Con temporary. An OldDisease, a. Pwinatal Problem. ]( ;n PRINCIPLES Q: PRACTICE. Pathogenesis. May/June 1992 J O G N N 209

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1 ]( ;n PRINCIPLES Q: PRACTICE JILL TILLMAN, RN, MSN An OldDisease, a Con temporary Pwinatal Problem he incidence of syphilis peaked during World War 11, with 600,000 new cases reported per year. Six thousand cases were reported in 1950, but by 1986,27,000 cases were reported. From 1986 to 1990, the number of reported cases of syphilis almost doubled, reaching 49,000 (Centers for Disease Control [CDC], 1991), with the highest rates in urban areas. Epidemiologists believe there are three unreported cases of syphilis for every one reported. In addition, an individual who has one sexually transmitted disease usually harbors at least two others (Spence, 1989; Krieger, 1984). Because the infections may have a potentiating effect, more harm occurs than if each sexually transmitted disease were present alone (Brunham, Holmes, & Eschenback, 1984). An estimated two to five infants are infected with congenital syphilis for every 100 women diagnosed with primary or secondary syphilis (CDC, 1988). In 1983, 158 cases of congenital syphilis were reported nationally. By 1986, the number had risen to 365 cases, indicating that 1 in 10,000 live-born infants in the United States were diagnosed with congenital syphilis (CDC, 1988). In 1991, the Centersfor Disease Control (CDC) established a new definition of congenital syphilis and new guidelines for surveillance of the disease (CDC, in press). These guidelines are consistent with treatment standards, provide criteria that can be satisfied soon after delivery, and offer a standardized approach to reporting. A study in Los Angeles showed a fivefold increase in reported cases when the new definition was applied (Cohen, Boyd, Pabhudas, & Mascola, 1990). In 1989, a total of 1,747 cases of congenital syphilis was reported to the CDC. Of these, 1,017 were from New York City, which had already adopted the new definition. Pathogenesis The incidence of syphilis in the United States has almost doubled in the past 5 years, with some cltles posting an Increase of more than 500% during this period. As a result, greater numbers of neonates infected with congenital syphilis are treated in nurseries across the country. The Incidence, pathogenesis, diagnosis, and treatment of syphilis are described, and the nursing Impllcatlons associated with caring for those at risk of contracting the dtsease are presented. Accepted: July 1991 Syphilis is a complex systemic illness caused by the thin, motile spirochete Treponerna pallidurn. Although the mechanism by which this bacterium penetrates mucocutaneous tissue is unknown, T. pallidurn is capable of invading all tissues and persists within the cells. Several antibodies that arise after the onset of clinical symptoms have been identified, but the disease persists even with increasing antibody titers. In addition, although some antibodies remain for the lifetime of the host, reinfection may occur (Mandell, Doublas, & Bennett, 1985). Syphilis can be acquired through sexual intercourse, the mode of transmission in the majority of May/June 1992 J O G N N 209

2 P R I N C I P L E S A N D P R A C T I C E An estimated two to Jive infants are infected with congenital syphilis for every 100 women diagnosed with primary or secondary syphilis. cases. Congenital syphilis can be acquired by the fetus via the placenta or during passage through the birth canal. Wet nurses often spread the disease to infants they attend through direct contact with active lesions. Blood transfusion has been identified as a method of acquiring syphilis; however, current testing requirements and the short survival period of T. pallidurn under the conditions of blood bank storage make this method of transmission rare. Syphilis also can be acquired by accidental direct inoculation, as in a needle prick. Thus, the finger is most often identified in rnedical personnel as the site of syphilis transmission (Mandell et al., 1985). Soon after T. pallidurn penetrates its host, it enters the bloodstream or lymphatic system and disseminates through the body. The organism divides every hours. When a concentration of approximately 10 million organisms per gram of tissue is reached, clinical lesions appear. The incubation period varies from patient to patient and is directly proportional to the amount of the inoculum (Mandell et al., 1985). stages of Syphilis Syphilis is categorized into four clinical stages: incubation, primary, secondary, and tertiary. The incubation period varies from 3 to 90 days, with a median duration of 3 weeks. At this stage, syphilis can be transmitted accidentally by blood donation. The fetus also can be infected at this time through transplacental infection (Mandell et al., 1985). The primary stage occurs when the primary lesion, or chancre, develops at the site of inoculation. The chancre is a painless ulcer with a raised, indurated border that is seen on the cervix in 25% of infected women and also can be seen on the vagina and the vulva. Extragenital lesions may