Chest
Volume 131, Issue 4, April 2007, Pages 1224-1231
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Special Feature
Special Considerations for Patients With Cystic Fibrosis Undergoing Lung Transplantation

https://doi.org/10.1378/chest.06-1163Get rights and content

This article reviews lung transplantation in patients with cystic fibrosis (CF). Lung transplantation is commonly utilized for patients with end-stage CF. There are several characteristics of CF that present unique challenges before and after lung transplantation. There is new information available that can be utilized to predict outcomes in patients with end-stage CF, and therefore can help in decisions of referral and listing for lung transplantation. The new lung allocation score, which allocates organs to patients who are on the lung transplant waiting list in the United States, presents new challenges and opportunities for patients with end-stage CF. In addition, the effect of the presence of microbiological flora prior to lung transplantation has been better linked to outcomes after lung transplantation. It is now known that, other than those patients harboring Burkholderia cepacia in their lungs before transplantation, most CF patients can undergo transplantation successfully. Nutrition remains an important issue among CF patients, and diabetes is a common problem after lung transplantation. In contrast, liver disease does not usually present major problems but, if it is severe, can necessitate liver and lung transplantation. Mechanical ventilation prior to transplantation might not be an absolute contraindication for CF patients. CF lung transplant recipients have good outcomes after lung transplantation compared with those of other lung transplant recipients. Quality of life is dramatically improved. However, they are still prone to common complications that all lung transplant recipients are prone to, including primary graft dysfunction, acute and chronic rejection, a variety of infections and malignancies, and renal failure.

Section snippets

Timing of Lung Transplantation

Several studies456789 from the past few years have attempted to assess the risk of death in cohorts of CF patients based on several baseline characteristics. According to these studies, several factors, in addition to FEV1, have been shown as important predictors. According to the largest studies,4 using data from the Cystic Fibrosis Registry, pulmonary function, number of exacerbations, age, gender, weight, presence of diabetes, pancreatic insufficiency, and type of bacterial colonization are

Bacterial Colonization/Infection

The majority of CF patients are colonized/infected with Pseudomonas aeruginosa by the time they are considered for lung transplantation. In most cases, there are mucoid and nonmucoid strains of the organism in the lungs of the transplant recipient.16 The impact of the resistance pattern of the Pseudomonas strains before lung transplantation appears to be minimal on survival after lung transplantation. Three smaller studies171819 have provided conflicting results, while a larger study20 has

Colonization/Infection With Fungi and Mycobacteria

There is less information available on the significance of colonization/infection with fungi prior to lung transplantation. Aspergillus species are isolated before transplantation in 10 to 25% of patients with CF.1525 Their presence does not appear to predict the isolation of fungal species after transplantation and it does not predict outcome.2627 Aspergillus is frequently isolated in patients with anastomotic problems after transplantation, whether they have CF or not.28

A rarer form of

Diabetes Mellitus

The prevalence of diabetes mellitus in patients with CF has been increasing, since more patients are living longer.34 This is a problem for pancreatic-insufficient patients, who comprise 85 to 90% of patients with CF. In a recent study,35 approximately 30% of adult patients undergoing lung transplantation had CF-related diabetes. In addition, diabetes develops in approximately 20% of patients posttransplant, usually in the early postoperative period.35

The presence of diabetes has been

GI and Hepatic Issues

CF patients with pancreatic insufficiency require enzyme supplementation in order to absorb nutrients. There is no compelling evidence, but most experts have suggested that pancreatic enzymes should be given along with immunosuppressive medications in order to improve absorption.36 Therapy with pancreatic enzymes should be started as soon as possible after the transplant operation. It can be more difficult to achieve therapeutic levels for CF patients with either cyclosporine A or tacrolimus.37

Sinusitis

Sinusitis is very common in patients with CF. They are frequently colonized/infected with organisms similar to those in their lungs. Fear of spreading the infection to the new lungs in the setting of immunosuppression has prompted many centers to attempt preemptive operations and drainage of the sinuses.44 However, outcomes did not change, and at this time routine surgical drainage of the sinuses prior to lung transplantation cannot be recommended. A more recent study45 has suggested that

Nutrition

Malnutrition is an independent predictor of poor outcomes in CF patients. Studies4647 have shown that poor nutritional status leads to worse outcomes after lung transplantation and while the patient is on the transplant waiting list. Efforts to improve nutrition by using supplementation via nasogastric/nasojejunal or with percutaneous gastrostomy/jejunostomy tube insertion are usually undertaken. No absolute level of malnutrition precludes patients from undergoing lung transplantation, but

