Download PDF
Semin Respir Crit Care Med 2001; 22(5): 499-508
DOI: 10.1055/s-2001-18422
Copyright © 2001 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Cystic Fibrosis and Bronchiectasis: Selection, Timing, and Outcome

Janet R. Maurer
  • LifeSource, CIGNA Healthcare, Bloomfield, Connecticut
Further Information

Publication History

Publication Date:
14 November 2001 (online)

    Permissions and Reprints

ABSTRACT

Of the nearly 30,000 Cystic Fibrosis (CF) patients alive in the United States, more than 300 die each year. Of CF deaths at least 75% are from respiratory failure due to bronchiectasis. CF patients make up about 40% of the patients undergoing bilateral transplant. Because of the shortage of donor organs relative to the demand for them, it is important to choose candidates who are in the ``transplant window.'' International criteria have been developed to assist in proper selection of potential candidates, but new data suggest that some refinement of current criteria may improve this process. Appropriate prognostic models, however, are not yet completely validated. Bronchiectasis patients are usually evaluated according to the same protocols as CF patients because no large studies exist that would allow us to derive separate prognostic indicators.

Outcomes for CF patients are comparable to those for other diagnoses, except in the case of living donor recipients, which are slightly lower. CF patients may face particular issues following transplant-like infections and some immunosuppression complications possibly related to their underlying disease process. In addition, CF patients seem to be at particular risk of posttransplant lymphoproliferative disorder.

