Cystic Fibrosis (CF)
“When I kiss my daughter, her skin tastes like salt.”
Salty-tasting skin is a symptom of CF. It is caused by a high chloride and sodium concentration produced by the sweat glands.
2. Q: Which documentation further supports the diagnosis of CF?
A: A history of frequent respiratory infections.
The respiratory system is affected by abnormally thick, sticky secretions that cause airway obstruction to the lungs. Other clinical manifestations of CF include poor growth and/or weight loss, a dry, non-productive cough, and increased bleeding tendencies caused by a deficiency of the fat-soluble Vitamin K.
Parents report foul-smelling stools that have white streaks and are sticky.
The term for undigested fat in the stools for clients with CF is steatorrhea. The foul smell is a result of the presence of protein. Large, loose, and sticky are characterizations of stools of clients with CF.
3. It is a simple, painless, reliable test that measures the amount of chloride in sweat.
The sweat test has been the gold standard for diagnosing CF for the past 40 years.
David and Paula have been at Debbie’s bedside since she was admitted to the hospital. David tells the nurse that Paula is telling everyone that Debbie is going to be fine, and that there has been a mistake and everything will be all right as long as they pray.
4. Make arrangements to meet with Paula privately at the first opportunity.
The nurse should meet with Paula to assess the situation before taking further action.
5. “Paula, I know this has to be a very scary and difficult time for you. What can I do to help you?”
This response promotes good (beneficence) by offering a nonjudgmental, supportive reply.
6. “I will sit with Debbie so you and your wife can talk. We have a quiet room that you can use that is private and close by.”
The parents are more likely to leave the room to deal with their crisis if they are assured that Debbie with not be alone.
7. Q: What nursing diagnosis has the highest priority?
A: Altered nutrition: less than body requirements related to poor intestinal absorption.
Oxygen, food, and water are the priority physiological needs according to Maslow’s Hierarchy of Needs. These biological needs must be satisfied before a person can move to a higher level need.
8. Q: Which is an expected outcome for this nursing diagnosis: Ineffective airway clearance related to excessive pulmonary secretions.
A: The child will be able to remove mucus from the airway by coughing.
9. Standards of care are published statements that describe the level of care that the client with cystic fibrosis (or other diseases) can expect from nurses.
10. “It is care like vision, hearing, and scoliosis screening that Debbie will get when she starts school.”
Secondary nursing care includes all types of screenings to help with early intervention.
11. Both of Debbie’s biological parents must have the CF gene in order for Debbie to get it.
CF is transmitted as an autosomal recessive trait, which means that both parents must carry the gene for the child to be affected. Each offspring of these parents has a 1 in 4 chance of having the disease.
12. Perform chest percussion and postural chest drainage 2 times a day.
This will loosen secretions and move them from peripheral airways into the main airways where they can be expectorated.
13. Discuss the importance of playing games that require Debbie to do physical activity, such as running, jumping, or skipping.
Research suggests that children with CF who regularly participate in aerobic exercise such as jogging or swimming have fewer pulmonary complications.
14. Mobilizing secretions is an important aspect of nursing care for children with CF.
A: Teach Debbie to take a deep breath and then exhale while whispering the word “huff.”
This is called “huffing,” which is a controlled coughing technique that has proved to help mobilize secretions.
Instruct the parents to performs chest physiotherapy (CPT) either 1 hour before meals or 2 hours after if possible.
This is done to reduce GI upset and the possibility of vomiting.
Explain the importance of increasing fluid intake during acute exacerbations.
This helps to thin the secretions.
15. Open the capsules and mix the beads in a food like applesauce.
Debbie is 3 years old and probably unable to swallow a whole capsule.
16. Increase the pancreatic enzymes with each meal and snack.
The dosage must be increased (if patient has loose, fatty stools) to obtain a well-formed, normal stool.
17. Community resources often provide support groups for parents of children with CF.
Meeting other parents who have experienced similar situations can provide hope, help, encouragement, and support.
18. Document the observations, but take no action at this time.
(Debbie builds a tower of 8 blocks, is able to say her first and last name, and state her age while holding up 3 fingers.) These behaviors indicate that Debbie is developmentally on-target.
19. “Play therapy uses toys and games to meet Debbie’s physical and emotional needs.”
Play therapy uses therapeutic play to address growth and developmental needs, to practice social skills, and to express feelings using toys instead of words. Play therapy takes place in a specially designed and decorated room that is furnished with carefully selected toys and special equipment.
20. Explain that Debbie should receive all routine childhood immunizations.
21. “The second MMR is given between 4 and 6 years of age.”
22. WBC count is 20,000.
This is elevated, indicating that Debbie has an infection.
23. Administer oxygen 6 L/min via simple face mask.
According to Maslow’s Hierarchy of Needs, oxygenation is the priority. (Debbie’s father reports that she started coughing, choking, unable to catch her breath, and then passed out. Debbie is admitted to ER.) The nurse should first apply oxygen, assess oxygen saturation, then start the IV line and administer an antibiotic, then contact the CF nurse educator.
24. The HCP prescribes Rocephin 12.5mg IVPB for tx of pneumonia. The medication comes in 12.5mg/25mL and is to be infused over 1 hour. At what rate should the nurse set the infusion pump?
A: 25
25. Assess Debbie and the child that received the wrong medication.
Assessment must be completed first to ensure that both children suffered no adverse effects from being given the wrong medication. Then the HCP should be notified to receive follow-up instructions, and then if appropriate the correct medications can be administered to both clients. A final step is the completion of an incident report.
26. Empty the Foley bag.
This task can be safely delegated to the UAP.
27. Explain that play therapy is an intervention and should not be interrupted for vital signs.
Play therapy is an important part of hospitalization and should only be interrupted for emergency situations.
28. Suggest that they add extra salt to Debbie’s diet and watch her for dehydration.
There is an increased risk for electrolyte imbalance secondary to dehydration during hot weather.
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