Cystic Fibrosis Nursing Exam 2026/2027 |
95 NCLEX-Style Questions with Answers
& Explanations | CFTR Modulators,
Respiratory Management & CFRD
Description:
Master cystic fibrosis nursing concepts with 95 exam questions updated for 2026/2027.
Includes CFTR modulators (Trikafta), airway clearance, pancreatic enzymes, CFRD,
and NCLEX-style rationales.
Download the complete CF examination paper with answers and explanations to ace your
pediatric pulmonology certification or nursing final exam today.
, Cystic Fibrosis NCLEX Exam 2026
Section 1: Diagnostic Testing for Cystic Fibrosis
Question 1
Which diagnostic tests are used to confirm the diagnosis of cystic fibrosis? Select all that
apply.
A. Cystic fibrosis transmembrane regulator (CFTR) mutation analysis
B. Sweat chloride test
C. Newborn screening test
D. Chest radiography
E. Complete blood count
Answer: A, B, C
Explanation: Confirmatory diagnosis of cystic fibrosis requires evidence of CFTR
dysfunction. Sweat chloride testing (chloride ≥60 mmol/L on two separate occasions)
remains the gold standard. CFTR mutation analysis identifies disease-causing variants on
both alleles. Newborn screening typically involves immunoreactive trypsinogen (IRT)
measurement followed by DNA testing or sweat testing; however, it is a screening tool that
requires confirmatory testing. Chest radiography and complete blood count are supportive but
not diagnostic.
Section 2: Genetics and Inheritance Patterns
Question 2
Which scenario will most likely result in a child having cystic fibrosis (CF)?
A. One parent has CF and the other parent has no CFTR gene mutations
B. Both parents have CF and each passes the mutated gene to the child
C. Both parents are mutated gene carriers, and each passes the mutated gene to the child
D. One parent is a mutated gene carrier and the other parent has no CFTR mutations
Answer: C
Explanation: Cystic fibrosis follows an autosomal recessive inheritance pattern. For a child
to have CF, they must inherit two mutated CFTR genes, one from each parent. When both
parents are carriers (heterozygous), each pregnancy has a 25% chance of producing an
affected child (homozygous recessive), a 50% chance of producing an asymptomatic carrier,
and a 25% chance of an unaffected non-carrier.
,Section 3: Multi-System Involvement in Cystic Fibrosis
Question 3
Which systems are affected by cystic fibrosis? Select all that apply.
A. Respiratory system
B. Gastrointestinal system
C. Endocrine system
D. Integumentary system
E. Reproductive system
Answer: A, B, C, E
Explanation: CFTR dysfunction affects multiple organ systems. The respiratory system
demonstrates thick mucus, chronic infection, and bronchiectasis. The gastrointestinal system
shows pancreatic insufficiency, meconium ileus, and distal intestinal obstruction syndrome.
The endocrine system is affected via CF-related diabetes mellitus due to pancreatic islet cell
damage. The reproductive system shows congenital bilateral absence of the vas deferens in
males and reduced fertility in females. The integumentary system is not primarily affected,
though salt crystals on skin may be observed due to elevated sweat chloride.
Section 4: Interprofessional Care Coordination
Question 4
The nurse is caring for a family and child who is newly diagnosed with cystic fibrosis in an
acute care setting. Which professionals could the nurse consult to support the client and
family? Select all that apply.
A. Respiratory therapist
B. Registered dietitian
C. Social worker
D. Child life specialist
E. Psychologist
F. Pharmacist
Answer: A, B, C, D, E, F
Explanation: All listed professionals are essential members of the multidisciplinary CF care
team. The respiratory therapist provides airway clearance education. The dietitian addresses
nutritional needs and pancreatic enzyme management. The social worker assists with
, insurance, school accommodations, and community resources. Child life specialists help
younger children understand procedures. Psychologists address anxiety, depression, and
treatment adherence. Pharmacists review medication interactions and educate about proper
medication administration.
Section 5: Nutrition and Enzyme Management Assessment
Question 5
The nurse is caring for an adolescent with cystic fibrosis who has been losing weight and
having increased incidence of steatorrhea. Indicate whether each assessment question is
applicable or not applicable to gathering information related to the problem.
"Can you tell me how you usually take your enzymes with meals?"
Answer: Applicable
Explanation: Enzyme administration timing with meals directly affects nutrient absorption.
Enzymes must be taken immediately before or with the first bite of a meal to be effective.
"Can you tell me how you usually take your enzymes with snacks?"
Answer: Applicable
Explanation: Enzymes are required with snacks as well as meals, as snacks contribute
significant calories and fat. Missed snack doses can cause steatorrhea.
"Can you tell me about times when you might not take your enzymes?"
Answer: Applicable
Explanation: Identifying patterns of nonadherence (forgetting, social embarrassment, side
effects) is essential for addressing weight loss and steatorrhea.
"Can you tell me what you ate yesterday?"
Answer: Applicable
Explanation: Dietary recall helps determine if enzyme dosing matches fat intake. A high-fat
meal without sufficient enzymes causes steatorrhea.
"Where do you store your enzymes?"