KEYWORD

Cystic fibrosis - lung transplantation - bronchiectasis - end-stage disease

REFERENCES

  • 1 Liou T G, Adler F R, FitzSimmons S C, Cahill B C, Hibbs J R, Marshall B C. Predictive 5-year survivorship model of Cystic Fibrosis.  Am J Epidemiol . 2001;  153(4) 345-352
    CrossrefPubMedGoogle Scholar
  • 2 Cystic Fibrosis Foundation Patient Registry Annual Data Report. 1998.  Bethesda, MD: Cystic Fibrosis Foundation 1999
    Google Scholar
  • 3 Collins F S. Cystic Fibrosis: Molecular Biology and therapeutic implications.  Science . 1992;  256 774-779
    PubMedGoogle Scholar
  • 4 Yankaskas J, Mallory G. Lung Transplantation in Cystic Fibrosis Consensus Conference Statement.  Chest . 1998;  113 217-226
    CrossrefPubMedGoogle Scholar
  • 5 Fiel S, FitzSimmons S, Suhidlow D. Evolving Demographics of cystic fibrosis.  Semin Respir Crit Care Med . 1994;  15 349-355
    PubMedGoogle Scholar
  • 6 Maurer J, Frost A, Estenne M, Higenbottam T, Glanville A. International Guidelines for the Selection of Lung Transplant Candidates.  J Heart Lung Transplant . 1998;  17(7) 703-709
    PubMedGoogle Scholar
  • 7 Anthony H, Paxton S, Catto-Smith A, Phelan P. Physiological and phsychosocial contributors to malnutrition in children with cystic fibrosis.  Clin Nutr . 1999;  18(6) 327-335
    PubMedGoogle Scholar
  • 8 Borowitz D. The interrelationship of nutrition and pulmonary function in patients with cystic fibrosis.  Curr Opin Pulm Med . 1996;  2(6) 457-61
    PubMedGoogle Scholar
  • 9 Pencharz P B, Durie P R. Pathogenesis of malnutrition in cystic fibrosis, and its treatment.  Clin Nutri . 2000;  19(6) 387-394
    PubMedGoogle Scholar
  • 10 Wilson D C, Pencharz P B. Nutrition and cystic fibrosis.  Nutrition . 1998;  14(10) 792-795
    CrossrefPubMedGoogle Scholar
  • 11 Collins C E, O'Loughlin E V, Henry R. Discrepencies between males and females with cystic fibrosis in dietary intake and pancreatic enzyme use.  J Pediatr Gastroenterol Nutr . 1998;  26(3) 258-262
    PubMedGoogle Scholar
  • 12 Vaisman N, Pencharz P B, Corey M. Energy expenditure of patients with cystic fibrosis.  J Pediatr . 1987;  111 496-500
    CrossrefPubMedGoogle Scholar
  • 13 Kerem E, Reisman J, Corey M, Canny G, Levison H. Prediction of mortality in patients with cystic fibrosis.  N Engl J Med . 1992;  326(18) 1187-1191
    CrossrefPubMedGoogle Scholar
  • 14 Smyth R, Walters S. Oral calorie supplements for cystic fibrosis.  Cochrane Database Syst Rev . 2000;  (2) CD000406
    PubMedGoogle Scholar
  • 15 Snell G I, Bennetts K, Bartolo J, Levvey Bronwyn, Griffiths A, Williams T, Rabinov M. Body mass index as a predictor of survival in adults with cystic fibrosis referred for lung transplantation.  J Heart Lung Transpl . 1998;  17(11) 1097-1103
    PubMedGoogle Scholar
  • 16 Sharples L, Hathaway T, Dennis C, Caine N, Higenbottam T, Wallwork J. Prognosis of patients with cystic fibrosis awaiting heart and lung transplantation.  J Heart Lung Transplant . 1993;  12(4) 671-674
    PubMedGoogle Scholar
  • 17 Shapiro B, Veeraraghavan S, Barbers R. Lung transplantation for cystic fibrosis: an update and practical considerations for referring candidates.  Curr Opin Pulm Med . 1999;  5 365-370
    CrossrefPubMedGoogle Scholar
  • 18 Henderson R C, Madsen C D. Bone mineral content and body composition in children and young adults with cystic fibrosis.  Pediatr Pulmonol . 1999;  27(2) 80-84
    CrossrefPubMedGoogle Scholar
  • 19 Aris R M, Renner J B, Winders A D, Buell H E, Riggs D B, Lester G E, Ontjes D A. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis.  Ann Intern Med . 1998;  128(3) 186-193
    CrossrefPubMedGoogle Scholar
  • 20 Shane E, Papadopoulos A, Staron R B. Bone loss and fracture after lung transplantation.  Transplantation . 1999;  68(2) 220-227
    PubMedGoogle Scholar
  • 21 Spima A, Gutieruez C, Chaparro C. Osteoporosis and lung transplantation: a prospective study.  Chest . 2000;  117 476-481
    CrossrefPubMedGoogle Scholar
  • 22 Aringer M, Kiener H P, Koeller M D. High turnover bone disease following lung transplantation.  Bone . 1998;  23(5) 485-488
    CrossrefPubMedGoogle Scholar
  • 23 Sokol R J, Durie P R, for the Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. Recommendations for management of liver and biliary tract disease in cystic fibrosis.  J Pediatr Gastroenterol Nutr . 1999;  28(1) S1-S13
