J.R., a 13-year-old with cystic fibrosis, is being seen in the outpatient clinic for a biannual evaluation. J.R. lives at home with his parents and 7-year-old sister, C.R., who also has cystic fibrosis. J.R. reports that he doesn't feel good, explaining that he has missed the last week of school, doesn't have any energy, is coughing more, and is having a hard time breathing.
- What additional data should be obtained from J.R. and his parents?
- Describe the pathophysiology of cystic fibrosis (CF). Be sure to address the multisystem
component of this disorder
J.R. is admitted to the hospital for a suspected respiratory infection. Your assessment includes the following: color, pale pink with bluish tinged nail beds; respiratory rate, 28 breaths/min and somewhat labored; oral temperature, 38.8 C (101.8 F); Sa O2 , 88%; rhonchi noted throughout; thorax has a barrelchest appearance; appears thin, weighs 30 kg.
- Why is J.R. at risk for developing pulmonary infections?
- What are the common microorganisms that cause respiratory infections in children with
cystic fibrosis?
Medication Orders Ceftazidime (Fortaz) 2 g IV q8h Gentamicin (Gentak) 100 mg IV q8h Vancomycin (Vancocin) 450 mg IV q8h
- You review the drugs that have been ordered to treat J.R.'s suspected infection. What will you
do before administering these drugs?
- Use a nursing drug reference book to find the safe dosage ranges for the previously
mentioned antibiotics, and calculate the dosage of each antibiotic J.R. will receive with the
previous orders. Are the prescribed doses within the safe ranges? (Show all work.)
J.R. weighs 30 kg.
- What factor will affect the selection of antibiotics and dosages?
- You are reviewing the physician orders for respiratory care. Which of these nursing
interventions would you expect to perform, and why?
a. Administer aerosolized albuterol (a bronchodilator).
b. Administer chest physiotherapy (CPT) before administering the bronchodilator
c. Monitor continuous pulse oximetry
d. Administer aerosolized Pulmozyme (dornase alfa) after administration of bronchodilator
e. Administer nebulized NS (normal saline).
f. Administer antibiotic via JET nebulizer.
g. Limit fluid intake.
- J.R.'s weight is below the 5th percentile. He has been on a high-calorie, high-protein diet at
home; however, he reports that he hasn't been hungry and really hasn't been eating much.
Describe the link between malnutrition and cystic fibrosis.
- Which of these actions can be delegated to the nursing assistive personnel (NAP)?
a. Charting daily weights and intake and output
b. Instructing the parents on correct administration of normal saline nebulizers.
c. Administering pancreatic enzymes from home supply with each snack.
d. Increasing O 2 during an episode of desaturation.
- Which of these strategies are appropriate to manage the GI dysfunction that patients with CF
often experience? (Select all that apply.)
a. Administer fat-soluble vitamins daily.
b. Administer pancreatic enzymes with meals and snacks.
c. Restrict fat intake.
d. Encourage a high-protein diet.
e. Breastfeeding is contraindicated in infants with CF.
f. Encourage snacks between meals
- What clinical sign assists in determining the effective dosage of pancreatic enzymes
- Discuss the common GI disorders that children with CF might be prone to.
J.R. will be spending 14 to 21 days in the hospital for treatment of his pulmonary infection. How will this
hospitalization affect J.R.'s normal development?
- How can you foster his development while he is hospitalized?
- J.R. is an adolescent and asks you, Will I be able to have children when I grow up? Your
best response would be:
a. You should discuss this with your parents. I will let them know you asked.
b. Most males have a significant chance of being sterile and you won't need to consider use
of contraception.
c. Although nearly 95% of males are sterile, you can discuss this with your physician and
family.
d. CF does not affect the male reproductive system; however, it does affect the female
reproductive tract.
J.R. improves with antibiotic therapy and is being discharged to home.
- Discuss health promotion behaviors that need to be reinforced with J.R. and his parents.
Full Answer Section
- Respiratory Complications: Chronic lung infections, inflammation, and impaired lung function.
- Pancreatic Dysfunction: Insufficient production of digestive enzymes, leading to malabsorption of nutrients.
- Gastrointestinal Issues: Pancreatitis, intestinal obstructions, and difficulty with bowel movements.
- Infertility: Reduced fertility in both males and females.
3. Risk Factors for Pulmonary Infections in CF Patients
Individuals with CF are at increased risk of pulmonary infections due to:
- Impaired mucociliary clearance: The thick mucus obstructs the airways, making it difficult to clear bacteria and other pathogens.
- Altered airway defense mechanisms: Decreased production of antimicrobial peptides and impaired immune response.
