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Psychosocial Issues In Cystic Fibrosis, Part 1. Susan Horky, LCSW University of Florida Pediatric Pulmonary Center. We’ll be talking about:. What cystic fibrosis is How families feel At the time of diagnosis Over time. What is CF?. http://www.medicalnewstoday.com/articles/147960.php.
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Psychosocial Issues In Cystic Fibrosis, Part 1 Susan Horky, LCSW University of Florida Pediatric Pulmonary Center
We’ll be talking about: • What cystic fibrosis is • How families feel • At the time of diagnosis • Over time
What is CF? http://www.medicalnewstoday.com/articles/147960.php (Then scroll down) Cystic Fibrosis is a life-shortening chronic illness...
An abnormal gene causes problems in the transport of sodium and chloride across cell membranes Because salt can’t cross into the airway lining, water doesn’t follow, leading to thick, sticky mucus
The genetic defect results from problems with CFTR • CFTR (Cystic Fibrosis Transmembrane conductance Regulator) is a protein that creates a chloride channel • Chloride flows out of the cell through the center of the channel and water follows behind it • The water keeps mucus thin so it can move out of the lungs.
CFTR Function Non-CF CF
Categories of Defect in CFTR • Class I-Nonsense gene, Insufficient CFTR made • Class II-Folding problem, CFTR can’t get to cell surface • Class III- Gating problem, CFTR gets to cell surface but is largely inactive • Class IV-CFTR Gating problem CFTR gets to cell surface but works inadequately • Class V- Trafficking problem: Not enough CFTR gets to cell surface • Class VI-CFTR pulls away from surface; not enough left
http://aotc-insidecftr.org/index.html Watch “Inside CFTR” and the 3D animation