be seen on the lips, tongue, tonsils, nipples, fingers, and anus (Siegel & Washington, 1987). The chancre is infectious and will probably cause transmission of the disease in 30-50% of exposed individuals. Multiple chancres may develop and heal spontaneously in 2 to 8 weeks. If chancres are not treated, a serologic response will develop in the host (Mandell et al., 1985). The secondary, or disseminated, stage of syphilis occurs 2-12 weeks after the original exposure. The systemic response is characterized by fever and malaise. At this time, the greatest number of treponemes are present in the body, particularly in the bloodstream. Skin manifestations also are present and include wartlike genital condylomata, mucous patches, and red-bronze macules on the palms of the hands or soles of the feet. A generalized lymphadenopathy and alopecia may occur, as may changes to organ systems. Circulating immune complexes can damage the kidney and cause nephrotic syndrome. Hepatitis with hepatomegaly, arthritis, cranial nerve palsies, and meningitis may occur. Relapses of secondary syphilis may occur up to 4 years after contact, with 75% of the relapses occurring within the first year (Mandell et al., 1985). In 1991, the Centers for Disease Control established a new dejinition of and new guidelines for surveillance of congenital syphilis. A study in Los Angeles showed a Jivefold increase in reported cases when the new dejnition was applied. When the secondary stage subsides, the patient enters a latent period. During this time, there are no clinical manifestations of the disease, and a diagnosis can be made only by obtaining a positive serologic test for syphilis. A pregnant woman can still infect her fetus in utero at this stage (Mandell et al., 1985). Approximately one third of untreated patients will develop a clinically apparent tertiary disease referred to as late syphilis. This is a slowly progressive inflammatory disease that can produce clinical illness in as few as 2 years but as many as 40 years after the initial infection. Late syphilis is divided into three basic categories: gummatous, cardiovascular, and neurosyphilis (Siegel & Washington, 1987). Gummatous syphilis is an inflammatory process that results in the formation of a necrotic mass called a gumma in soft tissue or viscera. Gummata appear to be a host reaction to small amounts of persistent treponemes producing reactive obliterative endarteritis (Mandell et al., 1985). Cardiovascular syphilis occurs after a latency period of years. It commonly presents as an aneurysm of the ascending aorta (Mandell et al., 1985). Neurosyphilis, a complex disorder with many forms, is divided into two phases, asymptomatic and symptomatic, and includes meningeal, meningovascular, parenchymal, and gummatous neurosyphilis (CDC, in press). 210 J O G N N Volume 21 Number 3

3 Syphilis Diagnosis Syphilis and congenital syphilis have been reported since biblical times, and the development of diagnostic testing devices has progressed over the past 70 years (Cohen et al., 1990). The first serologic test for syphilis was devised by Wasserman in In 1922, Kahn developed the first precipitating test for syphilis, using an alcoholic extract of beef heart. In 1940, Panglorn combined lecithin and cholesterol with cardiolipin (mitochondria1 membrane) and improved the sensitivity and specificity of the precipitating test. This improvement in the antigen led to the development of a slide microflocculation test by the Venereal Disease Research Laboratory (VDRL) of the United States Public Health Service. The VDRL test is simple and inexpensive and has been the mainstay for syphilis screening in many hospitals and municipal, state, and federal public health laboratories since the 1950s (Mandell et al., 1985). The accuracy of syphilis testing varies widely, depending on the test used and the stage in which diagnosis is attempted. To date, all available tests are problematic in some respect. Testing falls into three broad categories: darkfield examination and two serologic types, nonspecific nontreponemal reaginic antibody and specific antitreponemal antibody. The dark-field examination is the quickest and most direct method of diagnosis in primary, secondary, and early congenital syphilis. False-negative results can be as high as 35%, and the procedure may be unreliable in inexperienced hands. As a result, repeated dark-field testing, serologic testing, and follow-up are often necessary (Larsen, 1989). Nontreponemal tests measure antibodies directed against lipoidal antigen that results from the interaction of host tissues with T. pallidurn or from T. pallidurn itself. The tests that fall into this category include the VDRL test, rapid plasma reagin card test, and automated reagin test. Nontreponemal tests may be falsely negative, and positive nontreponemal tests should be confirmed by a specific treponemal test to exclude a false-positive test, which can be caused by a condition such as systemic lupus erythematous. A fourfold decrease in titer of the nontreponemal test after treatment indicates successful therapy (Mandell et al., 1985). Specific treponemal tests