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Burkholderia Cepacia in Cystic Fibrosis: Implications for Nursing Practice.

Cystic fibrosis (CF) is an autosomal recessive disorder affecting approximately 30,000 Americans. In Caucasians, it is considered the most common lethal genetic disorder, with a carrier frequency of 1 in 25 and an incidence of 1 in 3,200 live births (Cystic Fibrosis Foundation Annual Patient Registry, 1998). CF is the result of mutations in the gene that codes for the cystic fibrosis transmembrane regulator (CFTR) protein. CFTR plays a major role in the transport of chloride, sodium, and water across the apical membrane of the epithelial cells (Walsh & Smith, 1995). The abnormal movement of sodium, chloride, and water results in abnormally thick airway secretions, causing decreased mucociliary clearance and chronic bronchial infections. The resultant, irreversible damage to the pulmonary tree leads to bronchiectasis and early death. A variety of other organ systems are affected, including the pancreas, sweat glands, hepatobiliary system, and reproductive organs (Walsh & Smith, 1995).

Burkholderia Cepacia (B. cepacia) is an important pathogen in cystic fibrosis, and yet little more is known about it today than when it was first identified as a clinically significant pathogen in CF by Isles and his colleagues in 1984. Although it usually affects only 5%-10% of people with CF (Govan, Hughes, & Vandamme, 1996), the consequences of colonization are enormous and epidemic spread is possible. Medically, it has been associated with a more rapid decline in pulmonary function and increased frequency of hospitalization (Muhdi et al., 1996). Lewin, Byard, and Davis (1990) reported an increase in mortality in the year following colonization with B. cepacia. They also reported a more severe decline in pulmonary function in the 3 years before and the 2 years after B. cepacia was isolated in a patient's sputum. The Cystic Fibrosis Foundation Annual Patient Registry reported in 1998 that the mean predicted forced expiratory volume in 1 second (FEV1) for patients colonized with B. cepacia was 54.8%, compared to 87.7% for patients with normal lung flora, and 64.5% for patients colonized with Pseudomonas Aeruginosa. These observations suggest that colonization with B. cepacia in individuals with CF is an independent prognostic factor (LiPuma, 1998). It is innately resistant to multiple antibiotics, leaving limited treatment options, except for symptomatic management. Eradication with antibiotic treatment is virtually impossible (Hoiby, 1995). In about 20% of colonized patients, "cepacia syndrome" manifests as fulminant septicemia, necrotizing pneumonia, and death (Isles et al., 1984).

Historical Perspective

In 1950, Burkholder identified the saprophyte, which causes onion rot. It was originally called Pseudomonas Cepacia (Govan et al., 1996). Cepia is Latin for onion. Prior to the 1980s, reports of infections in humans caused by B. cepacia were infrequent and limited, primarily, to hospitalized patients who were infected with contaminated solutions. In the early 1980s, a rising incidence of B. cepacia infections was noted. In 1984, Isles and associates published their seminal paper describing B. cepacia as an emerging pathogen in CF. Within 5 years, reports began to circulate of rapidly fatal outcomes in some patients infected with B. cepacia.

In the early 1990s, RNA group II pseudomonads were recognized as the new genus Burkholderia. Cepacia is the type species. Bacteria that cannot be consistently identified by phenotype should not be given a new species name; taxonomically, such groups should be referred to as Genomovar I, Genomovar II, etc. (Govan et al., 1996). There are at least five genomovars of B. cepacia; Genomovars I, III, and IV are referred to as the B. cepacia complex. Genomovar II was recently given the name B. Multivorans. Genomovar V has been called B. Vietnamiensis (Vandamme et al., 1997).

