Discussion: Cystic Fibrosis Study

Discussion: Cystic Fibrosis Study

Discussion: Cystic Fibrosis Study

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Module 2: Discussion Melissa, a 12-year-old girl with cystic fibrosis comes to the primary care office with complaints of increased cough and productive green sputum over the last week. She also complains of increasing shortness of breath. She denies sore throat or nasal congestion. On physical examination her temperature is 101 and she has inspiratory wheezes bilaterally. Negative lymphadenopathy noted. Posterior pharynx is pink without exudate. BP 112/72 HR 96 RR 28. 1. In cystic fibrosis, the airway microenvironment favors bacterial colonization. In a minimum of 150 words explain the pathophysiological reason for this occurrence.

Cystic fibrosis affects at least 30,000 people in the United States; between 900
and 1,000 new cases are diagnosed every year (1). One in 29 people of Caucasian ancestry is an unaffected carrier of the CF gene mutation. In the United
States, cystic fibrosis occurs at a rate of 1 in 3,400 births. While it occurs in
persons of all racial and ethnic backgrounds, it is most common in Caucasians
of Northern European ancestry. Historically, half of affected individuals were
diagnosed by five months of age, though the average age at diagnosis was five
years, and some individuals were not diagnosed until adulthood

Clogged mucus secretions in the digestive tract can lead to malnutrition and
vitamin deficiencies. The genital tract abnormality can lead to infertility in men
and women. Other complications include CF-related diabetes, liver cirrhosis,
bowel obstruction, chronic sinusitis, and osteoporosis. There is a high prevalence of depression and anxiety in CF patients.
Environmental exposures worsen CF lung disease. Children who are exposed
to tobacco smoke have lower lung function and more pulmonary exacerbations
than those who live in smoke-free environments. High levels of air pollution are
associated with an increased rate of adverse pulmonary events.
How is it prevented, treated, and managed?
Prevention, treatment, staying healthy, prognosis
Cystic fibrosis carrier testing is recommended for Caucasian women who are
considering pregnancy or who are pregnant. This can allow a couple who is at
risk of having a child with CF to use reproductive technologies to avoid having a
baby with CF, or to prepare for the birth of an infant with CF. Diagnosing CF
before a child is born, or by newborn screening, allows earlier referral to a CF
center and initiation of treatment with pancreatic enzymes before symptoms of
abnormal absorption or poor growth occur.
In the United States, most people with CF are treated at specialized CF
centers accredited by the Cystic Fibrosis Foundation. Multidisciplinary teams of
physicians, nurses, respiratory therapists, dietitians, and social workers care for
both adult and pediatric patients.
Individuals with CF who have better nutrition have higher lung function and
longer life expectancy. Nutritional management with pancreatic enzymes and
a high-calorie, liberal-fat diet is recommended from the time of diagnosis. Some
people with CF benefit from supplemental feedings given overnight by a tube
placed into the stomach. Specialized vitamin preparations are prescribed in
order to reduce the risk of deficiency of certain fat-soluble vitamins.
Although most infants and young children have only intermittent symptoms
of cough and wheezing, recent research shows that there are structural and
functional abnormalities of the lung as early as the first few months of life. Most
CF treatments for lung disease have been tested primarily in patients aged five

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