While originally characterized as a collection of related syndromes, cystic fibrosis (CF) is now recognized as a single disease whose diverse symptoms stem from the wide tissue distribution of the gene product that is defective in CF, the ion channel and regulator, cystic fibrosis transmembrane conductance regulator (CFTR). Defective CFTR protein impacts the function of the pancreas and alters the consistency of mucosal secretions. The latter of these effects probably plays an important role in the defective resistance of CF patients to many pathogens. As the modalities of CF research have changed over the decades from empirical histological studies to include biophysical measurements of CFTR function, the clinical management of this disease has similarly evolved to effectively address the ever-changing spectrum of CF-related infectious diseases. These factors have led to the successful management of many CF-related infections with the notable exception of chronic lung infection with the gram-negative bacterium Pseudomonas aeruginosa. The virulence of P. aeruginosa stems from multiple bacterial attributes, including antibiotic resistance, the ability to utilize quorum-sensing signals to form biofilms, the destructive potential of a multitude of its microbial toxins, and the ability to acquire a mucoid phenotype, which renders this microbe resistant to both the innate and acquired immunologic defenses of the host.

Lung Infections Associated with Cystic Fibrosis

This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and microbiology of pulmonary disease in cystic fibrosis (CF). The molecular basis of CF lung disease including the impact of defective cystic fibrosis transmembrane regulator (CFTR) protein function on airway physiology, mucociliary clearance, and establishment of Pseudomonas aeruginosa infection is described. An extensive review of the microbiology of CF lung disease with particular reference to infection with P. aeruginosa is provided. Other pathogens commonly associated with CF lung disease including Staphylococcal aureus, Burkholderia cepacia, Stenotrophomonas maltophilia, Achromobacter xylosoxidans and atypical mycobacteria are also described. Clinical presentation and assessment of CF lung disease including diagnostic microbiology and other measures of pulmonary health are reviewed. Current recommendations for management of CF lung disease are provided. An extensive review of antipseudomonal therapies in the settings of treatment for early P. aeruginosa infection, maintenance for patients with chronic P. aeruginosa infection, and treatment of exacerbation in pulmonary symptoms, as well as antibiotic therapies for other CF respiratory pathogens, are included. In addition, the article discusses infection control policies, therapies to optimize airway clearance and reduce inflammation, and potential future therapies.

Pathophysiology and management of pulmonary infections in cystic fibrosis.

The emergence of multidrug-resistant gram-negative bacteria and the lack of new antibiotics to combat them have led to the revival of polymyxins, an old class of cationic, cyclic polypeptide antibiotics. Polymyxin B and polymyxin E (colistin) are the 2 polymyxins used in clinical practice. Most of the reintroduction of polymyxins during the last few years is related to colistin. The polymyxins are active against selected gram-negative bacteria, including Acinetobacter species, Pseudomonas aeruginosa, Klebsiella species, and Enterobacter species. These drugs have been used extensively worldwide for decades for local use. However, parenteral use of these drugs was abandoned approximately 20 years ago in most countries, except for treatment of patients with cystic fibrosis, because of reports of common and serious nephrotoxicity and neurotoxicity. Recent studies of patients who received intravenous polymyxins for the treatment of serious P. aeruginosa and Acinetobacter baumannii infections of various types, including pneumonia, bacteremia, and urinary tract infections, have led to the conclusion that these antibiotics have acceptable effectiveness and considerably less toxicity than was reported in old studies.

Colistin: The Revival of Polymyxins for the Management of Multidrug-Resistant Gram-Negative Bacterial Infections

The diagnosis, investigation and particularly management of bronchiectasis has been largely empirical and the subject of relatively few controlled clinical trials. There are no clear guidelines, although an Australian position statement has been published concerning bronchiectasis in children. The purposes of these guidelines were therefore threefold: (1) to identify relevant studies in non-cystic fibrosis (CF) bronchiectasis; (2) to provide guidelines on management based on published studies where possible or a consensus view; and (3) to identify gaps in our knowledge and identify areas for future study.

https://thorax.bmj.com/content/thoraxjnl/65/Suppl_1/i1.full.pdf

British Thoracic Society guideline for non-CF bronchiectasis.

