PEDIATRICS Vol. 107 No. 6 June 2001, pp. 1419-1420

COMMENTARY:
Lung Transplantation and Cystic Fibrosis: The Psychosocial Toll

Lung and heart-lung transplantation have been available as treatment options for cystic fibrosis (CF) since the mid-1980s.^1-3 The last decade has seen progress in the understanding of complications such as rejection as well as improvements in surgical and anesthetic techniques that expand the number of CF patients for whom transplantation is an option. As clinical problems such as airway colonization with antibiotic-resistant organisms, sinus disease, and malnutrition yield to continuing research, the potential CF transplant population will increase further. Although it is clear that lung transplantation is of great benefit to some patients with CF, it is also clear that transplantation comes with psychological, social, medical, and financial costs that are different from the burden experienced by patients, families, and CF caregivers in the pretransplant period. Each of the changes from CF patient to CF patient awaiting transplant to CF patient with lung transplant necessitates major alterations in attitudes toward CF care by patients, families, and caregivers. These in turn may result in uncertainty in the areas of control of patient care and continuity. Indeed, our experience leads us to believe that for some CF patients the psychosocial price paid for transplantation coupled with the risks inherent in transplantation together outweigh the potential benefit of the procedure.

Patients and families often have the idea that transplantation is uniformly effective and carries a relatively low risk of significant complications, neither of which is the case.^3-5 In discussing lung transplantation, Trulock⁶ has stated, "Lung transplantation is a treatment, not a cure, and it is not a panacea." A common maxim among transplantation physicians is "Transplantation involves trading one disease for another." For CF, however, a more correct saying might be "Lung transplantation for CF involves trading part of one disease for an entire second disease." One trades the lung disease of CF for the entirety of potential complications inherent in lung transplantation, complications ranging from surgical to infectious to drug-related.⁷ Even after lung transplantation, the nonpulmonary manifestations of CF such as electrolyte abnormalities, pancreatic insufficiency, and risk of bowel obstruction will remain. The proximal, nontransplanted, respiratory tract including the sinuses and native trachea can harbor microorganisms that can in turn infect the transplanted lung.

In their eagerness to embrace a potential solution to the unrelenting progression of CF lung disease, families and patients may adopt unrealistic expectations of the procedure as they naively accept or ignore the risks of transplantation. If the posttransplant course is complicated, the consequences of this mind-set on the part of the family may include anger directed at CF and transplant physicians, guilt, or both. The difficulty of predicting outcome after transplantation coupled with an often unrealistic expectation of the procedure leads us to question the possibility of truly informed consent for lung transplantation in this population. This may be especially true for children with CF, who may comprehend little of the significance of the choices they and their parents make.

Because most CF patients are not listed for transplant at their own CF center, 2 separate health care teams are often involved in their care before transplantation: their own CF team and the transplant team. This can result in confusion and insecurity in patients and their families. In some instances, disagreements as to how to treat patients arise between the 2 health care teams. For example, severely ill CF patients are commonly treated with intravenous anti-pseudomonal antibiotics. Because the repeated use of such aggressive therapy has been associated with the emergence of antibiotic-resistant airway flora,⁸ which is a potential contraindication to lung transplantation,^9,10 the transplant team may intervene to minimize this form of therapy. Unless the reasons for this change in management are explained clearly to the patient and family, they may distrust the clinical skills of the transplant physicians. If the change is explained well to the family and patient without including the regular CF care team, the family's confidence in and their relationship with their long-term CF physician may be undermined.

Traditionally, the doctor-patient relationship between CF caregivers and CF patients has been a close one, with the shared (and somewhat paradoxical) goals being vigorous treatment to preserve lung function coupled, at the end of life, with a dignified death and the avoidance of heroic measures. The transplant and CF teams may have a different opinion about aspects of CF care designed to prolong life while awaiting transplantation. More worrisome, however, is the possibility that the transplant team will alter the physician-patient relationship in such a way as to interfere with the dying process. The use of mechanical ventilation in CF patients is generally avoided by most CF physicians, because of the difficulty of successfully extubating a patient.¹¹ However, transplant teams are now documenting successful transplantation of patients who have been mechanically ventilated.^12,13 This fact, coupled with the unpredictability of organ availability, may lead to uncertainty on the part of the dying patient and his family as to whether or not they should accept death. Patients may not be allowed the same dignity and comfort in dying because of the possibility that organs may become available; and patients, families, and CF physicians may avoid important aspects of terminal care for fear of losing status as an active candidate.⁴ This in turn will result in more suffering by patients, and, if a patient dies without appropriate terminal care, lasting guilt on the part of the family and CF caretakers.

