Lyme Disease: . An Elusive Diagnosis
Jean Newsome Maran, MSN, CANP, RN, and Kathy A. Crispell, BSN, CANP, CCRN, RN
Lyme disease presents health care practitioners with a complex set of challenges. The history, physical assessment, and clinical evaluation must all be meticulously performed, as Lyme disease can prove to be an exceptionally elusive entity. This article details the pathogenesis, clinical manifestations, nursing history, and health care implications of Lyme disease. Education is emphasized as the key to both the detection and prevention of this rapidly increasing syndrome. j PEDIATR HEALTH CARE. (1989). 3, 60-66.
yme disease, the most common tick-borne illness in the United States today (Relman, Schoolnik, & Swartz, 1988), was initially recognized in 1975. In the past 13 years, the clinical recognition and reported incidence have increased steadily. Lyme disease is named for a Connecticut town, one of several rural communities in which a high incidence of juvenile rheumatoid arthritis (JRA) cases was noted (Bruhn, 1984; Relman et al., 1988). Concerned mothers brought this illness to medical attention when 39 of their children were diagnosed as having JRA, an incidence 100 times that expected (Steere et al., 1977). Today, there are three endemic areas in the United States for Lyme disease: (a) wooded locations in New York, Massachusetts, Connecticut, Rhode Island, and other coastal northeastern and Middle Atlantic states; (b) much of Wisconsin and Minnesota; and (c) coastal and wooded areas of California and southwestern Oregon (Relman et al., 1988). Although 90% of the reported cases have occurred in these areas (Lyme disease, 1988; Relman et al., 1988), it is important to note that as of mid-1988, cases have been reported in 34 states (Schwartz, 1988), more than double the number in 1980. Approximately 6000 cases have been recorded officially at the various state health agencies since
Jean Newsome Maran is currently an Assistant Professor of Nursing at Northeast Louisiana University School of Nursing, in Monroe, Louisiana. Kathy A. Crispell is currently a third-year medical student versity School of Medicine, New Orleans, Louisiana. Reprint requests: Jean Newsome Oaks Drive, Monroe, LA 71201.
at Tulane UniRN, 2606
1980. However, because reporting is not required, the exact incidence of the disease is unknown. Lyme disease also occurs in Europe, Canada, and Australia (Bruhn, 1984; Relman et al., 1988; Steere, et al., 1983). All ages are affected, with a slight male predominance (Bruhn, 1984). Lyme disease occurs primarily in the summer and early autumn, but may occur anytime from May through November (Bruhn, 1984; Lyme disease, 1988; Steere et al., 1983).
D ogs can contract implicated
Lyme disease, and it is in the arthritis of many dogs.
Lyme disease is generally not life-threatening, although a few deaths from complications have been reported. However, it can be a debilitating and painful disorder that can produce a lifetime of misery. If diagnosed and treated early, however, these sequelae may be avoided. Unfortunately, the disease often goes undetected, or is not considered in the differential diagnosis. Lyme disease is a bacterial illness. The causative organism, Bowelia burgdmferi, is a spirochete (a corkscrew-shaped bacterium) that is carried by ticks and, possibly, fleas (Goldings & Jericho, 1986). The bacterium is transmitted when the tick bites its host. The primary vectors are the hard ticks Ixodes dummini in the midwestern and northeastern regions of the JOURNAL
Lyme Disease: An Elusive Diagnosis
1 Stages and accompanying
U.S., and Ixodes pa$cur in the Pacific northwest (Bruhn, 1984; Goldings & Jericho, 1986; Relman et al., 1988; Steere et al., 1983). The ticks are very small-less than 2 mm long-when non-engorged, becoming about 4 mm long after feeding. They feed
ypical sites are the thighs, buttocks, and axilla.
heart. As a result, there are protean manifestations (Relman et al., 1988; Steere et al., 1983). The spirochete can cross the placental barrier and spread to organs of the fetus, with potentially devastating effects if not treated (Markowitz, Steere, Benach, Slade, & Broome, 1986; Schlesinger, Duray, Burke, Steere, & Stillman, 1985). The organism may survive for many years in the body. There are several clinical stages; exacerbations and remissions are typical (Steere et al., 1983). n
primarily on white-tailed deer and white-footed mice. Other hosts include a wide variety of wild and domesticated mammals, wild birds, and human beings (Bruhn, 1984; Geldings & Jericho, 1986; Lyme disease, 1988; Relman et al., 1988). Dogs can contract Lyme disease, and it is implicated in the arthritis of many dogs (Goldings & Jericho, 1986). The causative spirochete prefers high-protein tissues, such as the skin, joints, nervous system, and
Three stages characterize Lyme disease; the stages may be distinct, overlapping, or absent (Relman et al., 1988). Stale I is characterized by a pathognomanic sequence of dermatologic events in which isolated macular or papular lesions result from the offending tick bite. Typical sites are the thighs, buttocks, and axilla (Bruhn, 1984; Steere et al., 1983). About one week later (with a range of 3 to 32 days), a progressive expansion of the erythema surrounding
Maran and Crispell
TABLE 2 Differential
Complied from Bruhn (1984); et al. (1983).
