Cystic Fibrosis Lung Transplant Care Consensus Statements

The Cystic Fibrosis Foundation assembled a multidisciplinary committee to create consensus statements that provide recommendations to lung transplant clinicians on the management of CF-specific comorbidities immediately before and after transplantation. The committee developed 32 recommendation statements and organized them into seven categories.

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Summary

People with cystic fibrosis who have had a lung transplant have unique comorbidities that require specialized management by providers with expertise in CF.
The Cystic Fibrosis Foundation assembled a multidisciplinary committee that created recommendations to lung transplant clinicians on managing CF-specific comorbidities immediately before and after transplantation.
This consensus document is based on a comprehensive literature review and expert opinion.

Shah P, Lowery E, Chaparro C, et. al. Cystic fibrosis foundation consensus statements for the care of cystic fibrosis lung transplant recipients. J Heart Lung Transplant. 2021 Jul;40(7):539-556. doi: 10.1016/j.healun.2021.04.011. Epub 2021 Apr 22. PMID: 34103223.

Purpose/Background

Cystic fibrosis is a common indication for lung transplantation worldwide. Adult CF lung transplant recipients (CFLTRs) have excellent outcomes and the greatest survival after lung transplantation of any disease indication. CFLTRs, however, have unique comorbidities that require specialized management by providers with expertise in CF. The availability of CF-specific expertise at transplant centers may improve the long-term survival of CFLTRs.

The Cystic Fibrosis Foundation assembled a multidisciplinary committee composed of physicians from adult and pediatric transplant pulmonology, infectious diseases, gastroenterology, and endocrinology, as well as a transplant coordinator, dietician, pharmacist, psychologist, two adult CFLTRs, and a caregiver. This committee created consensus statements that provide recommendations to lung transplant clinicians on managing CF-specific comorbidities immediately before and after transplantation. This consensus document is based on a comprehensive literature review and expert opinion.

Methodology

Between June 2018 and March 2020, the committee:

Divided into three workgroups focused on infectious disease, extra-pulmonary CF considerations, and psychologic and pharmacologic considerations
Conducted a relevant literature search identifying 7,875 references, of which 869 full-text articles were assessed for eligibility, with 357 of those studies included in the development of recommendation statements
For topics without sufficient evidenced-based literature to guide best practice, recommendations were based on expert opinion consensus.
Developed draft recommendations.
Voted on recommendation statements with an 80% agreement for acceptance.
Developed a manuscript of recommendations that was distributed for public comment.

The committee developed 32 recommendation statements and organized them into seven categories:

General recommendations, with two recommendation statements
Infectious disease, with eight recommendation statements
Sinus disease, with four recommendation statements
Nutrition and gastrointestinal complications, with six recommendation statements
Diabetes and bone health, with three recommendation statements
Mental health and family planning, with four recommendation statements
Pharmacology and therapeutics, with five recommendation statements

Of the 32 recommendation statements, the following are the key recommendations most crucial and relevant to the CF clinician. Refer to the published guideline for a complete list of recommendations.

