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Hospital & Regional Medical Center Guideline of Care KAWASAKI DISEASE (KD) Mucocutaneous Lymph Node syndrome

I.

Description: KD is an acute inflammation of systemic blood vessels (systemic vasculitits) of unknown cause. It is the second most common vasculitic illness of childhood. Most cases occur in children < 5 years old (80%). About 20% of those affected will develop cardiac sequelae.

II.

Important Considerations: Cardiac complications include coronary and peripheral artery aneurysms, cardiac tamponade, cardiac failure, myocarditis, pericarditis, and myocardial infarction. There are reports of seasonal variation with peak occurrences in winter and spring. There is no diagnostic test for KD. It is diagnosed based on clinical criteria and after other viral or bacterial illnesses have been ruled out. Clinical criteria include fever for 4 days or more, non vesicular rash or non-purulent conjunctivitis plus four of the following five criteria: Conjunctivitis A. Lymphadenopathy B. Rash, non vesicular C. Changes of lips or oral mucosa (red cracked lips, strawberry tongue) D. Changes of extremities (erythema, edema, peeling of skin) E. Incomplete cases of KD occur and have an increased risk of complications due to misdiagnosis. To lessen the risk of complications children who present with unexplained fever for >=4days, rash or conjunctivitis will be considered for the KD pathway which will include at least an echocardiogram and a cardiology consult.

III.

Assessment: A.

History/Phase of Illness (children tend to be very irritable during the course of KD) Acute phase lasts 7-14 davs Progressive inflammation of small blood vessels (vasculitis) 1. High fever 2. Inflammation of the pharynx 3. Conjunctivitis 4. Rash 5. Lethargic, irritable 6. Strawberry tongue 7. Red, cracked lips 8. Swollen, reddened joints 9. Hepatic dysfunction 10. Aseptic meningitis 11. Lymphadenopathy (note: as internal lymph nodes swell, children may 12. develop abdominal pain, anorexia, and diarrhea)

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B.

Subacute phase, day 10-24 Inflammation of larger vessels 1. Thrombocytosis and hypercoagulability 2. Aneurysms may form which may lead to sudden death from accumulating 3. thrombi or rupture of the aneurysm 4. Fever, rash, and lymphadenopathy resolve 5. Irritability, anorexia, and conjunctivitis persist 6. Desquamation (shedding of the epidermis) of palms and soles 7. Arthralgia (pain in joints) 8. Arthritis Convalescent phase, day 25-40 1. Clinical signs of KD resolve 2. Lab results return to normal usually 6-8 weeks from onset 3. Follow up with cardiology is important to monitor for new and/or resolving cardiac complications Comfort 1. See “Comfort” GOC. 2. Assess for increased levels of anxiety (possibly due to cardiac complications or pain). 3. Neurological a. Assess neurologic status per unit routine. b. Assess for extreme irritability or seizure activity. Patient may develop aseptic meningitis. 4. Respiratory a. Assess for respiratory distress (may be related to coronary abnormalities: CHF, coronary artery thrombosis, myocarditis, pericarditis, cardiogenic shock). i. Tachypnea ii. Nasal flaring iii. Retractions iv. Grunting v. Decreased oxygen saturations vi. Diminished or abnormal breath sounds Assess for chest pain, type, location and severity. b. CXR as ordered to assess lung fields and cardiac silhouette. c. 5. Cardiovascular a. Continuous EKG monitoring while in bed. When out of room or off unit, patient will be accompanied by staff, family member or volunteer. b. Assess and report chest pain c. VS unit routine and clinical status of the patient d. Assess perfusion. i. Peripheral pulses ii. Capillary refill iii. Skin temperature, temperature line of demarcation iv. Color v. Urine output

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Page 3: Children’s Guideline of Care: KAWASAKI DISEASE (KD) e.

6. 7.

8.

9. IV.

Assess for cardiac arrhythmias, abnormal heart sounds due to coronary aneurysm, myocarditis, pericarditis, mitral regurgitation, Cardiac manifestations may develop and persist long-term. f. If in ICU, assess for decreased ventricular function and valvular insufficiency. Monitor labs as ordered. Integumentary System a. Assess skin for signs and symptoms of rash, skin integrity. b. Assess mouth for sores and fissures. c. Assess eyes for conjunctivitis. Renal a. Assess for signs and symptoms of urethritis. b. Strict I&O per unit routine. c. Daily weight GI a. Assess q shift for abdominal pain, diarrhea and vomiting.

Interventions: A.

