探索对CFTR调节器的看法和决策.
Lee Landess1, Mary G Prieur2, Ashley R Brown2
1University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA.
Pediatric pulmonology
|March 8, 2024
概括
囊性纤维化跨膜调节器 (CFTR) 调节器启动需要深思熟虑的讨论,解决患者的情绪和知识差距. 护理团队应支持所有患者,包括那些不符合调节器资格的患者.
科学领域:
- 肺部病理学 肺部病理学
- 医学遗传学 医学遗传学
- 患者护理 患者护理
背景情况:
- 囊性纤维化 (CF) 治疗越来越多地使用高效的调节器.
- 对CF护理团队关于教育患者关于调节器启动的指导是有限的.
- 这项研究探讨了患者,护理人员和临床医生的CFTR调节器观点.
研究的目的:
- 探索患者,护理人员和临床医生对CFTR调节器的看法.
- 为了确定关于开始CFTR调节器治疗的决定的影响.
- 为CF护理团队在讨论调节器启动时提供最佳实践信息.
主要方法:
- 与CF临床医生,CF成年人和CF儿童的照顾者进行半结构化采访.
- 通过编码响应来识别中心主题,分析了采访录音.
- 这项研究包括8名CF临床医生,9名CF成年人和11名护理人员.
主要成果:
- 关键主题包括情绪反应,决策影响,副作用问题以及与调节器相关的未来规划.
- 临床医生注意到,在elexacaftor/tezacaftor/ivacaftor获得批准后,处方对话发生了变化.
- 多学科的CF护理团队在支持治疗决策方面发挥着至关重要的作用.
- 还确定了无法接受调节器的患者的独特需求.
结论:
- 临床医生应该对CFTR调节器进行彻底和深思熟虑的对话.
- 利用多学科护理模式来解决患者/护理人员的情绪和知识差距.
- 讨论除了临床益处之外的治疗目标,并为不符合条件的患者提供富有同情心的支持.
相关概念视频
Cystic Fibrosis: Management
164
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
164
Cystic Fibrosis: Pathogenesis
239
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
239
Antiasthma Drugs: Leukotriene Modifiers
298
Leukotriene modifiers, or cysteinyl leukotriene receptor antagonists, are medications used to manage chronic asthma. These agents target specific inflammatory mediators produced during arachidonic acid metabolism, an essential process in generating inflammation in the body.
Leukotriene modifiers work through two distinct mechanisms:
Leukotriene modifiers work through two distinct mechanisms:
298
Drugs for Treatment of Constipation-Predominant IBS
163
Pharmacological therapies for IBS-C are designed to alleviate abdominal discomfort and enhance bowel function. In patients with IBS-C, fiber supplements may help soften stools and decrease straining, but may also lead to increased gas production and bloating. Osmotic laxatives like milk of magnesia are frequently used to soften stools and increase stool frequency in IBS-C patients. In addition, two drugs approved for use in severe IBS-C adult cases are linaclotide (Linzess) and lubiprostone...
163
Factors Affecting Perception
1.6K
Perception is influenced by perceptual set, context, motivation, and emotion. Perceptual set, or perceptual expectancy, refers to the tendency to perceive things in a particular way, influenced by previous experiences and expectations. This phenomenon affects the interpretation of stimuli, creating a set of mental tendencies and assumptions that impact sensory perceptions of sound, taste, touch, and sight.
An illustrative example of a perceptual set is the scenario where an airline pilot told...
An illustrative example of a perceptual set is the scenario where an airline pilot told...
1.6K
Drugs Affecting GI Tract Motility: Serotonin Receptor Agonists
252
Serotonin, a crucial neurotransmitter synthesized by enterochromaffin cells, plays a cardinal role in regulating gastrointestinal (GI) motility. With over 90% of the body's total serotonin in the GI tract, its influence on digestive processes is profound. Serotonin is swiftly released upon various stimuli, such as food boluses or certain drugs, triggering intrinsic sensory neurons in the myenteric plexus and extrinsic vagal and spinal sensory neurons. This leads to the activation of the...
252