Key Recommendations

Recommendations	Evaluation of the Evidence
The CF Foundation recommends that CF lung transplant recipients follow up with a multidisciplinary CF care team within 6–12 months of transplant to resume extra-pulmonary CF care. Communication between the transplant and CF care teams is essential for coordination of care.	100% Consensus
The CF Foundation recommends that noninvasive CF-specific bacterial, fungal, and acid-fast bacilllus (AFB) respiratory cultures be obtained by the transplant or CF center every three months in actively waitlisted transplant candidates, and that clinicians review prior pathogen history to guide the perioperative antibiotic regimen.	100% Consensus
The CF Foundation recommends an intraoperative CF bacterial, fungal, and AFB culture of the native lung be obtained at the time of lung transplantation.	100% Consensus
The CF Foundation recommends consideration of perioperative and/or early post-transplant inhaled antibiotics for bacterial pathogens isolated prior to transplant as a complement to systemic antimicrobials in CF lung transplant recipients.	100% Consensus
The CF Foundation recommends screening CF lung transplant recipients for symptoms of chronic rhinosinusitis (CRS) at least annually.	100% Consensus
The CF Foundation recommends that CF lung transplant recipients with moderate or severe symptomatic CRS be seen in consultation with an otolaryngologist experienced in CF for consideration of optimal topical therapies and endoscopic sinus surgery.	100% Consensus
The CF Foundation recommends that CF lung transplant recipients who have had multiple bacterial allograft infections be seen in consultation with an otolaryngologist with CF expertise regardless of their CRS symptoms.	100% Consensus
For CF lung transplant recipients, the CF Foundation recommends ongoing consultation with a dietitian with CF expertise, in order to receive individualized nutritional therapy to achieve an established BMI or weight-for-length goal.	100% Consensus
In CF lung transplant recipients, the CF Foundation recommends continuation of vitamin D supplementation, discontinuation of combination vitamin A, D, E, K supplements after lung transplantation, measuring fat-soluble vitamin levels by three months after transplant, and individually repleting and following levels as needed.	100% Consensus
The CF Foundation recommends daily symptom assessment for early signs of obstipation and obstruction in hospitalized patients that might herald emergence of distal intestinal obstruction syndrome (DIOS), particularly within the immediate post-operative period and with any opiate medication administration.	100% Consensus
In CF lung transplant recipients who develop DIOS, the CF Foundation recommends consideration of early enteral lavage. Refractory DIOS should be managed in coordination with experts in CF gastrointestinal complications to reduce risk for prolonged obstruction and potential need for operative management.	100% Consensus
The CF Foundation recommends that CF lung transplant recipients have liver enzyme monitoring for CF liver disease (CFLD) at least annually, and when elevated, noninvasive imaging techniques for initial evaluation.	100% Consensus
In CF lung transplant recipients who do not have cystic fibrosis-related diabetes (CFRD), the CF Foundation recommends screening with an oral glucose tolerance test (OGTT) at 3–6 months after transplant, then annually following the recommended screening guidelines for CFRD.	95% Consensus
For CF lung transplant recipients, the CF Foundation recommends that bone density be assessed with dual energy X-ray absorptiometry (DEXA) at 6–12 months after transplant.	100% Consensus
The CF Foundation recommends that CF lung transplant recipients have mental health screening and consultation for depression, anxiety, and post-traumatic stress disorder (PTSD) within six months of transplant, then resume annual screening per the International Committee on Mental Health (ICMH) depression and anxiety guidelines.	100% Consensus
The CF Foundation recommends that females with CF who are post-lung transplant and are considering pregnancy carefully assess their individual risks through shared decision-making with maternal fetal medicine and transplant providers.	100% Consensus
The CF Foundation recommends that females with CF who are post-lung transplant avoid pregnancy for at least the first two years after transplantation because of the increased risk of acute rejection, accelerated chronic rejection, and death.	100% Consensus
The CF Foundation found insufficient evidence to recommend for or against the use of cystic fibrosis transmembrane conductance regulator (CFTR) modulators for CF lung transplant recipients.	100% Consensus
Reduced renal function is common in CF lung transplant recipients, and serum creatinine is often a poor surrogate for renal function. Therefore, the CF Foundation recommends medication dosing appropriate for glomerular filtration rate (GFR), and when available, the use of therapeutic drug monitoring.	100% Consensus

Unanswered Questions

What is the role of CFTR modulators after lung transplant?
Should azithromycin be resumed after lung transplant in patients with CF?
What is the optimal interval of follow up for CF lung transplant recipients with the multidisciplinary CF team?

Use of This Position Paper

The CF Foundation intends for this executive summary of its consensus recommendations to summarize the published consensus recommendations. The published consensus recommendations summarize evidence and provide reasonable clinical recommendations based on that evidence to clinicians, patients, and other stakeholders. Care decisions regarding individual patients should be made using a combination of these recommendations, the associated benefit-risk assessment of treatment options from the clinical team, the patient’s individual and unique circumstances, as well as the goals and preferences of the patients and families that the team serves, as a part of shared decision-making between the patient and clinician. The consensus recommendations are intended to be location-agnostic and are applicable to individuals with CF in all settings including but not limited to outpatient clinics, hospitals, in-home, psychiatric facilities, long-term care facilities, incarceration, detention, and foster-care settings.

This executive summary was prepared by:

Alyssa A. Perez, MD, MEd (University of California San Francisco) and Ramsey Hachem, MD (Washington University School of Medicine)

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Purpose/Background

Methodology

Key Recommendations

Unanswered Questions

Further Reading

Use of This Position Paper