A major potential source of friction between a patient and the CF physician or transplant team is adherence to the medical regimen. CF care is notoriously complex, and it is difficult for any patient to be completely compliant.^14,15 However, the direct consequence of occasional noncompliance (for example, not doing all prescribed daily chest physiotherapy sessions) will be minimal for the average CF patient. Most patients and their families learn this, and many CF physicians also know and acquiesce to this behavior.¹⁶ In fact, many CF physicians accept this behavior as a normal part of CF patients' assuming responsibility for their care.¹⁷

Poor compliance, including haphazard immunosuppressive use, can have devastating, rapid, and sometimes irreversible consequences, affecting the survival of the lung graft and the development of renal insufficiency or other drug-related side effects.^18,19 Although posttransplant compliance is often impossible to predict,^18,20 a history of noncompliance is at least a relative contraindication to transplantation at most US and UK pediatric lung transplant centers.^21,22 This concern about noncompliance in the posttransplant period may influence the pretransplant period and the interactions of patients with their regular CF physician as well as the transplant team. For example, the transplant team may not accept the (relatively) small lapses in pretransplant compliance that had previously been accepted by the CF team. Patients will turn to their CF physicians only to discover that many of them will defer to the plan of the transplant team, even in those areas that had been tacitly accepted before. As a result, the patients may come into conflict with the transplant team and feel abandoned by their own CF physician.

Even if not transplanted at his regular CF center, the CF patient should maintain a relationship with and receive appropriate CF care from his regular CF physician after transplantation.¹⁹ Most lung transplant programs follow their recipients closely, especially in the initial 6 months posttransplant. Frequent visits to the transplant center or CF center as well as a requirement for home spirometry and frequent blood tests may leave a CF patient feeling overwhelmed by the level of monitoring. Before transplant, a mild increase in cough or a slight fall in pulmonary function tests may have been minor inconveniences. Posttransplant, however, they can be harbingers of significant posttransplant rejection or infection. This need for constant vigilance and amplified concern about symptoms can lead to major stresses on the part of patient, the CF physician, and the transplant team. This can be especially wearing on recipients who had previously been accepting of CF as a chronic illness, which would be expected to have a slow progression and would allow for some laxity in compliance.

Patients with CF who receive lung transplants are usually quite ill in the pretransplant period. In some ways, their lives have been a lengthy acceptance of chronic illness and ultimate death. For many such patients, their illness and the debility it brings defines their lives and who they are. A successful lung transplant dramatically changes this status, and some patients make the transition to wellness with enthusiasm and verve. For others, however, relative good health is something to distrust and disbelieve; they may find it difficult if not impossible to relinquish the previous mainstays of their lives, such as oxygen therapy. Transplant teams are often frustrated by such patients, encouraging them to be well only to see them tenaciously cling to their symbols of illness. Even as it tries to wean such patients to a better lifestyle, the transplant team undermines this mission by reminding the patients that their medications must be taken with near military exactness and that regular pulmonary function tests, chest radiographs, and lung biopsies are necessary to assess the status of the graft. The transplant team thus ensures that the patient will have a continuous reminder that his newly achieved well-being could suddenly disappear. In addition, the CF team must still follow the recipient because the CF phenotype is present in the gastrointestinal tract, sinuses, and naive trachea. It is not surprising that there are some transplant patients who are not fully prepared to enter into a life of health. An appreciation for the patient's pretransplant functional ability, perceived limitations, and reliance on therapy will help facilitate improvement posttransplant. In addition, it may be useful to recruit family and friends to help such patients do those activities associated with their new status.

RECOMMENDATIONS

For CF patients and caregivers, the very idea of lung transplantation will challenge basic tenets of care and attitudes toward illness. In addition, it is imperative that CF physicians and transplant teams recognize that the mere suggestion of transplantation as a treatment option is itself often stressful to a patient and family. The CF physician should be familiar with the risks of lung transplantation and with the processes involved in preparing for the procedure.⁵ CF care providers should communicate with physicians at the transplant center before patient evaluation for transplantation. At that time, a discussion should center on potential contraindications to transplantation (including patient compliance), patient and physician attitudes to transplantation, and reasonable expectations of transplantation.

If it is agreed that a patient is a candidate for additional evaluation, the CF physician should have a discussion with the patient, outlining the reasons for consideration for lung transplantation. It should be stressed that an evaluation by the transplant team is required before a decision concerning transplantation is possible. During the evaluation for transplantation, the transplant team must clearly state their goals and expectations for compliance. The risks of transplantation should also be clarified and discussed with the patient. The transplant team is obligated to state clearly their goals and expectations of patient behavior. In addition, the transplant team must present a realistic picture of the posttransplant course, underscoring the need for close monitoring. At the same time, it is not unreasonable to encourage the patient to look forward to an improved posttransplant quality of life.

By clearly stating their goals, by setting reasonable expectations of patient behavior, and by explaining the limitations of transplantation in addition to its hope, the transplant team, working with the referring CF physician, the patient, and family, can promote the changes in attitude and behavior that will facilitate the best possible result of the procedure.

