by Deborah S. Boroughs, RN, MSN, PA and Joan A. Dougherty, RN, BSN, CPN, CSN and Zoe A. Davies, RN, MSN, NP
Cystic fibrosis is a genetic disorder. It is a chronic, multisystem disorder that reduces life expectancy. The primary manifestations include progressive obstructive lung disease and pancreatic insufficiency, but patients also may suffer from chronic sinusitis, nasal polyps, malnutrition, distal intestinal obstructive syndrome, diabetes, liver cirrhosis, pancreatitis, gallstones and infertility. To have cystic fibrosis, a child must inherit two abnormal cystic fibrosis genes, one from each parent. The parents usually have just one abnormal gene, are asymptomatic and are called carriers. Each time two carriers have a child, there is a 25% chance the baby will have cystic fibrosis, a 50% chance the baby will be a carrier and a 25% chance the baby will be healthy. This module provides updated information on the treatment and management of people with cystic fibrosis.
The goal of this cystic fibrosis continuing education module is to help nurses and physical therapists gain a deeper understanding of cystic fibrosis and the treatments available, as well as to become informed about the latest research. After studying the information presented here, you will be able to:
- Describe the basic defect that causes cystic fibrosis and how it affects different organ systems
- Identify three therapies for the management of pulmonary and GI disease in patients with cystic fibrosis
- Explain teaching and support responsibilities
- Discuss research on and the future of cystic fibrosis
This course is available free of charge until 11/30/13 at midnight EDT.