Nebuliser systems for drug delivery in cystic fibrosis.

Background: Nebuliser systems are used to deliver medications to the lungs, to control the symptoms and the progression of lung disease in people with cystic fibrosis (CF). There are many different nebulised-medications prescribed for people with CF and there are many different types of nebuliser systems. Some of these nebulised medications are licenced for, and can be taken via only one type of nebuliser system; some are licensed for, and can be taken via more than one type of nebuliser system.

Role of the Physical Therapist in Cystic Fibrosis Care.

In looking back on 2020 and 2021, this Perspective reflects on the monumental impacts of the rollout of cystic fibrosis (CF) transmembrane conductance regulator highly effective modulator therapies and the COVID-19 pandemic on the management of CF. Advancements in the clinical management of people with CF have been both enormous and rapid, and physical therapists specializing in the care of people with CF have been at the forefront of driving this evolution in care. This year sees the 30th anniversary of the UK Association of Chartered Physiotherapists in Cystic Fibrosis and, as is inevitable in reaching such milestones, thoughts have turned to origins, role, impacts, and the future.

The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus.

Background: The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such guidelines.

Method: On 30 June and 1 July 2021, 39 stakeholders from 11 countries, including researchers, healthcare professionals and patients participated in a virtual conference to agree an evidence-based and informed expert consensus about PA and exercise for people with CF.

Clinimetric analysis of outcome measures for airway clearance in people with cystic fibrosis: a systematic review.

Background: Airway clearance techniques (ACTs) are integral to cystic fibrosis (CF) management. However, there is no consensus as to which outcome measures (OMs) are best for assessing ACT efficacy.

Objectives: To summarise OMs that have been assessed for their clinimetric properties (including validity, feasibility, reliability, and reproducibility) within the context of ACT research in CF.

Autogenic drainage for airway clearance in cystic fibrosis.

Background: Autogenic drainage is an airway clearance technique that was developed by Jean Chevaillier in 1967. The technique is characterised by breathing control using expiratory airflow to mobilise secretions from smaller to larger airways. Secretions are cleared independently by adjusting the depth and speed of respiration in a sequence of controlled breathing techniques during exhalation.

Respiratory muscle training for cystic fibrosis.

Background: Cystic fibrosis is the most common autosomal recessive disease in white populations, and causes respiratory dysfunction in the majority of individuals. Numerous types of respiratory muscle training to improve respiratory function and health-related quality of life in people with cystic fibrosis have been reported in the literature. Hence a systematic review of the literature is needed to establish the effectiveness of respiratory muscle training (either inspiratory or expiratory muscle training) on clinical outcomes in cystic fibrosis.

Investigating outcome measures for assessing airway clearance techniques in adults with cystic fibrosis: protocol of a single-centre randomised controlled crossover trial.

Introduction: Airway clearance techniques (ACTs) are a gold standard of cystic fibrosis management; however, the majority of research evidence for their efficacy is of low standard; often attributed to the lack of sensitivity from outcome measures (OMs) used historically. This randomised controlled trial (RCT) investigates these standard OMs (sputum weight, forced expiratory volume in 1 s) and new OMs (electrical impedance tomography (EIT), multiple breath washout (MBW) and impulse oscillometry (IOS)) to determine the most useful measures of ACT.

Methods And Analysis: This is a single-centre RCT with crossover design.

Pulmonary Exacerbations in Adults With Cystic Fibrosis: A Grown-up Issue in a Changing Cystic Fibrosis Landscape.

Pulmonary exacerbations (PExs) are significant life events in people with cystic fibrosis (CF), associated with declining lung function, reduced quality of life, hospitalizations, and decreased survival. The adult CF population is increasing worldwide, with many patients surviving prolonged periods with severe multimorbid disease. In many countries, the number of adults with CF exceeds the number of children, and PExs are particularly burdensome for adults as they tend to require longer courses and more IV treatment than children.

Randomised cross-over trial evaluating the short-term effects of non-invasive ventilation as an adjunct to airway clearance techniques in adults with cystic fibrosis.

Introduction: Non-invasive ventilation (NIV) is used in cystic fibrosis (CF) to support airway clearance techniques (ACTs) by augmenting tidal volumes and reducing patient effort. However, the evidence base for this is limited. We hypothesised that NIV, in addition to usual ACT, would increase sputum clearance.

Positive pressure--analysing the effect of the addition of non-invasive ventilation (NIV) to home airway clearance techniques (ACT) in adult cystic fibrosis (CF) patients.

Background: There is no published literature on the frequency of use of non-invasive ventilation (NIV) with airway clearance techniques (ACT) throughout the cystic fibrosis (CF) population; 3.9% (191 people of 5062 registered) of the United Kingdom CF population older than 16 years are reported to use NIV in registry data; however, it is not specified if this is for ACT or respiratory failure. Using NIV with ACT decreases work of breathing and fatigue during in-patient admissions for CF patients.

The expanding armamentarium of drugs to aid sputum clearance: how should they be used to optimize care?

Purpose Of Review: This review examines the evidence for use of agents that improve sputum clearance in cystic fibrosis.

