ADN 520 - Oxygenation- Cystic Fibrosis Questions and Answers Graded A+
What is cystic fibrosis (CF)? - Answers - A respiratory disorder that results from inheriting a
mutated gene
- Leads to obstruction of pancreas, lungs, liver, small intestine, and reproductive system
What are characteristics of CF? - Answers - Mucus glands that secrete an increased quantity of
thick, tenacious mucus
- Increased organic and enzymatic constituents in the saliva
- Increase in sodium and chloride content of sweat
- Autonomic nervous system abnormalities
What are risk factors associated with CF? - Answers - Both biological parents carry the
recessive trait for CF
- Caucasian ethnicity
What are respiratory manifestations with CF? - Answers - Wheezing, rhonchi
- Dry, nonproductive cough
- Dyspnea
- Obstructive emphysema
- Atalectasis
- Cyanosis
- Barrel chest
- Clubbing of fingers and toes
- Multiple episodes of bronchitis and bronchopneumonia
What are gastrointestinal manifestations of CF? - Answers - Large, frothy, bulky, greasy, foul-
smelling stools (streatorrhea)
- Voracious appetite early on, then decreased appetite
- Failure to gain weight or weight loss
- Delayed growth patterns
- Distended abdomen
, - Thin arms and legs
- Deficiency of fat soluble vitamins
- Anemia
What are skin manifestations of CF? - Answers - Sweat, tear, and saliva have an excessively high
content of sodium and chloride
What are endocrine and reproductive manifestations of CF? - Answers - Viscous cervical fluid
- Decreased or absent sperm
What labs can indicate CF? - Answers - Blood panel to detect a deficiency of fat-soluble vitamins
(A, D, and E)
- Sputum culture for detection of infection
What diagnostic tests are used to diagnose CF? - Answers - DNA testing
- Pulmonary function tests: decrease in forced vital capacity and expiratory volume, increased
residual volume
- Chest x-ray: hyperinfiltration, bronchial wall thickening, atelectasis, infiltration
- Abdominal x-ray
- Stool analysis
- Sweat chloride tests
- Pulse oximetry: decreased
- Bronchoscopy
What is a normal value for a chloride sweat test and what value might a patient with CF exhibit?
- Answers - Less than 40 is normal
- CF patients: greater than 60
What are nursing interventions for pulmonary management when caring for a patient with CF? -
Answers - Assist with airway clearance therapy (ACT)
- Avoid ACT immediately before of after meals
- Assist with chest physiotherapy, active cycle of breathing techniques, autogenic draining, and
high frequency chest compressions
- Administer aerosol therapy ad prescribed prior to ACT
What is cystic fibrosis (CF)? - Answers - A respiratory disorder that results from inheriting a
mutated gene
- Leads to obstruction of pancreas, lungs, liver, small intestine, and reproductive system
What are characteristics of CF? - Answers - Mucus glands that secrete an increased quantity of
thick, tenacious mucus
- Increased organic and enzymatic constituents in the saliva
- Increase in sodium and chloride content of sweat
- Autonomic nervous system abnormalities
What are risk factors associated with CF? - Answers - Both biological parents carry the
recessive trait for CF
- Caucasian ethnicity
What are respiratory manifestations with CF? - Answers - Wheezing, rhonchi
- Dry, nonproductive cough
- Dyspnea
- Obstructive emphysema
- Atalectasis
- Cyanosis
- Barrel chest
- Clubbing of fingers and toes
- Multiple episodes of bronchitis and bronchopneumonia
What are gastrointestinal manifestations of CF? - Answers - Large, frothy, bulky, greasy, foul-
smelling stools (streatorrhea)
- Voracious appetite early on, then decreased appetite
- Failure to gain weight or weight loss
- Delayed growth patterns
- Distended abdomen
, - Thin arms and legs
- Deficiency of fat soluble vitamins
- Anemia
What are skin manifestations of CF? - Answers - Sweat, tear, and saliva have an excessively high
content of sodium and chloride
What are endocrine and reproductive manifestations of CF? - Answers - Viscous cervical fluid
- Decreased or absent sperm
What labs can indicate CF? - Answers - Blood panel to detect a deficiency of fat-soluble vitamins
(A, D, and E)
- Sputum culture for detection of infection
What diagnostic tests are used to diagnose CF? - Answers - DNA testing
- Pulmonary function tests: decrease in forced vital capacity and expiratory volume, increased
residual volume
- Chest x-ray: hyperinfiltration, bronchial wall thickening, atelectasis, infiltration
- Abdominal x-ray
- Stool analysis
- Sweat chloride tests
- Pulse oximetry: decreased
- Bronchoscopy
What is a normal value for a chloride sweat test and what value might a patient with CF exhibit?
- Answers - Less than 40 is normal
- CF patients: greater than 60
What are nursing interventions for pulmonary management when caring for a patient with CF? -
Answers - Assist with airway clearance therapy (ACT)
- Avoid ACT immediately before of after meals
- Assist with chest physiotherapy, active cycle of breathing techniques, autogenic draining, and
high frequency chest compressions
- Administer aerosol therapy ad prescribed prior to ACT
