Cystic fibrosis care is a daily balancing act between the lungs and the gut, and the exam tests whether you understand both halves. These questions cover airway clearance and chest physiotherapy, pancreatic enzyme replacement and its timing, the high-calorie nutritional needs these children have, and the infection-prevention rules that protect them from each other.
Question 1
111 of 150. A 3-year-old child with chronic daily cough and nasal polyps has a sweat chloride level of 72 mmol/L on a properly collected sample.
- A) Confirm the cystic fibrosis diagnosis and refer to a specialist.✓
- B) Classify the result as indeterminate and order a repeat test.
- C) Reassure the family that this is a normal developmental finding.
- D) Prescribe daily oral corticosteroids to reduce the nasal polyp size.
💡 Key Takeaway
A sweat chloride level of 60 mmol/L or greater confirms the diagnosis of cystic fibrosis.
Show rationale
A sweat chloride level of 60 mmol/L or greater on a valid sample is diagnostic for cystic fibrosis. Given the child's clinical symptoms of chronic cough and nasal polyps, the correct action is to confirm the diagnosis and immediately refer the patient to an accredited cystic fibrosis care center for comprehensive management. Classifying the result as indeterminate is incorrect because it exceeds the 59 mmol/L threshold. Reassuring the family is inappropriate for a positive diagnostic test. Prescribing oral corticosteroids is not the primary intervention for a new cystic fibrosis diagnosis.
Question 2
36 of 150. A 16-year-old with end-stage cystic fibrosis has had five hospitalizations in six months and now requires continuous BiPAP for respiratory support. He tells the nurse, “I’m so tired of all this – I just want to stay home and be comfortable.” His parents are still pursuing a lung transplant evaluation. Which action should the nurse take?
- A) Encourage the parents to honor their son’s wishes for comfort care.
- B) Arrange a goals-of-care conference including the adolescent and family.✓
- C) Explain that transplant evaluation is still reasonable given his age.
- D) Ask the social worker to begin the hospice referral process.
💡 Key Takeaway
Goals-of-care conferences align adolescent and family preferences when treatment goals diverge.
Show rationale
The adolescent’s statement reveals suffering and readiness to forgo aggressive treatment, yet his parents remain focused on transplant. A goals-of-care conference (B) includes the adolescent’s voice and allows the family to collaboratively explore the best path forward. Option A pressures parents without a structured conversation. Option C disregards the child’s experience and may promote false hope. Option D prematurely initiates hospice without a shared decision. The nurse’s action to arrange a conference aligns with pediatric advance care planning principles, which stress that mature minors should participate in decisions about their own end-of-life care when disagreements arise. This approach preserves trust and ensures that any treatment shift is ethically sound. By facilitating this communication, the nurse helps the family reconcile divergent goals and protect the adolescent’s comfort.
Question 3
37 of 150. A 4-year-old with cystic fibrosis presents with large, greasy, foul-smelling stools and poor weight gain over the past month. Which intervention should the nurse anticipate incorporating into the care plan?
- A) Decrease the child's daily dietary fat intake immediately.
- B) Increase the pancreatic enzyme dosage before each meal.✓
- C) Switch the child to a low-protein, high-carbohydrate diet.
- D) Administer a daily over-the-counter oral antidiarrheal medication.
💡 Key Takeaway
Signs of malabsorption in cystic fibrosis require an upward adjustment of pancreatic enzyme replacement therapy.
Show rationale
Greasy, foul-smelling stools and poor weight gain are classic signs of steatorrhea and malabsorption, indicating that the current enzyme dose is insufficient. The standard approach is to increase the enzyme dosage to match dietary intake. Option A is incorrect because children with cystic fibrosis require a high-fat diet to meet their elevated caloric needs; fat should not be restricted. Option C is incorrect because a high-protein diet is essential for growth and muscle maintenance in this population. Option D is incorrect because antidiarrheals do not address the underlying pancreatic insufficiency causing the symptoms.
