Cystic Fibrosis Flashcards
What organs do the xteristic thick, viscous secretion affect? Effect?
The thick mucus mostly affects the:
Lungs, Pancrese, Liver and Intestine
Primarily causes:
Difficulty breathing and lung infections as well as digestive complications
List the classic sx of cystic fibrosis (CF)?
Salty tasting skin
Poor growth and poor weight gain despite adequate food intake
Thick and sticky mucus production
Freq chest infection
Coughing
Shortness of breathe
List digestive sx associated with CF
Steatorrhea
Malnutrition due to poor absorption of nutrients, including fat-soluble vitamins, and a failure to thrive if not treated
List tx for lung problems ass with CF
Inhaled bronchodilators
Hypertonic saline (HyperSal)
DNAse enzyme therapy
Inhaled antibiotics
Oral Azithromycin
Transplantation
What’s the use of inhaled bronchodilators in CF?
Open the airways
What’s the use of Hypertonic saline (HyperSal) in CF?
To hydrate the airway mucus secretion
And
Facilitate mucociliary clearance
What’s the use of DNAse enzyme therapy in CF?
Used to breakdown extracellular DNA from accumulated neutrophils.
DNAse therapy is designed to THIN MUCUS and facilitate mucociliary clearance
What’s the use of inhaled antibiotics in CF?
To prevent and treat lung and sinus infections
What’s the use of oral Azithromycin in CF?
To reduce airway inflammation and disrupt P. aeruginosa biofilm formation
What’s the use of transplantation in CF?
In pts with end-stage dx
List tx for intestinal and nutritional problems
High fat and calorically-dense diet to help with nutrition
Pancreatic enzyme replacement to optimize growth and nutritional status and promote healthy bowels
Proton pump inhibitors to prevent degradation of pancreatic enzymes in stomach and for tx of GERD
Vitamin supplements, especially the fat-soluble vits A, D, E, K
Insulin for tx of CF-related diabetes mellitus
What’s used to help with nutrition in CF (tx for intestinal and nutritional problems)?
High-fat and calorically-dense diet
What’s used to optimize growth and nutritional status and promote healthy bowels in CF (tx for intestinal and nutritional problems)?
Pancreatic enzyme replacement
What’s used to prevent degradation of pancreatic enzymes in the stomach and for tx of GERD in CF (tx for intestinal and nutritional problems)?
Proton pump inhibitors
What vitamin supplements are essential in CF (tx for intestinal and nutritional problems)?
Fat-soluble vitamins A, D, E and K
What’s used for tx of CF-related diabetes mellitus (tx for intestinal and nutritional problems)?
Insulin
Types of CF infections?
Intermittent infection
Chronic infection
In intermittent lung infection due to CF, which organisms are most the common culprits?
Staphylococcus aureus and Haemophilus influenza
Followed by
P. aeruginosa
What xterizes acute pulmonary Exacerbations in CF?
Increase in cough
Sputum pdt with change in sputum color (greenish)
Shortness of breathe
Rapid decline in FEV1
Loss of appetite and weight
How do u treat a pt with P. aeruginosa infection in CF?
2 drugs given IV are recommended to provide potential synergy and prevent resistance:
Aminoglycosides
Beta lactams
Quinolones
And others that cover p. aeruginosa
What organism is associated with chronic lung infections in CF?
P. aeruginosa
What may be used to eradicate P. aeruginosa from the lungs?
Inhaled antibiotics
What must be given prior to using inhaled antibiotics in CF?
If pt is on bronchodilator and/or mucolytic, make sure to give these before using inhaled Antiobiotic
List inhaled antibiotics used in CF
Tobramycin inhaled solution (TOBI)
Tobramycin inhalation powder (TOBI Podhaler)
Tobramycin inhalation solution (Bethkis)
Aztreonam Lysine Inhalation Solution (Cayston)
What are the main active ingredients in inhaled antibiotics?
Tobramycin (in many forms e.g. Solution, powder)
Aztreonam
What’s the brand name of Tobramycin Inhaled Solution (used in chronic lung inf in CF)?
TOBI
What’s the brand name of Tobramycin Inhalation Powder (used in chronic lung inf in CF)?
TOBI Podhaler
What’s the brand name of Aztreonam Inhalation Solution (used in chronic lung inf in CF)?
Cayston
SE of Tobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?
Ototoxicity
Tinnitus
Voice alteration
Dizziness
Bronchospasm
How should u use the doses of Tobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?
Doses should be taken at least 6 hrs apart