Cystic Fibrosis (15 Questions)

1. Baby Lucas Tremblay, 2 days old, is in the newborn nursery. His parents report that he has not passed any stool since birth. Lucas has a distended abdomen and is vomiting greenish (bilious) fluid. The nurse notes that attempts at rectal stimulation bring no stool.

Medical Orders:
*Abdominal X‑ray.
*NPO.
*IV fluids.
*Consult pediatric surgery.

Question:
What underlying condition does the nurse suspect based on these findings? *

Pyloric stenosis.

Cystic Fibrosis with meconium ileus.

Lactose intolerance.

Gastroesophageal reflux disease (GERD).

2. M. and Mme. Gagnon have a 4‑year‑old son diagnosed with Cystic Fibrosis. They are expecting another baby and ask the nurse, "How did this happen? None of us are sick in the family. What are the chances this will happen again?"

Medical Orders:
*Refer to genetic counselling.

Question:
What is the best explanation of CF inheritance? *

It is a sex‑linked disorder passed from mother to son only.

It is a dominant disorder; any child of an affected parent will have CF.

It is caused only by environmental exposure, not genetics.

It is an autosomal recessive disorder; both parents must carry one defective gene for a child to be affected.

3. M. Alexandre Gagnon, 16 years old, is admitted for a pulmonary exacerbation. The nurse is preparing to administer his morning respiratory treatments.

Assessment:
*Productive cough with thick green sputum.
*SpO2 93% on room air.

Medical Orders:
Salbutamol (Ventolin) MDI 2 puffs.
Dornase Alfa (Pulmozyme) nebulizer.
Chest Physiotherapy (CPT) / High-frequency chest wall oscillation.
Tobramycin inhaled antibiotic.

Question:
In which sequence should the nurse administer these therapies to maximize effectiveness? *

Tobramycin -> Pulmozyme -> CPT -> Salbutamol.

CPT -> Salbutamol -> Pulmozyme -> Tobramycin.

Salbutamol -> Pulmozyme -> CPT -> Tobramycin.

The order does not matter as long as they are all given.

4. Mme. Sophie Roy, 4 years old, is hospitalized. The nurse brings her lunch tray and her medication. Sophie refuses to swallow the Pancrelipase (Creon) capsule whole because "it's too big."

Medical Orders:
*Regular diet High Fat/High Calorie.
*Pancrelipase (Creon) 3 capsules PO with meals.

Question:
What is the correct nursing action? *

Crush the capsule into a fine powder and mix it with milk.

Withhold the medication and notify the physician.

Dissolve the capsule in a glass of warm water.

Open the capsule and sprinkle the beads onto a small amount of acidic food like applesauce; instruct her not to chew the beads.

5. Émile Roy, 7 years old, has CF with pancreatic insufficiency. He takes pancreatic enzyme capsules with meals and snacks. This morning, he begins eating lunch in hospital before the nurse arrives with his enzymes. His mother asks, "Can he just take them after he finishes?"

Medical Orders:
*Pancrelipase (pancreatic enzymes) with all meals and snacks.

Question:
What should the nurse instruct? *

"It does not matter when he takes them, as long as it’s the right daily dose."

"He should take the enzymes immediately before or with the first bites of food to help digest the meal."

"He should take them at bedtime so they work overnight."

"He only needs them if he eats fatty foods."

6. M. Étienne Bouchard, 14 years old, is seen in the CF clinic. He has lost 2 kg since his last visit. The nurse is reviewing his dietary habits to plan a catch-up growth diet.

Medical Orders:
*Dietary consult.
*Vitamin A, D, E, K supplements daily.

Question:
Which diet is indicated for a patient with Cystic Fibrosis? *

Low fat, high carbohydrate, sodium restricted.

High calorie, high protein, high fat (unrestricted), and liberal salt intake.

Gluten-free, low calorie, low fat, and low salt intake.

High fiber, low protein, low calorie, and low fat (restricted).

7. Mme. Claire Dubé, mother of 10‑year‑old Maxime, who has CF, says, "I keep trying to limit fats and calories because I don’t want him to become overweight."

Medical Orders:
*High‑calorie, high‑fat diet.
*Multivitamins including fat‑soluble vitamins A, D, E, K.

Question:
What dietary teaching is most appropriate? *

"You are right to limit fats; CF patients should eat a low‑fat diet."

"Children with CF need a diet high in calories, protein, and fats."

"He should avoid all fried foods but otherwise eat a normal diet."

"Calories should be restricted because he is less active."