Question 4
75 of 150. The mother of a 4-year-old healthy boy reports he recently resumed wetting the bed and demanding a pacifier since his older sister began cystic fibrosis treatments. What is the most accurate interpretation of this sibling's behavior?
- A) It indicates a high likelihood of an underlying urinary tract infection.
- B) It represents expected developmental regression due to altered family dynamics.✓
- C) It suggests the child is mimicking the sister's chronic illness symptoms.
- D) It demonstrates early signs of severe oppositional defiant disorder development.
💡 Key Takeaway
Behavioral regression is a typical stress response in preschool-aged siblings facing major family disruptions.
Show rationale
Behavioral regression, such as bedwetting or using a pacifier, is a typical stress response in preschool-aged siblings facing major family disruptions like a new chronic illness treatment regimen. It reflects a need for parental attention rather than pathology. While a urinary tract infection could cause enuresis, the simultaneous demand for a pacifier points clearly to regression. Mimicking symptoms would involve respiratory or digestive complaints similar to cystic fibrosis, not developmental regression. Oppositional defiant disorder involves a pattern of angry, vindictive behavior, not simple regression seeking comfort.
Question 5
114 of 150. A 14-year-old with cystic fibrosis and an active AND order is admitted for acute severe bone pain following a minor fall. The parents request immediate intervention for the pain.
- A) Administer prescribed analgesics to manage the acute pain episode.✓
- B) Withhold analgesics since the AND order precludes acute interventions.
- C) Request a temporary revocation of the AND order for treatment.
- D) Consult the ethics committee before providing any pain medications.
💡 Key Takeaway
An AND order applies to resuscitation and life-sustaining measures, not the treatment of reversible acute pain.
Show rationale
An AND or DNR order specifically dictates the withholding of cardiopulmonary resuscitation in the event of an arrest; it does not mean "do not treat." Providing comfort care and managing reversible acute conditions, such as pain from a fall, remains a primary clinical obligation. Option B is incorrect because withholding analgesics violates the core principles of palliative and standard medical care. Option C is incorrect because treating acute pain does not conflict with an AND order, so no revocation is necessary. Option D is incorrect because administering pain medication for an acute injury is a standard, expected nursing intervention that does not require an ethics consultation, even for a patient with an active AND order.
Question 6
119 of 150. A 6-year-old child is 8 hours post-tonsillectomy. The child is sleeping but exhibits frequent swallowing motions and a heart rate of 135 bpm.
- A) Gently awaken the child to inspect the oropharynx using a penlight.✓
- B) Administer the prescribed intravenous analgesic for throat discomfort.
- C) Reposition the child to a prone position to facilitate oral drainage.
- D) Document the findings as normal sleep patterns after anesthesia.
💡 Key Takeaway
Frequent swallowing in a sleeping post-tonsillectomy patient is a classic early sign of active bleeding.
Show rationale
Frequent swallowing and tachycardia in a sleeping child are classic early signs of postoperative hemorrhage. Blood trickling down the throat triggers the swallowing reflex. You must gently awaken the child to inspect the oropharynx using a penlight to confirm active bleeding. Administering analgesics ignores the potential emergency and masks symptoms. Repositioning to a prone position might help with airway clearance if vomiting occurs, but it delays the critical assessment of the surgical site. Documenting the findings as normal is incorrect because these are specific red flags for bleeding, not typical sleep behaviors. Early recognition of frequent swallowing prevents progression to hypovolemic shock.
Question 7
29 of 150. The nurse delegates the setup of a room for a 12-year-old adolescent admitted with new-onset unprovoked seizures. Which observation of the completed room setup indicates the nurse must correct the unlicensed assistive personnel?
- A) The bed is locked and placed in the lowest position.
- B) Commercial seizure pads are secured to the upper side rails.
- C) An appropriately sized oxygen mask is connected to the flowmeter.
- D) The rigid suction tip is stored inside the supply room.✓
💡 Key Takeaway
All components of the suction setup must be kept at the bedside for immediate emergency access.
