CysticFibrosis Flashcards
T/F: CF mainly involves the exocrine glands
TRUE
What is the typical presentation of CF?
Failure to thrive, recurrent pneumonia, meconium ileus, nasal polyps, uncontrolled asthma, chronic sinusitis, salty taste to the skin
How is CF diagnosed?
One or more characteristic signs/symptoms, hx of CF in a sibling, positive newborn screening test and evidence of CFTR abnormality (sweat test or a known mutation)
What does newborn screening measure?
Cationic trypsinogen
CF pts need to have quarterly visits. What should be done each visit?
Vitals, hx, PE, review of therapies, detailed dietary hx, spirometry, lab work
What are the GI manifestations of CF?
Deficient secretion of digestive enzymes, fat soluble vitamin deficiency, insulin deficiency, intestinal obstruction, reflux
What is the classic symptom of maldigestion of nutrients in CF?
Steatorrhea
What are symptoms of pancreatic insufficiency and how is this diagnosed?
Steatorrhea, frequent loose stools, flatulence, cramping, bloating, and voracious appetite. 72 hour fecal fat collection
What is the treatment for enzyme deficiency?
Pancreatic enzyme replacements that contain lipase, protease, and amylase
How often do pt with CF take enzyme replacements?
1000 lipase units /kg/meal. 500 lipase units/kg/snack
EVERYTIME THEY EAT
What are the clinical issues related to enzyme replacement?
Inadequate enteric coating and poor acid resistance that leads to denaturing enzymes. Poor outcomes when switching between brand and generic name
What are some long term GI- therapies
High calorie diet with added salt and fat soluble vitamin replacement
What is DIOS? What are the symptoms and treatment?
Distal intestinal obstruction syndrome. Vomit, abdominal distention, and pain
Electrolyte lavage solutions
GERD often accompanies CF. what are treatment options?
Antacids, H2 blockers, proton pump inhibitors
Can proton pump inhibitors be taken with other GERD treatments? Why or why not?
NO, need an acidic environment
What drug can decrease liver inflammation and bile duct proliferation?
Ursodeoxycholate
What is the goal for the pulmonary components of CF
Decrease the long term rate of lung function decline
What are treatments for the pulmonary involvement in CF?
Airway clearance techniques, anti-inflammatory agents, chronic antibiotics, mucolytics, and bronchodilators
What does ACT involve and how often should it be done?
Chest/back percussion w/ vigorous coughing, flutter valve, exercise, and mechanical vest. BID
What is a non-steroidal anti-inflammatory agent that decreases the rate of FEV1 decline?
Ibuprofen
What is an antimicrobial and an anti-inflammatory agent that can be used in CF for pulmonary involvement?
Azithromycin
How often is azithromycin given and what does is do?
3x weekly, slows decline in FEV1. in CF pt with pseudomonas colonization it can improve FEV1 by 6% and decrease pulmonary exacerbations, well tolerated drug
What are the mucolytics?
Pulmozyme and mucomyst
What does pulmozyme do and what are its ADR?
Decreases viscosity of sputum, modest, FEV1 improvement , ADR: hoarseness, voice alterations, and pharyngitis
What are some problems with mucomyst?
Not typically used chronically, Irritating and causes bronchoconstriction, Smells real ratchet
How does the nebulized hypertonic saline increase the ability to cough out mucus?
Draw H2O into the airways
What is the major pulmonary pathogen in CF pts in the first year of life?
Staph aureus
What is the major pulmonary pathogen in CF pts by age 3?
H. influenzae
What is the major pulmonary pathogen in CF pts by age 5?
Colonized pseudomonas aeruginosa
How are chronic antibiotics useful in CF?
They prolong the time between acute exacerbations
What are the chronic antibiotics used for colonized P. aeruginosa and how are they given?
Tobramycin: BID 28 days on, 28 days off, Cayston: TID 28 days on and 28 days off, Treated regardless of infection status
What are the signs and symptoms of acute pulmonary exacerbations in CF pts?
Increased: cough frequency and duration, chest congestion, and sputum production, Decreased: exercise tolerance, PFTS, weight, O2 sat, Use of accessory muscle use and +/- fever
What is the goal of therapy for acute pulmonary exacerbation?
Decrease pulmonary signs and symptoms, Eradicate P. aeuroginosa
When do you dose at the upper end of the range in CF pts?
When they have increased volume distribution and increased clearance of the drug
What is the treatment for acute pulmonary exacerbations?
Typically aminoglycosides (systemic) and antipseudomonal penicillin
