This is particularly problematic for the lungs because the thick mucus impairs clearance of invasive particles and infectious agents to maintain a sterile environment.
Microbial contamination of airway surfaces triggers an inflammatory response, including a massive invasion by neutrophils (Rodrigues et al., 2008). As the neutrophils react to bacterial contamination of the lung tissue they generate considerable amounts of cellular debris that increases the viscosity of the mucous, primarily by the deposition of genomic DNA and elastase proteins. The result of this process is chronic microbial lung infection, chronic immune-mediated inflammation, and progressive tissue damage. Because cystic fibrosis lung disease is so severe the life expectancy used to be late teens or early adulthood, but the development of more effective methods for disease management has almost doubled life expectancy in recent years (Cystic Fibrosis Foundation, n.d.).
Haematology
There is increasing evidence that CFTR is expressed in polymorphonuclear leukocytes and may be required for proper phagosome acidification and bacterial killing (Conese et al., 2011). Impaired phagolysosomal function would explain why microbial lung infections are resistant to an immune response. In addition, platelets play an important role in haemostasis and may mediate some aspects of an inflammatory response. In individuals with cystic fibrosis, platelets display a number of defects and secrete proteins that signal the end of an inflammatory response. These findings have caused some researchers to debate whether stem cell therapy might be an effective treatment for cystic...
Cystic Fibroids Cystic fibrosis Cystic fibrosis is a disease that can be passed down from one generation to the other. It affects secretary glands that produce mucus and sweat. The disease results after the fibrosis transmembrane conductance regulator (CFTR) gene that is found on chromosome 7 has undergone some sort of mutation. Mutation on chromosome 7 alters the production and function of CFTR glycoprotein (Scott, 2013). Studies have identified more than 1600
Cystic Fibrosis (CF) is genetically inherited through a defective gene, which results in the body producing "abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food." (PubMed Health, 2011) Reports state that millions of Americans carry the defective Cystic Fibrosis gene however; most do not have any symptoms since
Cystic Fibrosis: The Facts is a comprehensive, informative, and well-written book about the disease and its treatments. Ann Harris and Maurice Super address the book to a general audience, making the book extremely accessible to laypeople. Cystic Fibrosis: The Facts is an ideal source for people suffering from cystic fibrosis or for people who have loved ones suffering from the disease. Harris and Super divide the 129-page book into several
" (Karem et al., 1073) With an increased focus today on the genetic implications of the condition, enhanced abilities to understand the behaviors of human DNA are opening the door to a more perceptive response to the condition in question. As the text by Davis (1993) indicates, "as mutational analysis and patient data continue to be compiled, patient genotyping should prove useful in both prognosticating and providing a framework for evaluating
Cff.org/will each be the source of information and professional peer reviewed articles will be cited from these sources and identified by source as they cited. There is a wealth of available information, data and studies on CF. What it all means to the patients who suffer from this debilitating and life-threatening disease will be understood as this essay proceeds. Chapter One Diagnosis and the Anatomy and Physiology of a Life Threatening Disease: Cystic
However, treatments can be used to reduce, if not nearly completely diminish, symptoms and minimize any other complications. Here are the conditional means: Therapy: People with cystic fibrosis need a way to physically remove thick mucus from their lungs. This is often done by manually clapping with cupped hands on the front and back of the chest -- a procedure that's best performed with the person's head over the edge of
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