    PubMedGoogle Scholar
  • 24 Kotloff R M, Zuckerman J B. Lung transplantation for cystic fibrosis: special considerations.  Chest . 1996;  109(3) 787-798
    PubMedGoogle Scholar
  • 25 Yankaskas J R, Aris R. Outpatient care of the cystic fibrosis patient after lung transplantation.  Curr Opin Pulm Med . 2000;  6 551-557
    PubMedGoogle Scholar
  • 26 Halvorson D J, Dupree J R, Porubsky E S. Management of chronic sinusitis in the adult cystic fibrosis patient.  Ann Otol Rhinol Laryngol . 1998;  107(11 pt 1) 946-952
    PubMedGoogle Scholar
  • 27 Aris R M, Gilligan P H, Neuringer I P. The effects of panresistant bacteria in cystic fibrosis patients on lung transplant outcome.  Am J Resp Crit Care Med . 1997;  155 1699-1704
    PubMedGoogle Scholar
  • 28 Chaparro C, Maurer J, Gutierrez C. Infection with Burkholderia cepacia in cystic fibrosis: outcome following lung transplantation.  Am J Respir Crit Care Med . 2001;  163 43-48
    CrossrefPubMedGoogle Scholar
  • 29 Ryan P, Stableforth D. Referral for lung transplantation: experience of a Birmingham Adult Cystic Fibrosis Centre between 1987 and 1994.  Thorax . 1996;  302-305
    PubMedGoogle Scholar
  • 30 Egan T M, Detterbeck F C, Mill M R. Lung transplantation for cystic fibrosis: effective and durable therapy in a high risk group.  Ann Thorac Surg . 1998;  66 337-346
    CrossrefPubMedGoogle Scholar
  • 31 Nunley D R, Ohori P, Grgurich W F. Pulmonary aspergillosis in cystic fibrosis lung transplant recipients.  Chest . 1998;  114(5) 1321-1329
    CrossrefPubMedGoogle Scholar
  • 32 Cahill B C, Hibbs J R, Savik K. Aspergillus airway colonization and invasive disease after lung transplantation.  Chest . 1997;  112(5) 1160-1164
    CrossrefPubMedGoogle Scholar
  • 33 Calvo V, Borro J M, Morales P. Antifungal prophylaxis during the early postoperative period of lung transplantation. Valencia Lung Transplant Group.  Chest . 1999;  115(5) 1301-1304
    CrossrefPubMedGoogle Scholar
  • 34 Westney G E, Kesten S, De Hoyos A, Chapparro C, Winton T, Maurer J R. Aspergillus infection in single and double lung transplant recipients.  Transplantation . 1996;  61(6) 915-919
    CrossrefPubMedGoogle Scholar
  • 35 Meyers B F, Lynch J P, Battafarano R J. Lung transplantation is warranted for stable, ventilator-dependent recipients.  Ann Thorac Surg . 2000;  70(5) 1675-1678
    CrossrefPubMedGoogle Scholar
  • 36 Hosenpud J D, Bennett L E, Keck B M, Boucek M M, Novick R J. The Registry of the International Society for Heart and Lung Transplantation: seventeenth official report-2000.  J Heart Lung Transplant . 2000;  19 909-931
    CrossrefPubMedGoogle Scholar
  • 37 Massard G, Shennib H, Metras D. Double-lung transpantation in mechanically ventilated patients with cystic fibrosis.  Ann Thorac Surg . 1993;  55(5) 1087-1091
    CrossrefPubMedGoogle Scholar
  • 38 Dodd M E, Webb A K. Understanding noncompliance with treatment in adults with cystic fibrosis.  J R Soc Med . 2000;  93(suppl 38) 2-8
    PubMedGoogle Scholar
  • 39 Corey M, Edwards L, Levison H, Knowles M. Longitudinal analysis of pulmonary function decline in patients with cystic fibrosis.  J Pediatr . 1997;  131(6) 809-814
    CrossrefPubMedGoogle Scholar
  • 40 Frangolias D D, Mahenthiralingam E, Rae S. Burkholderia cepacia in cystic fibrosis: variable disease course.  Amer J Respir Crit Care Med . 1999;  160 1572-1577
    PubMedGoogle Scholar
  • 41 Rosenfeld M, Davis R, FitzSimmons S, Pene M, Ramsey B. Gender gap in cystic fibrosis mortality.  Am J Epidemiol . 1997;  145(9) 794-803
    PubMedGoogle Scholar
  • 42 Doershuk C, Stern R. Timing of referral for lung transplantation for cystic fibrosis.  Chest . 1999;  115 782-787
    CrossrefPubMedGoogle Scholar
  • 43 Milla C, Warwick W. Risk of death in cystic fibrosis patients with severely compromised lung function.  Chest . 1998;  113 1230-1234
    CrossrefPubMedGoogle Scholar
  • 44 Robinson W, Waltz D A. FEV(1) as a guide to lung transplant referral in young patients with cystic fibrosis.  Pediatr Pulmonol . 2000;  30(3) 198-202
    CrossrefPubMedGoogle Scholar
  • 45 Kadikar A, Maurer J, Kesten S. The six-minute walk test: a guide to assessment for lung transplantation.  J Heart Lung Transplant . 1997;  16(3) 313-319
