ACUTE RHEUMATIC FEVER

Acute rheumatic fever (ARF) is an acute autoimmune disease that occurs as a sequel of group A beta-hemolytic streptococcal infection. It is characterized by inflammatory lesions of connective tissue and endothelial tissue, primarily affecting the joints and heart. Pathophysiology and Etiology

Most first attacks of ARF occur 1 to 5 weeks (average 3 weeks) after a streptococcal infection of the throat or of the upper respiratory tract. Peak incidence occurs in children ages 6 to 15. Incidence after a mild streptococcal pharyngeal infection is 0.3% and after a severe streptococcal infection is 1% to 3%. Family history of rheumatic fever is usually positive. Streptococcal infection abates with or without treatment; however, auto-antibodies attack the myocardium, pericardium, and cardiac valves.
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Asch offs bodies (fibrin deposits) develop on the valves, possibly leading to permanent valve dysfunction, especially of the mitral and aortic valves. Severe myocarditis may cause dilation of the heart and CHF.

Inflammation of the large joints causes a painful arthritis that may last 6 to 8 weeks.

Involvement of the nervous system causes chorea (sudden involuntary movements).

Clinical Manifestations Documented or undocumented group A beta-hemolytic streptococcal infection is usually followed in several weeks by fever, malaise, and anorexia. Major symptoms of ARF may appear several weeks to several months after initial infection. Major Manifestations

Carditis manifested by systolic or diastolic murmur, prolonged PR and QT intervals on ECG, and possibly by signs of CHF. Polyarthritis pain and limited movement of two or more joints; joints are swollen, red, warm, and tender. Chorea purposeless, involuntary, rapid movements commonly associated with muscle weakness, involuntary facial grimaces, speech disturbance, and emotional lability. Erythema marginatum nonpruritic pink, macular rash mostly of the trunk with pale central areas; migratory. Subcutaneous nodules firm, painless nodules over the scalp, extensor surface of joints, such as wrists, elbows, knees, and vertebral column.

Minor Manifestations

History of previous rheumatic fever or evidence of preexisting rheumatic heart disease.

Arthralgia pain in one or more joints without evidence of inflammation, tenderness, or limited movement. Fever temperature greater than 100.4 F (38 C). Laboratory abnormalities elevated erythrocyte sedimentation rate (ESR), positive C-reactive protein, elevated white blood cell count. ECG changes prolonged PR interval.

Diagnostic Evaluation

Diagnosed clinically through use of the Jones criteria from the American Heart Association presence of two major manifestations or one major and two minor manifestations (as listed above), with supporting evidence of a recent streptococcal infection. ECG done to evaluate PR interval and other changes. Laboratory tests listed above. In addition, group A streptococcal culture and/or anti-streptolysin-O titer to detect streptococcal antibodies from recent infection. Chest X-ray for cardiomegaly, pulmonary congestion, or edema.

Management

Course of antibiotic therapy to completely eradicate streptococcal infection (may be given despite previous treatment).
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Usually benzathine penicillin is given I.M. in a single dose. Oral erythromycin may be used for children who are allergic to penicillin.

Oral salicylates (aspirin) or non-steroidal anti-inflammatory drugs (naproxen sodium) usually used to control pain and inflammation of arthritis. Aspirin is continued for 4 to 6 weeks for carditis. Corticosteroids used in severe cases to try to control cardiac inflammation. Phenobarbital, diazepam, or other neurologic agent to control chorea. Bed rest during the acute phase (until ESR decreases, C-reactive protein becomes negative, and pulse rate returns to normal) to rest the heart. Bed rest may need to be maintained for 2 to 4 months in cases of severe carditis. Mitral valve replacement may be necessary in some cases. Secondary prevention of recurrent ARF:
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Risk of recurrence greatest within first 5 years, with multiple episodes of ARF, and with rheumatic heart disease. Prophylactic antibiotic treatment may be lifelong. For those at low risk for recurrence, antibiotic prophylaxis may be continued for 5 years or longer. Antibiotic regimens may include the following:

Benzathine penicillin I.M. every 28 days. Penicillin V or erythromycin 250 mg twice per day. Sulfisoxazole (Pediazole) 0.5 to 1 g (dosage calculated according to patient's weight) once per day.

