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
Storing ofTobramycin Inhaled Solution (TOBI) used in chronic lung inf in CF?
Refrigerator
Can be kept at room up to 28 days
Store in FOIL, to protect from light
SE of TOBI Podhaler (used in chronic lung inf in CF)?
Same as TOBI
Ototoxicity, tinnitus, voice alteration, dizziness, bronchospasm
How should TOBI Podhaler be used? (CF)
Use with Podhaler
How should the doses of TOBI Podhaler be spaced?
Doses should be taken no less than 6 hrs apart, should be taken as close to 12 hrs (same as TOBI)
Storage of TOBI Podhaler?
Store capsules at room temp in DRY place
Different from TOBI, refrigerator recommended as storage
SE of Aztreonam Lysine Inhalation Solution (Cayston) used in CF?
Allergic rxns (may be severe)
Bronchospasm
Fever
Wheezing
Cough
Chest discomfort
How should the doses of Aztreonam (Cayston) be spaced (in CF)?
At least every 4 hours apart
Different from TOBI and TOBI Podhaler… Q 6 hrs
How should Aztreonam (Cayston) be used in CF?
Reconstitute with 1ml of sterile diluent. Give immediately
How should Aztreonam (Cayston) be stored in CF?
Recommend to refrigerate, can be kept at room temp up to 28 days
Protect from light
(Storage is same as TOBI)
List oral antibiotic used in CF
Azithromycin
Whats the recommended order of use, if pt is on multiple meds? (Hypertonic saline, Chest physiotherapy, Bronchodilator, inhaled antibiotics, Dornase alfa)
Bronchodilator -> Hypertonic saline -> Dornase alfa -> Chest physiotherapy -> Inhaled antiobiotics (use last)
List agents that promote mucus clearance in CF
Albuterol
Hypertonic saline (HyperSal)
Dornase alfa (Pulmozyme)
What’s the brand name of Hypertonic Saline used to promote mucus clearance in CF?
HyperSal
What’s the brand name of Dornase alfa used to promote mucus clearance in CF?
Pulmozyme
Howz Hypertonic saline (HyperSal) used?
In small ampules delivered via a nebulizer
How should Dornase alfa (Pulmozyme) used in CF be stored?
Store ampules in refrigerator. (Don’t expose to room temp >= 24 hrs)
Protect from light
Can Dornase alfa (Pulmozyme) be mixed with another drug in the nebulizer?
No!
Don’t mix with any other drug in nebulizer
How many tabs of multivitamin do pts with CF use daily?
At least 1-2 tabs of multivitamins with A, D, E, K
What’s Pancrelipase? Used as a Pancreatic Enzyme Pdt (PEPs) in CF
Pancrelipase is a natural pdt harvested from porcine pancreatic glands which contains a combination of LIPASE, AMYLASE, and PROTEASE.
Howz Pancrelipase dosed?
Dose is individualized for each pt and is based on the LIPASE component
How’s the dose of Pancrelipase adjusted to a pt with CF?
Once enzyme therapy is started, dose is adjusted Q 3-4 days UNTIL STOOLS ARE NORMALIZED
What’s the max dose of Pancrelipase? Why?
> 6,000 units/kg/meal of lipase
Due to colonic structure risk
When is Pancrelipase used?
Enzymes are given PRIOR to meals and snacks
Full doses before meals
50% of mealtime dose before snacks
What causes a bump in Pancrelipase dose used?
Meals with high fat content req higher doses
Can the capsules of Pancrelipase be chewed or crush in CF?
Don’t chew or crush the capsules
What can be done if a pt can’t swallow Pancrelipase?
It can be sprinkled on soft food with low PH that doesn’t req chewing
Applesauce, gelatin, baby food etc
Don’t use with yogurt, pudding etc as they have a higher PH
How should Pancrelipase be used wrt entire dose and liquid?
Take entire dose at the beginning of each meal or snack
Take with a generous amt of liquid
Should u retain Pancrelipase in your mouth? Why or why not?
Don’t retain in mouth before swallowing; swallow immediately
Retention may cause mucosal irritation and stomatitis
Cam pancreatic enzyme pdts be substituted?
No!
Don’t substitute pancreatic enzyme pdts. This is an FDA recommendation
How do u store Pancrelipase pdts?
They don’t require refrigeration
List brand names of Pancrelipase
Creon
Lip-Prot-Amyl
Pancreaze
Pertzye
Ultresa
Viokace
Zenpep
SE of Pancrelipase use?
Mucosal irritation
Abdominal pain (stomach pain)
Nausea
Headache
Neck pain
Are all formulations of Pancrelipase interchangeable?
No!
Should u mix Pancrelipase with dairy pdts such as milk or yogurt?
No! Don’t mix
Hydration and Pancrelipase use?
Drink lots of non-caffeinated liquids every day
T/F? Is it important to follow the diet plan to get adequate nutrition and to keep as healthy as possible?
True
How u deal with missed Pancrelipase?
Give as soon as u remember
But, if it’s close to time for the next dose, skip it and go back to usual schedule
What’s used to target the G1551D-CFTR Protein Defect in CF?
Ivacaftor
What’s Ivacaftor?
Agent used to target the G1551D-CFTR Protein Defect in CF
How should Ivacaftor be used wrt food?
Take with high-fat containing food
List agents used in CF that DO require refrigeration
TOBI (can be kept at room temp for 28 days only)
Aztreonam (Cayston) (can be kept at room temp for 28 days only)
Dornase alfa (Pulmozyme) (can be kept at room temp for 24 hrs only)
What’s the usual dose of Ivacaftor (Kalydeco)?
150mg PO Q12H
How does CYP450 3A4 mod inhibitor or moderate-severe hepatic impairment affect Ivacaftor (Kalydeco) dosing?
Reduce daily dose - 150mg PO Daily (usually 150mg PO Q12H)
How does CYP34A strong inhibitors affect dosing of Ivacaftor (Kalydeco)?
150mg twice weekly (usual dose is 150mg Q12h)
What xteristic of cystic fibrosis?
Thick, viscous secretions