Geoffrey Kurland, MD^*,
David M. Orenstein, MD^*,
* The Antonio J. and Janet Palumbo Cystic Fibrosis Center
and
 The Pediatric Cardiothoracic Transplant Program
Children's Hospital of Pittsburgh
Pittsburgh, PA 15213

FOOTNOTES

Received for publication May 26, 2000; accepted Sep 19, 2000.

Address correspondence to Geoffrey Kurland, MD, Division of Pediatric Pulmonology, Children's Hospital of Pittsburgh, 3705 Fifth Ave, Pittsburgh PA 15213. E-mail: kurlang{at}chplink.chp.edu

ABBREVIATIONS

CF, cystic fibrosis.

REFERENCES

1. Armitage JM, Fricker FJ, Kurland G, et al. Pediatric lung transplantation. The years 1985 to 1992 and the clinical trial of FK 506. J Thorac Cardiovasc Surg. 1993:105:337-345; discussion 346
2. Cohen RG, Barr ML, Starnes VA Pediatric lung transplantation. Semin Pediatr Surg 1993; 2:279-288 [Medline]
3. Noyes BE, Kurland G, Orenstein DM, Fricker FJ, Armitage JM Experience with pediatric lung transplantation [see comments]. J Pediatr 1994; 124:261-268 [Medline]
4. Warner JO Heart-lung transplantation: all the facts. Arch Dis Child 1991; 66:1013-1016 [Abstract/Free Full Text]
5. Shapiro BJ, Veeraraghavan S, Barbers RG Lung transplantation for cystic fibrosis: an update and practical considerations for referring candidates. Curr Opin Pulm Med 1999; 5:365-370 [CrossRef][Medline]
6. Trulock EP Lung transplantation: state of the art. Am J Respir Crit Care Med 1997; 155:789-818 [Medline]
7. Noyes BE, Kurland G, Orenstein DM State of the art: pediatric lung and heart-lung transplantation. Pediatr Pulmonol 1997; 23:39-48 [CrossRef][Medline]
8. Mouton JW, den Hollander JG, Horrevorts AM Emergence of antibiotic resistance amongst Pseudomonas aeruginosa isolates from patients with cystic fibrosis. J Antimicrob Chemother 1993; 31:919-926 [Abstract/Free Full Text]
9. Snell GI, de Hoyos A, Krajden M, Winton T, Maurer JR Pseudomonas cepacia in lung transplant recipients with cystic fibrosis [see comments]. Chest 1993; 103:466-471 [Abstract/Free Full Text]
10. Noyes BE, Michaels MG, Kurland G, Armitage JM, Orenstein DM Pseudomonas cepacia empyema necessitatis after lung transplantation in two patients with cystic fibrosis. Chest 1994; 105:1888-1891 [Abstract/Free Full Text]
11. Davis PB, di Sant'Agnese PA Assisted ventilation for patients with cystic fibrosis. JAMA 1978; 239:1851 [Abstract/Free Full Text]
12. Egan TM, Westerman JH, Lambert CJ Jr, et al. Isolated lung transplantation for end-stage lung disease: a viable therapy. Ann Thorac Surg. 1992;53:590-5; discussion 595-596
13. Massard G, Shennib H, Metras D, et al. Double-lung transplantation in mechanically ventilated patients with cystic fibrosis. Ann Thorac Surg. 1993;55:1087-1091; discussion 1091-1092
14. Eigen H, Clark NM, Wolle JM NHLBI Workshop Summary. Clinical-behavioral aspects of cystic fibrosis: directions for future research. Am Rev Respir Dis 1987; 136:1509-1513 [Medline]
15. Orenstein DM, Wachnowsky DM Behavioral aspects of cystic fibrosis. Ann Behav Med 1985; 7:17-20
16. Lask B Non-adherence to treatment in cystic fibrosis. J R Soc Med 1994; 87:25-27 [Abstract]
17. Conway SP, Pond MN, Hamnett T, Watson A Compliance with treatment in adult patients with cystic fibrosis. Thorax 1996; 51:29-33 [Abstract/Free Full Text]
18. Schweizer RT, Rovelli M, Palmeri D, Vossler E, Hull D, Bartus S Noncompliance in organ transplant recipients. Transplantation 1990; 49:374-377 [Medline]
19. Yankaskas JR, Mallory GB Jr Lung transplantation in cystic fibrosis: consensus conference statement. Chest 1998; 113:217-226 [Abstract/Free Full Text]
20. Howard L, Fahy T, Wong P, Sherman D, Gane E, Williams R Psychiatric outcome in alcoholic liver transplant patients. Q J Med 1994; 87:731-736 [Abstract/Free Full Text]
21. Kurland G Pediatric lung transplantation: indications and contraindications. Semin Thorac Cardiovasc Surg 1996; 8:277-285 [Medline]
22. Madden BP, Geddes DM Which patients should receive lung transplants? Monaldi Arch Chest Dis 1993; 48:346-352 [Medline]