of Lyme disease
the primary pattern is noted (Steere et al., 1983). This is known as erythema chronicum migrans (ECM) and occurs in about 31 percent of those patients who recall a tick bite (Goldings & Jericho, 1986; Steere et al., 1983). The diameter of the redness may range from 3 to 68 centimeters (Steere et al., 1983). During expansion of the erythema, the lesion itself usually has bright red, flat (or occasionally raised) peripheries, with incomplete clearing at the center (Steere et al., 1983). However, necrosis, induration of the vesicle, or marked erythema may appear at the center. Steere et al. (1983) reported that “if ECM were on the head, only a linear streak might be seen emerging from the hairline” (pp. 7778). Several other physical signs and symptoms typically accompany the dermatologic changes of Stage I (for a listing of these, refer to Table 1).
ultiple secondary lesions appear within a few days following the onset of ECM in about half of the affected patients.
Multiple secondary lesions appear within a few days following the onset of ECM in about half of the affected patients (Relman et al., 1988; Steere et al., 1983). These secondary lesions are typically not as large as the primary site and usually do not have indurated centers (Steere et al., 1983). The palms and soles are the only body surfaces not susceptible to the secondary presentations. When not
treated with antibiotics, the skin lesions disappear within 3 to 4 weeks (Bruhn, 1984). If left untreated, a few patients may have dermatologic recurrences for up to a year. With appropriate antibiotic therapy, the skin lesions resolve in a matter of days. Conjunctivitis or a malar rash are other forms of skin manifestations that may present in the early course (Goldings & Jericho, 1986; Steere et al., 1983). Stade II is identified by cardiac and neurologic manifestations (see Table 1). Cardiac symptoms present in 8 to 10 percent of patients with Lyme disease (Goldings &Jericho, 1986; Steere et al., 1980). Cardiac manifestations are significant but are typically short lived, often lasting less than 1 week (Bruhn, 1984; Goldings & Jericho, 1986). Atrioventricular (AV) block is the most commonly reported cardiac complication. First degree AV block, Wenckebach second degree AV block, and even complete AV block requiring temporary pacemaker insertion are not uncommon (Goldings &Jericho, 1986; Relman et al., 1988). Interestingly, valvular dysfunction has not yet been reported in conjunction with Lyme disease (Relman et al., 1988). Myocarditis is a possibility; cardiomegaly and pericarditis have been reported in a small percentage of patients studied (Goldings & Jericho, 1986).
he knee is the most frequently joint.
Neurologic complications occur in approximately 11 percent of the affected population (Goldings & Jericho, 1986). Complaints ranging from headache, stiff neck, and photophobia to more profound neurologic inflammation are reported (Relman et al., 1988). Neurologic complications occur most commonly during ECM or within 6 weeks of its disappearance (Relman et al., 1988). Goldings and Jericho (1986) reported a recurrent course of meningitis and encephalitis frequently accompanied by unilateral or bilateral Bell’s palsy. Other cranial nerves are unpredictable in their involvement, but cranial nerves III, V, and VIII appear most susceptible (Relman et al., 1988). Radiculopathies and peripheral neuropathies are common (Goldings & Jericho, 1986; Relman et al., 1988). StaBe II. with rheumatologic findings, presents in approximately half the population with Lyme disease. Intermittent episodes of musculoskeletal pain
Journal of Pediatric Health
with no accompanying swelling, followed by long, asymptomatic periods is the initial rule, followed by chronic involvement of one or more joints. The knee is the most fi-equently affected joint (see Table 1). Once a chronic pattern has been established, the involved joints appear markedly swollen. Popliteal cysts are common in chronic knee involvement;, Patients usually deny other complaints associated with the arthritis, and the overwhelming majority of serologic tests for rheumatoid disease are negative (Goldings & Jericho, 1986) (see Table 2 and the Box). n
fore, the diagnosis of Lyme disease can be very elusive, and should be included in the differential diagnosis of these other illnesses, especially if the patient is from (or has recently visited) one of the three endemic areas.
rythromycin and parenteral penicillin are demonstrating curative results in the later stages of Lyme disease.
