NR 507 Respiratory Disorders and Alterations in Acid/Base Balance, Fluid and Electrolytes
NR 507 Respiratory Disorders and Alterations in Acid/Base Balance, Fluid and Electrolytes
What is the etiology of cystic fibrosis?
This topic is of great interest to me because of this subject that I will be doing my presentation on, so I did a detailed search. The peer review article revealed that according to Munder& Tummler (2015), cystic fibrosis lung disease result from impaired chloride and bicarbonate epithelial transport, defective mucociliary transport, and acidification of the airway-surface liquid. In many ways, the pathophysiological process differs according to the different evidenced-based practice. “The pathophysiology of cystic fibrosis is deduced from the loss or dysfunction of CFTR in the apical epithelial membrane” Stoltz, (2015). The contributing factor is an attenuated bacteria-killing capability has reported in monocytes and macrophages isolated from people with cystic fibrosis.
The response to the peer review is” We agree with Munder and Tümmler that abnormal function in myeloid cells (i.e., neutrophils, monocytes, or macrophages) may contribute to impaired host defense in cystic fibrosis.” Stoltz, 2015. The article contains other pertinent information to describe the depth of this disease. “Specific cell types involved (monocytes, neutrophils, or macrophages) and proposed mechanisms for defects (e.g., organelle acidification, complement-mediated phagocytosis, and intraphagosomal production of hypochlorous acid) vary substantially among studies.” Stoltz (2015).
Describe in detail the pathophysiological process of cystic fibrosis.
Cystic fibrosis is the abnormal secretions that obstruct the respiratory, digestive, and reproductive tract. According to (McCance et al., 2013) research shows that there may be additional CF-associated primary defects, such as a natural proinflammatory state and abnormal local immune defenses in the lungs. “Cystic fibrosis is also associated with cystic fibrosis transmembrane conductance regulator (CFTCR) gene mutation results in the abnormal expression of cystic fibrosis transmembrane conductance regulator (CFTCR) protein, which is a cyclic adenosine monophosphate (cAMP)–activated chloride channel present on the surface of many types of epithelial cells.” (McCance et al., 2013). These cells include the lining of the airways, bile ducts, the pancreas, sweat glands, and the vas deferens. Even though cystic fibrosis affects multisystem it ultimately most often affects the lung, which is the most critical site of involvement that leads to respiratory failure and death.
Identify hallmark signs identified from the physical exam and symptoms.
Cystic fibrosis often occurs primarily in the white population; it affects 1 in 29 whites in the united states. The symptoms typically start with the respiratory and digestive systems. Respiratory symptoms include a persistent cough or wheeze, sputum production, and recurrent or severe pneumonia, and chronic sinusitis and nasal polyps. “Persistence of infection incites chronic local inflammation, airway damage, bronchiectasis, microabscess formation, and foci of hemorrhagic pneumonia.
Describe the pathophysiology of complications of cystic fibrosis.
There is progressive damage to the typical architecture of the lung with a decline in pulmonary function.” (McCance et al., 2013. CF causes reduced hydration of airway mucus which results in increased adherence of mucus to the epithelium, making it easier for bacteria to stick to and increasing the chances of infection (McCance et al., 2013)
What teaching related to her diagnosis would you provide the parents?
Due to the severity of the disease, it will be overwhelming and devastating to the parents, as an advanced clinical nurse I will provide them with all the information and videos available. I would also refer them to join a group of parents with children of a similar diagnosis. Advising the patient to prevent infection, adequate nutrition, and an increase in caloric intake is the primary goal to maintain a healthy lifestyle. Lastly, I would provide them with my information for them to call with any further questions.
Antje Munder, M.D. Burkhard Tümmler, M.D., Ph.D.
Hanover Medical School, Hannover, Germany (2015)
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David A. Stoltz, M.D., Ph.D. David K. Meyerholz, D.V.M., Ph.D.
Michael J. Welsh, M.D.
