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J R Soc Med. 2002 June; 95(6): 278–279.
PMCID: PMC1279907

Can patients with cystic fibrosis have a career in the health service?

Patients with cystic fibrosis (CF) grow up in contact with a wide variety of health professionals, so it is hardly surprising that some wish to have a career in the health service. In an attempt to answer the question posed by the title, this editorial looks at three issues—the infection risks to the CF healthcare worker; the infection risks to the CF healthcare worker's patients; and the challenges of a health service career for an individual who already has to cope with the considerable demands of a CF therapy regimen.

Infection risks to the CF healthcare worker

Patients and a healthcare environment pose a risk to all healthcare workers, but for workers with CF the risks may be greater. Box 1 lists the main organisms that are likely to be of concern. The fact is that cross-infection has become a nightmare for all patients with CF. At first the fear was just of colonization with Burkholderia cepacia1,2,3, an organism that exists in several different forms, each with its own pathogenicity. However, it is now known that patients with CF can all too easily acquire Pseudomonas aeruginosa from other patients4,5,6. For the patient not already colonized, a matter of minutes in the company of a patient who is colonized may be sufficient for cross-infection to occur, and the worry is even greater in the case of multi-antibiotic resistant strains of the organism. Staphylococcus aureus has always been a threat to patients with CF, and the advent of methicillin-resistant strains has intensified efforts to prevent cross-infection. A common-place strategy is to cohort patients, and many CF units now run separate ‘Pseudomonas’, ‘Non-pseudomonas’ and ‘Cepacia’ clinics. Although there is controversy about how far one should take measures to prevent cross-infection—objective data are scarce—many units make strenuous efforts to keep CF patients apart from each other: gone are the outings for patients with CF to Alton Towers, the day trips to Blackpool, the CF holiday camps. But keeping patients with CF apart at school can be extremely problematic, and keeping CF siblings apart is well-nigh impossible. Some units in North America have persuaded their patients to wear a mask whenever attending hospital; but, as with most other strategies in CF, there is no objective evidence in favour of this practice.

Box 1
Potential infection risks created by healthcare workers with cystic fibrosis (CF)

Against this background, a healthcare environment such as a hospital is a minefield for the healthcare worker with CF, who is likely to be at far greater risk of harm from cross-infection than a non-CF healthcare worker. Plainly some areas in the hospital or community environment are riskier than others. Probably the most hazardous environment of all is the CF unit—a miserable conclusion, because this is just the place where having a healthcare worker with CF could be of particular value. There is a dearth of published evidence in this whole area, and there are no data on the relative risks of, say, working in an intensive care unit, a burns unit, an infectious disease unit or a respiratory unit. Similarly there are no data on the value of measures to protect the CF healthcare worker, such as wearing masks, gowns or gloves.

Risks to patients from CF healthcare workers

The risk to patients will depend on the nature of the organisms colonizing the CF healthcare worker's respiratory tract. Box 1 lists some of the more likely pathogens. If the CF individual has no cough, produces no sputum, and has no pathogenic bacteria in the respiratory tract, then there is no increased risk to patients. Alas, few individuals with CF have a completely symptomless respiratory tract. The large majority pose a threat to a greater or lesser degree, and regrettably there is no objective way to measure the hazard they present. Some situations are self-evidently very high risk—for example, a CF patient expectorating S. aureus on a surgical ward, or a CF patient with P. aeruginosa on a burns unit—but in most circumstances any risk will be less obvious.

The challenges of a health service career

Many individuals with CF are highly motivated to work7,8. In one small study of 15 CF adolescents who were followed up, 10 were working in professional, semi-professional or clerical jobs, 3 were attending college and just 2 were unemployed because of CF9. Not surprisingly, the most powerful predictors of being able to work are low disease severity and high self-esteem9.

A patient with CF who is considering a health service career—or any other career for that matter—needs information on the duration and requirements of training, as well as the demands of the career. These factors must be considered against the need to maintain a therapeutic regimen which may include daily exercise, twice daily or more physiotherapy, various medications, and possibly admission to hospital. A patient with CF considering a health service career must also give thought to the long term, and what will happen if and when his or her health deteriorates.

Conclusions

This editorial paints a rather bleak and negative picture. The other side of the coin is that some people with CF have had immensely successful health service careers, and a diagnosis of CF should not be seen as a complete barrier to such a career. The individual with CF who is contemplating a career in the health service needs to consider the issues of risk, and to discuss these with both a CF physician and a specialist in occupational health. For the reader who wishes to explore the subject in greater depth, it is one of several topics covered in this month's supplement to the JRSM, based on the latest Section of Paediatrics symposium on CF10.

References

1. Holmes A, Nolan R, Taylor R, et al. An epidemic of Burkholderia cepacia transmitted between patients with and without cystic fibrosis. J Infect Dis 1999;179: 1197-205 [PMC free article] [PubMed]
2. Jones AM, Dodd ME, Webb AK. Burkholderia cepacia: current clinical issues, environmental controversies and ethical dilemmas. Eur Respir J 2001;17: 295-301 [PubMed]
3. Govan JR. Infection control in cystic fibrosis: methicillin-resistant Staphylococcus aureus, Pseudomonas aeruginosa and the Burkholderia cepacia complex. J R Soc Med 2000;93(suppl 38): 40-5 [PMC free article] [PubMed]
4. Ojeniyi B, Frederiksen B, Hoiby N. Pseudomonas aeruginosa cross-infection among patients with cystic fibrosis during a winter camp. Pediatr Pulmonol 2000;29: 177-81 [PubMed]
5. Jones AM, Govan JR, Doherty CJ, et al. Spread of multiresistant strain of Pseudomonas aeruginosa in an adult cystic fibrosis clinic. Lancet 2001;358: 557-8 [PubMed]
6. McCallum SJ, Corkill J, Gallagher M, Ledson MJ, Hart CA, Walshaw MJ. Superinfection with a transmissible strain of Pseudomonas aeruginosa in adults with cystic fibrosis chronically colonised by P. aeruginosa. Lancet 2001;358: 558-60 [PubMed]
7. Gillen M, Lallas D, Brown C, Yelin E, Blanc P. Work disability in adults with cystic fibrosis. Am J Respir Crit Care Med 1995;152: 153-6 [PubMed]
8. Goldberg RT, Isralsky M, Shwachman H. Vocational development and adjustment of adolescents with cystic fibrosis. Arch Phys Med Rehab 1979;60: 369-74 [PubMed]
9. Goldberg RT, Isralsky M, Shwachman H. Prediction of rehabilitation status of young adults with cystic fibrosis. Arch Phys Med Rehab 1985;66: 492-5 [PubMed]
10. Walters S. Health service careers for people with cystic fibrosis. J R Soc Med 2002;95(suppl 41): 41-51 [PMC free article] [PubMed]

Articles from Journal of the Royal Society of Medicine are provided here courtesy of Royal Society of Medicine Press