    PubMedGoogle Scholar
  • 46 Moorcroft A J, Dodd M E, Webb A K. Exercise testing and prognosis in adult cystic fibrosis.  Thorax . 1997;  52(3) 291-293
    PubMedGoogle Scholar
  • 47 Fraser K L, Tullis D E, Sasson Z, Hyland R H, Thornley K S, Hanley P J. Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia.  Chest . 1999;  115(5) 1321-1328
    CrossrefPubMedGoogle Scholar
  • 48 Venuta F, Rendina E A, Rocca G D. Pulmonary hemodynamics contribute to indicate priority for lung transplantation in patients with cystic fibrosis.  J Thorac Cardiovasc Surg . 2000;  119(4 pt 1) 682-689
    CrossrefPubMedGoogle Scholar
  • 49 Vizza C D, Yusen R D, Lynch J P, Fedele F, Alexander Patterson G, Trulock E P. Outcome of patients with cystic fibrosis awaiting lung transplantation.  Am J Respir Crit Care Med . 2000;  162(3 Pt 1) 819-825
    CrossrefPubMedGoogle Scholar
  • 50 Fogarty A, Hubbard R, Britton J. International comparison of median age at death from cystic fibrosis.  Chest . 2000;  117 1656-1660
    CrossrefPubMedGoogle Scholar
  • 51 Ryan G, Mukhopadhyay S, Singh M. Nebulized anti-pseudomonal antibiotics for cystic fibrosis.  Cochrane Database Syst Rev . 2000;  (2) CD001021
    PubMedGoogle Scholar
  • 52 Dezataux C, Crighton A. Oral non-steroidal anti-inflammatory drug therapy for cystic fibrosis.  Cochrane Database Syst Rev . 2000;  (2) CD001505
    PubMedGoogle Scholar
  • 53 Curtis J R, Burke W, Kossner A W. Absence of health insurance is associated with decreased life expectancy in patients with cystic fibrosis.  Amer J Resp Crit Care Med . 1997;  155 1921-1924
    PubMedGoogle Scholar
  • 54 Matouk E, Ghezzo R H, Gruber J, Hidvegi R, Gray-Donald K. Internal consistency reliability and predictive validity of a modified N. Huang clinical scoring system in adult cystic fibrosis patients.  Eur Respir J . 1997;  10(9) 2004-2013
    CrossrefPubMedGoogle Scholar
  • 55 Hayllar K M, Williams S G, Wise A E, Pouria S, Lombard M, Hodson M E, Westaby D. A prognostic model for prediction of survival in cystic fibrosis.  Thorax . 1997;  52(4) 313-317
    CrossrefPubMedGoogle Scholar
  • 56 Liou T G, Adler F R, FitzSimmons S C. Predictive 5-year survivorship model of cystic fibrosis.  Am J Epidemiology . 2001;  153 345-352
    CrossrefPubMedGoogle Scholar
  • 57 Barr M L, Schenkel F A, Cohen R G. Recipient and donor outcome in living related and unrelated lobar transplantation.  Transplant Proc . 1998;  30(5) 2261-2263
    CrossrefPubMedGoogle Scholar
  • 58 Battafarano R J, Anderson R C, Meyers B G. Perioperative complications after living donor lobectomy.  J Thorac Cardiovasc Surg . 2000;  120(5) 909-915
    CrossrefPubMedGoogle Scholar
  • 59 Kotloff R M, Zuckerman J B. Lung transplantation for cystic fibrosis: special considerations.  Chest . 1996;  109(3) 787 Review-798 Review
    PubMedGoogle Scholar
  • 60 Harringer W, Wiebe K, Struber M. Lung transplantation: 10-year experience.  Eur J Cardiothorac Surg Nov . 1999;  16(5) 546-554
    CrossrefPubMedGoogle Scholar
  • 61 Meyers B F, Lynch J, Trulock E P, Guthrie T J, Cooper J D, Patterson G. Lung transplantation: a decade of experience.  Ann Surg . 1999;  230(3) 362-370
    CrossrefPubMedGoogle Scholar
  • 62 Mendeloff E N, Huddleston C B, Mallory G B. Pediatric and adult lung transplantation for cystic fibrosis.  J Thorac Cardiovasc Surg . 1998;  115(2) 404-414
    CrossrefPubMedGoogle Scholar
  • 63 Hosenpud J, Bennett L. Effect of diagnosis on survival benefit of lung transplantation for end-stage lung disease.  Lancet . 1998;  351 24-27
    CrossrefPubMedGoogle Scholar
  • 64 Aurora P, Whitehead B, Wade A. Lung transplantation and life extension in children with cystic fibrosis.  Lancet . 1999;  354(9190) 1591-1593
    CrossrefPubMedGoogle Scholar
  • 65 Barlow C W, Robbins R C, Moon M R, Akindipe O, Theodore J, Reitz B A. Heart-lung versus double-lung transplantation for suppurative lung disease.  J Thorac Cardiovasc Surg Mar . 2000;  119(3) 466-476
    CrossrefPubMedGoogle Scholar
  • 66 Oelberg D A, Systrom D M, Markowitz D H. Exercise performance in cystic fibrosis before and after bilateral lung transplantation.  J Heart Lung Transplant . 1998;  17(11) 1104-1112
    PubMedGoogle Scholar
