Cystic Fibrosis Foundation consensus guidelines for the care of individuals with advanced cystic fibrosis lung disease

BACKGROUND: Advanced cystic fibrosis lung disease (ACFLD) is common, is associated with reduced quality of life, and remains the most frequent cause of death in individuals with cystic fibrosis (CF). These consensus guidelines provide recommendations to the CF community on management of both common and unique issues that arise when individuals reach a state of ACFLD. METHODS: The CF Foundation assembled a multidisciplinary expert panel consisting of three workgroups: Pulmonary management; Management of comorbid conditions; Symptom management and psychosocial issues. Topics were excluded if the management considerations did not differ in ACFLD from in the overall CF population or if already addressed in other published guidelines. Recommendations were based on a systematic literature review combined with expert opinion when appropriate. RESULTS: The committee formulated twenty-three recommendation statements specific to ACFLD that address the definition of ACFLD, pulmonary and intensive care unit management, management of selected comorbidities, symptom control, and psychosocial issues. CONCLUSIONS: These recommendations are intended to be paired with previously published management guidelines for the overall CF population, with the objective of reducing practice variability and improving overall care, quality of life, and survival in those with ACFLD

A survey identifying nutritional needs in a contemporary adult cystic fibrosis cohort

Abstract Background Cystic fibrosis (CF) is a disease in which nutritional barriers are diverse and common, with malnutrition greatly influencing pulmonary trajectory and overall outcomes. Despite this, the most effective methods to optimize CF nutrition are unknown, and literature describing patients’ perspectives on their specific nutritional needs is lacking, particularly in the modern era of CF care. This study aimed to identify the most important nutritional needs and desired health-improvement resources in a contemporary adult CF cohort. Methods A 14-question investigator-designed survey addressing nutrition concerns, preferred health-improvement resources, and dietary/exercise routines was administered to CF adults. Clinical characteristics and survey responses are presented with descriptive statistics, and responses compared by body mass index (BMI) category (< 18.5 kg/m2; 18.5–24.99 kg/m2; 25–29.99 kg/m2; ≥30 kg/m2), gender, and socioeconomic status using Chi square or Fisher’s Exact testing. Results Of 66 total patients, nine (13.6%) were underweight (BMI < 18.5 kg/m2), while 19 (28.8%) were overweight or obese (BMI ≥ 25 kg/m2). In the overall cohort, the most common primary concern was preventing weight loss [in 20/66 patients (30.3%)], but there were significant differences by BMI (p < 0.001), with the most common concern in the overweight subgroup being preventing weight gain. Fifteen (46.9%) men (BMI mean 20.7, range 16.4–29.2 kg/m2) listed preventing weight loss as the primary concern, compared to only 5 (14.7%) women (BMI mean 18.4, range 16.2–19.9 kg/m2), representing a trend toward a difference in primary concerns by gender (p = 0.066). The most commonly desired health-improvement resource was online CF nutrition and fitness information, found in 26 patients (39.4%) in the overall cohort, without significant differences by BMI (p = 0.814) or gender (p = 0.199). Financial assistance was the preferred resource in 17 (26.2%), without differences by socioeconomic status (p = 0.367). Conclusions We identified a wide variety of nutritional needs in CF adults, including a high prevalence of overweight status, many patients desiring weight loss, and many seeking financial resources. Our findings support the individualization of modern-day CF nutrition programs and development of online resources, in an effort to address the heterogeneous barriers that exist in the contemporary CF population and improve outcomes in patients with the disease

Satisfaction and Effectiveness of Opioid Pain Management Among Adults with Cystic Fibrosis: A Mixed Methods Study

Background Chronic pain is common among people living with cystic fibrosis (CF) and associated with worse clinical outcomes. Despite this, little is known about how pain is managed and how opioids are used to treat pain. The purpose of this convergent mixed methods study was to examine self-reported satisfaction and effectiveness of pain management strategies among a sample of adults with CF who are prescribed opioids. Methods We developed an online survey querying 4 domains - demographics, pain characteristics, pain communication, and management strategies. This was distributed nationally to adults with CF (n=48) via various online platforms. We obtained quantitative and qualitative responses regarding satisfaction and effectiveness of pain management. Emerged themes from qualitative data were compared with responses from quantitative survey domains. Results Participants reported high levels of satisfaction and effectiveness with their opioid pain management plans. However, qualitative themes emerged regarding fears of addiction, experiences of feeling stigmatized by the healthcare system and ineffectiveness and inefficiency of alternative therapies for adequate pain relief. Conclusions Adults with CF reported opioids as an important component of their current pain management plans despite risks associated with opioid use. CF-specific pain management guideline development is warranted as is further research exploring pain development

A middle rate of failed extubation is desirable?: Questions unanswered (reply)

Cartas al editorRevisión por pare

A middle rate of failed extubation is desirable?: Questions unanswered (reply).

Cartas al editorRevisión por pare

Consensus document for the selection of lung transplant candidates:An update from the International Society for Heart and Lung Transplantation

Tens of thousands of patients with advanced lung diseases may be eligible to be considered as potential candidates for lung transplant around the world each year. The timing of referral, evaluation, determination of candidacy, and listing of candidates continues to pose challenges and even ethical dilemmas. To address these challenges, the International Society for Heart and Lung Transplantation appointed an international group of members to review the literature, to consider recent advances in the management of advanced lung diseases, and to update prior consensus documents on the selection of lung transplant candidates. The purpose of this updated consensus document is to assist providers throughout the world who are caring for patients with pulmonary disease to identify potential candidates for lung transplant, to optimize the timing of the referral of these patients to lung transplant centers, and to provide transplant centers with a framework for evaluating and selecting candidates. In addition to addressing general considerations and providing disease specific recommendations for referral and listing, this updated consensus document includes an ethical framework, a recognition of the variability in acceptance of risk between transplant centers, and establishes a system to account for how a combination of risk factors may be taken into consideration in candidate selection for lung transplantation.</p

International Society for Heart and Lung Transplantation consensus statement for the standardization of bronchoalveolar lavage in lung transplantation

Bronchoalveolar lavage (BAL) is a key clinical and research tool in lung transplantation (LTx). However, BAL collection and processing are not standardized across LTx centers. This International Society for Heart and Lung Transplantation-supported consensus document on BAL standardization aims to clarify definitions and propose common approaches to improve clinical and research practice standards. The following 9 areas are covered: (1) bronchoscopy procedure and BAL collection, (2) sample handling, (3) sample processing for microbiology, (4) cytology, (5) research, (6) microbiome, (7) sample inventory/tracking, (8) donor bronchoscopy, and (9) pediatric considerations. This consensus document aims to harmonize clinical and research practices for BAL collection and processing in LTx. The overarching goal is to enhance standardization and multicenter collaboration within the international LTx community and enable improvement and development of new BAL-based diagnostics