cystic fibrosis3
Terms
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- What is the gold standard diagnostic test for cystic fibrosis?
- The Sweat Test
- _______ mEq/L or higher is diagnostic
- ?
- Most CF patients can be diagnosied using the sweat test by _____ of age
- 7months
- What alterations in clinical status may indicate cystic fibrosis?
-
1)chronic or repeated respiratory conditions
2)exocrine pancreatic insufficiency
3)meconium ileus
4)family history - What are the 4 physiologic systems affected by cystic fibrosis?
-
1)reproductive
2)exocrine/endocrine
3)gastrointestinal
4)pulmonary - Reproductive system effects
-
1)late maturation/delayed puberty
2)90% of males sterile
3)60% of females are unable to have children - Exocrine/Endocrine
-
1)18 years of age they may clinically appear to be insulin deficient (CFDM)
2)presents as if a type2 diabetic - Treatment for CFDM
-
1)Humulin R or Humalog on SSI when in hospital
2)Split-mixed dosing w/ Humulin N and Humulin R or 1-dose lantus w/ premeal Humalog for outpatient management - GI system pathophysiology from cystic fibrosis
-
1)increased viscosity of mucus secretions
2)deficiency of pancreatic enzymes to aid in digestion - GI system effects
-
1)initially may present as meconium ileus or as distal intestinal obstructive syndrome (DIOS) if later in life
2)lifelong complications related to pancreatic deficiency presenting as maldigestion and malnutrition - Pancreatic enzyme deficiency leads to _____, protein, and carbohydrate malabsorption
- fat?
- Malabsorption also leads to decreased absorption of 4 fat soluble vitamins which are
- ADEK
- Treatment for GI effects
-
1)nutrition
2)vitamin replacement
3)pancreatic enzyme replacement - Specific nutrition treatment
-
1)foods/energy sources high n calories and easily absorbed
2)Ensure like products PO throughout day or via NG drip at night
3)cyclical TPN if problems persist -
Pancreatic replacement therapy
a)how should doses be titrated -
To patient response
1)decrease stools
2)less complains of steatorrhea and bloating
3)weight gain -
Pancreatic replacement therapy
b)how is dose based? - 1)number of lipase units with corresponding ratio of Protease and Amylase
-
Pancreatic replacement therapy
c)what products are preferred - 1)microencapsulated-protect compound from stomach acid degradation
- For INFANTS: Give _______ units lipase per ____mL bottle
-
1)2000-4000units
2)120mL -
For all others: preferred dosing is by weight-
1)Give ____ units lipase per Kg prior to each meal
2)Give ____ units lipase prior to each snacks -
1)?
2)? - Pulmonary system effects
-
1)thickened mucus in lungs
a)chronic issue: mucus makes gas exchange difficult resulting in hypoxia and COPD-like syndrome; Cor pulmonale (rt sided HF)
b)bacteria growth medium - Pulmonary system-Acute exacerbation common pathogens
-
1)PSEUDOMONAS AERUGINOSA
2)staph aureus
3)burkholderia capacia
4)haemophilus influenza - Acute exacerbation treatment
-
COMBO TREATMENT
1)Extended-spectrum penicillin (betalactamase inhibition) AND tobramycin
2)3rd or 4th gen cephalosporin (antipseudomonal) AND tobramycin - Aminoglycoside dosing in CF patients
- 1)INCREASED ability to clear aminoglycosides
- Tobramycin dosing
- 1)initial 7.5-9mg/kg/day divided q8h or q12h
- Target peaks for tobramycin dosing should be _____mcg/mL
- ?
- What should be monitored closely with tobramycin therapy?
- CPK-dosing interval may need to be as close as q6h
- Duration of antibiotic treatments
- 14-21days
- What are the goals of chronic care for the pulmonary system?
-
1)minimize obstruction/gas exchange symptomatology while lengthening time between acute exacerbations
2)leads to decreased systemic consequences and lower degrees of airway inflammation/destruction - What is the cornerstone of pulmonary therapy?
- percussion and postural drainage
- Prior to ONE OF THE CPT SESSIONS the patient should receive a nebulized dose of __________.
- Pulmozyme (DNase or Dornase Alpha)
- Pulmozyme effects
-
1)reduces visosity of CF sputum by breaking down DNA and other proteins in mucus
2)lengthens time between acute exacerbations and improves QOL - Pulmozyme dose
- 2.5mg QD
- What bacteria is the predominate organism for colonization in CF patients
- Pseudomonas aeruginosa
- When is chronic supressive therapy indicated in CF patients?
- 1)4 exacerbations in 6months or 6 in 1year
- What is used for chronic suppressive therapy?
- tobramycin nebulizations (TOBI)
- Dose of tobramycin for chornic suppressive therapy
- 300mg BID for 28 days on then 28 days off therapy, then repeat