Complications

CHF. Pericarditis, pericardial effusion. Permanent damage to the aortic or mitral valve, possibly requiring valve replacement.

Nursing Assessment

Assess for signs of cardiac involvement by auscultation of the heart for murmur and cardiac monitoring for prolonged PR interval. Monitor pulse for 1 full minute to determine heart rate. Assess temperature for elevation. Observe for involuntary movements: stick out tongue or smile; garbled or hesitant speech when asked to recite numbers or the ABCs; hyperextension of the wrists and fingers when trying to extend arms. Assess child's ability to feed self, dress, and do other activities if chorea or arthritis present. Assess pain level using scale appropriate for child's age. Assess parents' ability to cope with illness and care for child. Assess need for home schooling while patient is on bed rest.

Nursing Diagnoses

Decreased Cardiac Output related to carditis

Acute and Chronic Pain related to arthritis Risk for Injury related to chorea

Nursing Interventions Improving Cardiac Output

Explain to the child and family the need for bed rest during the acute phase (approximately 2 weeks) and as long as CHF is present. In milder cases, light indoor activity is allowed. In severe cases, organize care so that the child will not have to exert self and will have hours of uninterrupted rest. Maintain cardiac monitoring if indicated. Administer course of antibiotics as directed. Be alert to adverse effects, such as nausea, vomiting, and GI distress. Administer medications for CHF as directed. Monitor BP, intake and output, and heart rate.

Relieving Pain

Administer anti-inflammatory antipyretics as directed.

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medication,

analgesics,

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Monitor for signs of aspirin toxicity, such as tinnitus, nausea and vomiting, and headache. Monitor for signs of corticosteroid use GI distress, acne, weight gain, emotional disturbances or long-term effects, such as rounded face, ulcer formation, and decreased resistance to infection.

Administer all anti-inflammatory medications with food to reduce GI injury. Be aware that anti-inflammatory may not alter the course of myocardial injury.

Teach family the importance of maintaining dosage schedule, continuing medication until all signs and symptoms of the ARF have gone, and tapering the dose as directed by health care provider. Assist child with positioning for comfort and protecting inflamed joints. Suggest diversional activities that do not require use of painful joints.

Protecting the Child with Chorea

Use padded side rails if chorea is severe. Assist with feeding and other fine-motor activities as needed. Assist with ambulation if weak. Avoid the use of straws and sharp utensils if chorea involves the face. Make sure that child consumes nutritious diet with recommended vitamins, protein, and calories. Be patient if speech is affected, and offer emotional support. Protect the child from stress.

Administer phenobarbital or other medication for chorea as directed. Observe for drowsiness.

Family Education and Health Maintenance

Teach the appropriate administration of all medications, including prophylactic antibiotic. Encourage all family and household members to be screened for streptococcus and receive the appropriate treatment. Instruct on additional prophylaxis for endocarditis with dental procedures and surgery as indicated. Encourage following activity restrictions, resuming activity gradually, and resting whenever tired. Encourage keeping appointments for follow-up evaluation by cardiologist and other health care providers. Advise the parents that child cannot return to school until health care provider assesses that all disease activity is gone. Parents may need to discuss with teachers how the child can catch up with schoolwork. Instruct on follow-up with usual health care provider for immunizations, well-child evaluations, hearing and vision screening, and other health maintenance needs. Provide general health education about early identification and treatment seeking for any possible streptococcal infection (fever, sore throat). Compliance with 10 to 14 days of antibiotics can greatly reduce the risk of ARF and other poststreptococcal sequelae.

Evaluation: Expected Outcomes

Heart rate and PR interval within normal range for age; no signs of CHF Compliant with anti-inflammatory therapy; reports pain as 1 to 2 on scale of 1 to 10 Feeds self, washes face and hands, and ambulates to bathroom without injury