Cystic Fibrosis Flashcards
1
Q
CF
A
- Cystic fibrosis: incurable hereditary disease caused by mutation in CFTR
- Causes abnormal transport of sodium, chloride, and bicarbonate ions
- Leads to thick, viscous secretions that affect lungs, kidneys, liver, and intestines
- Therefore causes breathing problems, lung infections, and digestive issues
- Most patients diagnosed by 2 yo
2
Q
Diagnosis
A
- Newborn screening done to test for CF risk factors and other disease
- Sweat test confirms diagnosis if risk is identified (elevated chloride in sweat)
3
Q
CF Signs/Sxs
A
- Salty tasting skin
- Poor growth and weight gain
- Thick/sticky mucus production
- Frequent lung infections
- Coughing
- SOB
- Steatorrhea (fatty stools)
- Malnutrition if not properly treated
4
Q
Inhaled Medication Order
A
- Inhaled bronchodilator (albuterol) - opens airways
- Hypertonic Saline (HyperSal) - mobilizes mucus
- Dornase alfa (Pulmozyme) - decreases mucus viscosity
- Chest physiotherapy - mobilizes mucus
- Inhaled antibiotics - control infections
5
Q
Intermittent Infections
A
- Most common in early disease: S. aureus and H. influenzae
- P. aeruginosa is more common in adolescents and adults
- Acute exacerbation characterized by increase in sputum and rapid decline in FEV1
6
Q
P. Aeruginosa Tx Guidelines
A
- Use two IV drugs
- Promotes synergy and prevents resistance
- Includes aminoglycosides, b-lactams, quinolones, and others
- Doses tend to be larger than normal due to altered CF kinetics
7
Q
Chronic Lung Infections
A
- Inhaled antibiotics recommended for those with chronic P. aeruginosa infections
- Treatment is cycled: 28 days on/off
- Azithromycin doesn’t act against Pseudomonas directly but disrupts the biofilm (oral and reduces exacerbations), given when not responding
8
Q
HyperSal
A
- Hypertonic saline
- Delivered by nebulizer
- Used in CF to mobilize mucus in airway
9
Q
TOBI/TOBI Podhaler
A
- Tobramycin
- Inhaled antibiotics used to target P. aeruginosa
- Capsules are for inhalation in Podhaler specifically
- Vios compressor for nebulizer formulation
10
Q
Tobramycin Information
A
- SE: ototoxicity, tinnitus, voice alteration, mouth/throat pain
- Dosed Q12h, each dose MUST be at least 6 hours apart
- TOBI: refrigeration recommended, can be kept at room temp for 28 days (nebulizer)
- Don’t mix with any other meds in nebulizer
- TOBI Podhaler: can be kept at room temp
11
Q
Cayston/Azactam
A
- Aztreonam (inhaled/IV respectively)
- SE: allergic rxns (severe), bronchospasm, fever, wheezing, cough, chest discomfort (more inhalation rxn)
- Doses given Q8h, MUST be at least 4 hours apart (TID instead of BID with TOBI)
- Refrigeration recommended and don’t mix with other agents in nebulizer
12
Q
Pancreatic Enzymes in CF
A
- Most need to supplement with PEP to help breakdown fat, starches, and proteins (PERT)
- Pancrelipase - harvested from porcine pancreatic glands
- Contains lipase, amylase, and protease
- Formulated to dissolve at basic pH of duodenum
- Dose is individualized based on lipase
- Adjust dose Q3-4d until stool is normalized
- Formulations are NOT interchangeable (change based on lipase dose)
13
Q
Pancrelipase Information
A
- Brand Names: Creon, Zenpep, or Viokace
- Max lipase: =< 10,000 u/kg/d
- Warning: colonic strictures (high doses) and mucosal irritation
- SE: abdominal pain, flatulence, nausea
- Formulations are not interchangeable
- Can open capsules and sprinkle on acidic food
- Use half meal dose with snacks
14
Q
CFTR Modulators
A
- Either work by increasing the time the CFTR channel remains open increasing the amount of CFTR delivered to cell surface
- Genotype testing must be performed to determine which medication can be used
- Most common mutation is homozygous F508del mutation
- ALL increase LFTs and cause cataracts in kids
15
Q
Kalydeco
A
- Ivacaftor
- Works by increasing time CFTR channel is open
- Not approved for homozygous F508del
- Take with high-fat containing food
