Bilateral lung transplantation in cystic fibrosis with hepatitis C infection – a study of two cases

Address for correspondence: Nikola Ruszel, Students’ Research Club of the Department of Thoracic Surgery and Transplantation. Department of Thoracic Surgery and Transplantation, Pomeranian Medical University, 11 Sokołowskiego St, 70-891 Szczecin-Zdunowo, Poland, e-mail: nikola_ruszel@yahoo.com, abkubisa@hotmail.com Received: 15.05. 2019, accepted: 29.07. 2019. Cystic fibrosis (CF) is one of the most common hereditary diseases in Caucasians – it affects more than 70 000 patients worldwide [1]. Mutation in the CFTR gene leads to inappropriate synthesis of the chloride anion channel, which interferes with the regular function of multiple organs. Pulmonary-wise, CF manifests by the presence of thick, immobile secretions in the lungs, which increases the incidence of respiratory tract infections. Median survival rate for patients in the last decades has increased – over a half of the children with CF, born between 2013 and 2017, are expected to live 44 years or more [1]. The development of pharmacotherapy and a variety of remedial solutions give a high chance for longer survival of CF patients, and it is predicted that the number of effectively cured adults will continue to grow in future. However, some CF patients require a more radical life-saving solution to tackle the respiratory insufficiencies – lung transplantation (LuTx). Identification of transplant candidates is still an evolving discipline which aims to provide the best survival benefit for all of the patients qualified for this procedure [2]. In this Letter, the history of two male patients who underwent a double LuTx (1st in 2015; 2nd in 2016) after eradication of hepatitis C virus (HCV) infection will be presented. Their cases were the first of this kind in Poland. As seen in the case of patient 1, a carefully planned operation and intensive care after the procedure can lead to satisfactory therapeutic results and significant improvement of the life standard of critically ill patients. However, LuTx can also be a highly challenging procedure. As was seen in the case of patient 2, asymmetry of the native lungs, hilar fibrosis and the risk of potential HCV viral multiplication lead to repetitive exclusion of the patient from this procedure in multiple centers in Europe and United States. However, LuTx was performed with a therapeutic outcome surpassing initial predictions. The main intention of lung transplantation was to improve the outcomes in patients suffering from end-stage lung disease. The most important indications for LuTx referral and the factors which may be further related to posttransplantation survivorship are presented in Table I. In accordance with the International Society for Heart and Lung Transplantation [3], the main indications for LuTx referral in the presented CF patients were: forced expiratory volume in 1 s (FEV1) below 30%, 6-minute walk test results under 400 m, development of pulmonary hypertension in the absence of a hypoxic exacerbation (patient 1), episodes of acute respiratory failure, increasing antibiotic resistance with poor clinical recovery from exacerbations and life-threatening haemoptysis (patient 1). However, in the presented cases there were pre-transplant risk factors which gave a prognosis of poor long-term survival after LuTx – insulin-dependent diabetes mellitus (patient 1) and low BMI (patient 2). The main reason for postponing the procedure in both cases was presence of HCV viral mRNA (which was successfully eradicated before the LuTx). At that time it was listed as one of the absolute contraindications, as it was said to significantly increase the risk of graft rejection [4]. In the case of patient 2, he first presented with hepatitis C infection in 2006, and then in 2009 he underwent interferon-a and ribavirin treatment. In 2014 laboratory results showed the HCV reactivation and the patient was treated with pegylated interferon and sofosbuvir, but the therapy had to be interrupted due to pulmonary exacerbations. Finally, in June 2015 the level of HCV-RNA was under the cut-off line and the patient was continued to be treated with ribavirin 800 mg with sofosbuvir 400 mg, and in late 2015 he underwent LuTx. Since then, he has presented no viral RNA, with follow-up blood tests in July, AuBilateral lung transplantation in cystic fibrosis with hepatitis C infection – a study of two cases