8. Thomas, 13 years old with CF, is hospitalized with increased cough and thick sputum. He says he is tired and refuses his chest physiotherapy (postural drainage and percussion), saying, "It doesn’t help anyway."

Medical Orders:
*Chest physiotherapy (CPT) with postural drainage BID and PRN.
*Salbutamol nebulization prior to CPT.

Question:
What should the nurse explain about the purpose of CPT? *

"It helps your heart pump stronger."

"It helps loosen and mobilize thick mucus."

"It reduces inflammation in your pancreas."

"It replaces the need for antibiotics."

9. Mme. Valérie Fortin, 19 years old, with Cystic Fibrosis, is in the waiting room of the pulmonary clinic. She sees her friend M. Luc (who also has CF) across the room and gets up to go sit next to him to chat.

Medical Orders:
*Routine CF clinic visit.

Question:
What is the nurse’s priority action? *

Encourage the social interaction to improve mental health.

Intervene and ask them to maintain a distance of at least 2 meters (6 feet).

Allow them to sit together if they are both wearing masks.

Place them immediately in negative pressure rooms.

10. Alex and Julien, both 15 years old and diagnosed with CF, are admitted to the same unit for tune‑up therapy. Their mothers suggest that the boys share a room to "support each other." The nurse reviews Alex’s chart and notes chronic colonization with Pseudomonas aeruginosa.

Medical Orders:
*Contact/Droplet precautions as per CF infection control policy.
*Separate rooms if colonized with different organisms.

Question:
What is the most appropriate nursing decision? *

Agree and room them together to support socialization.

Put them together but ensure they wear masks.

Place them in separate rooms to prevent cross‑infection.

Allow them to share a room but separate their beds by at least 2 meters.

11. M. Sylvain Cote, 22 years old, is admitted for IV antibiotics. While coughing vigorously during chest physiotherapy, he suddenly grabs his chest.

Assessment:
*Reports sudden, sharp pleuritic chest pain on the left side.
*Severe dyspnea.
*SpO2 dropped from 94% to 86%.
*Decreased breath sounds on the left side.

Medical Orders:
*Oxygen PRN.
*Chest X-ray STAT.

Question:
What complication does the nurse suspect? *

Pulmonary Embolism.

Spontaneous Pneumothorax.

Rib fracture.

Myocardial Infarction.

12. M. Pierre Lavoie, 22 years old, has CF and works landscaping outdoors in July. He presents to the clinic with fatigue, muscle cramps, dizziness, and very salty‑tasting sweat noticed by his partner. He reports decreased urine output.

Medical Orders:
*Oral rehydration solution.
*Assess serum electrolytes.

Question:
What is the nurse’s interpretation of these symptoms? *

Normal effect of heavy exercise.

Excessive salt and fluid loss in sweat.

Early onset of kidney failure unrelated to CF.

Overdose of pancreatic enzymes.

13. Mme. Julie Desrosiers, 30 years old, has CF and is admitted for weight loss and fatigue despite good enzyme use and caloric intake. Her random blood glucose is 14 mmol/L. She asks, "Does this mean I have regular diabetes like my grandmother?"

Medical Orders:
*Fasting blood glucose and HbA1c.
*Sliding scale insulin if confirmed CFRD.

Question:
What is the nurse’s best explanation? *

"No, CF never affects blood sugar levels."

"Many adults with CF develop a type of diabetes."

"This will go away once your infection clears."

"You will only need pills, not insulin."

14. M. Antoine Côté, 26 years old, has CF and is planning marriage. He asks the nurse, "Can I have children of my own naturally?"

Medical Orders:
*Refer to fertility specialist/genetic counseling.

Question:
What information should the nurse provide? *

"Men with CF are always completely sterile and can never have biological children."

"Most men with CF produce normal sperm but are usually infertile because the vas deferens is blocked or absent."

"CF affects only the lungs, not fertility."

"Only women with CF are infertile, not men."

15. Lucas, 16 years old with CF, is admitted with an acute pulmonary exacerbation. He is tachypneic (RR 30), coughing thick green sputum, and appears fatigued. His SpO₂ is 89% on room air, and he is febrile at 38.7°C.

Medical Orders:
*Oxygen 1–3 L/min via nasal cannula to maintain SpO₂ ≥ 92%.
*IV Piperacillin‑Tazobactam.
*Chest physiotherapy QID.
*High‑calorie diet.

Question:
What is the nurse’s priority immediate intervention? *

Arrange a dietitian consult.

Start 1 L/min of oxygen via nasal cannula.

Begin chest physiotherapy.

Titrate oxygen to 4 L/min as ordered.