EKG/Comfort/Mobility 1. "Comfort" GOC with be utilized. 2. Decrease external stimulation (i.e. lights and noise) for extreme irritability. 3. Use non-pharmacological interventions to promote comfort to joints and swollen extremities: a. Warm environment b. Warm compresses to joints c. Adequate hydration/mouth care d. Supportive positioning e. Distraction and relaxation techniques

B.

Respiratory 1. Maintain oxygen saturations ≥95%. 2. Administer O2 prn with chest pain, as ordered. Cardiovascular Prepare for and administer IVIG and aspirin treatment as ordered. 1. Perform EKGs with any cardiac symptoms (e.g., abdominal/chest pain or 2. onset of CHF). Cardiology consult on all patients with KD and expect echocardiogram 3. and possibly and EKG. Skin Good personal hygiene for comfort and to promote skin integrity. 1. Skin care with lotions to alleviate itching that may occur with rash. 2. Mouth care q 2-4 hours. 3. Renal Ensure adequate hydration. 1. Strict I&O. 2. Daily weight 3.

C.

D.

E.

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G. H. I. J. K. L. V.

Outcomes: A. B. C. D. E. F. G. H.

VI.

Expected treatment interventions: Treatment is aimed at reducing inflammation and preventing complications of thrombosis and aneurysms. 1. In the acute phase, aspirin is as an anti-inflammatory at 30-50 kg/day and may be given up to 100mg/kg/day. During the subacute phase, aspirin may be decreased to 2-5mg/kg/day and continued dependent on results of cardiac echo. In addition to aspirin, immunoglobulin is given to the patient in a high dose single administration. IVIG is usually ordered as 2g/kg in a single dose over 8-12 hours. In resistant cases, the dose may be repeated. In infants and children with fragile cardiac status, the infusion may be divided to prevent cardiac compromise from the IVIG fluid volume. (See Children’s Formulary of Medications (online) for information on IVIG infusion including information about premedication, rate of administration, and vital sign monitoring.) Monitor for aspirin toxicity (tinnitus, headache, dizziness, confusion) Administer coumadin or heparin as ordered for treatment of severe coronary findings. Apply soothing ointments to lips and provide gentle mouth care Provide soft, nonirritating foods as tolerated Provide cool liquids to maintain hydration and reduce mouth tenderness Consult Child Life Therapist for distraction and non pharmacological options for comfort and irritability, assess family and child’s coping with hospitalization

Observe for 18-24 hours after completion of IVIG infusion Child symptoms are improving and overall the child condition is improved No fever for at least 18 hours prior to discharge Echocardiogram complete Cardiologist has seen child Child’s PMD has been contacted and discharge instructions and follow up plans have been faxed to the doctors office. Child has a confirmed appointment with PMD within 48 hours of discharge Cardiology follow up appointment(s) are scheduled

Patient/Family Education: See attached interdisciplinary teaching tool, typically initiated and completed on the acute care unit, once child has transferred from ICU

VII.

Health Issues/Plan of Care/Problem List: A. B. C. D. E. F.

Potential cardiac complications: cardiology consult, echocardiogram Administration of aspirin: dose for acute phase and then decrease for subacute phase, ongoing plan developed at time of discharge. IVIG administration Community follow-up plan: PMD identified and appointment made Cardiology clinic appointment and follow up plans made Long-term care and follow up needs communicated in writing to parents.

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Page 5: Children’s Guideline of Care: KAWASAKI DISEASE (KD) VIII. References: Barron KS. (1998). Kawasaki disease in children Current Opinion In Rhematology, 10:29-37. Belkengren R and Sapala S. (1997). Pediatric management problems. Kawasaki Disease. Pediatric Nursing, 23(4):404-405. Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, Klein N, Booy R, Levin M, Dilon MJ (2002) Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. ndArch dis Child. 86, 286-290. Jaffe, M (1998). Pediatric nursing, careplans,(2 edition). Englewood, Co: Skidmore-Roth Pahl E. (1997). Kawasaki disease: cardiac sequelae and management. Pediatric Annals, 26(2):112-115. Payling .J. (1997). Kawasaki disease. Professional Nurse, 13(2):108-109. Pillitteri, A. (1995) Maternal and Child Health Nursing: Care of the Childbearing and nd Childrearing Family. (2 ed) Philadelphia, PA: Lippincott. Rubin B and Cotton DM. (1998). Kawasaki disease: a dangerous acute childhood illness. Nurse Practitioner, 23(2):34, 37-38, 44-48. Shulman S, DeInocencio J, and Hirsch, R. (1995). Kawasaki Disease. Pediatric Rheumatology. 42(5), 1205-1222. Takahashi M. (1997). Kawasaki disease. Current Opinion in Pediatrics, 9(5):523-529. Originated by:

Debra Ridling, RN, MSN, PICU CNS Jerry Zimmerman, MD, PICU Medical Director Kawasaki Pathway Workgroup (Brian D. Johnstom, MD, MPH, Facilitator) Kristi Klee, RN, MSN, Medical Unit CNS Linda Crandall RN, Medical Unit

Reviewed by: Revised by:

APPROVED BY:

Richard Molteni, MD Vice President & Medical Director ORIGINATED: REVIEWED: REVISED:

Susan Heath, RN, MN Nurse Executive

5/03

Additional Key Words:

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Learner needs and expected outcomes The patient and family will express understanding of or demonstrate the following: Kawasaki disease is an illness that causes swelling and inflammation of blood vessels. This swelling and inflammation may occur in vessels of the heart. This is why we monitor your child so closely. The patient and family will express understanding of or demonstrate the following: Need for CRM and possibly oximetry

The patient and family will express understanding of or demonstrate the following: Medications used to treat inflammation Aspirin

The patient and family will express understanding of or demonstrate the following: Medications used to treat inflammation IVIG

The patient and family will express understanding of or demonstrate the following: Need for echocardiogram and possibly EKG during admission and as outpatient to follow up cardiac status

The patient and family will express understanding of or demonstrate the following: Administration of Aspirin as part of home regimen until outpatient f/u with cardiology. Do not stop aspirin unless discuss with PMD/Cardiology

Learner Parent Child Other Level of understanding

❑ ❑ ❑

Initital teaching, needs reinforcement Partial understanding/demonstrates with help Independent understanding/demonstrates skill

Learner Parent Child Other Level of understanding

❑ ❑ ❑

Teaching Methods Verbal instruction Audio Visual Written instruction Class Demonstration Other Time Date

Initital teaching, needs reinforcement Partial understanding/demonstrates with help Independent understanding/demonstrates skill

Learner Parent Child Other Level of understanding

❑ ❑ ❑

Teaching Methods Verbal instruction Audio Visual Written instruction Class Demonstration Other Time Date

Initital teaching, needs reinforcement Partial understanding/demonstrates with help Independent understanding/demonstrates skill

Learner Parent Child Other Level of understanding

❑ ❑ ❑

Teaching Methods Verbal instruction Audio Visual Written instruction Class Demonstration Other Time Date

Initital teaching, needs reinforcement Partial understanding/demonstrates with help Independent understanding/demonstrates skill

Learner Parent Child Other Level of understanding

❑ ❑ ❑

Teaching Methods Verbal instruction Audio Visual Written instruction Class Demonstration Other Time Date

Initital teaching, needs reinforcement Partial understanding/demonstrates with help Independent understanding/demonstrates skill

Learner Parent Child Other Level of understanding

❑ ❑ ❑

Teaching Methods Verbal instruction Audio Visual Written instruction Class Demonstration Other Time Date

Teaching Methods Verbal instruction Audio Visual Written instruction Class Demonstration Other Time Date

Initital teaching, needs reinforcement Partial understanding/demonstrates with help Independent understanding/demonstrates skill

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Page 7: Children’s Guideline of Care: KAWASAKI DISEASE (KD) Learner needs and expected outcomes The patient and family will express understanding of or demonstrate the following: Patient may not have any live virus vaccines for 11 months post IVIG.

The patient and family will express understanding of or demonstrate the following: Coping strategies (family and child) Child life referral and follow of plan even at home as needed for compliance.

Learner Teaching methods Parent Verbal instruction Audio Visual Child Written instruction Class Other Demonstration Other Level of understanding Date Time ❑ Initial teaching, needs reinforcement ❑ Partial understanding/demonstrates with help ❑ Independent understanding/demonstrates skill

Teaching methods Learner Parent Verbal instruction Audio Visual Child Written instruction Class Other Demonstration Other Level of understanding Date Time ❑ Initial teaching, needs reinforcement ❑ Partial understanding/demonstrates with help ❑ Independent understanding/demonstrates skill

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Page 8: Children’s Guideline of Care: KAWASAKI DISEASE (KD) PATIENT AND FAMILY EDUCATION SUMMARY NOTES DATE/TIME

NOTES

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DATE/SIGNATURE