Anaesthesia for Adults with Cystic Fibrosis

Cystic fibrosis is an autosomal recessive disorder which affects one in 2500 live births. It is a multisystem disease and has a variety of presentations. The major clinical features affect the gastrointestinal and respiratory tracts. Severe respiratory disease, diabetes and gastroesophageal reflux are common features of concern to anaesthetists. Improved care of young patients has allowed many to survive into adulthood. Lung transplantation has significantly improved the outlook for many patients. At Alfred Hospital, 74 patients with cystic fibrosis underwent 149 procedures from January 1978 to January 1994, with a mortality of 0.6% (95% CI 0.4%-0.8%). This retrospective cohort study describes the anaesthetic management and perioperative care of these patients. Most of the anaesthetics were for procedures related to cystic fibrosis but 12% were for unrelated conditions. Cystic fibrosis related procedures include diagnostic, venous access, enteral feeding procedures, treatment of complications of cystic fibrosis and lung transplantation. Despite extremely poor respiratory function, these patients can be managed with acceptably low postoperative mortality (1%). Pre- and postoperative care must be directed towards optimal clearance of viscous respiratory secretions. Procedures need to be planned so that optimal care can be given by each member of the team caring for cystic fibrosis patients.

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A Roadmap to the Brittle Bones of Cystic Fibrosis

Journal of Osteoporosis ◽

10.4061/2011/926045 ◽

2011 ◽

Vol 2011 ◽

pp. 1-10 ◽

Cited By ~ 4

Author(s):

Ashwini P. Gore ◽

Soon Ho Kwon ◽

Antine E. Stenbit

Keyword(s):

Cystic Fibrosis ◽

Lung Transplantation ◽

Bone Disease ◽

Bone Health ◽

Autosomal Recessive ◽

Multidisciplinary Approach ◽

Early Recognition ◽

Autosomal Recessive Disorder ◽

Fatal Disease ◽

Brittle Bones

Cystic fibrosis (CF) is an autosomal recessive disorder which despite advances in medical care continues to be a life-limiting and often fatal disease. With increase in life expectancy of the CF population, bone disease has emerged as a common complication. Unlike the osteoporosis seen in postmenopausal population, bone disease in CF begins at a young age and is associated with significant morbidity due to fractures, kyphosis, increased pain, and decreased lung function. The maintenance of bone health is essential for the CF population during their lives to prevent pain and fractures but also as they approach lung transplantation since severe bone disease can lead to exclusion from lung transplantation. Early recognition, prevention, and treatment are key to maintaining optimal bone health in CF patients and often require a multidisciplinary approach. This article will review the pathophysiology, current clinical practice guidelines, and potential future therapies for treating CF-related bone disease.

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ECMO for Lung Transplantation in Cystic Fibrosis Patients

Case Medical Research ◽

10.31525/ct1-nct03919604 ◽

2019 ◽

Author(s):

Keyword(s):

Cystic Fibrosis ◽

Lung Transplantation

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Thoracoscopic Bilobectomy after Bilateral Single Sequential Lung Transplantation in a Cystic Fibrosis Patient with Post-Transplant Airway Stenosis

The Journal of Heart and Lung Transplantation ◽

10.1016/j.healun.2021.01.2042 ◽

2021 ◽

Vol 40 (4) ◽

pp. S503-S504

Author(s):

N. Mayer ◽

P. Perikleous ◽

G. Doukas ◽

J. Finch ◽

E. Beddow ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Transplantation ◽

Cystic Fibrosis Patient ◽

Airway Stenosis ◽

Post Transplant

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Outcomes Of Non‐Cystic Fibrosis Related Bronchiectasis Post Lung Transplantation

Internal Medicine Journal ◽

10.1111/imj.15256 ◽

2021 ◽

Author(s):

Jessica Kennedy ◽

Anne Walker ◽

Claire M. Ellender ◽

Kate Steinfort ◽

Catherine Martin ◽

...

Keyword(s):

Cystic Fibrosis ◽

Lung Transplantation

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1458. Uncharted territories: applying “precision medicine” to understand the treacherous landscape of extensively and multidrug resistant (XDR and MDR) Pseudomonas aeruginosa in a patient with cystic fibrosis and lung transplantation

Open Forum Infectious Diseases ◽

10.1093/ofid/ofaa439.1639 ◽

2020 ◽

Vol 7 (Supplement_1) ◽

pp. S731-S731

Author(s):

Laura J Rojas ◽

Mohamad Yasmin ◽

Jacquelynn Benjamino ◽

Steven Marshall ◽

Kailynn DeRonde ◽

...

