Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis

Abstract

There are no European recommendations on issues specifically related to lung transplantation (LTX) in cystic fibrosis (CF). The main goal of this paper is to provide CF care team members with clinically relevant CF-specific information on all aspects of LTX, highlighting areas of consensus and controversy throughout Europe. Bilateral lung transplantation has been shown to be an important therapeutic option for end-stage CF pulmonary disease. Transplant function and patient survival after transplantation are better than in most other indications for this procedure. Attention though has to be paid to pretransplant morbidity, time for referral, evaluation, indication, and contraindication in children and in adults. This review makes extensive use of specific evidence in the field of lung transplantation in CF patients and addresses all issues of practical importance. The requirements of pre-, peri-, and postoperative management are discussed in detail including bridging to transplant and postoperative complications, immune suppression, chronic allograft dysfunction, infection, and malignancies being the most important. Among the contributors to this guiding information are 19 members of the ECORN-CF project and other experts. The document is endorsed by the European Cystic Fibrosis Society and sponsored by the Christiane Herzog Foundation.

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“…2,14 Additionally, although an "ideal" lung donor has a smoking history of less than 20 years, 15 that benchmark often is not achieved, as evidenced by the 16.4% of donors listed in this study with a history of cigarette use. There are currently no studies that directly quantify how much past donor smoking is safe when accepting lungs for transplant; however, retrospective reviews have shown that more than 40 pack-years negatively influences outcomes in non-CF patients.…”

“…1 Lung transplant (LTx) is a therapeutic option for end-stage or acutely declining CF lung disease. 2 As such, CF is the second most frequent indication for bilateral LTx worldwide. 3 While LTx in CF is associated with improved median survival compared with other transplant indications, optimization of posttransplant outcomes and determination of risk factors for acute allograft rejection is ongoing in CF.…”

Nutritional status in patients with cystic fi brosis before and after lung transplantation

“…Использование прокинетиков и уменьшение объема питания поз-воляют избежать данных осложнений. Диета с высоким содержанием углеводов может стать причиной усиления продукции углекислого газа и усугубления дыхательной недостаточности, что является основанием для применения неинвазив-ной вентиляции легких [59].…”

“…Подходы к поддержанию адекватного СП больных МВ непосредственно после ТЛ ввиду отсутствия достаточной базы данных основаны на стандартных рекомендациях для общей популя-ции пациентов хирургического профиля и боль-ных, перенесших трансплантацию других орга-нов [60]. Пероральная диета у пациентов, эксту-бированных на 1-е или 2-е сутки после операции, постепенно расширяется в зависимости от усво-ения больным продуктов питания с обязатель-ным применением заместительной ферментной терапии.…”

Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis

“…The adult with CF should remain under the primary care of the transplantation team after a successful transplant and there should be shared care for pulmonary and non-pulmonary complications with the CF team. Primary care could be progressively handed over to CF teams with continued close liaison with the transplant centre [44].…”