Various other drug-based treatments, including the use of mucolytics to break up thick, viscous sputum and the use of osmotic agents to keep airways moist, are well established in CF treatment protocols, but research continues in order to refine and improve on these approaches as well.
A novel therapeutic method being tried for CF is "the modulation of non-CFTR ion channels in epithelial cells." (Jones & Helm, 2009). The drug Lancovutide (Milil901, duramycin) "activates an alternative chloride channel in epithelial cells by elevating intracellular calcium levels, and thus, may potentially compensate for CFTR deficiency in the airway epithelium." (Jones & Helm, 2009). These agents have reached the clinical trial stage, and, if successful, will be able to offer a new category of treatment option for CF patients.
Gene therapies, which seek to correct "the underlying gene effect, either by agents that help to correct the dysfunctional CFTR . . . Or by gene transfer" (Jones & Helm, 2009), are particularly promising. The gene defect that causes CF was first identified in 1989, and since then researchers have explored treatments that would replace the patient's defective CFTR with "wild-type" CFTR (Jones & Helm, 2009).
For example Zhang, et al. have focused on the development of gene transfer strategies using epithelial cells. They used "an in vitro model of human CF ciliated surface airway epithelium…to test whether a human parainfluenza virus vector engineered to express CFTR could deliver sufficient CFTR…to restore mucus transport, thus correcting the CF phenotype." (Zhang, et al., 2008). The team was able to determine that normal mucus transport rates were restored after "CFTR delivery to 25% of surface epithelial cells." (Zhang, et al., 2008). Additional study will help develop this corrective gene transfer approach and determine how best to use the technique and how far it can go towards restoring normal mucus transport function in CF patients.
One factor contributing to the advancement of research to treat CF is the availability of stem cells for experimental use. Recently, two new human embryonic stem cells lines have been produced affected by CF. (Deleu et al., 2009) This new source of "feeder cells" offers researchers ease and availability of a medium for continued research studies in the laboratory prior...
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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