University of Iowa Carver College of Medicine, Iowa City, IA (2015)
McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2013). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, MO: Mosby.
Your post on cystic fibrosis (CF) is very informative. Your post explains the etiology, pathophysiology, and symptoms
of cystic fibrosis in detail. I think we should also look to studying EBP nursing care plans to better assist our patients diagnosed with cystic fibrosis. Living with cystic fibrosis comes with many medical, social, and financial challenges for patients, their caretakers, and family members. Having a productive and fulfilling life with cystic fibrosis is possible, as long as patients are educated about how to cope with the condition. The Cystic Fibrosis Foundation (2018) outlines four areas they would like CF patients to focus on so they can live a their best lives: daily life, treatments and therapies, transitions, and CF resources. The Adult Guide to Cystic Fibrosis is a resource that helps adults with CF manage their lives, as adults with CF outnumber children with the disease. This guide discusses and makes recommendations for basic lung care, menopause, having a social life, birth control, pregnancy, exercise, traveling, having a career, and more. FNPs should also study this guide in order to create individual care plans for their CF patients.
Griesenbach and Alton (2015) mention that several therapies have progressed including amongst novel antibiotics (inhaled tobramycin and macrolides) and novel mucolytics (dornase alpha–a recombinant DNase) and hypertonic saline that have been approved through clinical trials and offered as mainstream treatment. New CF drug developments have progressed to CFTR correctors to treat classes I-III mutations (Griesenbach and Alton, 2015). Our understanding of cystic fibrosis pathophysiology and genetics has expanded tremendously and has led to vast improvements for patients. Griesenbach and Alton (2015) note the recent licensing of ivacaftor, a drug targeting the molecular defect in the CFTR protein in approximately 5 percent of cystic fibrosis patients, is one of the current treatments making a difference in treatment efforts.
Griesenbach, U., & Alton, E. W. F. W. (2015). Recent advances in understanding and managing cystic fibrosis transmembrane conductance regulator dysfunction. F1000Prime Reports, 7, 64. http://doi.org/10.12703/P7-64
National Cystic Fibrosis Foundation. (2018). Adult Guide to Cystic Fibrosis. Retrieved from
I really appreciated how in the education section you mentioned to the mother what kind of symptoms would require immediate medical attention. This is not something that I thought about mentioning, but it is, of course, extremely important information! In my education part, I focused more on helping the mother feel better about the diagnosis and the long-term effects on her daughter. You did that, but you went a step further and that caught my eye! When a healthy child contracts the flu, it can be a scary time for any parent. But when that child has Cystic Fibrosis and the flu, it can be an actual life or death situation. The flu on it’s own could lead to a worsening in symptoms for the child, but that flu could develop into pneumonia leading to a marked decrease in lung function that could ultimately result in death (CFF, n.d.). I found an interesting study, however, of a patient with cystic fibrosis that seemed to present with flu, but it turned out to be a completely different and rare disease that is not usually associated with cystic fibrosis; Kawasaki Disease. So, this teaches us that we should never take the easy way and just assume, but “when the clinical course does not follow a typical pathway including persistent, high fever, alternate etiologies must be considered” (Osborne, Stillwell, Zemanick, & Dominguez, 2017). Thank you for your very informative post!
Cystic Fibrosis Foundation (CFF). (n.d.). Influenza (the Flu). Cystic Fibrosis Foundation.Retrieved from https://www.cff.org/Life-With-CF/Daily-Life/Germs-and-Staying-Healthy/What-Are-Germs/The-Flu/ (Links to an external site.)
Osborne, C M., Stillwell, P C., Zemanick, E., & Dominguez, S R. (2017). An unusual cause of high fever in a patient with cystic fibrosis (cf): Kawasaki disease. American Journal of Respiratory and Critical Care Medicine, 195(1). Retrieved from https://search.proquest.com/openview/e68de8491d52556b1abe5eac540add79/1.pdf?pq-origsite=gscholar&cbl=40575