Environmental Versus Clinical Debate

In agricultural microbiology, environmental awareness and the development of increasingly pesticide-resistant pathogens have led to an interest in B. cepacia as a potential agent for biological control (such as a pesticide) and soil decontamination. It produces several antimicrobial substances that inhibit bacterial and fungal phytopathogens and suppress plant diseases. It also has the ability to degrade industrial waste and herbicides, including the main ingredient in Agent Orange (Govan et al., 1996). There is a great deal of concern about the relationship between environmental and human isolates of B. cepacia and the potential dangers of using B. cepacia for the purpose of soil and plant remediation. The natural environment of B. cepacia is not well defined. There is a common misconception that it is ubiquitous, and although it is found in soil, water, and vegetation, it is not frequently recovered. Butler, Dogherty, Hughes, Nelson, and Govan (1995) surveyed 55 samples of soil, water, and plants and found a yield of only 12 B. cepacia isolates. The potential hazard that environmental isolates present to the CF community is unclear. Environmental isolates do have pathogenic potential, as evidenced by the invasive B cepacia lesions called "swamp foot," which were acquired by military personnel during jungle training. Conversely, human isolates will readily macerate onion, where environmental isolates have typically been found. Most environmental isolates belong to Genomovar I, whereas isolates associated with acute clinical decline in humans typically are identified as belonging to Genomovar III. It has yet to be proven whether B. cepacia can adapt itself to either human or plant environments, or whether certain strains occur exclusively in one setting.

Transmission

There are many different strains of B. cepacia, with varying degrees of virulence and transmissibility. There is a tendency to link transmissibility and virulence, categorizing strains as either transmissible and virulent or nontransmissible and avirulent (Govan et al., 1996). This is contradicted by the fact that in epidemic outbreaks where patients are colonized with the same strain, some patients may be asymptomatic, while others succumb to fatal disease.

Transmission occurs in three ways: direct patient-to-patient contact, nosocomial spread, and autoinfection (Smith et al., 1993). Initial studies did not clearly document person-to-person transmission, and this resulted in a delay in the initiation of strict infection control practices. Early difficulties in consistently culturing and accurately identifying B. cepacia also contributed to slow recognition of the increase in the number of affected individuals. Subsequently, several reports have shown strong evidence in support of direct contact transmission (Govan et al., 1996; LiPuma, Dasen, Nielson, Stern, & Stull, 1990; Pegues et al., 1994; Smith et al., 1993). One of the most widely reported studies was of a transcontinental epidemic via the Edinburgh/Toronto strain (Sun et al., 1995). The exact mechanism of transmittal across the Atlantic Ocean is still not known.

Individuals who are at reasonable risk for infection are those who have skin contact with affected persons, use contaminated equipment, take improper care of their nebulizers, are school mates of affected persons, or are in the same room as a cepacia-positive patient undergoing chest physiotherapy. People at high risk for cross contamination through contact with affected individuals are those who use the same cutlery or drinking vessels or otherwise exchange saliva, kiss, cohabit, are a sibling of an affected individual, or share nebulizer tubing/sets with a B. cepacia-positive person. There is also the unknown risk associated with contact with other CF patients who may harbor the bacteria but have not yet had a positive culture for B. cepacia.

Nosocomial transmission has been well documented in the literature and occurs in a number of ways (Govan et al., 1996; LiPuma et al., 1990; Pegues et al., 1994; Smith et al., 1993; Whiteford et al., 1995). B. cepacia has been isolated from blood and respiratory secretions of individuals without CF who are mechanically ventilated in intensive care units (ICUs) (Hamill et al., 1995). Spread has occurred through the use of multidose medication vials (Hamill et al., 1995; Reboli et al., 1996), contaminated nebulizer equipment (Hutchinson et al., 1996), and affected containers of disinfectants and antiseptics (Oie & Kamiya, 1996). Although non-CF patients can become infected with B. cepacia, transmission occurs more easily in individuals with CF, and the morbidity associated with B. cepacia is more severe in individuals with cystic fibrosis.

Drabick and colleagues (1996) studied the survival of B. cepacia on environmental surfaces and found that the bacteria was able to survive for long periods of time in respiratory droplets. They also found that B. cepacia was able to survive longer suspended in CF sputum than in either non-CF sputum or saline, suggesting that there are factors in CF sputum that may contribute to its survival. Other investigators found that B. cepacia suspended in sputum was transmissible by shaking hands for up to 180 minutes and that hand washing in contaminated sinks led to positive hand cultures (Dorning et al., 1996). Ensor and colleagues (1996) found that 40% of air samples taken in B. cepacia-positive patients' rooms before, during, and after chest physiotherapy were positive for the organism, suggesting that transmission may also occur through airborne dissemination. Hutchinson and colleagues (1996) studied homeuse nebulizers and found that 69% of nebulizers were contaminated, with up to sixteen different organisms identified, one of which was B. cepacia. They also found that patients who followed recommended care guidelines for their nebulizers had minimal or no contamination.