measure specific treponemal antibodies and include the microhemagglutination test, the hemagglutination treponemal test for syphilis, the bio-enzabead test, and the fluorescent treponemal antibody absorption test. The T. pallidurn immobilization test is used rarely today. Even with suc- The accuracy of syphilis testing varies widely, depending on the test used and the stage in which diagnosis is attempted. cessful therapy, positive fluorescent treponemal antibody absorption and microhemagglutination tests usually remain reactive for life. A condition such as systemic lupus erythematous also may give a positive or borderline positive fluorescent treponemal antibody absorption test result (Mandell et al., 1985). The nontreponemal reaginic tests usually are used for testing large numbers of sera. The specific treponema1 tests are used to confirm the diagnosis. Then, the quantitative nontreponemal antibody tests (rapid plasma reagin and VDRL) are used to assess the efficacry of treatment (Mandell et al., 1985). Congenital Syphilis Congenital syphilis is the result of maternal infection with spirochetemia during pregnancy. The risk of congenital syphilis has been estimated to be 50% in primary and secondary syphilis, 40% in early latent syphilis, and 10% in late syphilis (Gabbe, Niebyl, & Simpson, 1986). The disease causes fetal or perinatal death in 40% of the infants affected (Brunham et al., 1984) and may result in stillbirth, intrauterine growth retardation, nonimmune hydrops, and preterm labor. The congenital effects are irreversible. Early congenital syphilis (the infantile form of the disease) is diagnosed before 2 years of age. The earliest sign is usually rhinitis. Hepatosplenomegaly and mucocutaneous eruptions also may appear. The architecture of the skeletal system may be affected, and diffuse inflammatory changes may be present in any organ of the body. If the child is untreated and survives the first 6-12 months of life, he or she enters a latent period that resembles the disease in adults (Report of the Committee, 1988). Late congenital syphilis is diagnosed after 2 years of age. It is a multisystem disease that, like early congenital syphilis, can involve the central nervous system, bone, teeth, skin, and cartilage (Gabbe et al., 1986). The first transplacental transmission from an asymptomatic infected mother was described in 1906 (CDC, 1988). Serologic testing for syphilis in pregnant women first began in the 1950s (CDC, 1989). Congenital syphilis is preventable with antibiotic treatment of the mother during pregnancy. The CDC May/June 1992 J O G N N 211

4 P R I N C I P L E S A N D P R A C T I C E Congenital syphilis causes fetal or perinatal death in 40% of the infants affected and may result in stillbirth, intrauterine growth retardation, nonimmune hydrops, and preterm labor. The congenital effects are irreversible. recommends that no infant be released from the hospital until the serologic status of its mother is known. Treatment and Follow-up The treatment of syphilis involves counseling and drug therapy. Penicillin, the preferred drug, is the only drug that has been widely used for patients with neurosyphilis, congenital syphilis, or syphilis during pregnancy. Skin testing, with desensitization, if necessary, is optimal for patients who are allergic to penicillin (Cates & Holmes, 1986). Centers for Disease Control guidelines (CDC, 1989) are as follows: For early syphilis (less than 1 year s duration), administer 2.4 million units Benzathine penicillin G intramuscularly with clinical and serologic reexamination at 3 and 6 months. For late latent syphilis (more than 1 year s duration), gummatous syphilis, and cardiovascular syphilis, administer 7.2 million units Benzathine penicillin G intramuscularly, given as three doses of 2.4 million units, 1 week apart for 3 weeks consecutively, with quantitative nontreponemal serologic tests at 6 and 12 months. For neurosyphilis, administer million units aqueous crystalline penicillin G intravascularly, given as 2-4 million units every 4 hours for days, with cerebrospinal fluid examination every 6 months until the cell count is normal. For congenital syphilis, administer 100, ,000 units/kg aqueous crystalline penicillin G daily (50,000 units/kg intravascularly every 8-12 hours) or 50,000 units/kg of procaine penicillin daily (once intramuscularly), for days, with follow-up at 1, 2,3,6, and 12 months of age. Infants should be treated if maternal treatment was inadequate, was unknown, was given during the last 4 weeks of pregnancy, or was with drugs other than penicillin (Report of the Committee, 1988). Patients undergoing treatment for syphilis should be advised that a Jarisch-Herxheimer reaction, a flulike response more common in patients with early syphilis, may occur. Pregnant women should be warned that they may experience early labor. No proven methods exist for preventing this reaction, and it is not a contraindication to treatment. Nursing Implications Individuals of low socioeconomic status, adolescents, and young adults are at increased risk for sexually transmitted diseases (Gravett, 1984; Holmes, Bell, & Berger, 