounds, ECG patterns, presence and strength of pulses, skin or tissue status, renal output, and mentation; level of activity; characteristics of lesions and ulcer classification; condition of oral mucous membranes and routine oral care; the plan of care and teaching plan; responses to interventions, teaching, and actions; attainment or progress toward outcomes; and modifications to the plan.