Answer: Applicable
95 NCLEX-Style Questions with Answers
& Explanations | CFTR Modulators,
Respiratory Management & CFRD
Description:
Master cystic fibrosis nursing concepts with 95 exam questions updated for 2026/2027.
Includes CFTR modulators (Trikafta), airway clearance, pancreatic enzymes, CFRD,
and NCLEX-style rationales.
Download the complete CF examination paper with answers and explanations to ace your
pediatric pulmonology certification or nursing final exam today.
, Cystic Fibrosis NCLEX Exam 2026
Section 1: Diagnostic Testing for Cystic Fibrosis
Question 1
Which diagnostic tests are used to confirm the diagnosis of cystic fibrosis? Select all that
apply.
A. Cystic fibrosis transmembrane regulator (CFTR) mutation analysis
B. Sweat chloride test
C. Newborn screening test
D. Chest radiography
E. Complete blood count
Answer: A, B, C
Explanation: Confirmatory diagnosis of cystic fibrosis requires evidence of CFTR
dysfunction. Sweat chloride testing (chloride ≥60 mmol/L on two separate occasions)
remains the gold standard. CFTR mutation analysis identifies disease-causing variants on
both alleles. Newborn screening typically involves immunoreactive trypsinogen (IRT)
measurement followed by DNA testing or sweat testing; however, it is a screening tool that
requires confirmatory testing. Chest radiography and complete blood count are supportive but
not diagnostic.
Section 2: Genetics and Inheritance Patterns
Question 2
Which scenario will most likely result in a child having cystic fibrosis (CF)?
A. One parent has CF and the other parent has no CFTR gene mutations
B. Both parents have CF and each passes the mutated gene to the child
C. Both parents are mutated gene carriers, and each passes the mutated gene to the child
D. One parent is a mutated gene carrier and the other parent has no CFTR mutations
Answer: C
Explanation: Cystic fibrosis follows an autosomal recessive inheritance pattern. For a child
to have CF, they must inherit two mutated CFTR genes, one from each parent. When both
parents are carriers (heterozygous), each pregnancy has a 25% chance of producing an
affected child (homozygous recessive), a 50% chance of producing an asymptomatic carrier,
and a 25% chance of an unaffected non-carrier.
,Section 3: Multi-System Involvement in Cystic Fibrosis
Question 3
Which systems are affected by cystic fibrosis? Select all that apply.
A. Respiratory system
B. Gastrointestinal system
C. Endocrine system
D. Integumentary system
E. Reproductive system
Answer: A, B, C, E
Explanation: CFTR dysfunction affects multiple organ systems. The respiratory system
demonstrates thick mucus, chronic infection, and bronchiectasis. The gastrointestinal system
shows pancreatic insufficiency, meconium ileus, and distal intestinal obstruction syndrome.
The endocrine system is affected via CF-related diabetes mellitus due to pancreatic islet cell
damage. The reproductive system shows congenital bilateral absence of the vas deferens in
males and reduced fertility in females. The integumentary system is not primarily affected,
though salt crystals on skin may be observed due to elevated sweat chloride.
Section 4: Interprofessional Care Coordination
Question 4
The nurse is caring for a family and child who is newly diagnosed with cystic fibrosis in an
acute care setting. Which professionals could the nurse consult to support the client and
family? Select all that apply.
A. Respiratory therapist
B. Registered dietitian
C. Social worker
D. Child life specialist
E. Psychologist
F. Pharmacist
Answer: A, B, C, D, E, F
Explanation: All listed professionals are essential members of the multidisciplinary CF care
team. The respiratory therapist provides airway clearance education. The dietitian addresses
nutritional needs and pancreatic enzyme management. The social worker assists with
, insurance, school accommodations, and community resources. Child life specialists help
younger children understand procedures. Psychologists address anxiety, depression, and
treatment adherence. Pharmacists review medication interactions and educate about proper
medication administration.
Section 5: Nutrition and Enzyme Management Assessment
Question 5
The nurse is caring for an adolescent with cystic fibrosis who has been losing weight and
having increased incidence of steatorrhea. Indicate whether each assessment question is
applicable or not applicable to gathering information related to the problem.
"Can you tell me how you usually take your enzymes with meals?"
Answer: Applicable
Explanation: Enzyme administration timing with meals directly affects nutrient absorption.
Enzymes must be taken immediately before or with the first bite of a meal to be effective.
"Can you tell me how you usually take your enzymes with snacks?"
Answer: Applicable
Explanation: Enzymes are required with snacks as well as meals, as snacks contribute
significant calories and fat. Missed snack doses can cause steatorrhea.
"Can you tell me about times when you might not take your enzymes?"
Answer: Applicable
Explanation: Identifying patterns of nonadherence (forgetting, social embarrassment, side
effects) is essential for addressing weight loss and steatorrhea.
"Can you tell me what you ate yesterday?"
Answer: Applicable
Explanation: Dietary recall helps determine if enzyme dosing matches fat intake. A high-fat
meal without sufficient enzymes causes steatorrhea.
"Where do you store your enzymes?"
Answer: Applicable
