PEDIATRIC DISORDER

SCENARIO

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.”

1. What additional data should be obtained from J.R. and his parents?

What medications and respiratory regimen does the child follow, and does he

or she take any over-the-counter supplements? Is the child in contact with any sick

people? Is the child physically active, and if so, how long and how frequently? What

exactly is the definition of a child's BM?

2. Describe the pathophysiology of cystic fibrosis (CF). Be sure to address the

multisystem component of this disorder.

A chronic multisystem disorder is an autosomal recessive trait disorder with

exocrine gland dysfunction. The exocrine gland's mucus is abnormally thick and

copious, obstructing the small passageways of the affected organs, particularly the

respiratory, gastrointestinal, and reproductive systems.

 Respiratory- Mucus stagnation in the airway leads to bacterial colonization and

lung tissue destruction, putting the patient at risk for respiratory infections.
 Gastrointestinal- Mucus thickness can prevent pancreatic enzymes from

reaching the duodenum, resulting in malabsorption syndrome.

 Reproductive- Males are usually sterile but not impotent as a result of abnormal

secretions blocking the vas deferens or the failure of normal duct structure

development.

 Skin and electrolyte balance- When there are abnormally high concentrations of

sodium and chloride in the sweat due to impaired sodium and chloride re-

absorption, the patient can develop hyponatremia , hypochloremia , and

dehydration.

CASE STUDY PROGRESS

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); SaO2,

88%; rhonchi noted throughout; thorax has a barrel-chest appearance; appears thin,

weighs 30 kg.

3. Why is J.R. at risk for developing pulmonary infections?

JR is susceptible to respiratory infections because his lungs cannot clear the

mucous due to its dense, tenacious nature. Because mucous is so thick and sticky,

breaking it up and expelling it from the lungs is difficult, especially through the

smaller lung airways. It has the potential to affect all airways in the long run. Because

of the obstructions and stasis of atypical materials, this is an ideal breeding ground
for bacteria, and many respiratory infections become chronic due to the difficulty in

eliminating them.

4. What are the common microorganisms that cause respiratory infections in

children with cystic fibrosis?

Common pathogens include Pseudomonas aeruginosa, Staphylococcus

aureus, and Hemophilus influenzae.

CHART VIEW

Medication Orders :

Ceftazidime (Fortaz) 2 g IV q8h

Gentamicin (Gentak) 100 mg IV q8h

Vancomycin (Vancocin) 450 mg IV q8h

4. You review the drugs that have been ordered to treat J.R.'s suspected infection.

What will you do before administering these drugs?

I will obtain a blood glucose level before administering the medications.

6. What factor will affect the selection of antibiotics and dosages?

ceftazidime (Fortaz) 2g IV q8h

(IV or IM use, treat or prevent bacterial infections, broad spectrum antibiotic,

indicated treatment
for lower respiratory infection and pneumonia. Comes 1-2 g viles. )

Safe does for >12 years: 1-2 g IV q8hr

30-50mg/kg X 30kg/ day = 900-1500 mg/dose = 0.9-1.5 g/dose however 30-50 mg/kg

IV. q8hr; not to exceed 6 g/day or 2g/dose (higher end of dosing range reserved for

patients with immunocompromised, meningitis, or cystic fibrosis)

This dose is safe.

gentamicin 300mg IV q24hr

Treats wide variety of bacterial infections, stops bacterial growth

Cystic Fibrosis patients7.5-10.5 mg/kg/day IV. 7.5-10.5 mg/kg X 30 kg = 225 mg- 315

mg/day

This dose is safe.

vancomycin 450mg IV q8h

Antibiotic used to treat a number of bacterial infections. It is recommended IV 40

mg/kg/day IV 40 mg X 30 kg = 1200 mg/day divided by 3X/ day = 400 mg/ dose safe

dose pediatrics. 450mg per dose is too high ! (However the patient may needs the

extra 50 mg per dose with cystic fibrosis if their body is having a hard time

metabolizing the drug)

7. 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).

 Yes, this medication will aid in the opening of the bronchi for easier

expectoration.
b. Administer chest physiotherapy (CPT) before administering the bronchodilator.

c. Monitor continuous pulse oximetry

 to assess pulmonary function and gas exchange

d. Administer aerosolized Pulmozyme (dornase alfa) after administration of

bronchodilator.

 This medication acts as a mucolytic, meaning it thins secretions and improves

lung function.

e. Administer nebulized NS (normal saline).

 it will help with airway hydration and clear mucus

f. Administer antibiotic via JET nebulizer.

 antibiotics administered through a JET nebulizer will allow direct antimicrobial

exposure

g. Limit fluid intake.

7. 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.

Mucus thickness can prevent essential nutrients from being absorbed and

pancreatic enzymes from reaching the duodenum, resulting in malabsorption

syndrome.

9. 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 O2 during an episode of desaturation.

10. 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.

11. What clinical sign assists in determining the effective dosage of pancreatic

enzymes?

Examine the stool- if it floats, it has more fat, indicating that it hasn't been

absorbed, and if it sinks, it has been absorbed and is receiving an effective dosage.

12. Discuss the common GI disorders that children with CF might be prone to.

 Meconium ileu - This feces is very thick and sticky. Meconium ileus is a bowel

obstruction caused by thicker and stickier meconium in your child's intestine,

resulting in a blockage in the ileum, a section of the small intestine. Cystic

 fibrosis affects the majority of infants with meconium ileus. The first signs of

meconium ileus are abdominal distention (a swollen stomach), bilious (green)

vomit, and a lack of meconium passage.

 Rectal prolapse - When the rectum falls through the anus, this is referred to as

rectal prolapse. In complete rectal prolapse, the entire rectum wall falls through

the anus. In partial rectal prolapse, only the rectum lining passes through the

anus.

 Constipation - Constipation occurs when bowel movements become less

frequent and stools become difficult to pass. It is usually caused by a change in

diet or routine, or by a lack of fiber. Contact your doctor if you have severe pain,

blood in your stools, or constipation that lasts more than three weeks.

Constipation occurs when your colon absorbs too much water from waste

(stool/poop), causing the stool to harden and become difficult to push out of

your body.

CASE STUDY PROGRESS

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?

13. How can you foster his development while he is hospitalized?

Encourage age-appropriate independence in self-care. Arrange for

schoolwork to be brought to the patient so that he can complete assignments and

not fall behind in his studies.

14. 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.”

CASE STUDY OUTCOME:

J.R. improves with antibiotic therapy and is being discharged to home

15. Discuss health promotion behaviors that need to be reinforced with J.R. and his

parents.

Maintain immunization and flu vaccine records; consume nutritious foods

and snacks containing pancreatic enzymes; engage in regular exercise; and educate

parents and children on the signs of complications and appropriate actions to take,

as well as maintain follow-up appointments.