NR 507 Week 2: Respiratory Disorders and Alterations in Acid/Base Balance, Fluid and Electrolytes
NR 507 Week 2: 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.
References:
Antje Munder, M.D. Burkhard Tümmler, M.D., Ph.D.
Hanover Medical School, Hannover, Germany (2015)
tuemmler.burkhard@mh-hannover.de
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.
Cystic Fibrosis is a genetically inherited disease secondary to an autosomal recessive gene within chromosome 7. There are close to 2000 gene mutations; however, there are 6 prominent mutations that lead to pulmonary insufficiency. Gene mutations classified as 1 to 3 or closely correlated with severe pulmonary dysfunction whereas genes 4 thru 6 present a mild version of pulmonary insufficiency without pancreatic compromise. Affecting predominately Caucasians, African and Asian Americans. Over 70 percent of Cystic Fibrosis diagnoses occur around 6 months to 1 year of age. Affecting an estimated “30,000 individuals in the United States and 70,000 worldwide” (McCance, Huether, Brashers, & Rote, 2014, p. 1310).
Describe in detail the pathophysiological process of cystic fibrosis.
Cystic fibrosis is characterized by abnormal amounts of copious secretions that are heavily saturated and obstructive to the respiratory, digestive and reproductive organs. Secondary to altered chloride transport creating obstructions of the exocrine glands (Sommers & Fannin, 2015). Secretions are resultant of the gene mutation referred to as “cystic fibrosis transmembrane conductance regulator (CFTCR) protein. A cyclic adenosine monophosphate (cAMP) activated chloride channel” that lines the epithelial cells of specific secretory organs such as the airway, pancreas, bile ducts and in the male the vas deferens (McCance et al., 2014, p. 1311). Alterations in the epithelial sodium channel (ENaC) leads to excessive excretion of chloride with enhanced sodium retention. Contributing to dehydration of epithelial cells leading to restriction of ciliary tracts ability to expel mucus. With consequential overproduction of secretions creating a host environment for bacteria (McCance et al., 2014).
Identify hallmark signs identified from the physical exam and symptoms.
Hallmark signs and symptoms consist of parents reporting a salty taste to their infant’s skin often identified during moments of affection such through kissing the child. Parents and the patient’s medical history may indicate a history of chronic and/or repetitive upper respiratory infections ranging from a croup sounding cough to being diagnosed with pneumonia. In addition to excessive foul-smelling stools, insufficient weight gain not correlating with excessive appetite. As well as a failure of the infant to develop fine and gross motor skills at anticipated developmental milestones. Roughly 5 to 15 percent of infants will be diagnosed with a meconium ileus, spontaneous failure to thrive, abnormal breath sounds from rhonchi to rales. Body alignment and redisposition of the chest wall and clubbing of the fingernails as the child ages (Sommers & Fannin, 2015).
Describe the pathophysiology of complications of cystic fibrosis.
Complications consist of multiple organ insufficiency ranging from pulmonary insufficiency and failure secondary to antibiotic-resistant bacterial infections. A leading cause of death in patients diagnosed with Cystic Fibrosis. Electrolyte imbalances affected by chloride reabsorption limitations and excessive sodium excretion. Vitamins A, D, E and K deficiency related to malabsorption and metabolic abnormalities. In addition to secretions blocking the pancreatic ducts decreasing the production and secretion of pancreatic enzymes (Dodge, 2015). Additional complications include insulin resistance, reproductive complications as evidenced by fertility in women and sterility in men (McCance et al., 2014).
What teaching related to her diagnosis would you provide the parents?
Parental education would include teaching them how to cope with their child’s diagnosis spiritually, mentally, and physically. Also included in patient education, will consist of any additional disease confirmatory tests available such as “Quantitative electrolyte sweat tests, genotyping and confirmatory 72-hour stool sampling to assess pancreatic function” (Sommers & Fannin, 2015, p. 304). The importance of medication, dietary and physical exercise regimens for their child. Parents will be educated on treatment modalities to manage potential respiratory infections, how to prepare and monitor meals high in caloric intake. As well as how to perform various physical exercises, and what assistive medical equipment is available should the child need advanced therapy to help mobilize excessive secretions (Sommers & Fannin, 2015).
References
Dodge, J. A. (2015). A millennial view of Cystic Fibrosis. Developmental Period Medicine, 19, 9-13. Retrieved from https://eds-a-ebscohost-com.chamberlainuniversity.idm.oclc.org/eds/pdfviewer/pdfviewer?vid=3&sid=115f2582-1c0a-4d30-9d64-0367a7a38097%40sessionmgr4008
McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2014). Pathophysiology: The biologic basis for disease in adults and children (7th ed.). St. Louis, Missouri: Mosby. Sommers, M. S., & Fannin, E. (2015). Cystic Fibrosis. In Diseases & disorders: A nursing therapeutics manual (5th ed., pp. 302-306). Philadelphia, Pennsylvania: F.A. Davis
