NUR 201 Test 6 - Answer hereditary disorder of the exocrine glan...

NUR 201 Test 6

• cystic fibrosis (CF)

Answer hereditary disorder of the exocrine glands characterized by excess mucus production in the respiratory tract, pancreatic deficiency, and other symptoms

• Pseudomonas

Answer Common causes of pneumonia in CF

• CF most at risk ethnicity is

Answer caucasians

• CFTR gene

Answer a gene that codes for a protein involved in chloride and water transport across membranes. In patients with cystic fibrosis, a mutation in this gene disrupts chloride and water transport across membranes.

• CFTR is an disorder

Answer autosomal recessive

• CF pathophysiology

Answer The CFTR protein promotes chloride transport at the cellular level. Mutations disrupt these functions to varying degrees.

Major result is the formation of thick sticky mucus in the epithelial cells of the lungs, pancreas, liver, salivary glands, and testes, causing reduced organ function over time. 1 / 4

• Chloride (electrolyte)

Answer Aids in maintaining a normal balance of bodily fluids

• CF mucus secretions have too much and too little

Answer salt, water

• Pancrelipase (Creon)

Answer *class*

pancreatic enzyme

*Indication*

pancreatic insufficiency, ductal obstruction

*Action*

replacement of pancreatic enzymes

lipase, amylase, protease

*Nursing Considerations*

• contraindicated with pig products allergy
• can cause shortness of breath, nausea, diarrhea, rash
• assess nutritional status
• monitor for steatorrhea 2 / 4

• may increase uric acid levels
• instruct patient to follow diet
• take with meals and snacks

• CF clinical manifestations

Answer -chronic cough

-sinusitis (nasal polyps) -meconium ileus (7-10% of newborns) -pancreatic insufficiency -rectal prolapse -frequent respiratory infections -persistent coughing -audible wheezing -dyspnea -thick sputum -very foul fatty stools

• CF testing

Answer -confirmatory test = sweat chloride test (>60mEq/L is positive for CF)

(gold standard)

• CF Digestive system

Answer mucus can block tubes or ducts in pancreas and prevent enzymes from reaching intestines.infants do not pass meconium. gas, severe constipation, poor weight gain/growth, diarrhea

• CF reproductive system

Answer

• delayed puberty in females

• fertility can be inhibited by highly viscous cervical secretions, which act as a plug and block 3 / 4

sperm entry

• males are usually sterile as a result of the blockage of the vas deferens by abnormal

secretions or the failure of the normal development of duct structures

• CF Nutrition

Answer -Use age appropriate BMI to assess ht + wt

-pancreatic enzymes w/ meals + snacks -High pro, kcal, fat, salt -15-20% pro

-CHO 45-55%

-35-40% fat

• Additional appropriate doses water sol vit/min
• Supp water sol forms fat sol (A,D, E, K)

• CF medications

Answer

• Bronchodilators

• Antibiotics
• O2 only during acute episodes, maybe at night - because they live with a chronical- ly high CO2
• if they are over oxygenated will give them problems because repressing breathing

• Kalydeco (ivacaftor)

Answer first-in-class oral cystic fibrosis medication that treats the G551D-CFTR protein defect

• Dornase alfa (Pulmozyme)

Answer decreases viscosity of mucus and improves lung function.

• chest percussion vest

Answer rhythmic percussion of a patient's chest to loosen retained respiratory secretions.

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