1984; Mosher & Aral, 1985; Stone, Grimes, & Magder, 1986). Control of syphilis is essential, particularly in the pregnant woman and the neonate. The most effective control is through early detection and treatment (Holmes et al., 1984; Mosher & Aral, 1985). National screening, prevention, and education efforts are aimed at the population at risk and healthcare providers (CDC, 1988). The CDC has established model clinic and training sites around the country to train health-care providers in the prevention, detection, and treatment of sexually transmitted diseases. The most effective control of syphilis is through early detection and treatment. Nurses must take a multifaceted approach to the problem. First, education programs should be developed and implemented for health-care providers and their clients. Second, nurses should become involved in assuring that facilities in their community provide adequate prenatal care, because the absence of early prenatal care and diagnosis is a leading factor in the rising incidence of congenital syphilis. Nurses need to advocate adherence to the 1988 recommendations of the United States Public Health Service that all women be screened serologically for syphilis early in pregnancy and at delivery with a nontreponemal (rapid plasma reagin or VDRL) test and that infants not be released from the hospital until the serologic status of the mother is known (CDC, 1988). Third, nurses should perform thorough assessments for their clients, including follow-up for newborns, in whom signs and symptoms of infection may not develop until several weeks after birth. Syphilis has been prevalent for thousands of years, and it appears that maintaining the status quo is all that can be expected today. The eventual health-care services those afflicted with syphilis need and the cost of that care are staggering. Conversely, many untreated syphilis patients will become disabled. Thus, for economic and societal reasons, we must take steps to detect and treat syphilis effectively. 212 J O G N N Volume 21 Number 3

5 Syphilis References Brunham, R., Holmes, K., & Eschenback, D. (1984). Sexually transmitted diseases in pregnancy. In K. Holmes, J. Sparling, & J. Wiesner (Eds.), Sexually transmitted diseases. New York: McGraw-Hill. Cates, W., Jr., & Holmes, K. (1986). Sexually transmitted diseases. In J. Last (Ed.), Public health andpreventive medicine (12th ed.). New York: Appleton-Century- Crofts. Centers for Disease Control. (1988, January 15). Policy guidelines for prevention and control of congenital syphilis. Morbidity and Mortality Weekly Report, 37, Centers for Disease Control. (1989, September 1) sexually transmitted diseases treatment guidelines. Morbidity and Mortality Weekly Report, 38, Centers for Disease Control. (1991, January 4). Morbidity and Mortality Weekly Report, 39, 952. Centers for Disease Control. (in press). Case definitions for public health surveillance. Morbidity and Mortality Weekly Report. Cohen, D., Boyd, D., Pabhudas, I., & Mascola, L. (1990). The effects of case definition, maternal screening, and reporting criteria on rates of congenital syphilis. American Journal of Public Health, 80, Gabbe, S., Niebyl, J., & Simpson, J. (Eds.). (1986). Obstetrics: Normal and problem pregnancies. New York: Churchill Livingstone. Gravett, M. (1984). Causes of preterm delivery. Seminars in Perinatology, 8(4), Holmes, K., Bell, T., & Berger, R. (1984). Epidemiology of sexually transmitted diseases. Urology Clinics of North America, 21(1), Krieger, J. (1984). Biology of sexually transmitted diseases. Urology Clinics of North America, 12(1), Larsen, S. (1989). Syphilis. Clinics in Laboratory Medicine, 3(3), Mandell, G., Doublas, R. G., & Bennett, J. E. (1985). Principles andpractice of infectious diseases (2nd ed.). New York: John Wiley & Sons. Mosher, W., &Aral, S. (1985). Factors related to infertility in the United States, Sexually Transmitted Diseases, 12(3), Report of the Committee on Infectious Diseases. (1988). Elk Grove Village, IL: American Academy of Pediatrics. Siegel, D., & Washington, A. (1987). Syphilis: Updated approach to an old disease. Postgraduate Medicine, 8(1), Spence, M. (1989). Epidemiology of sexually transmitted diseases. Obstetrics and Gynecology Clinics of North America, 16(3), Stone, K., Grimes, D., & Magder, L. (1986). Primary prevention of sexually transmitted diseases: A primer for clinicians. Journal of the American Medical Association, 225(13), Address for correspondence: Jill Tillman, RN, MSN, 432 Bolsover Rd., Wynnewood, PA Jill Tillman, RN, MSN, is the special projects coordinator for the Department of Nursing at Hahnemann University Hospital in Philadelphia. Ms. Tillman is a member of NAACOG. Notice to Copiers Authorization to photocopy items for internal or personal use, or the internal or personal use of specific clients, is granted by NAACOG, a division of the American College of Obstetricians and Gynecologists, for libraries and other users registered with the Copyright Clearance Center (CCC), provided that the base fee of $3 per copy is paid directly to CCC, 21 Congress St.. Salem, MA $3. May/June 1992 JOG" 213