Lyme disease is a great imitator, and its clinical presentation varies widely. Because of its three stages, an inexact progression from one stage to the next, and an often indistinct pattern, a vague and confusing picture frequently results. Some patients may present with signs and symptoms suggesting a nonspecific viral illness. Others may be quite ill, with arthritis, headaches, meningitis, facial paralysis, and depression. In still other patients, there may be a loss of muscle coordination, as in multiple sclerosis. There-
Lyme disease is primarily a clinical diagnosis. Relman et al. (1988, p. 9) state it is “suggested by the presence of the ECM lesion, and the subsequent development of neurologic, cardiac, or articular manifestations.” Other clues may be the geographic clustering of many cases and their seasonal occurrence. A history positive for tick bite, or the lack of such a history, can be misleading, because many people do not recall being bitten, and many tick bites do not
FIGURE 1 Dermatologic A, An early lesion (arrow)
Volume 3, Number L March-April 1989
manifestations of Lyme disease. A-G, Erythema chronicum migrans is seen 4 days after detection. 6, lo-day-old lesion (arrows) has gradually expanded to 10 cm; a bright red border is common. C, In a 17-day-old lesion, the distinct outer ring (arrows) is larger and there is much central clearing. D, The center of an 8-day-old lesion is intensely red, (area indicated by arrows), raised, and indurated. E, The center (left arrow) of this 7-day-old lesion (right arrow) has become vesicular and necrotic. F, A target lesion (black arrow; white arrows indicate the parameter of the entire lesion) is seen at 8 days. C, At 12 days, as this lesion expands, the center has turned blue (indicated by lower arrow) rather than clear. H, Four days after onset of erythema chronicum migrans, similar secondary lesions have appeared and several of their borders have merged. I, Same patient as in 1H; secondary lesions are seen on the legs (arrows). Reprinted with permission from Kathleen Case, for Steere et al. (1983). The Early Clinical Manifestations of Lyme disease. Anna/s of internal Medicine, 99, p. 77.
lead to disease (Bruhn, 1984; Steere et al., 1983). When the pathognomonic ECM lesion is not noted, or never appears, the diagnosis can be more difficult, and many other diseases should be considered in the differential diagnosis (Table 2). The differential diagnosis may vary, depending upon whether the primary manifestations are neurologic, cardiac, or artitular in nature. Bowelia bup!q%i can be cultured and has been isolated from the skin, blood, CSF, and synovia (Bruhn, 1984; Goldings & Jericho, 1986; Relman et al., 1988). Antibodies to the spirochete can be identified in almost all people with later stages of Lyme disease, using immunofluorescent antibody (IFA) testing or enzyme-linked immunosorbent assay (ELISA) testing. These antibody tests may not aid in diagnosing early Lyme disease (Relman et al., 1988).
iscussing with patients the need to take certain precautions to help prevent Lyme disease is essential.
Any child with a recurrent or chronic illness, or a severe acute illness as described earlier, should have the possibility of Lyme disease included in the differential diagnosis. Parents may wish to suggest this to the practitioner, regardless of geographic location.
pertinent personal information that would help the practitioner in recognizing Lyme disease (see Box); and (c) contribute to long-term planning and teaching in order to resolve and prevent Lyme disease. While all nursing histories should be comprehensive, the critical areas on which to focus when dealing with a possible Lyme disease patient are listed in the Box. n
Prevention is the goal of patient teaching. Discussing with parents the need to take certain precautions to help prevent Lyme disease is essential. Some helpful hints for persons whose lifestyle or environment make them candidates for Lyme disease include: 9 Always check thoroughly for ticks following a day in the woods, grass, or brush. Don’t forget to check the head, back and neck. Children should be inspected twice a day. This may be one of the best ways to prevent Lyme disease. n Insect repellant is not a guarantee against ticks, but it can help. n Make sure vour clothing is tucked in-shirts in pants, pants in boots. n Long sleeves reduce some of the surface area available for ticks. n Light-colored clothing makes it easier to see ticks (Lyme disease, 1988). If someone finds a tick attached to the skin, it is important to remove it promptly and properly. It may take several hours for the tick to transmit the disease, so removal of the ticks upon discovery is a first-line preventive measure. Children should not be allowed to remove ticks.
The drug of choice is tetracycline, which has thus far proved to be the most prompt and effective drug for combating Lyme disease in its early stages. Oral doses of 0.25 to 0.5 grams four times a day for 10 to 20 days appear not only to eradicate the early manifestations of the disease, but to prevent the later complications as well. Erythromycin and parenteral penicillin are demonstrating curative results in the later stages of Lyme disease. Patients who do not respond to penicillin can be given cefiriaxone, 2 grams a day for 14 days (Treatment of Lyme Disease, 1988). n
The goals of a nursing history should be to: (a) compile a record of comparative and contrasting signs and symptoms that would help in formulating an accurate differential diagnosis (Table 2); (b) elicit
ong sleeves reduce some of the surface area available for ticks.