Keyword(s):

Cystic Fibrosis ◽

Pseudomonas Aeruginosa ◽

Lung Transplantation ◽

Precision Medicine ◽

Clinical Sample ◽

Phylogenetic Reconstruction ◽

Multidrug Resistant ◽

Population Diversity ◽

Before And After ◽

Research Grant

Abstract Background Pseudomonas aeruginosa is a persistent and difficult-to-treat pathogen in many patients, especially those with cystic fibrosis (CF). Herein, we describe our experience managing a young woman suffering from CF with XDR P. aeruginosa who underwent lung transplantation. We highlight the contemporary difficulties reconciling the clinical, microbiological, and genetic information. Methods Mechanism-based-susceptibility disk diffusion synergy testing with double and triple antibiotic combinations aided in choosing tailored antimicrobial combinations to control the infection in the pre-transplant period, create an effective perioperative prophylaxis regimen, and manage recurrent infections in the post-transplant period. Thirty-six sequential XDR and PDR P. aeruginosa isolates obtained from the patient within a 17-month period, before and after a double-lung transplant were analyzed by whole genome sequencing (WGS) and RNAseq in order to understand the genetic basis of the observed resistance phenotypes, establish the genomic population diversity, and define the nature of sequence changes over time Results Our phylogenetic reconstruction demonstrates that these isolates represent a genotypically and phenotypically heterogeneous population. The pattern of mutation accumulation and variation of gene expression suggests that a group of closely related strains was present in the patient prior to transplantation and continued to evolve throughout the course of treatment regardless of antibiotic usage.Our findings challenge antimicrobial stewardship programs that assist with the selection and duration of antibiotic regimens in critically ill and immunocompromised patients based on single-isolate laboratory-derived resistant profiles. We propose that an approach sampling the population of pathogens present in a clinical sample instead of single colonies be applied instead when dealing with XDR P. aeruginosa, especially in patients with CF. Conclusion In complex cases such as this, real-time combination testing and genomic/transcriptomic data could lead to the application of true “precision medicine” by helping clinicians choose the combination antimicrobial therapy most likely to be successful against a population of MDR pathogens present. Disclosures Federico Perez, MD, MS, Accelerate (Research Grant or Support)Merck (Research Grant or Support)Pfizer (Research Grant or Support) Robert A. Bonomo, MD, Entasis, Merck, Venatorx (Research Grant or Support)

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Targeted Drug Delivery Technologies Potentiate the Overall Therapeutic Efficacy of an Indole Derivative in a Mouse Cystic Fibrosis Setting

Cells ◽

10.3390/cells10071601 ◽

2021 ◽

Vol 10 (7) ◽

pp. 1601

Author(s):

Matteo Puccetti ◽

Marilena Pariano ◽

Giorgia Renga ◽

Ilaria Santarelli ◽

Fiorella D’Onofrio ◽

...

Keyword(s):

Cystic Fibrosis ◽

Targeted Drug Delivery ◽

Fungal Infections ◽

Murine Models ◽

Multisystem Disease ◽

Aryl Hydrocarbon ◽

Target Organs ◽

Infection And Inflammation ◽

Anti Inflammatory ◽

Inflammatory Toxicity

Inflammation plays a major role in the pathophysiology of cystic fibrosis (CF), a multisystem disease. Anti-inflammatory therapies are, therefore, of interest in CF, provided that the inhibition of inflammation does not compromise the ability to fight pathogens. Here, we assess whether indole-3-aldehyde (3-IAld), a ligand of the aryl hydrocarbon receptor (AhR), may encompass such an activity. We resorted to biopharmaceutical technologies in order to deliver 3-IAld directly into the lung, via dry powder inhalation, or into the gut, via enteric microparticles, in murine models of CF infection and inflammation. We found the site-specific delivery of 3-IAld to be an efficient strategy to restore immune and microbial homeostasis in CF organs, and mitigate lung and gut inflammatory pathology in response to fungal infections, in the relative absence of local and systemic inflammatory toxicity. Thus, enhanced delivery to target organs of AhR agonists, such as 3-IAld, may pave the way for the development of safe and effective anti-inflammatory agents in CF.

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