Changing landscape: psychological care in the era of cystic fibrosis transmembrane conductance regulator modulators

Purpose of review The current review provides an overview of key psychological issues and challenges for the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator era of care. It discusses research from diagnosis and beyond, to patient-team communication with a particular focus on medical trials, adherence and living with CFTR modulators. Recent findings The impact of the diagnosis on parents is immense and the complexity of treatment now and in the future, are a challenge for both parents and teams. Communicating digitally is starting to become daily practice for many in CF care, with coronavirus disease 2019 accelerating this process. Participating in trials has a psychological impact, but most of all the (delayed) access and timing of accessing CFTR modulators is an important theme. Adherence remains of significance, both to ‘old’ and ‘new’ treatments. Living with CF in the era of CFTR modulators is beginning to impact on patients’ quality of life, including new possibilities, opportunities and challenges. Summary Psychological care needs to engage and keep pace with the rapid medical changes. Some care priorities remain the same, including psychological screening and assessment, as well as psychoeducation, communication training and psychotherapy. The presence of CF psychologist in the CF clinic remains as important as ever.
Source: Current Opinion in Pulmonary Medicine - Category: Respiratory Medicine Tags: CYSTIC FIBROSIS: Edited by Peter J. Barry and Barry J. Plant Source Type: research

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Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has disrupted clinical trials worldwide [1]. This could delay the approval of new medicines and reduce access to investigational treatments via clinical trials. This particularly impacts patients with rare diseases such as cystic fibrosis (CF).
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: Correspondence Source Type: research
(University of Leeds) A new and quicker method of diagnosing diseases in patients has been created by researchers. The team has developed a system of examining individual molecules to detect the presence of disease in blood.
Source: EurekAlert! - Infectious and Emerging Diseases - Category: Infectious Diseases Source Type: news
Severe acute respiratory syndrome – coronavirus-2 (SARS-CoV-2), has caused a pandemic of unknown example to date [1]. The World Health Organization described the disease caused by SARS-CoV-2 coronavirus disease 2019 (COVID-19) [2]. The COVID-19 pandemic impacted in health care systems worldwide, its long-term consequences are stil l to be seen. To date, predictors for the development of severe COVID-19 have not been well established; however, patients with chronic lung diseases among other risk factors are more likely to suffer [3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
Background: In COVID 19 related lung disease, which is a leading cause of death from this disease, cytokines like tumor necrosis factor-alpha (TNF alpha) may be pivotal in the pathogenesis. TNF alpha reduces fluid absorption due to impairment of sodium and chloride transport required for building an osmotic gradient across epithelial cells, which in the airways maintains airway surface liquid helping to keep airways open and enabling bacterial clearance and aids water absorption from the alveolar spaces. TNF alpha can, through Rho-kinase, disintegrate the endothelial and epithelial cytoskeleton, and thus break up intercell...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
BOSTON (CBS) — Harvard University researchers are working with two Boston hospitals to try a new way to help the sickest coronavirus patients. Boston Children’s Hospital and Brigham and Women’s Hospital will test a drug approved by the FDA to treat people with cystic fibrosis on those experiencing severe COVID-19 pneumonia and respiratory failure. The drug is called Dornase alfa, also known as DNase 1 or Pulmozyme, and it works to prevent lung infections by breaking up thick mucus. That may make oxygen easier to deliver via ventilator, researchers say. There’s also hope that the drug might break up ...
Source: WBZ-TV - Breaking News, Weather and Sports for Boston, Worcester and New Hampshire - Category: Consumer Health News Authors: Tags: Boston News Covid-19 Boston, MA Health Syndicated CBSN Boston Syndicated Local Boston Children's Hospital Brigham and Women's Hospital Coronavirus Harvard University Source Type: news
By now, we all know the drill: Maintain physical distance. Wear a mask. Wash your hands. Avoid people who are sick and stay away from others if you are sick. While these measures may seem simple enough, they are not easy to keep up month after month. Yet they are likely to be with us for a while. But what about those who cannot comply? Certain conditions can make the standard measures to stay safe during the pandemic seem impossible. At the same time, some of those likely to have the most trouble following the guidelines — such as older people with dementia — are at higher risk for illness and death if they do ...
Source: Harvard Health Blog - Category: Consumer Health News Authors: Tags: Alzheimer's Disease Anxiety and Depression Asthma Caregiving Coronavirus and COVID-19 Source Type: blogs
As our attention has been distracted to address issues related to the novel coronavirus (COVID-19) and we are hoping to be relieved of its disruption to our lives, we must remember that when it is all over, our patients and their families are still dealing with the consequences of cystic fibrosis (CF). All that we had been working on prior to the pandemic remains as important as before. Through all of this the Journal of Cystic Fibrosis (JCF) has continued to receive submitted manuscripts. We appreciate the dedication of our Associate Editors and the reviewers who have continued to respond to the work involved in the peer review process.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Editorial Source Type: research
The newly emergent human coronavirus (HCoV), known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has become a worldwide threat and the most severe public healthcare concern. The impact of SARS-CoV-2 on cystic fibrosis (CF) remains poorly understood, still there are serious concerns over its potential effect due to deficiencies in respiratory tract physiology of CF patients that prevent efficient clearance of pathogens from the airways. SARS-CoV-2 infections have been observed in few CF patients and were associated to a not severe infection [1].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
The newly emergent human coronavirus (HCoV), known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has become a worldwide threat and the most severe public healthcare concern. The impact of SARS-CoV-2 on cystic fibrosis (CF) remains poorly understood, still there are serious concerns over its potential effect due to deficiencies in respiratory tract physiology of CF patients that prevent efficient clearance of pathogens from the airways. SARS-CoV-2 infections have been observed in few CF patients and were associated to a not severe infection [1].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
AbstractObjectivesSevere Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) may trigger severe pneumonia in coronavirus disease of 2019 (COVID-19) patients through release of damage-associated molecular patterns (DAMPs) and recruitment of neutrophils in the lungs. Activated neutrophils induce inflammation and severe alveolar injury by releasing neutrophil extracellular traps (NETs). The backbones of many DAMPs and NETs are made of extracellular, cell-free DNA decorated with highly toxic compounds such as elastase, myeloperoxidase and citrullinated histones. Dornase alfa is a FDA-approved recombinant human DNAse 1 for th...
Source: Trials - Category: Research Source Type: clinical trials
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