CF is a multisystem disorder. Describe the condition and its physiologic effect on the following
systems: respiratory system, gastrointestinal system, reproductive system, and skin and
electrolyte balance.
Focus on factors that place J.R. at risk for developing respiratory infections.
CF is the most common inherited genetic disorder affecting Caucasians in the pediatric and young
adult population. It is inherited as an autosomal recessive trait affecting the protein gene code called
the CF transmembrane regulator. The primary factor that is responsible for most manifestations
is mechanical obstruction as a result of increased viscosity of the mucus-secreting glands. In a CF
patient, chloride secretion is decreased, sodium absorption is increased, and water flow across cells
is decreased; these changes result in viscous and dry mucus. Mucous plugs form within and damage
the respiratory, GI (pancreas, liver, intestines), and reproductive systems.
Respiratory tract: Increased bronchial mucus viscosity decreases ciliary action and leads to
progressive lung dysfunction. The lungs become filled with thick, sticky mucus (stasis), causing
air trapping, hyperinflation, atelectasis, and a medium for bacterial growth, increasing risk for
respiratory infections.
GI tract: GI involvement varies. The increased viscosity of mucus might affect the pancreas, inhibiting
pancreatic enzymes from reaching the duodenum. This creates a malabsorption syndrome.
Meconium ileus is the earliest sign of GI involvement. Thick intestinal secretions might be
problematic throughout life and lead to intestinal obstructions.
Reproductive system: CF affects the reproductive systems of males and females. Viscous cervical
secretions might affect fertility in females. Most men are sterile because of blockage of the vas
deferens or decreased sperm production.
Skin: The impaired reabsorption of sodium and chloride can lead to abnormally high sodium and
chloride concentrations in sweat. This may manifest as hyponatremia, hypochloremia, and
dehydration.
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A patient experienced a 33% TBSA burn 72 hours ago. The nurse observes that the patient's hourly urine output has been steadily increasing over the past 24 hours. How should the nurse best respond to this finding?
A) Obtain an order to reduce the rate of the patient's IV fluid infusion. B) Report the patient's early signs of acute kidney injury (AKI). C) Recognize that the patient is experiencing an expected onset of diuresis. D) Administer sodium chloride as ordered to compensate for this fluid loss.
You work in a cardiac ICU and are caring for a patient with a suspected MI. What drugs would expect the physician to have ordered for this patient?
A) Calcium channel blocker B) Coumadin C) IV beta-blocker D) Tylenol
How often is mature sperm generated?
A) Every 90 days B) Every 80 days C) Every 70 days D) Every 60 days
Usually, the family is advised to arrive at the birth setting at the beginning of the active phase of labor or when which of the following occur? Note: Credit will be given only if all correct and no incorrect choices are selected
Select all that apply. 1. Rupture of membranes (ROM) 2. Increased fetal movement 3. Decreased fetal movement 4. Any vaginal bleeding 5. Regular, frequent uterine contractions (UCs)