Assessment Findings
What do both parents need to have in order for a child to present with cystic fibrosis? (think about their genes)
"Both biological parents have to carry the recessive trait for cystic fibrosis." (ATI, Nursing Care of Children, Chapter 19).
What is our priority in caring for patients with Cystic Fibrosis?
Airway= lung sounds, respiratory status, O2 sat
(ATI, Nursing Care of Children, Chapter 19)
Which vitamins should a patient with Cystic Fibrosis be taking daily?
Fat soluble vitamins (ADEK)
(ATI, Nursing Care of Children, Chapter 19)
What kind of genes do patients have to inherit in order to have cystic fibrosis?
recessive genes from both parents
(ATI, Nursing Care of Children, Chapter 19)
True or False:
It is important for the patient to stay up to date with vaccinations.
TRUE
(ATI, Nursing Care of Children, Chapter 19)
What are some respiratory findings of Cystic Fibrosis?
-multiple respiratory infections due to stasis of mucous
-wheezing, rhonchi, dry and nonproductive cough
- dyspnea, paroxysmal cough, obstructive emphysema
- cyanosis, barrel-shaped chest, clubbed fingers or toes
(ATI, Nursing Care of Children, Chapter 19)
What is a non-pharmacological way we can help a patient with Cystic Fibrosis clear their airway secretions?
Chest PT
(ATI, Nursing Care of Children, Chapter 19)
What are considerations when administering pancreatic enzymes to a client with cystic fibrosis?
Administer within 30 minutes of eating a meal or snack, monitor weight, monitor for adequate amount of stools, increase enzymes with high fat meals.
(ATI, Nursing Care of Children, Chapter 19)
What is the problem in cystic fibrosis?
mucous glands that secrete an increase of thick, tenacious mucous that leads to obstruction of organs.
(ATI, Nursing Care of Children, Chapter 19)
True of False:
We should educate parents and patients that exercise could be harmful to the patients.
FALSE:
Patients should be performing physical exercise regularly. We should be encouraging this.
(ATI, Nursing Care of Children, Chapter 19)
What are some GI manifestations related to Cystic Fibrosis?
foul-smelling stools (steatorrhea), failure to gain weight of weight loss, deficiency of fat-soluble vitamins, reflux
(ATI, Nursing Care of Children, Chapter 19)
What kind of food should we be encouraging a patient with Cystic Fibrosis to eat?
High protein and High calorie diet
(ATI, Nursing Care of Children, Chapter 19)
Albuterol
(ATI, Nursing Care of Children, Chapter 19)
What organs are affected from cystic fibrosis?
- pancreas
- lungs
-liver
- small intestine
-reproductive system
(ATI, Nursing Care of Children, Chapter 19)
True or False:
A support group would be helpful for patients with cystic fibrosis.
TRUE
(ATI, Nursing Care for Children, Chapter 19)
What is the diagnostic test done for cystic fibrosis?
A Sweat Chloride Test
(ATI, Nursing Care of Children, Chapter 19)
What other healthcare team members should be involved in the care of a patient with Cystic Fibrosis?
Respiratory therapy, physical therapy, social services, pulmonology, dietician
(ATI, Nursing Care of Children, Chapter 19)
What are some things to monitor in patients taking albuterol?
tremors, tachycardia
(ATI, Nursing Care of Children, Chapter 19)
Which two electrolytes are increased in Cystic Fibrosis?
sodium and chloride
(ATI, Nursing Care of Children, Chapter 19)
If a client is taking fluticasone propionate/ salmeterol what should we educate them to do after taking their medication?
Rinse out mouth after taking this medication.
(ATI, Nursing Care for Children, Chapter 19)
What other lab test can be done when trying to diagnose cystic fibrosis?
- blood specimen to see deficiency in fat soluble vitamins (ADEK)
- sputum culture to detect any respiratory infections
(ATI, Nursing Care of Children, Chapter 19)
What is important to provide to a family member or patient newly diagnosed with Cystic Fibrosis?
Emotional support
(ATI, Nursing Care of Children, chapter 19)
What does Dornase Alfa (aerosol) do for a patient with Cystic Fibrosis?
decreases vicosity of mucous and produces lung function.
(ATI, Nursing Care of Children, Chapter 19)
the first few years of their lives.
What are respiratory complications we should educate patients with cystic fibrosis about?
respiratory infections, bronchial cysts, emphysema, pneumothorax, and nasal polyps
(ATI, Nursing Care of Children, Chapter 19)