Term
| Newborn screening: only identifies infants at risk for CF; high value necessitates genetic testing |
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Definition
| Immunoreactive Trypsinogen (IRT) |
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Term
| 80-90% of patients with this have CF |
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Definition
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|
Term
| ~20% of patients with this present with Meconium Ileus |
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Definition
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|
Term
| diagnostic gold standard; 2nd confirmatory test required |
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Definition
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Term
| Cystic Fibrosis: Genetics |
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Definition
| Autosomal recessive disease |
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Term
| most severe form of cystic fibrosis |
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Definition
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Term
|
Definition
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|
Term
| usually pancreatic sufficient |
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Definition
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Term
| most common genetic mutation in CF |
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Definition
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Term
| normally regulates sodium and chloride transport across the cell membrane |
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Definition
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Term
| impaired chloride transport --> dehydrated cell surface liquid --> mucus plugging --> organ dysfunction |
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Definition
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|
Term
| thickened mucus --> decreased mucus clearance --> bacterial overgrowth and colonization ---> plugging, increased cough, SOB --> decreased O2, salts, lung scarring--> eventual mortality |
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Definition
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|
Term
| Other lung complications in CF |
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Definition
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Term
| Thick secretions in bile ducts and pancreas |
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Definition
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Term
| In utero blockage of vas deferens... thickened cervical mucus or malnutrtion |
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Definition
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|
Term
| Thick secretions in bile ducts and pancreas |
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Definition
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|
Term
| Recommend genetic counseling |
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Definition
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|
Term
| preferred CF-specific vitamin |
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Definition
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|
Term
|
Definition
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|
Term
| Consider if T/Z score is equal to or less than -2.0 |
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Definition
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|
Term
| Zenpep, Creon, Pancreaze, ultresa, Viokase (DO NOT SUBSTITUTE) |
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Definition
| Pancreatic Enzyme Replacement Therapy (PERT) |
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Term
| Order of Respiratory Meds: #1 |
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Definition
|
|
Term
| Order of Respiratory Meds: #2 |
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Definition
| Hypertonic saline, then Pulmozyme (Vest simultaneously) |
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|
Term
| Order of Respiratory Meds: #3 |
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Definition
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|
Term
| Order of Respiratory Meds: #4 |
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Definition
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|
Term
|
Definition
| drugs: as recommended by your physician |
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Term
| sidestream nebulizer (use with 50 psi compressor, i.e., MobilAire) |
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Definition
| Drugs: Pulmozyme (dornase alpha); Albuterol (salbutamol) |
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Term
| Pari LC Plus (use with 25 psi compressor, i.e., PulmoAide) |
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Definition
| Drugs: Colmycin (steril colistimethate sodium, USP); Albuterol; Pulmozyme; Pulmicort (budesonide) |
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|
Term
| Pari LC Star (use with 25 psi compressor, i.e. PulmoAide) |
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Definition
| Drugs: Colymycin, Albuterol, Pulmozyme, Pulmicort |
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|
Term
| Altera with eFlow technology |
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Definition
|
|
Term
| a substitute for the Pari-LC Plus nebulizer that allows for rapid administration of meds |
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Definition
|
|
Term
| increases hydration of airway surface liquid; not FDA approved for CF |
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Definition
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|
Term
| Mucolytic; breaks up long DNA strands, decreases mucus viscosity |
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Definition
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|
Term
|
Definition
| may cause increased cough, wheeze, hoarseness, skin rash, fever, HA, malaise |
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|
Term
| CF Anti-inflammatory therapies |
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Definition
| Ibuprofen and Azithromycin |
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|
Term
| studies show benefit for nutritional status and fewer pulmonary infections; monitor for atypical mycobacterium |
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Definition
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|
Term
|
Definition
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|
Term
| oral antipseudomonal antibiotics |
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Definition
|
|
Term
| inhaled antipseudomonal antibiotics |
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Definition
| Tobi (tobramycin); Cayston (aztreonam) Colistimethate |
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|
Term
| may cause coughing, wheeze, sore throat, hoarseness; little-to-no systemic absorption |
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Definition
|
|
Term
| SEs: cough, bronchospasm, hoarseness; supplied by only a few specialty pharmacies in the US |
|
Definition
| Cayston (aztreonam lysine) |
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|
Term
| IV Antipseudomonal Antibiotics |
|
Definition
| penicillins, cephalosporins, carbapenems, tobramycin, ciprofloxacin, aztreonam |
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|
Term
| Piperacillin-Tazobactam (Zosyn) |
|
Definition
| Penicillin Antipseudomonal IV antibiotic |
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|
Term
|
Definition
| Cephalosporin Antipseudomonal IV antibiotics |
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|
Term
|
Definition
| Carbapenem Antipseudomonal IV antibiotics |
|
|
Term
| first choice among carbapenems for Antipseudomonal |
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Definition
|
|
Term
| 30 mg/kg/day IV required for CF children due to increased clearance |
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Definition
|
|
Term
| oral antibiotics for MRSA |
|
Definition
| high dose septra, clindamycin, minocycline, doxycycline, linezolid |
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|
Term
| Bactrim is the DOC; Cefepime or Timentin; Respiratory quinolones, minocycline are alternatives; resistant to aminoglycosides and carbapenems |
|
Definition
| Stenotrophomonas maltophilia |
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|
Term
| Bactrim is DOC; Respiratory quinolones, minocycline are alternatives; may be resistant to aminoglycosides, piperacillin, ceftazidime |
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Definition
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|
Term
| Treats and prevents CF liver disease |
|
Definition
|
|
Term
|
Definition
| constipation, N/V, diarrhea, backache, dizziness, rash ,cough, pharyngitis/bronchitis |
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|
Term
| MOA: decreased biliary cholesterol concentration --> breakdown of gallstones; increases bile flow, protects liver by decreasing toxicity of bile acids |
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Definition
|
|
Term
|
Definition
| LFTS; dose titration dependent upon LFTs |
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|
Term
|
Definition
| Periactin (cyproheptadine) |
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|
Term
|
Definition
| drowsiness, diarrhea, N/V |
|
|
Term
| MOA: histamine, serotonin, and acetylcholine antagonist; likely alters serotonin activity in the appetite center of the hypothalamus |
|
Definition
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|
Term
| Dual mechanism of action in CF; improved enzyme efficacy: decreased bicarb production by the pancreas inhibitors release of enzymes from microspheres |
|
Definition
|
|
Term
| Pancreatic duct blockage and scarring, loss of beta cells prevents insulin secretion; insulin resistance may develop over time |
|
Definition
| CF-Related Diabetes (CFRD) |
|
|
Term
|
Definition
| oral anti-diabetic agents |
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|
Term
|
Definition
| CI in hypoxia due to risk of lactic acidosis; GI side effects |
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|
Term
|
Definition
|
|
Term
|
Definition
| may bind and inhibit the CFTR |
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|
Term
| Primary treatment of CFRD |
|
Definition
| insulin therapy; results in increased BMI, improved FEV1, improved survival |
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|
Term
| Indicated for CF patients > 6 years old with G441D mutation |
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Definition
|
|
Term
| Kalydeco contraindications |
|
Definition
|
|
Term
|
Definition
| URI, stomach ache, HA, rash, diarrhea, dizziness |
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|
Term
|
Definition
| CFTR potentiator; facilitates opening of chloride channel |
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|
Term
| Cost $25,000/month; additional studies underway for other gating mutations and for use in combination with VX809 |
|
Definition
|
|
Term
| CFTR Corrector; facilitates movement of the CFTR protein to the cell surface |
|
Definition
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