Other Issues

Osteoporosis is common in patients with end-stage lung disease.49 CF patients are particularly vulnerable to osteoporosis because of vitamin D absorption issues, in addition to chronic lung disease. Osteoporosis tends to worsen after lung transplantation because of steroid and calcineurin inhibitor use.50 Aggressive calcium and vitamin D supplementation with concomitant use of bisphosphonates can lead to maintenance of or improvement in bone density.51 Severe, symptomatic osteoporosis is a

Living Donor Lung Transplantation

The shortage of donor organs and the previous UNOS lung allocation system (which gave priority to patients based on time spent on the waiting list) led to a significant number of deaths of patients who were on the list.2 Living-donor lung transplantation was undertaken for selected patients in order to circumvent the above situation. In that operation, two lower lobes are obtained from taller healthy donors and are placed in the chest cavity of a smaller recipient.53

The operation has been

Complications of Lung Transplantation

There are several transplant-specific complications that occur in transplant recipients, irrespective of pretransplant diagnosis. These will only be mentioned briefly in the article, and the list is not inclusive. CF patients are at risk, like all other transplant recipients; however, they need to be counseled about the possibility of these complications.

In the early postoperative period, primary graft dysfunction occurs in approximately 10% of patients, with patients presenting with multilobar

Expected Outcomes

CF patients achieve normal lung function after lung transplantation.60 The improvement continues in the first posttransplant year, but lung function declines as chronic rejection starts to occur. In addition, normalization of the blood gases is expected early after transplantation, usually before hospital discharge.

Exercise tolerance significantly improves after lung transplantation. CF patients achieve improvement in their New York Heart Association (NYHA) class and their 6-min walk distances

Conclusions

Lung transplantation occurs in an increasing number of CF patients. There are several issues unique to this population requiring special attention and management. However, as a group their outcomes are somewhat better than those of non-CF patients. Progress has been made in understanding the factors that affect outcomes before and after transplantation. Nevertheless, there are several areas where improved understanding of the mechanisms that lead to complications after transplantation could

References (61)

  • HadjiliadisD et al.

    Outcome of lung transplantation in patients with mycetomas

    Chest

    (2002)
  • BrennanAL et al.

    Clinical importance of cystic fibrosis-related diabetes

    J Cyst Fibros

    (2004)
  • KnoopC et al.

    Tacrolimus pharmacokinetics and dose monitoring after lung transplantation for cystic fibrosis and other conditions

    Am J Transplant

    (2005)
  • GilljamM et al.

    GI complications after lung transplantation in patients with cystic fibrosis

    Chest

    (2003)
  • LamireauT et al.

    Epidemiology of liver disease in cystic fibrosis: a longitudinal study

    J Hepatol

    (2004)
  • MadillJ et al.

    Nutritional assessment of the lung transplant patient: body mass index as a predictor of 90-day mortality following transplantation

    J Heart Lung Transplant

    (2001)
  • SingerLG et al.

    Weight gain after lung transplantation

    J Heart Lung Transplant

    (2003)
  • Caplan-ShawCE et al.

    Osteoporosis in diffuse parenchymal lung disease

    Chest

    (2006)
  • RutherfordRM et al.

    Functional status and quality of life in patients surviving 10 years after lung transplantation

    Am J Transplant

    (2005)
  • BartzRR et al.

    Pre-transplant mechanical ventilation and outcome in patients with cystic fibrosis

    J Heart Lung Transplant

    (2003)
  • BarrML et al.

    Living donor lobar lung transplantation: current status and future directions

    Transplant Proc

    (2005)
  • KumarD et al.

    Clinical impact of community-acquired respiratory viruses on bronchiolitis obliterans after lung transplant

    Am J Transplant

    (2005)
  • ReamsBD et al.

    Posttransplant lymphoproliferative disorder: incidence, presentation, and response to treatment in lung transplant recipients

    Chest

    (2003)
  • YoungLR et al.

    Lung transplantation exacerbates gastroesophageal reflux disease

    Chest

    (2003)
  • EganT et al.

    Improved results of lung transplantation for patients with cystic fibrosis

    J Thorac Cardiovasc Surg

    (1995)
  • OrensteinDM et al.

    Transplantation

  • LiouTG et al.

    Predictive 5-year survivorship model of cystic fibrosis

    Am J Epidemiol

    (2001)
  • KeremE et al.

    Prediction of mortality in patients with cystic fibrosis

    N Engl J Med

    (1992)
  • VizzaCD et al.

    Outcome of patients with cystic fibrosis awaiting lung transplantation

    Am J Respir Crit Care Med

    (2000)
  • Mayer-HamblettN et al.

    Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality

    Am J Respir Crit Care Med

    (2002)
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    The author has reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

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