Purpose Of Review: With improving life expectancy and quality of life, sexual and reproductive health (SRH) has become an increasingly important aspect of patient-centered cystic fibrosis care. This review aims to describe advances in cystic fibrosis-related SRH and highlight optimal practices.
Recent Findings: Recent publications suggest that people with cystic fibrosis follow a similar trajectory of sexual development and activity as their noncystic fibrosis peers, although contraception use is lower. Although fertility is reduced in patients with cystic fibrosis, improved survival and assisted reproductive technologies have led to an increasing pursuit and incidence of pregnancy. Cystic fibrosis transmembrane regulator modulators that correct the underlying cystic fibrosis defect might improve fertility and thus far appear safe in pregnancy, though data are limited.Despite medical knowledge of SRH in cystic fibrosis, patients continue to report they lack sufficient education about these aspects of their healthcare, and cystic fibrosis multidisciplinary teams are ill prepared to counsel their patients.
Summary: Understanding of the effects of cystic fibrosis on SRH continues to improve, although many questions remain regarding optimal care from the choice of contraception to the safety of cystic fibrosis-specific medications in pregnancy. Further development of cystic fibrosis-informed interdisciplinary specialist networks and a wider framework of practice would both enhance health outcomes and better support patients.
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http://dx.doi.org/10.1097/MCP.0000000000000731 | DOI Listing |
Background: Studies showed significant physical improvement after starting elexacaftor/tezacaftor/ivacaftor (ETI). However, some patients reported new mental health symptoms.
Aim: This study explores the impact of ETI on end-stage cystic fibrosis patients, focusing on mental health.
Sci Rep
January 2025
CEINGE-Biotecnologie avanzate Franco Salvatore, Via G. Salvatore 486, Naples, 80145, Italy.
The development of targeted therapies that correct the effect of mutations in patients with cystic fibrosis (CF) and the relevant heterogeneity of the clinical expression of the disease require biomarkers correlated to the severity of the disease useful for monitoring the therapeutic effects. We applied a targeted metabolomic approach by LC-MS/MS on saliva samples from 70 adult CF patients and 63 age/sex-matched controls to investigate alterations in metabolic pathways related to pancreatic insufficiency (PI), Pseudomonas aeruginosa (PA) colonization, CF liver disease (CFLD), and CF related diabetes (CFRD). Sixty salivary metabolites were differentially expressed, with 11 being less abundant and 49 more abundant in CF patients.
View Article and Find Full Text PDFA 3-year-old girl treated with intravenous chemotherapy for bilateral retinoblastoma (RB) and a standard technique of intravitreal topotecan for vitreous seeds in the left eye developed a conjunctival nodule at the injection site. Ultrasound biomicroscopy showed normal underlying sclera and ciliary body. Fundus examination of the left eye showed partly calcified vitreous seeds.
View Article and Find Full Text PDFAnn Am Thorac Soc
January 2025
University of Washington, Genomic Sciences, Seattle, Washington, United States.
Rationale: Chronic () airway infection is common and a key contributor to diminished lung function and early mortality in persons with cystic fibrosis (PwCF). Risk factors for chronic among PwCF include cystic fibrosis transmembrane conductance regulator genotype, genetic modifiers, and environmental factors. Intensive antibiotic therapy and highly effective modulators do not eradicate in most adolescents and adults with cystic fibrosis.
View Article and Find Full Text PDFTuberk Toraks
December 2024
Division of Pediatric Pulmonology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Türkiye.
Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction observed in asthma as well as cystic fibrosis (CF) patients due to the colonization of the airways by Aspergillus fumigatus. While ABPA is most commonly observed in CF patients (2-9%), it is seen at a rate of 1-2% in patients diagnosed with asthma. ABPA is mostly seen in steroid dependent adult asthma patients and has rarely been reported in pediatric asthma patients.
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