  • 67 Stavem K, Bjortuft O, Lund M B, Kongshaug K, Geiran O, Boe J. Health-related quality of life in lung transplant candidates and recipients.  Respiration . 2000;  67(2) 159-165
    CrossrefPubMedGoogle Scholar
  • 68 Lanuza D M, Lefaiver C A, Farcas G A. Research on the quality of life of lung transplant candidates and recipients: an integrative review.  Heart Lung . 2000;  29(3) 180-195
    CrossrefPubMedGoogle Scholar
  • 69 Limbos M M, Joyce D P, Chan C K, Kesten S. Psychological functioning and quality of life in lung transplant candidates and recipients.  Chest . 2000;  118(2) 408-416
    CrossrefPubMedGoogle Scholar
  • 70 Anyanwu A C, McGuire A, Rogers C A, Murday A J. Assessment of quality of life in lung transplantation using a simple generic tool.  Thorax . 2001;  56 218-222
    CrossrefPubMedGoogle Scholar
  • 71 Deierhoi M H, Haug M. Review of select transplant subpopulations at high risk of failure from standard immunosuppressive therapy.  Clin Transplant . 2000;  14(5) 439-448
    CrossrefPubMedGoogle Scholar
  • 72 Cooper J D, Billingham M, Egan T. A working formulation for the standardization of nomenclature for clinical staging of chronic dysfunction in lung allografts: International Society for Heart and Lung Transplantation.  J Heart Lung Transpl . 1993;  12 713-716
    PubMedGoogle Scholar
  • 73 Metras D, Viard L, Kreitmann B. Lung infections in pediatric lung transplantation: experience in 49 cases.  Eur J Cardiothorac Surg . 1999;  15(4) 490-494
    CrossrefPubMedGoogle Scholar
  • 74 Snell G I, deHoyos A, Krajden M, Winton T, Maurer J R. Pseudomonas cepacia in lung transplant recipients with cystic fibrosis.  Chest . 1993;  103(2) 466-471
    CrossrefPubMedGoogle Scholar
  • 75 Palmer S M, Alexander B D, Sanders L L. Significance of blood stream infection after lung transplantation: analysis in 176 consecutive patients.  Transplantation . 2000;  69(11) 2360-2366
    CrossrefPubMedGoogle Scholar
  • 76 Goldstein L S, Haug M T, Perl J, Perl M K, Maurer J R, Arroliga A C, Mehta A C, Kirby T, Higgins B, Stillwell P C. Central nervous system complications after lung transplantation.  J Heart Lung Transplant . 1998;  17(2) 185-191
    PubMedGoogle Scholar
  • 77 Goldstein A B, Goldstein L S, Perl M K, Haug M T, Arroliga A C, Stillwell P C. Cystic fibrosis patients with and without central nervous system complications following lung transplantation.  Pediatr Pulmonol . 2000;  30(3) 203-206
    CrossrefPubMedGoogle Scholar
  • 78 Maurer J R. The spectrum of colonic complications in a lung transplant population.  Ann Transplant . 2000;  5(3) 54-57
    PubMedGoogle Scholar
  • 79 Aris A M, Maia D M, Neuringer I P. Post-transplantation lymphoproliferative disorder in the Epstein-Barr virus-naïve lung transplant recipient.  Am J Respir Crit Care Med . 1996;  154(6 pt 1) 1712-1717
    CrossrefPubMedGoogle Scholar
  • 80 Wong J Y, Tait B, Levvey B. EBV mismatching and HLA matching post lung transplantation: key risk factors for posttransplant lymphoproliferative disease (PTLD).  J Heart Lung Transplant . 2001;  20(2) 199
    PubMedGoogle Scholar
  • 81 Boyle G J, Michaels M G, Webber S A. Post-transplantation lymphoproliferative disorders in pediatric thoracic organ recipients.  J Pediatr . 1997;  131(2) 309-313
    PubMedGoogle Scholar
  • 82 Cohen A H, Sweet S C, Mendeloff E. High incidence of posttransplant lymphoproliferative disease in pediatric patients with cystic fibrosis.  Am J Respir Crit Care Med . 2000;  161(4 Pt 1) 1252-1255
    CrossrefPubMedGoogle Scholar
  • 83 Ifthikharuddin J J, Mieles L A, Rosenblatt J D, Ryan C K, Sahasrabudhe D M. CD-20 expression in post-transplant lymphoproliferative disorders: treatment with rituximab.  AM J Hematol . 2000;  65(2) 171-173
    CrossrefPubMedGoogle Scholar
  • 84 Milpied N, Vasseur B, Parquet N. Humanized anti-CD20 monoclonal antibody (Rituximab) in posttransplant B-lymphoproliferative disorder: a retrospective analysis on 32 patients.  Ann Oncol . 2000;  11(S1) 113-116
    PubMedGoogle Scholar
  • 85 Cohen R G, Starnes V A. Living donor lung transplantation.  World J Surg . 2001;  25 244-250
    CrossrefPubMedGoogle Scholar
  • 86 Keistinen T, Saynajakangas O, Tuuponen T, Kivela S L. Bronchiectasis: an orphan disease with a poorly-understood prognosis.  Eur Respir J . 1997;  10 2784-2787
    CrossrefPubMedGoogle Scholar
      >