This paper focused on the effectiveness of a parent empowerment intervention based on nursing education (PEINE). This study examined whether the intervention improved the quality of life of children with cystic fibrosis (CF) and improved their caregivers learn about the disease and develop problem-solving and coping skills. This randomized-controlled trial used a pretest-posttest parallel-group research design. The sample consisted of 48 parents (caregivers) of children with CF. Participants were randomly assigned to an intervention ( = 24) and a control group ( = 24). The intervention group received PEINE and standard care and treatment for ten weeks. The control group received standard care and treatment. Data were collected using a Disease Information Survey (DIS), the Ways of Coping Inventory (WCI), the Problem-Solving Inventory (PSI), and the Cystic Fibrosis Questionnaire (CFQ-R). After the intervention, the intervention group DIS scores (d: 1,627 [CI: 0.934,2.305], had more correct answers than the control group. Nursing interventions were effective ( < .001). There was no significant difference in the mean pretest-posttest PSI scores (d: 0.378 [CI: -0.221-0.972], posttest WCI scores (d: 0.239 [CI:-0.356-0.831]) between the intervention and control groups (>.05). There was a significant difference in the mean posttest CFQ-R scores between the intervention and control groups (d: 1.363 [CI: l.698, 2.015]);( < .001). PEINE increased the intervention group participants develop disease-management skills. However, the increase in their PSI and WCI scores was statistically insignificant. PEINE also increased the quality of life of children with CF. Parents of children followed in pediatric pulmonary diseases participated in the study. Parents were informed during outpatient clinic visits. After the first meeting, the children and parents who voluntarily agreed to participate in the research were contacted by phone. The outpatient nurse assisted in communicating with children and parents.
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http://dx.doi.org/10.1080/24694193.2024.2411986 | DOI Listing |
Infection
January 2025
Department of Thoracic Surgery, Saarland University, 66421, Homburg/Saar, Germany.
Background: Lung transplantation is the ultimate treatment option for patients with advanced cystic fibrosis. Chronic colonization of these recipients with multidrug-resistant (MDR) pathogens may constitute a risk factor for an adverse outcome. We sought to analyze whether colonization with MDR pathogens, as outlined in the German classification of multiresistant Gram-negative bacteria (MRGN), was associated with the success of lung transplantation.
View Article and Find Full Text PDFEur J Pediatr
January 2025
Service de Physiologie Pédiatrique-Centre du Sommeil-CRMR Hypoventilations Alvéolaires Rares, INSERM NeuroDiderot, Université Paris-Cité, AP-HP, Hôpital Robert Debré, Paris, France.
Unlabelled: It is known that in most cases of congenital central hypoventilation syndrome (CCHS), apnoeas and hypoventilation occur at birth. Nevertheless, a detailed description of initial symptoms, including pregnancy events and diagnostic tests performed, is warranted in infants with neonatal onset of CCHS, that is, in the first month of life. The European Central Hypoventilation Syndrome Consortium created an online patient registry from which 97 infants (44 females) with CCHS of neonatal onset and PHOX2B mutation from 10 countries were selected.
View Article and Find Full Text PDFJ Med Chem
January 2025
AbbVie Inc., 1 North Waukegan Road, North Chicago, Illinois 60064, United States.
Cystic fibrosis (CF) is a genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which regulates ion and fluid transport across epithelial cells. Mutations lead to complications, with life-limiting lung disease being the most severe manifestation. Traditional treatments focused on managing symptoms, but advances in understanding CF's molecular basis led to small-molecule CFTR modulators.
View Article and Find Full Text PDFInt J Gen Med
January 2025
Department of Respiratory and Sleep Medicine, Department of Internal Medicine, School of Medicine, The University of Jordan, Amman, Jordan.
Background: Bronchiectasis, a respiratory ailment, significantly impacts the life expectancy of individuals. This study aimed to explore the prevalence of multidrug-resistant organisms (MDROs) among bronchiectasis patients, the resistance patterns within various antibiotic classes, and the associated factors with these organisms.
Methods: A retrospective observational analysis was conducted on adult bronchiectasis patients attending clinics at Jordan University Hospital.
Case Rep Anesthesiol
January 2025
Department of Medicine, University of California Riverside School of Medicine, Riverside, California, USA.
Recent advancements in therapeutics and risk reduction in the management of cystic fibrosis have increased the life expectancy of cystic fibrosis patients to the fifth decade of life. As the life expectancy of cystic fibrosis patients has increased, more cystic fibrosis patients have opted to pursue pregnancy. Normal pregnancy is accompanied by physiological changes that affect anesthetic management.
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