Feb 23, 2024 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
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.
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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.
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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
Participation for MSN
Threaded Discussion Guiding Principles
The ideas and beliefs underpinning the threaded discussions (TDs) guide students through engaging dialogues as they achieve the desired learning outcomes/competencies associated with their course in a manner that empowers them to organize, integrate, apply and critically appraise their knowledge to their selected field of practice. The use of TDs provides students with opportunities to contribute level-appropriate knowledge and experience to the topic in a safe, caring, and fluid environment that models professional and social interaction. The TD’s ebb and flow is based upon the composition of student and faculty interaction in the quest for relevant scholarship. Participation in the TDs generates opportunities for students to actively engage in the written ideas of others by carefully reading, researching, reflecting, and responding to the contributions of their peers and course faculty. TDs foster the development of members into a community of learners as they share ideas and inquiries, consider perspectives that may be different from their own, and integrate knowledge from other disciplines.
Participation Guidelines
Each weekly threaded discussion is worth up to 25 points. Students must post a minimum of two times in each graded thread. The two posts in each individual thread must be on separate days. The student must provide an answer to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week. If the student does not provide an answer to each graded thread topic (not a response to a student peer) before the Wednesday deadline, 5 points are deducted for each discussion thread in which late entry occurs (up to a 10-point deduction for that week). Subsequent posts, including essential responses to peers, must occur by the Sunday deadline, 11:59 p.m. MT of each week.
Direct Quotes
Good writing calls for the limited use of direct quotes. Direct quotes in Threaded Discussions are to be limited to one short quotation (not to exceed 15 words). The quote must add substantively to the discussion. Points will be deducted under the Grammar, Syntax, APA category.
Grading Rubric Guidelines
Performance Category109840ScholarlinessDemonstrates achievement of scholarly inquiry for professional and academic decisions.Provides relevant evidence of scholarly inquiry clearly stating how the evidence informed or changed professional or academic decisionsEvaluates literature resources to develop a comprehensive analysis or synthesis.Uses valid, relevant, and reliable outside sources to contribute to the threaded discussionProvides relevant evidence of scholarly inquiry but does not clearly state how the evidence informed or changed professional or academic decisions.Evaluates information from source(s) to develop a coherent analysis or synthesis.Uses some valid, relevant, reliable outside sources to contribute to the threaded discussion.Discusses using scholarly inquiry but does not state how scholarly inquiry informed or changed professional or academic decisions.Information is taken from source(s) with some interpretation/evaluation, but not enough to develop a coherent analysis or synthesis.Little valid, relevant, or reliable outside sources are used to contribute to the threaded discussion.Demonstrates little or no understanding of the topic.Discusses using scholarly inquiry but does not state how scholarly inquiry informed or changed professional or academic decisions.Information is taken from source(s) without any interpretation/evaluation.The posting uses information that is not valid, relevant, or reliableNo evidence of the use of scholarly inquiry to inform or change professional or academic decisions.Information is not valid, relevant, or reliablePerformance Category 109840Application of Course Knowledge -Demonstrate the ability to analyze, synthesize, and/or apply principles and concepts learned in the course lesson and outside readings and relate them to real-life professional situationsPosts make direct reference to concepts discussed in the lesson or drawn from relevant outside sources;Applies concepts to personal experience in the professional setting and or relevant application to real life.Posts make direct reference to concepts discussed in the lesson or drawn from relevant outside sources.Applies concepts to personal experience in their professional setting and or relevant application to real lifeInteractions with classmates are relevant to the discussion topic but do not make direct reference to lesson contentPosts are generally on topic but do not build knowledge by incorporating concepts and principles from the lesson.Does not attempt to apply lesson concepts to personal experience in their professional setting and or relevant application to real lifeDoes not demonstrate a solid understanding of the principles and concepts presented in the lessonPosts do not adequately address the question posed either by the discussion prompt or the instructor’s launch post.Posts are superficial and do not reflect an understanding of the lesson contentDoes not attempt to apply lesson concepts to personal experience in their professional setting and or relevant application to real lifePosts are not related to the topics provided by the discussion prompt or by the instructor; attempts by the instructor to redirect the student are ignoredNo discussion of lesson concepts to personal experience in the professional setting and or relevant application to real lifePerformance Category 54320Interactive DialogueReplies to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week, and posts a minimum of two times in each graded thread, on separate days.(5 points possible per graded thread)Exceeds minimum post requirementsReplies to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week, and posts three or more times in each graded thread, over three separate days.Replies to a post posed by faculty and to a peerSummarizes what was learned from the lesson, readings, and other student posts for the week.Replies to each graded thread topic posted by the course instructor, by Wednesday, 11:59 p.m. MT, of each week, and posts a minimum of two times in each graded thread, on separate daysReplies to a question posed by a peerSummarizes what was learned from the lesson, readings, and other student posts for the week.Meets expectations of 2 posts on 2 different days.The main post is not made by the Wednesday deadlineDoes not reply to a question posed by a peer or facultyHas only one post for the weekDiscussion posts contain few, if any, new ideas or applications; often are a rehashing or summary of other students’ commentsDoes not post to the threadNo connections are made to the topic Minus 1 PointMinus 2 PointMinus 3 PointMinus 4 PointMinus 5 PointGrammar, Syntax, APANote: if there are only a few errors in these criteria, please note this for the student in as an area for improvement. If the student does not make the needed corrections in upcoming weeks, then points should be deducted.Points deducted for improper grammar, syntax and APA style of writing.The source of information is the APA Manual 6th Edition2-3 errors in APA format.Written responses have 2-3 grammatical, spelling, and punctuation errors.Writing style is generally clear, focused, and facilitates communication.4-5 errors in APA format.Writing responses have 4-5 grammatical, spelling and punctuation errors.Writing style is somewhat focused.6-7 errors in APA format.Writing responses have 6-7 grammatical, spelling and punctuation errors.Writing style is slightly focused making discussion difficult to understand.8-10 errors in APA format.Writing responses have 8-10 grammatical, spelling and punctuation errors.Writing style is not focused, making discussion difficult to understand.Post contains greater than 10 errors in APA format.Written responses have more than 10 grammatical, spelling and punctuation errors.Writing style does not facilitate communication.The student continues to make repeated mistakes in any of the above areas after written correction by the instructor0 points lost -5 points lostTotal Participation Requirementsper discussion threadThe student answers the threaded discussion question or topic on one day and posts a second response on another day. The student does not meet the minimum requirement of two postings on two different daysEarly Participation Requirementper discussion threadThe student must provide a substantive answer to the graded discussion question(s) or topic(s), posted by the course instructor (not a response to a peer), by Wednesday, 11:59 p.m. MT of each week. The student does not meet the requirement of a substantive response to the stated question or topic by Wednesday at 11:59 pm MT.
NOTE: To receive credit for a week’s discussion, students may begin posting no earlier than the Sunday immediately before each week opens. Unless otherwise specified, access to most weeks begins on Sunday at 12:01 a.m. MT, and that week’s assignments are due by the next Sunday by 11:59 p.m. MT. Week 8 opens at 12:01 a.m. MT Sunday and closes at 11:59 p.m. MT Wednesday. Any assignments and all discussion requirements must be completed by 11:59 p.m. MT Wednesday of the eighth week.
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