Question

J.R., a 13-year-old with cystic fibrosis (CF), 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 CF. 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.” J.R. is admitted to the hospital for a suspected respiratory infection and CF exacerbation. His mother helps get him settled into the room. Your assessment includes the following: vital signs: 115/76, 85, 28, 101.8 ° F (38.8 ° C) (oral) and Spo2: 88% on room air. J.R. weighs 30 kg. Color: pale and dry with bluish-tinged nail beds and clubbing; capillary refill 3 seconds. Respiratory effort labored; coarse productive cough; rhonchi noted throughout; states he has pain of 3 to 4 of 10 with coughing. Thorax has a barrel-chest appearance; patient appears thin with decreased muscle mass. Last void and bowel movement this morning, no problems. Patient anxious and answers questions in short phrases. He is currently prescribed the following: • Ceftazidime (Fortaz) 2 g IV q8h • Gentamicin (Gentak) 100 mg IV q8h • Vancomycin (Vancocin) 450 mg IV q8h J.R.'s weight is below the 5th percentile. He has been on a high-calorie, high-protein diet at home; J.R. reports that he hasn't been hungry and really hasn't been eating much. JR. will be spending 14 to 21 days in the hospital for treatment of his pulmonary infection.  

1. J.R.'s weight is below the 5th percentile. He has been on a high-calorie, high-protein diet at home; J.R. reports that he hasn't been hungry and really hasn't been eating much. Describe the link between malnutrition and CF.

2. Which of the following 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 NS nebulizers

c. Administering pancreatic enzymes from the home supply with each snack

d. Increasing O2 during an episode of desaturation

3. J.R. asks you, “Will I be able to have children when I grow up?” Keeping his age in mind, 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 should discuss this with your physician and family.”

d. “CF does not affect the male reproductive system; however, it does affect the female reproductive tract.”

4. Which of the following GI comorbidities might you see in a patient with CF?

Select all that apply.

a. Celiac disease

b. Meconium ileus

c. Rectal prolapse

d. Chronic vomiting

e. Constipation

5. J.R.'s condition improves with antibiotic therapy and he is being discharged to home. As you provide your discharge teaching, discuss health promotion points to reinforce with J.R. and his parents. List health promotion points in priority order.

1.

2.

3.

4.

5.

6.

7.

8.

9.

10.

Answer 1

1.CFTR protein which is normally present in the lining of exocrine portions of pancreatic duct.The link between Cystic fibrosis are the mutation in the gene Cystic fibrosis transmemebrane regulator on the chromsome 7, affect the protein called CFTR, produces thick and sticky mucus. this thick and sticky mucus causes obstruction in the pancreatic duct and its branches and that leads to fibrosis of acinar gland of pancreas. thereby it reduces function of pancreas and loss of ability to produce pancreatic enzymes which is responsible for digestion of food in the intestine. so, fat and fat soluble vitamin and proterin malabsorption caused by lack of pancreatic enzymesand that leads to failure to grow and gain weight.

2.charting daily weight and intake and output

3. although 95% of males are sterile, you should discuss this with physician and family

4.meconiium ileus in infants with cystic fibrosis and constipation and vomiting(distal intestinal obstruction syndrome in ilealcecal area)

5.1. teach him and his family about sign of infection that would be fever, increased sputum production, increased mucus, chest tightness. wash hands often for 20 minutes

2. avoids crowds and also in winter times. avoid speaking with people who have infections like cold, fever.avoid passive smoking in case of exposure to passive smoking

3. educate him to sit with head slightely flexed and knee flexed to allow for air entry

4 instruct him to take breath slowly and to turn in the bed frequently in order to mobilise the secretions.

5.frequent rest periods in between exercise.continue deep breathing exercises

6. provide high calorie and high protein diet and small and frequent meals with adequate amount fluid intake. before each meal, cleaning mouth improves taste of food. provide him the diet that is interested to him

7.oxygen therapy is necessary when he partcipates in school or other any other activities

8.monitor for intake and output in order to assess fluid status in the body

9.continue medication and other measures as per physician order

10.regular follow up and tell him to contact health care provider if he has following symptoms like constipation, diarrohea, vomiting, abdominal pain, chest pain, change of color of sputum, dyspnea, fever