Show rationale
When assessing delegated tasks, the nurse must ensure all safety standards are met. Option D requires correction because the rigid suction tip (Yankauer) must be at the bedside, connected to the tubing and canister, to allow for immediate airway clearance during a seizure. Options A, B, and C represent correct implementations of seizure precautions. Keeping the bed low and locked prevents fall injuries, securing commercial pads protects the patient from striking hard side rails, and having connected oxygen ensures rapid response to potential hypoxia during or after the seizure event.
Question 8
46 of 150. The nurse is evaluating the room of a 14-month-old toddler admitted for complex febrile seizures. When assessing the crib setup for safety, which finding requires an immediate intervention by the nurse?
- A) The crib mattress is placed in the lowest possible position.
- B) Thick blankets are tied securely around the crib side rails.✓
- C) A working bag-valve-mask device is located on the bedside table.
- D) The suction canister is attached to the wall vacuum source.
💡 Key Takeaway
Commercial crib bumpers or pads should be used instead of loose blankets to prevent suffocation.
Show rationale
While padding side rails is a standard component of seizure precautions, using thick blankets for a toddler introduces a significant suffocation risk and violates safe sleep guidelines. Option B requires immediate intervention because commercial, breathable seizure pads should be used instead. Option A is a correct safety measure that prevents falls if the child climbs or stands. Option C is appropriate, as emergency ventilation equipment must be readily accessible. Option D is also a correct finding, ensuring that airway clearance tools are assembled and ready for immediate use.
Question 9
57 of 150. A 14-year-old male with cystic fibrosis has a BMI of 16. He expresses frustration about being "treated like a kid" and his lack of physical growth compared to his peers. Which response is most appropriate?
- A) Reassure him that physical growth will eventually catch up.
- B) Assess his specific concerns regarding peer relationships and dating.✓
- C) Refer him to an endocrinologist for growth hormone therapy.
- D) Advise him to increase his daily caloric intake immediately.
💡 Key Takeaway
Delayed puberty in chronic illness significantly impacts a teenager's psychosocial identity and requires targeted relational assessment.
Show rationale
Teens with cystic fibrosis often experience delayed puberty and poor weight gain, which can severely impact their self-esteem and identity development. Option B is the best choice because it directly assesses how his lack of physical growth is affecting his psychosocial well-being and peer interactions. Adolescents measure their normalcy against their peers, and feeling physically behind can lead to isolation or depression. Option A is dismissive of his current emotional distress and offers false reassurance without exploring his feelings. Option C jumps to a medical intervention without first completing a comprehensive psychosocial and endocrine assessment. Option D focuses purely on the physical aspect of caloric intake, completely ignoring the identity and body image concerns he is expressing. Validating his feelings builds trust and guides appropriate psychosocial support.
Question 10
83 of 150. During interdisciplinary rounds, the nurse presents a 4-year-old with a recent traumatic brain injury who exhibits frequent coughing during meals and difficulty holding a spoon. Which recommendation should the nurse prioritize to ensure safe care coordination?
- A) Advocate for an urgent speech-language pathology evaluation.✓
- B) Advocate for an urgent occupational therapy evaluation.
- C) Advocate for an urgent physical therapy evaluation.
- D) Advocate for an urgent respiratory therapy evaluation.
💡 Key Takeaway
Speech-language pathology is the primary discipline for evaluating and managing dysphagia to prevent aspiration.
Show rationale
Frequent coughing during meals strongly suggests dysphagia and a high risk for aspiration, making a speech-language pathology consult the immediate priority for airway protection and safe feeding. While occupational therapy is certainly needed to address the fine motor deficit of holding a spoon, airway safety always takes clinical precedence over fine motor skill building. Physical therapy focuses on gross motor rehabilitation and mobility, which does not address the immediate feeding safety concern. Respiratory therapy manages airway clearance and ventilation but does not perform clinical swallow evaluations or prescribe modified diets. By prioritizing speech therapy, the nurse ensures the child receives a proper swallowing assessment before continuing oral intake.