Prevention

The evidence that most strongly supports person-to-person transmission is the dramatic decline in the number of new cases since the implementation of segregation policies (Muhdi et al., 1996; Pegues et al., 1994; Thomassen, Demko, Doershuk, Stern, & Klinger, 1985). Implementation of segregation policies have included having separate physical out-patient settings for individuals with B. cepacia and hospitalizing cepacia-positive patients on hospital units other than those that care for cepacia-negative patients. In addition, many institutions have implemented infection control standards, which include the use of masks for all CF patients when they are in the health care facility. There is a significant challenge in implementing nursing staffing patterns that allow nurses to care for only positive or negative patients, so they are not going back and forth between patients, creating an increased risk of transmission of B. cepacia between patients.

Segregation has not been universally accepted by patients and families or by caregivers, particularly in centers where there is a low incidence of B. cepacia colonization. There are also logistical issues for hospitals; there may not be enough private rooms available, or staff may not be adequately trained to care for B. cepacia-positive patients on units other than where CF patients are usually hospitalized. It is important to remember that segregation is not perfect. It cannot protect against unidentified carriers and poor infection control practices.

Because of the serious consequences of colonization (psychological distress, antimicrobial resistance, and the possibility of deterioration in lung function even resulting in death), efforts must be focused on prevention. Cohorting is a hallmark of prevention. It has been proven to be effective in decreasing the incidence of new cases (Smith et al., 1993). Additionally, education is critical. Efforts should be directed toward patients and their families and CF center staff, including those who care for patients when they are admitted to the hospital.

Staff need to be reminded of the importance of handwashing between patients and should be familiar with hospital policies on the use of multidose vials of medications, the proper sterilization of equipment between patients, and the correct storage and handling of disinfectants and antiseptics to prevent contamination. Staff members should discourage socialization between B. cepacia-positive and negative patients and be careful to limit potential contact between them in common areas, such as radiology, the pulmonary function testing lab, physical therapy, and the hospital cafeteria. Caregivers who will be having contact with both positive and negative patients should see the positive patients last, for example, if equipment is to be shared between B. cepacia-positive and negative patients; the equipment should be used with the negative patients first. All equipment that is shared by patients should be washed with disinfectant as per hospital protocol between use.

Parents and older patients need to know the importance of cleaning and drying nebulizers after each use. In multi-sibling families, sharing of inhalation therapy equipment must be discouraged. Finally, it is important for CF centers to send B. cepacia cultures to reference laboratories for ribotyping to monitor for the appearance of epidemic strains of B. cepacia.

Psychosocial Issues

There are several problems with segregation, not the least of which is the psychosocial implications for patients Many researchers have tried to identify the stressors associated with chronic illness and the coping mechanisms that people use. Woodgate (1998) looked at a group of adoles. cents with chronic diseases and evaluated their coping strategies. Most teenagers said having a peer affected with the same illness improved their sense of well-being. They felt that the only people who could truly understand their situation were those who had the same illness. Burk and colleagues (1998) reported that individuals with chronic illnesses felt that social contact with peers with the same illness was an important part of their social development, especially during middle childhood and adolescence. They said that social contact with individuals with the same disease gave them important skills for living with the disease. Patients reported a sense of belonging and that they no longer felt isolated when in contact with a peer group affected with the same illness. In addition, they were able to gain a broader perspective of their disease and anticipate future implications. These same patients reported a great sense of loss when summer camps were discontinued secondary to risk for infection.

Many CF centers have traditionally encouraged socialization among patients and families through support groups, educational offerings, summer camps, holiday parties, and informal get-togethers. It has required a great deal of re-education to help staff to understand the risks of continuing these practices. Segregation policies have been widely adopted by most CF centers. Affected individuals have stated that these policies make them feel like pariahs. At a time when patients with CF need more emotional support than ever to deal with declining health, more frequent hospitalizations, and grim prognoses, they are isolated from the CF community of which they have been a part all their lives.

For affected individuals and their families who are struggling with the difficulties of isolation from the CF community because of B. cepacia, the Internet is a valuable resource. Children can communicate via e-mail and "chat" online with other affected individuals. Thus, affected individuals can still have contact and develop peer support without physical contact that may put them at risk.

