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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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

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.

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  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.

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

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

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

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

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 Reports7, 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

 

Diarrhea is characterized by an increase in the frequency, fluidity, or volume of stools. 
Most people associate diarrhea with an increase in stool frequency1, although the passing of loose stools is the most important symptom of diarrhea for the majority of people. 
Diarrhea is defined as passing stool with weight or volume of more than 200 or 200 mL in less than 24 hours. 
Diarrhea is regular occurrence, with most episodes lasting only few hours. 
In the course of year, however, about 5% of the US population suffers with chronic diarrhea, which is defined as watery stools that continue longer than four weeks. 
As result, diarrhea is significant source of morbidity. 
It’s critical to remember that diarrhea is symptom or indicator, not an illness, and that it can be caused by variety of factors. 
The examination and management of patient with diarrhea might be difficult due to the wide range of probable causes. 
Understanding the basic mechanisms of diarrhea can make diagnosis and treatment easier.

 

Pathophysiology is good place to start.

 

Incomplete absorption of water from intestine luminal contents is the root cause of all diarrheal disorders. 
Water is not actively carried through the intestinal mucosa, but it does so as result of osmotic forces caused by the movement of solutes including electrolytes and minerals. 
Absorption and secretion normally occur at the same time, but absorption is more quantitatively significant. 
Increased water in the lumen and diarrhea result from either decrease in absorption or an increase in secretion. 
Stool consistency suffers as result of too much water in the stool.

 

As result, diarrhea is problem with water and electrolyte movement in the intestine. 
Osmotic, secretory, inflammatory, and altered motility are among the pathophysiologic processes of diarrhea. 
Osmotic diarrhea is caused by an unabsorbed material that follows osmotic gradients and sucks water from the plasma into the intestinal lumen. 
Secretory diarrhea is caused by disruption in electrolyte transport and, despite the name, is more often caused by decreased absorption than by net secretion. 
Exudative, secretory, and osmotic diarrhea are all symptoms of inflammatory disorders. 
Changes in gut or colon motility might affect fluid absorption by increasing or decreasing the amount of luminal material exposed to the intestinal absorptive surface. 
However, no single cause of diarrhea is genuinely unifactorial from pathophysiologic standpoint.
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