NR 507 Week 2 Discussion One

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Describe in detail the pathophysiological process of cystic fibrosis

The types of complications in patients with cystic fibrosis contrast depending on the extent of transmutation of cystic fibrosis transmembrane conductance regulator (CFTR) (Brown, White, & Tobin, 2017). Generally CF is described by atypical excudations that actuate blockage issues within the pulmonary, gastrointestinal, and reproductive tracts (Brown, White, & Tobin, 2017). Literature proposes that there might be added CF-linked primary defects, like an intrinsic proinflammatory condition and anomalous immune protection in the lungs (Brown, White, & Tobin, 2017).  CF is brought about by a transmutation which is precipitated by a defect in the CF transmembrane conductance regulator gene. The CFTR polypeptide which is cyclic adenosine monophosphate (cAMP)- actuated chloride medium existing on the surface of numerous kinds of epithelial cells, in addition to those lining trachea, bile ducts, the pancreas, sweat glands, and the vas deferens (McCance, Huether, Brashers, & Rote, 2013). Even though CF is a multiorgan medical condition, lung pathosis is the root of sudden death of patients. In the lung this leads to airway surface liquid depletion and, since airway surface liquid is important to aid ciliary balance and functioning, ciliary collapse and reduced mucociliary transport (Brown, White, & Tobin, 2017). The result of this is a vicious circle of mucus retention is pestilence of the small airways and chronic inflammation (Brown, White, & Tobin, 2017).

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Describe in detail the pathophysiological process of cystic fibrosis.

CF is associated with deficient epithelial chloride ion transport (McCance et al., 2013). The CF gene can be found on chromosome 7 and has six classifications that differ in severity (McCance et al., 2013). Research states that “the cystic fibrosis transmembrane conductance regulator (CFTR or CFTR) gene mutation results in the abnormal expression of cystic fibrosis transmembrane conductance regulator protein, which is a cyclic adenosine monophosphate (cAMP)-activated chloride channel present on the surface of many types of epithelial cells including those lining the airways, bile ducts, the pancreas, sweat ducts, and the vas deferens” (McCance et al., 2013).

Most commonly the CF genetic mistake is the DF508 protein that results in hypochloremia that progresses to lung issues related to thick mucus which inhibits proper air exchange that leads to infections, lung damage, and could ultimately lead to respiratory failure (CFF, n.d.). Patients display a variety of symptoms from the salty-tasting skin, to a cough, frequent respiratory infections, wheezing, dyspnea, clubbing, poor weight gain even though there is a good appetite, and stools can range from greasy to difficulty in having bowel movements (CFF, n.d.). The patient is five months old (the median age at diagnosis is six months), is experiencing greasy, foul-smelling diarrhea, small for her age, coughing, and wheezing, which are classic symptoms of CF.  If the disease presents itself later in life, the patients typically have milder symptoms as opposed to diagnosing the patient within the first year of life. The severity of the disease depends on which class it falls in; classes 4-6 experience milder symptoms than those that fall within classes 1-3 (McCance et al., 2013).  CF must be diagnosed early to improve the patient’s quality and longevity of life. After recognizing the symptoms, one must confirm their differential before giving a definite diagnosis. Screening newborns for CF is a universal protocol in the United States.