Conclusion

The nursing care of patients with B. cepacia is a very complex issue. Not only is it necessary for nurses to follow the infection control guidelines at their institution of practice, which will directly impact the physical safety of their patients, they must also care for their patients' psychological well-being. Nurses cannot completely protect their patients from exposure to B. cepacia, but they can instill knowledge in their patients and their families so that they have the information they need to make appropriate decisions regarding their health. Nurses are in a good position to advocate for their patients in the health care environment and to ensure that appropriate infection control practices are in place to care for both affected and unaffected individuals. Additionally, the nurse can also serve as a patient advocate by educating patients, other health care professionals, and society in general regarding environmental hazards to individuals with CF. Nurses need to be aware of and empathetic about the impact that cohorting patients with B. cepacia has on individuals colonized with this bacteria. Nurses may also need to help them find alternative ways of coping, not only with the issue of colonization with B. cepacia but with the diagnosis and management of CF as well.

References

Burk, S.O., Kauffman, E., Costello, E., Wiskin, N., & Harrison, M.B. (1998). Stressors in families with a child with a chronic condition: An analysis of qualitative studies and a framework. Canadian Journal of Nursing Research, 30(1), 71-95.

Butler, S.L., Dogherty, C.J., Hughes, J.E., Nelson, J.W., & Govan, J.R. (1995). Burkholderia cepacia and cystic fibrosis: do natural environments present a potential hazard? Journal of Clinical Microbiology, 33(4), 1001-1004.

Cystic Fibrosis Foundation Annual Patient Registry. (1998). Bethesda, MD: Author.

Doming, G., Jansen, S., Noll, H., Grupp, H., Frank, F., Botzenhart, K., Magdorf, K., & Wahn, U. (1996). Distribution and transmission of pseudomonas aeruginosa and burkholderia cepacia in a hospital ward. Pediatric Pulmonology, 21(2), 90-100.

Drabick, J.A., Gracely, E.J., Heidecker, G.J., & LiPuma, J.J. (1996). Survival of burkholderia cepacia on environmental surfaces. Journal of Hospital Infection, 32(4), 267-276.

Ensor, E., Humphreys, H., Peckham, D., Webster, C., & Knox, A.J. (1996). Is burkholderia (pseudomonas) cepacia disseminated from cystic fibrosis patients during physiotherapy? Journal of Hospital Infection, 32, 9-13.

Govan, J.R., Hughes, J.E., & Vandamme, R (1996). Burkholderia cepacia: Medical, toxonomic, and ecological issues. Journal of Medical Microbiology, 45(6), 395-407.

Hamill, R.J., Hustson, E.D., Georghiou, P.R., Wright, C.E., Koza, M.A., Cadle, R.M., Goepfert, P.A., Lewis, D.A., Zenon, G.J., & Clarridge, J.E. (1995). Infection associated outbreak of B. cepacia (formally R cepacia) respiratory tract colonization with nebulized albuterol therapy. Annuals of Internal Medicine, 122(10), 762-766.

Hoiby, N. (1995). Isolation and treatment of cystic fibrosis patients with lung infections caused by pseudomonas (burkholderia) cepacia and multiresistant pseudomonas aeruginosa. Netherlands Journal of Medicine, 46(6), 280-287.

Hutchinson, G.R., Parker, S., Pryor, J.A., Duncan-Skingle, F., Hoffman, P.N., Hodson, M.E., Kauffman, M.E., & Pitt, T.L. (1996). Home-use nebulizers: a potential primary source of burkholderia cepacia and other colistin-resistant gram-negative bacteria in patients with cystic fibrosis. Journal of Clinical Microbiology, 34(3), 584-587.

Isles, A., Maclusky, I., Corey, M., Gold, R., Prober, C., Flemming, P., & Levinson, H. (1984). Pseudomonas cepacia infection in cystic fibrosis an emerging problem. Journal of Pediatrics, 104(2), 206-210.

Lewin, L.O., Byard, P.J., & Davis, P.B. (1990). Effect of Pseudomonas cepacia colonization on survival and pulmonary function of cystic fibrosis patients. Journal of Clinical Epidemiology, 45, 125-131.