The site should be disinfected before tick removal. The tick should not be removed with fingers; instead a pair of fine-tipped tweezers should be used (Lyme disease, 1988). The tweezers should grip the tick’s mouthparts as close to the skin as possible. The tick should then be pulled upward, firmly and steadily. Do not squeeze, crush, or puncture the body of the tick, as its fluids may contain bacteria (Lyme disease, 1988). Home remedies, such as nail polish, alcohol, matches, etc. are ineffective, and can provoke the tick
Maran and Crispell
to expel spirochetes into the skin (Schwartz, 1988). After removing the tick, disinfect the bite area again, and wash hands with soap and water. People who have had tick bites, especially in one of the endemic areas, can save the tick for identification. After its removal, the tick should be placed in a small container with a few drops of alcohol. The tick can then be taken to the practitioner for possible identification. When, then, should medical attention for a tick bite be sought? Certainly any person, adult or child, should seek medical attention if they have been bitten by a tick and a rash has developed at the bite site. Thus, it is important for parents to be aware of tick bites and rashes on their children. However, because Lyme disease can develop without a rash ever having been noted, the question of when to take an asymptomatic child to a practitioner is more difficult to answer. If the bite was received in one of the three endemic areas, medical attention should be sought
eople who have had tick bites, especially in one of the endemic areas, can save the tick for identification.
promptly. The practitioner may elect to administer antibiotics immediately, or may wait to see if signs and symptoms of Lyme disease develop. The nurse should remember, however, that awareness Es not panic. When teaching patients who have contracted or who are potential candidates for contracting Lyme disease, the practitioner must stress awareness of how the disease is acquired, along with preventative measures for self-protection. Emphasizing that the person’s lifestyle and hobbies need not necessarily be changed, and that the disease is easily and quickly curable if detected early, is crucial. Awareness need not give way to panic if the nurse offers information in a positive and constructive manner. Providing awareness of the mode of transmission of this disease, of its early signs and symptoms, and
of measures the persons themselves can take to prevent or help identify the disease is a nursing intervention that will have longlasting, positive results. 8 SUMMARY
Although Lyme disease is an uncommon illness, it is important for nurses to be aware of it. Its incidence is increasing and its geography is expanding. By educating parents and the public about this disease, knowledgeable nurses can play a role in its prevention and in early medical intervention. These actions may diminish the potentially devastating effects of Lyme disease. This is a relatively minor illness if treated early; however, serious sequelae may occur if it is unrecognized or left untreated. 4 REFERENCES
Bmhn, F. (1984). Lyme disease. American Children,
Goldings, E., &Jericho, J. (1986). Lyme disease. Clinia in Rheumatic Diceme,
Lyme disease: don’t panic. ( 1988). lJntve+v of Cal$imia, Berkeley WeUnix Lettev, 4(9), l-2. Markowitz, L., Steere, A., Benach, J., Slade, J., & Broome, C. (1986). Lyme disease during pregnancy. Journal ofthe American MeditulAmciahm,
Rehnan, D., School&, G., & Swarcz, M. (1988). Lyme disease. In E. Rubinstein (Ed.), [email protected]
American Medicine (pp. 611). New York: Scientific American, Inc. Schlesinger, P., Duray, P., Burke, B., Steere, A., & Stillman, T. (1985). Maternal-fetal transmission of the Lyme disease spirochete, Borrelia bur~~m’. Ann& of Internal Medicine, 103, 67-68. Schwartz, B. (1988). Personal communication. Centers for Disease Control. Atlanta, Georgia. Steere, A., Bartenhagen, N., Craft, J., Hutchinson, G., Newman, J., Rahn, D., Sigal, L., Speiler, I’., Stenn, K., &Malawista, S. (1983). The early clinical manifestations of Lyme disease.Annab of Internal
Steere, A., Batsford, W., Weinberg, M., Alexander, J., Berger, H., Wolfson, S., & Malawista, S. (1980). Lyme carditis: cardiac abnormalities of Lyme disease. Annals of Internal Medicine, 93, 8-15. Steere, A., Malawista, S., Snydman, D., Shope, R., Andirnan, W., Ross, M., & Steele, F. (1977). Lyme arthritis: an epidemic of oligoarticular arthritis in children and ‘adults in three Connecticut communities. A&r& and Rbeumuticm, 20, 7- 17. Treatment of Lyme Disease. (1988). The Medical Letter on Drugs and Tberapeutia, 30(769), 65-66.