Cystic Fibrosis Discussion – Immunopathology and Genetic Diseases

Cystic Fibrosis Discussion – Immunopathology and Genetic Diseases

Cystic Fibrosis (CF) is a genetic lung disease that causes persistent lung infections. These infections limit the ability to breathe over time (Merck Manual 2020). According to Bhagirath (2016), when the “Cystic Fibrosis transmembrane regulators (CFTR) gene,” a necessary protein is absent or dysfunctional.  This absent or dysfunctional protein’s reaction is an accumulation of thick, sticky mucous in the respiratory tract clogging the airways. When the CFTR is not working correctly, a component of salt – chloride is unable to be moved to the cell surface. Chloride attracts water to the cells regulating the mucous in various organs. When the mucous is thick and sticky in the lungs, infections, inflammation, respiratory failure, and other complications are caused by trapped bacteria. People with Cf need to minimize contact with germs to prevent colonization of the airways by microbial pathogens which cause bacterial infections (Bhagirath, 2016) Merck Manual states most diagnosis of CF is found in infancy. The United States and many other countries test for DF at birth. Early diagnosis affects long term prognosis. Cystic Fibrosis Discussion – Immunopathology and Genetic Diseases



    • Cystic FibrosisSubscribe
    • Melissa Morgan posted Jan 27, 2021 10:53 PM
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    • Patients with cystic fibrosis have decreased airway clearance which lessens lung function and eventually leads to infections. Our body naturally has microorganisms that live on the surface of the upper respiratory tract called normal microbiome (Huether, McCance & Brashers, 2020). These microorganisms are not harmful to the body under normal circumstances. However, cystic fibrosis is characterized by increased mucus production that makes it hard for people affected by this disease to keep their airways clear. “Impaired mucociliary clearance predisposes patients to recurrent infection, mucus plugging, inflammation, and airway obstruction that ultimately leads to progressive loss of lung function” (Mikesell, et al., 2017). Melissa has a cough, productive green sputum, febrile, and is wheezing. These clinical manifestations indicate an upper respiratory infection. A healthy person has a well-hydrated layer of periciliary caused by a higher osmotic pressure; this provides an appropriate amount of lubrication for ciliary activity and transport (Turcios, 2020). In a person with CF, they have “hyperconcentrated (dehydrated) mucus, impaired mucus transport, and mucus adhesion to airway surfaces” (Turcios, 2020). This means that they do not have the ability to keep their airways clear, and with this defect, they develop lung diseases more easily than the average person.Huether, S., McCance, K., and Brashers, V.  (2020).  Understanding Pathophysiology (7th ed.).  Elsevier.Turcios, N. L. (2020). Cystic Fibrosis Lung Disease: An Overview. Respiratory Care65(2), 233–251. UnreadUnread4 ViewsViews
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    • Mikesell, C. L., Kempainen, R. R., Laguna, T. A., Menk, J. S., Wey, A. R., Gaillard, P. R., & Regelmann, W. E. (2017). Objective Measurement of Adherence to Out-Patient Airway Clearance Therapy by High-Frequency Chest Wall Compression in Cystic Fibrosis. Respiratory Care62(7), 920–927.
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    • Dennies Jones Edited DiscussionSubscribe
    • Dennies Jones posted Jan 26, 2021 1:24 PM
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    •                                 Cystic fibrosis, the most common lethal recessive disease in white children, occurs in about 1 in 2500 births. Approximately 1 in 25 whites carry a copy of a mutation that causes cystic fibrosis (Huether et al., 2020). Defective transport of chloride ions leads to a salt imbalance, which results in secretions of abnormally thick, dehydrated mucus (Huether et al., 2020). According to Andrade et al. (2018), “cystic fibrosis is a progressive disease with lung function deterioration, malnutrition, and progressive exercise limitation” (p. 144).        less1 UnreadUnread12 ViewsViews
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    • Moore, J. E., & Mastoridis, P. (2017). Clinical implications of Pseudomonas aeruginosa location in the lungs of patients with cystic fibrosis. Journal of Clinical Pharmacy & Therapeutics42(3), 259–267.
    • Huether, Sue E., McCance, Kathryn L… Understanding Pathophysiology – E-Book (p. 169). Elsevier Health Sciences. Kindle Edition.
    • Andrade, R. C., da Silva e Silva, C. M., Diniz da Silva, A. L. L., Haun, S. R., de Souza, V. A., &    Ezequiel, D. J. S. (2018). Comparison of respiratory muscle strength, quality of life, and functional capacity among adolescents with cystic fibrosis with different bacteriological profiles. Fisioterapia e Pesquisa25(2), 143–150. Cystic Fibrosis Discussion – Immunopathology and Genetic Diseases
    •                     Inflammation of the airway obstructs the airflow, which causes inadequate ventilation. The impairment of pulmonary function is characterized by respiratory infection followed by colonization by bacteria (Andrade et al., 2018). Bacterial colonization is favorable due to its decreased lung function, nutrient-rich mucous pooled in oxygenated areas such as the respiratory tract, thus making an optimal breeding environment for bacteria. This makes for a brutal cycle of bacterial infection. Moore and Mastoridis (2017), stated that “P. aeruginosa infection in the lower airways correlates with declining lung function” (p. 260).


Melissa, a 12-year-old girl with cystic fibrosis comes to the primary care office with complaints of increased cough and productive green sputum over the last week. She also complains of increasing shortness of breath. She denies sore throat or nasal congestion. On physical examination her temperature is 101 and she has inspiratory wheezes bilaterally. Negative lymphadenopathy noted. Posterior pharynx is pink without exudate. BP 112/72 HR 96 RR 28.

1. In cystic fibrosis, the airway microenvironment favors bacterial colonization. In a minimum of 150 words explain the pathophysiological reason for this occurrence.

Post your initial response by Wednesday at midnight. Respond to one student by Sunday at midnight. Both responses must be a minimum of 150 words, scholarly written, APA formatted, and referenced. A minimum of 2 references are required (other than your text). Refer to grading rubric for online discussion. Cystic Fibrosis Discussion – Immunopathology and Genetic Diseases