NR 507 Week 2 Discussion 1

Week 2: Respiratory Disorders and Alterations in Acid/Base Balance, Fluid and Electrolytes

Week 2: Discussion Part One

A five-month-old Caucasian female is brought into the clinic as the parent indicates that she has been having ongoing foul-smelling, greasy diarrhea. She seems to be small for her age and a bit sickly but, her parent’s state that she has a huge appetite. Upon examination you find that the patient is wheezing and you observe her coughing. After an extensive physical exam and work-up, the patient is diagnosed with cystic fibrosis.

1. What is the etiology of cystic fibrosis?
2. Describe in detail the pathophysiological process of cystic fibrosis.
3. Identify hallmark signs identified from the physical exam and symptoms.
4. Describe the pathophysiology of complications of cystic fibrosis.
5. What teaching related to her diagnosis would you provide the parents?

In addition to the textbook, utilize at least one peer-reviewed, evidence based resource to develop your post.A five-month-old Caucasian female is brought into the clinic as the parent indicates that she has been having ongoing foul-smelling, greasy diarrhea.

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SAMPLE ANSWER

1. What is the etiology of cystic fibrosis?

In cystic fibrosis, a mutation or a defect in a gene changes the protein that regulates the movement of salt in and out of cells. Cystic fibrosis, or CF, is an autosomal recessive disorder that affects the lungs, pancreas, small and large intestines, liver, gallbladder, bile ducts, sweat and saliva glands and the vas deferens. The most common symptoms of CF include persistent respiratory infections (i.e., wheezing and coughing), pancreatic insufficiency (i.e., greasy, foul-smelling stools), and elevated sweat chloride levels (Katkin, 2017).  The result is thick, sticky mucus in the respiratory, digestive and reproductive systems, as well as increased salt in sweat. Many different defects can occur in the gene. The type of gene mutation is associated with the severity of the condition. Children need to inherit one copy of the gene from each parent to have the disease. If children inherit only one copy, they will not develop cystic fibrosis. However, they will be carriers and possibly pass the gene to their children. The mean survival rate is 40 years of age (Van Biervliet et al., 2016). CF is the most common and fatal autosomal recessive disease among Caucasians. About 10 percent of CF cases are diagnosed after the patient is ten years of age…….CONT’D

A five-month-old Caucasian female is brought into the clinic as the parent indicates that she has been having ongoing foul-smelling, greasy diarrhea

SAMPLE RESPONSE

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.

References:

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

https://www.cff.org/Life-With-CF/Daily-Life/Adult-Guide-to-CF.pd

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