LiPuma, J.J. (1998). Burkholderia cepacia management issues and new insights. Clinics in Chest Medicine, 19(3), 473-486.

LiPuma, J.J., Dasen, S.E., Nielson, D.W., Stern, R.C., & Stull, T.L. (1990). Person-to-person transmission of Pseudomonas cepacia between patients with cystic fibrosis. The Lancet, 336, 1094-1095.

Muhdi, K., Edenborough, F.P., Gumery, L., O'Hicky, S., Smith, E.G., Smith, D.L., & Stableforth, D.E. (1996). Outcome for patients colonized with Burkholderia cepacia in a Birmingham adult cystic fibrosis clinic and the end of an epidemic. Thorax, 51, 374-375.

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Pegues, D.A., Schidlow, D.V., Tublan, O.C., Carson, L.A., Clark, N.C., & Jarvis, W.R. (1994). Possible nosocomial transmission of Pseudomonas cepacia in patients with cystic fibrosis. Archives of Pediatric and Adolescent Medicine, 148, 803-811.

Reboli, A.C., Koshinski, R., Arias, K., Marks-Austin, K., Stiertz, D., & Stull, T.L. (1996). An outbreak of burkholderia cepacia lower respiratory tract infection associated with contaminated albuterol nebulization solution. Infection Control and Hospital Epidemiology, 17(11), 741-743.

Smith, D.L., Gumery, L.B., Smith, E.G., Stableforth, D.E., Kaufmann, M.E., & Pitt, T.L. (1993). Epidemic of Pseudomonas cepacia in the adult cystic fibrosis unit: Evidence of person to person transmission. Journal of Clinical Microbiology, 31(11), 3017-3022.

Sun, L., Jiang, R.Z., Steinbach, S., Holmes, A., Campanelli, C., Forstner, J., Sajjan, U., Tan, Y., Riley, M., & Goldstein, R. (1995). The emergence of a highly transmissible lineage of cbl+ pseudonomas (burkholderia) cepacia causing CF center epidemics in North America and Britain. Nature Medicine, 1(17), 661-666.

Thomassen, M.J., Demko, C.A., Doershuk, C.E, Stern, R.C., & Klinger, J.D. (1985). Pseudomonas cepacia: Decrease in colonization in patients with cystic fibrosis. American Review of Respiratory Disease, 131, 669-671.

Vandamme, P., Holmes, B., Vancanneyt, M., Coenye, T., Hoste, B., Coopman, R., Revets, H., Lauwers, S., Gillis, M., Kersters, K., & Govan, J.R. (1997). Occurrence of multiple genomovars of burkholderia cepacia in cystic fibrosis patients and proposal of burkholderia multivorans sp. nov. International Journal of Systematic Bacteriology, 47(4), 1188-1200.

Walsh, M.J., & Smith, A.E. (1995, December). Cystic fibrosis. Scientific American, 52-59.

Whiteford, M.L., Wilkinson, J.D., McColl, J.H., Colon, F.M., Michie, J.R., Evans, T.J., & Paton, J.Y. (1995). Outcome of burkholderia (Pseudomonas) cepacia colonization in children with cystic fibrosis following a hospital outbreak. Thorax, 50(11), 1194-1198.

Woodgate, R.L. (1998). Adolescents' perspectives of chronic illness: "It's hard." Journal of Pediatric Nursing: Nursing Care of Children & Families, 13(4), 210-223.

This column shares new ideas, new policies, new understandings behind diseases and interventions, new resources, new issues, and new roles for the pediatric nurse. For more information, contact Janice Selekman, DNSc, RN; Section Editor; Pediatric Nursing; East Holly Avenue Box 56; Pitman, NJ 08071-0056; (856) 256-2300 or FAX (856) 256-2345.

Jean E. Henskens, MSN, CRNP, is a Cystic Fibrosis Nurse Practitioner, The Children's Hospital of Philadelphia, Philadelphia, PA.

Susan K. VonNessen, MSN, CRNP, is a Pediatric Nurse Practitioner and Clinical Instructor, University of Pennsylvania School of Nursing, Philadelphia, PA.
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Author:Henskens, Jean E.; VonNessen, Susan K.
Publication:Pediatric Nursing
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Date:May 1, 2000
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