Recent Findings: The basic defect in cystic fibrosis causes abnormal airway surface liquid leading to mucus plugging and subsequent infection and inflammation in the airways. Agents that improve clearance of secretions should improve lung function, reduce infective exacerbations and may enhance survival.

An assessment of a short composite questionnaire designed for use in an interventional spine pain management setting.

Objective: To evaluate the reliability, validity, responsiveness, and practicality of a short composite questionnaire designed for use in an interventional spine pain management setting.

Setting: A rural interventional spine pain management practice.

Patients: Sixty subjects undergoing spinal intervention procedures (injection or neurotomy).

Is it useful to repeat sacroiliac joint provocative tests post-block?

Objective: To evaluate the diagnostic usefulness of repeating sacroiliac joint (SIJ) provocative tests post-block.

Design: Thirty-four patients with suspected unilateral mechanical SIJ pain participated. Eleven had confirmed SIJ origin pain (>79% pain relief with fluoroscopically guided comparative local anesthetic intra-articular blocks), whereas 23 were confirmed not to have SIJ origin pain (<80% pain relief with a single local anesthetic intra-articular block).

The effect of repeated zygapophysial joint radiofrequency neurotomy on pain, disability, and improvement duration.

Objective: To assess the effectiveness of repeated radiofrequency neurotomy (RFN) on pain, disability, and treatment effect duration.

Patients: One hundred-four patients who underwent repeat RFN for chronic neck or back pain were prospectively followed using a Pain Disability Questionnaire-Spine (PDQ-S). Complete data sets were available for 73, 73, and 36 patients for the 1st, 2nd, and 3rd RFN, respectively.

An evaluation of the dyslexia training program: a multisensory method for promoting reading in students with reading disabilities.

The development of reading and spelling skills in students with dyslexia, by definition, is delayed and often remains delayed despite years of instruction. Three qualities are thought to facilitate reading development in these children: the provision of a highly structured phonetic-instruction training program with heavy emphasis on the alphabetic system, drill and repetition to compensate for short-term verbal memory deficits, and multisensory methods to promote nonlanguage mental representations. The Dyslexia Training Program, a remedial reading program derived from Orton-Gillingham methods, embodies these qualities.

Developmental and transgenic analysis of two tomato fruit enhanced genes.

Tomato fruit development is characterized by distinct developmental stages: fruit set, periods of rapid cell division and cell expansion, and the period where processes associated with ripening are dominant. During each of these stages, different aspects of cellular metabolism are favored. Accompanying these developmental changes are dramatic differences in gene expression, with a subset of genes being expressed early and a subset being expressed later in development.

The stress response to trauma and critical illness.

The stress response is a protective system that defends the body from external and internal threats. When the body is confronted with an over-whelming threat, the response becomes uncontrolled, resulting in damage to the body and frequently death. Knowledge of the strengths and pitfalls of this system is important in understanding the responses of the body to trauma and critical illness.

Effect of interferon gamma on infection-related death in patients with severe injuries. A randomized, double-blind, placebo-controlled trial.

Objective: To assess the efficacy of interferon gamma in reducing infection and death in patients sustaining severe injury.

Design: Multicenter, randomized, double-blind, placebo-controlled trial with observation for 60 days and until discharge for patients with major infection on day 60.

Setting: Nine university-affiliated level 1 trauma centers.

Documentation of clinical interventions in nutritional support.

The implementation of this documentation system has been a success. The SPF pharmacists' participation has been excellent. All of the original objectives have been met.

Beyond honesty: choosing language for talking to children about pain and procedures.

Gene Stanford, long an influential and colorful member of the Association for the Care of Children's Health (ACCH), died on February 25, 1991, after a long illness. His presentation, "Talking to Children about Medical Procedures," at the 1985 ACCH conference in Boston was heard by and has influenced the thinking and clinical skills of many ACCH members. As an author and coauthor of many publications addressing communication skills and the needs of children in health care, it is fitting that his memorable presentation in 1985 incorporated both areas of Gene's expertise.

Adverse effect of therapeutic vasoconstrictors in experimental acute pancreatitis.

Alpha-adrenergic drugs commonly are used to treat hypotension resulting from severe acute pancreatitis. It was shown previously that although systemic arterial pressure is increased by phenylephrine, pancreatic microcirculatory perfusion is decreased. Because inadequate tissue perfusion may be critical in the progression of edematous pancreatitis to parenchymal necrosis, it was hypothesized that vasoconstrictors might be harmful in pancreatitis.

Use of inotropic agents in critical illness.

Inotropic agents are useful in increasing oxygen delivery in critically ill patients. The need for inotropic support requires careful assessment of all the available cardiovascular variables. Following the decision to stimulate contractility, the choice of inotrope should take into consideration the adrenergic receptor populations and their effects on the distribution of blood flow.

Factors affecting morbidity following hepatic trauma. A prospective analysis of 482 injuries.

During a 5-year period, 482 patients with liver injuries were studied prospectively: 65% resulted from penetrating and 35% from blunt injuries. The injuries were graded by the hepatic injury scale (grades I to VI); transfusion requirements and perihepatic abscesses correlated with increasing scores. Minor surgical techniques were needed in 338 patients and 144 patients required major techniques.