Cystic Fibrosis Adult Care: Consensus Conference Report

BACKGROUND: Cystic fibrosis is an inherited life-limiting disorder, characterised by pulmonary infections and thick airway secretions. Chest physiotherapy has been integral to clinical management in facilitating removal of airway secretions. Conventional chest physiotherapy techniques (CCPT) have depended upon assistance during treatments, while more contemporary airway clearance techniques are self-administered, facilitating independence and flexibility. OBJECTIVES: To compare CCPT with other airway clearance techniques in terms of their effects on respiratory function, individual preference, adherence, quality of life and other outcomes. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register which comprises references identified from comprehensive electronic database searches and handsearching of relevant journals and abstract books of conference proceedings. We also searched CINAHL from 1982 to 2002 and AMED from 1985 to 2002. Date of most recent search: January 2004. SELECTION CRITERIA: Randomised or quasi-randomised clinical trials including those with a cross-over design where CCPT was compared with other airway clearance techniques. Studies of less than seven days duration were excluded. DATA COLLECTION AND ANALYSIS: Two reviewers allocated quality scores to relevant studies and independently extracted data. If we were unable to extract data, we invited authors to submit their data. We excluded studies from meta-analysis when data were lost or study design precluded comparison. For some continuous outcomes, we used the generic inverse variance method for meta-analysis of data from cross-over trials and data from parallel-designed trials were incorporated for comparison. We also examined efficacy of specific techniques and effects of treatment duration. MAIN RESULTS: Seventy-eight publications were identified by the searches. Twenty-nine of these were included, representing 15 data sets with 475 participants. There was no difference between CCPT and other airway clearance techniques in terms of respiratory function measured by standard lung function tests. Studies undertaken during acute exacerbations demonstrated relatively large gains in respiratory function irrespective of airway clearance technique. Longer-term studies demonstrated smaller improvements or deterioration over time. Ten studies reported individual preferences for technique, with participants tending to favour self-administered techniques. Heterogeneity in the measurement of preference precluded these data from meta-analysis. AUTHORS' CONCLUSIONS: This review demonstrated no advantage of CCPT over other airway clearance techniques in terms of respiratory function. There was a trend for participants to prefer self-administered airway clearance techniques. Limitations of this review included a paucity of well-designed, adequately-powered, long-term trials.

/pdf/conventional-chest-physiotherapy-compared-to-other-airway-1d3w0fuek3.pdf

Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis.

“One case which I had was that of a newborn child with acquired atelectasis, which required my presence, or that of my assistant, for 24 hours. In such cases there is nothing, I believe, as efficacious as flagellation. I usually tell the attendant to take a rubber band and flip the soles of the feet whenever the child begins to tire of breathing.” 

Dr Sanders. In: Disturbances of respiration in the newborn. Transactions of the American Pediatric Society 1903; 15 :47.

At the beginning of this century, paediatrics was an art. Skills were learnt from a mentor, picking up tips and anecdotes while standing at your master’s side. Now, as practitioners of child health in the final years of this same century, life has changed. Anecdote and word of mouth have lost credibility and are replaced by scientific scrutiny and the rigour of evidence from carefully controlled and sufficiently powerful trials. We do our best to find the truth, but in many areas of care there remains a dearth of sufficient evidence. Often in the closets of our own practices, we continue to do what our teachers taught us and what, over time, we believe works.

The central function of chest physiotherapy in paediatric respiratory disease is to assist in the removal of tracheobronchial secretions. The intention is to remove airway obstruction, reduce airway resistance, enhance gas exchange, and reduce the work of breathing. In the acute situation, recovery should be hastened and in the child with a chronic respiratory disorder, the progression of the lung disease is hopefully delayed.

Chest physiotherapy can improve a patient’s respiratory status and expedite recovery. But in certain situations it may be a useless intervention or even harmful—perhaps by increasing bronchospasm, inducing pulmonary hypertension, repositioning a foreign body, or destabilising a sick infant. What …

https://adc.bmj.com/content/archdischild/80/4/393.full.pdf

Who needs chest physiotherapy? Moving from anecdote to evidence

Nebulised colistin is regularly used as antipseudomonal therapy in children with cystic fibrosis. We assessed bronchoconstriction in response to nebulised colistin in 58 children. Nebulised colistin significantly reduced FEV1 ,M EF25%, and SaO2 for 15 minutes. In 20 children the reduction was greater than 10% from baseline FEV1, and was still at that level in five at 30 minutes. Subjective assessment, baseline FEV1, and serum IgE were unable to identify susceptible children. It is recommended that children receiving colistin should be carefully assessed for bronchoconstriction. (Arch Dis Child 2001;84:432‐433)

https://adc.bmj.com/content/archdischild/84/5/432.full.pdf

Bronchoconstriction following nebulised colistin in cystic fibrosis

Conventional chest physiotherapy compared to other forms of chest physiotherapy for cystic fibrosis

Adolescent FEV 1 decline in cystic fibrosis may be an artefact of the spirometry reference equations used http://ow.ly/J0com

https://erj.ersjournals.com/content/erj/46/1/262.full.pdf

Global Lung Function Initiative equations improve interpretation of FEV1 decline among patients with cystic fibrosis.

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