4. Common Microorganisms
Common microorganisms that cause respiratory infections in CF patients include:
- Pseudomonas aeruginosa
- Staphylococcus aureus
- Burkholderia cepacia complex
5. Pre-Medication Considerations
Before administering these medications, it's crucial to:
- Assess renal function: Gentamicin can be nephrotoxic, so renal function should be monitored.
- Monitor for allergic reactions: Assess for previous allergic reactions to antibiotics.
- Check for drug interactions: Identify any potential drug interactions with other medications J.R. may be taking.
6. Dosage Calculations
- Ceftazidime: Safe dose range: 50-100 mg/kg/day divided into 2-3 doses.
- Dose for J.R.: 100 mg/kg/day ÷ 3 doses = 33.3 mg/kg/dose.
- For a 30 kg child: 33.3 mg/kg/dose × 30 kg = 999 mg/dose.
- Gentamicin: Safe dose range: 2-7.5 mg/kg/day divided into 2-3 doses.
- Dose for J.R.: 5 mg/kg/day ÷ 2 doses = 2.5 mg/kg/dose.
- For a 30 kg child: 2.5 mg/kg/dose × 30 kg = 75 mg/dose.
- Vancomycin: Safe dose range: 40-60 mg/kg/day divided into 2-4 doses.
- Dose for J.R.: 45 mg/kg/day ÷ 2 doses = 22.5 mg/kg/dose.
- For a 30 kg child: 22.5 mg/kg/dose × 30 kg = 675 mg/dose.
The prescribed doses for Ceftazidime and Vancomycin are within the safe range. However, the dose of Gentamicin is slightly higher than the recommended maximum dose.
7. Factors Affecting Antibiotic Selection and Dosage
- Age: Pediatric patients may have different dosing requirements compared to adults.
- Weight: The dose is often adjusted based on the patient's weight.
- Renal function: Kidney function can impact drug clearance and may require dose adjustments.
- Liver function: Liver function can affect drug metabolism and clearance.
- Allergy history: Previous allergic reactions to antibiotics should be considered.
- Microbiological data: Culture and sensitivity testing can guide antibiotic selection and dosage.
8. Respiratory Care Interventions
- a, c, d, and e: These interventions are appropriate for managing respiratory symptoms in CF.
- b: CPT should be administered after bronchodilator therapy to improve airway clearance.
- f: Antibiotics are typically administered intravenously, not via nebulizer.
- g: Fluid intake should be encouraged to help loosen secretions.
9. Malnutrition and CF
Malabsorption due to pancreatic insufficiency is a common complication of CF. This can lead to malnutrition, growth failure, and delayed development.
10. Delegation to NAP
- a: Charting daily weights and intake and output can be delegated to NAP.
- b, c, and d: These tasks require nursing judgment and should not be delegated.
11. Managing GI Dysfunction in CF
- a, b, d, and f: These strategies are appropriate for managing GI dysfunction in CF.
- c and e: Fat intake should not be restricted, and breastfeeding is encouraged for infants with CF.
12. Assessing Pancreatic Enzyme Dosage
Clinical signs of effective pancreatic enzyme replacement therapy include improved weight gain, reduced steatorrhea (fatty stools), and normal growth and development.
13. Common GI Disorders in CF
Common GI disorders in CF include:
- Pancreatitis: Inflammation of the pancreas.
- Intestinal Obstruction: Blockage of the intestines caused by meconium ileus or fecal impaction.
- Liver Disease: Cirrhosis and liver failure.
14. Fostering Development During Hospitalization
- Encourage age-appropriate activities: Provide educational materials, games, and social interaction.
- Maintain a normal routine: Establish a consistent daily schedule, including meal times, sleep, and activity periods.
- Involve parents in care: Encourage parents to participate in their child's care and spend time with them.
- Provide emotional support: Offer emotional support and reassurance to J.R. and his family.
15. Addressing Fertility Concerns
- c: Although many males with CF are infertile, some may be able to father children through assisted reproductive technologies. It's important for J.R. to discuss his specific situation with a healthcare provider.
16. Health Promotion Behaviors
- Adherence to medical regimen: Emphasize the importance of taking medications as prescribed, performing chest physiotherapy, and following a prescribed diet.
- Infection prevention: Teach techniques to prevent infection, such as handwashing and avoiding exposure to sick individuals.
- Regular medical check-ups: Schedule regular check-ups with healthcare providers to monitor lung function, nutritional status, and overall health.
- Psychosocial support: Encourage participation in support groups and counseling to address emotional and psychological needs.