Chapter 26: Kawasaki's Disease (Children) Flashcards
Kawasaki’s Disease
- “mucocutaneous lymph nose syndrome”
- multisystem disease affecting the cardiovascular system
- affects the blood vessels
- causes inflammation in the walls of medium-sized arteries throughout the body. It primarily affects children. The inflammation tends to affect the coronary arteries, which supply blood to the heart muscle.
- is sometimes called mucocutaneous lymph node syndrome because it also affects lymph nodes, skin, and the mucous membranes inside the mouth, nose and throat.
- cause unknown; defective immune response to an infectious process is thought to be responsible
- not congenital or contagious
- acute phase: diffuse vasculitis leads to long-term cardiovascular problems 1 out of 5 pt’s affected
- one long term sequelae= aneurysm formation in arterial vessels
Signs and Symptoms
s/s are the result of vasculitis (inflammation of the blood vessels) affecting all organ systems
>together with persistent fever (5 days or more spiking to 104 Degrees F) the patient has four of the five signs:
-skin rash
-cervical lymphadenopathy (lymph node swelling in the neck), typically unilateral, greater than 1.5 mm in diameter
-edema and erythema of the hands and feet with eventual peeling of skin
-irritation and inflammation of the mouth with “strawberry tongue”, erythema, and cracking lips
-conjunctivitis (pink eye) without exudate
Diagnosis
- no specific test
- children with a fever along with other s/s in the presence of vessel aneurysm (a dilatation in the wall of an artery supplying blood to a specific area)
- a CBC, erythrocyte sedimentation rate (ESR), electrocardiogram, and echocardiogram are done to help confirm diagnosis
- occasionally, cardiac cath is indicated to diagnose aneurysm formation
Prevention
b/c cause is unknown, it is difficult to discuss prevention
-proper balanced diet, hydration, and healthy living will aid the child in the healing process
Nursing Care
- supportive treatment of the symptoms and on giving the prescribed medications
- emotional and spiritual care may be necessary if the child suffers the sequalae of aneurisms and the family is facing a lifetime of treatment and monitoring
- pre- and post-op care only required if child undergoes surgery for aneurism repair
Medical Care
- begins with administration of IV immunoglobulin (IVIG) and aspirin (ASA), which are used primarily for their anti-inflammatory effects
- steroids, plasma exchange, or cytotoxic agents my be used if this initial therapy is ineffective
Anticoagulant Medication Precautions
instruct the family about precautions if the child is taking anticoagulant mediations
-child is monitored closely because of the risk of infection, bleeding, and bruising
Education/Discharge
- follow-up visits are essential
- if on anticoagulant therapy, appropriate blood testing and monitoring should be adhered to
- may have activity restrictions if aneurisms involved
Thrombus Formation
-teach parents that children with aneurysm formation as a result of Kawasaki’s disease require long-term follow-up for continued assessment related to other vascular changes such as stenosis or tortuosity (twisting)
-in the event of thrombus (blood clot) formation, the tx is the same as for any patient who is at risk for a myocardial infarction
>Thrombotic agents, such as streptokinase (Streptase), urokinase (Abbokinase), and alteplase (Activase), are used with some success in thrombus formation
>long-term use of anticoagulants such as warfarin (Coumadin) or clopidogrel (Plavix) may also be sued to prevent thrombus formation in the engorged or aneurysmal vessels
ATI: Kawasaki Disease
acute systemic vasculitis
- resolves in less than 8 weeks
- “mucocutaneous lymph node syndrome”
ATI: Acute Phase
onset of high fever, lasting 5 days to 2 weeks that is unresponsive to antipyretics
- irritability
- red eyes without drainage
- bright red, chapped lips
- strawberry tongue with white coating or red bumps on the posterior aspect
- red oral mucous membranes with inflammation including the pharynx
- swelling of hand and feet with red palms and soles
- non-blistering rash
- bilateral joint pain
- enlarged lymph nodes
- desquamation of the perineum
- cervical lymphadenopathy
- cardiac manifestations: Myocarditis, decreased ventricular function, pericardial effusion, and mitral regurgitation
ATI: Subacute Phase
resolution of fever and gradual subsiding of other manifestations
- irritability
- peeling skin around the nails, on the palms and soles
- temporary arthritis
ATI: Convalescent
no manifestations seen except altered laboratory findings
-resolution in about 6 to 8 weeks from onset
ATI: Laboratory Tests
- CBC
- CRP
- ESR
- Blood albumin
- elevated liver enzymes
- lumbar puncture to asses for aseptic meningitis and inflammation
ATI: Diagnostic Procedures
-Chest x-ray
-Echocardiogram and/or ECG: to indicate myocarditis, pericarditis, arthritis, meningitis, inflammation
>follow-up study recommended 6 to 8 weeks
