© Springer International Publishing Switzerland 2016
Antonio M. Esquinas (ed.)Noninvasive Mechanical Ventilation10.1007/978-3-319-21653-9_9090. Long-Term Noninvasive Ventilation Among Chronic Respiratory Failure Diseases (Cystic Fibrosis and Other Diseases) Awaiting Lung Transplantation: Key Determinants and Practical Implications
(1)
Respiratory Service, Instituto de Investigación Biomédica de A Coruña (INIBIC), Complejo Hospitalario Universitario de A Coruña (CHUAC), Sergas, Universidade da Coruña (UDC), Corunna, Spain
(2)
Sleep Unit, Araba University Hospital, Basque Country University, School of Medicine, Ciberes, BioaAraba Project, Vitoria-Gasteiz, Spain
90.1 Introduction
90.2 Long-Term NIV in Patients with Chronic Respiratory Failure Diseases Awaiting Lung Transplantation
90.2.1 Cystic Fibrosis
90.2.2 COPD
90.2.4 Advantages of NIV
90.3 Conclusion
Keywords
Noninvasive ventilationLung transplantationChronic obstructive pulmonary diseaseCystic fibrosisWaiting90.1 Introduction
Lung transplant prevalence continues to increase in spite of the recent advances that have been made in the knowledge and treatment of lung diseases in recent years. Today, it is a therapeutic option for patients with end-stage lung disease with poor prognosis in 2 year’s time to improve their chance of survival and quality of life.
In Spain in 2014, according to the National Transplantation Office Annual Report [1], interstitial lung disease (ILD, 38 %), chronic obstructive pulmonary disease (COPD, 35 %), and cystic fibrosis (CF, 11 %) are the three most frequent principal diagnoses among patients awaiting lung transplantation. Other indications include bronchiectasis, pulmonary hypertension, and retransplantation [1]. This distribution is similar in the international registry [2].
It is important to highlight the difference between the time the patient is referred to evaluation by the multidisciplinary transplantation team and the time to be placed to wait for lung transplantation [3, 4]. There are national and international consensus recommendations to manage this situation, and we must acknowledge that the decision to include a patient on the waiting list for lung transplant is complex and involves taking into account specific program and regional factors that affect such a decision [3, 4].
In 2014, 262 patients out of 601 listed underwent lung transplantation in Spain [1]. The average and median number of days spent on the waiting list were 241 (standard deviation (SD) 221) and 178 (range 75–329), respectively. The waiting list global mortality rate was 3.9 %. Nearly 1 out of 10 transplantation procedures was under emergency code, and the most common diseases were ILD (45 %) and CF (28 %). Mortality under emergency code has remained between 6 and 11 % during the past few years.
Although every country has its own transplant waiting list management system, territorial distribution criteria together with severity index and anthropometric features are taken into account for most of them. The Lung Allocation Score (LAS) is a statistical model used in some countries that considers both urgency and post-transplant survival and prioritizes listing and transplantation for high risk patients.
The treatment of symptomatic chronic respiratory failure (CRF) continues to be the cornerstone of the management of critically ill patients, and noninvasive ventilation (NIV) is the key element to its success. Initially, it was used as a bridge to lung transplantation strategy instead of invasive mechanical ventilation [5]. Gradually, its use has adopted a long-term view with the aim of stabilizing critically ill patients to improve access to lung transplantation as pre-transplant life expectancy increases, and to improve the probability of success as the patient undergoes surgery in better condition [3, 6–8]. Currently, the need for ventilatory support is an element of severity to be assessed while the patient is being evaluated to be a lung transplant candidate, and it is not considered as a contraindication [3, 4]. Nor does it bias the prioritization scales used in some countries. What determines a change in consideration and in priority is the beginning of invasive ventilation.
In spite of the spread of its use, the scientific evidence that supports long-term NIV for patients awaiting lung transplantation relies on observational studies. This is due to the ethical difficulty of designing a randomized trial for these patients. This chapter reviews the most representative studies of long-term NIV within this context.
90.2 Long-Term NIV in Patients with Chronic Respiratory Failure Diseases Awaiting Lung Transplantation
The long-term NIV approach for patients on a waiting list lung for transplantation is a situation that is mainly seen in patients with CF and COPD, as it is irrelevant to the pathogenesis and course of lung interstitial and vascular diseases. The role of invasive and noninvasive ventilation for CF patients was initially evaluated as a bridge to lung transplantation in patients with respiratory failure and severe advanced disease. It was then considered as a treatment option for respiratory failure in very advanced disease, irrespective of the patient’s status as a lung transplant candidate. The difficulty of not having randomized studies means that the scientific evidence for this approach is based on observational series alone. There are no data about short-term NIV or treating exacerbations. There are no clinical guidelines for how to start and adapt ventilation in these patients. Each team has its own program based on the available evidence and on its proven experience.
The highest level of evidence to support the use of NIV is achieved in the treatment of COPD patients with hypercapnic respiratory failure exacerbation. Benefits in survival, less orotracheal intubation (OTI), and hospital length of stay have been proven. However, its role in respiratory failure outside the context of an exacerbation scenario is more controversial. Nevertheless, after several scientific studies there is favorable evidence for its use in a group of patients with stable hypercapnic chronic respiratory failure. We have not found any specific studies about long-term NIV in COPD designed in the lung transplant context.
90.2.1 Cystic Fibrosis
Pulmonary alteration in patients with CF is characterized by bronchiectasis that damages lung parenchyma and by progressive bronchial obstruction due to bronchial wall inflammation and mucous plugs. Hart et al. [9] observed that, in children and young adults with CF and stable advanced lung disease, there was a correlation between forced expiratory volume in 1 s (FEV1) fall and muscle respiratory load rise. As a result, these patients develop a compensatory mechanism of rapid shallow breathing pattern. Although this respiratory strategy succeeds in maintaining the level of ventilation, pCO2 eventually increases, and the efficiency of the respiratory muscle pump in eliminating CO2 diminishes. Supportive treatment with NIV alleviates the muscle respiratory load and, therefore, respiratory muscle performance can be preserved. Despite this physiopathological basis, more studies are required to determine for whom, when, and how this therapy is proven to be beneficial.
In 2002, Madden et al. [6] produced the first and most extensive retrospective report on 113 patients with end-stage CF receiving nasal ventilation. Indication for ventilatory support was established when clinical condition and arterial blood gases where judged to be a severe risk to survival. They studied 23 non-transplant candidates and 90 patients who were either waiting for or being evaluated for a lung transplant. They looked after most patients on a general ward or at home. The mean duration of NIV support was 61 days (range 1–600) for those on the lung transplant waiting list, 53 days (range 1–279) for patients under evaluation and 45 days (range 0–379) among those patients who were not being considered for a lung transplant. They described their experience in treating respiratory failure until transplantation with NIV for advanced patients who are actually on the lung transplant waiting list or being evaluated for it. They also recommend caution in its use among non-candidate patients as it may unnecessarily delay the inevitable and prolong suffering.
There are few references to long-term NIV therapy. This chapter focuses on two more recent works. One is a French study based on the analysis of data from the CF National Register Centre, and the second is an English study that reviewed 20 years of clinical practice in a lung transplant center with a multidisciplinary team.
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