Term
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Definition
most common lethal, genetically inherited dx in Caucasians;
AUTOSOMAL RECESSIVE mode of inheritance;
- mutation found on Chromosome 7;
Cl- is main/initial electrolyte involved |
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Term
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Definition
| w/ the mutation, Cl stays in cell & Na & H2O stay in the cell --> dehydration & thickening of mucosa on lumen border --> CONCENTRATES electrolytes remaining on lumen border; |
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Term
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Definition
gold standard for diagnosis of CF;
>= 60 mEq/L is DIAGNOSTIC;
- most can be diagnosed by 7 months old |
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Term
| Initial Clinical Presentation may lead to diagnosis |
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Definition
chronic, repeated respiratory conditions;
- exocrine pancreatic insufficiency --> very fatty, foul-smelling stools;
- ANY family hx;
- meconium ileus (1st poop causes blockage) |
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Term
| CF Effects on Reproductive System |
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Definition
late maturation in both sexes w/ delayed puberty;
90% of males: destruction of epididymis/vas deferens/seminal vesicles;
60% of females: unable to have children --> abnormally thickened cervical mucosa;
Pregnant CF + women: watch nutrition & pulmonary status CLOSELY!!! - both baby & mom at HIGH RISK; |
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Term
| CF Effects on Exocrine/Endocrine System |
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Definition
Initially presents w/o sign. alterations in insulin;
By 18 yrs old, pt appears insulin deficient --> CF Related DM (CFDM) - may present as Type I or Type II;
- may be asymptomatic, discoverd when other labs are performed;
- if symptomatic, presents like untreated Type 2 DM (polyuria, polydypsia, polyphagia); |
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Term
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Definition
***NEED to treat w/ Insulin therapy AUTOMATICALLY***
1) Humulin R or Humalog on sliding scale when in hospital;
2) Split-mixed (Outpatient Therapy):
- Humlin N & Humulin R (2 injections/day)
OR
- Lantus (1 dose) + premeal Humalog (3 injections)
Oral Agents:
- Sulfonylureas DO NOT WORK!!! |
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Term
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Definition
related to increased viscosity of mucus secretions & deficiency of pancreatic enzymes to aid in digestion;
- 85% of CF pts have SOME degree of involvement;
- initially related to increased viscosity, may present as meconium ileus or distal intestinal obstructive syndrome (DIOS) later in life;
Lifelong Complications:
- pancreatic deficiency, maldigestion, malnutrition;
- Sx: failure to thrive, steatorrhea;
- fat, protein, & CHO malabsorption;
- decreased absorption of fat-soluble vitamins (A,D,E,& K); |
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Term
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Definition
1) Nutrition
2) Vitamin Replacement
3) Pancreatic Enzyme Replacement |
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Term
| Tx of GIT problems in CF - Nutrition |
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Definition
1) Encourage foods/energy sources high in calories & easily absorbed;
- Supplementation: Ensure PO throughout day or vid NG tube drip HS;
- if significant problems: cyclical TPN (AT NIGHT!!!) |
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Term
| Tx of CF GIT Problems - Vitamin Replacement |
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Definition
1) Take standard Multivitamin BID
OR
2) Take ADEK qdaily;
Alert pt & family to Sx of fat-soluble vitamin deficiency & ask about Sx at every visit;
K --> brusing, bleeding;
D --> bone breaks easily;
A --> vision problems, rough/dry skin;
E --> impaired balance, coordination, muscle weakness; |
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Term
| Treatment of GIT in CF pts - Pancreatic Enzyme Replacement |
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Definition
Initial dose, then titrate to pt response: decrease in # of stools/day, less steatorrhea/bloating, weight gain;
Dose based # of Lipase units w/ corresponding ratio of Protease & Amylase units;
Microencapsulated --> protect from stomach acid degradation;
INFANT Dosing:
- 2,000-4,000 units Lipase per 120 mL bottle;
Preferred Dosing Method (by weight):
- 1,000 units Lipase/kg prior to each meal
- 500 units Lipase/kg prior to each snack
***Give LOWEST # of caps while still meeting Lipase needs*** |
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Term
| ACute & Chronic Pulmonary Problems in CF pts |
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Definition
thickened mucus in lungs:
1) chronic: gas exchange is difficult, makes pt hypoxic --> COPD-like syndrome --> cor pulmonale found in many pts;
2) Acute:
- mucus is perfect growth medium for bacteria --> pulmonary infections (acute) & colonization (chronic) |
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Term
| Acute Exacerbation of Pulmonary Symptoms in CF pts |
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Definition
Common Pathogens:
- PSEUDOMONAS, Staph aureus, Burkholderia, H. influenzae;
Tx (ALWAYS use COMBO therapy):
- aminoglycoside (tobramycin) PLUS 3rd/4th gen. cephalosporin (ceftazidime, cefepime);
Continue therapy for 14-21 days;
- burst doses of steroids may be beneficial;
Target: return to pre-exacerbation condition, NOT elimination of pathogen; |
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Term
| Tobramycin Dosing for CF pts |
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Definition
- increased ability to clear AMGs;
Initial Dose:
- 7.5-9.0 mg/kg/day divided q8 or q12 hrs;
- target peaks: 10-14 mcg/mL;
Monitor closely: may need to change dosing interval to Q6 hrs |
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Term
| Goals of Chronic Pulmonary Care in CF pts |
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Definition
1) minimize obstruction/gas exchange symptomatology;
2) lengthen amount of time between acute exacerbations; |
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Term
| Percussion/Postural Drainage |
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Definition
cornerstone of pulmonary therapy in CF pts;
- providse less obstruction;
- decreases amount of "media" for bacterial growth;
MoA: dislodges mucus by manual means & allows it to be spit out, done 2-4 times/day (takes 30 min);
- may precede process with Sterile water or 0.9% NaCl nebulization, bronchodilators or mucolytics (N-actylcysteine), nebulized dose of Pulmozyme (very expensive, not routinely done) |
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Term
| Standard of Supportive Chronic Pulmonary Care in CF pts |
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Definition
1) Percussion/Postural drainage - CORNERSTONE;
Standard of Therapy (Precedes Percussion/postural Drainage):
1) Sterile water or 0.9% NaCl nebulizer;
2) Bronchodilators or mucolytics (N-acteylcysteine) may be added if benefit is seen;
3) Prior to ONE drainage session, pt should receive 1 nebulized dose of Pulmozyme:
- reduces viscosity of CF sputum by cleaving extracellular DNA & other proteins in mucus
- lengthen times between acute exacerbations & improves QOL
- dose: 2.5 mg daily or BID |
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Term
| Pulmozyme (DNase, dornase alpha) |
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Definition
Prior to at least ONE drainage session, pt should receive 1 nebulized dose:
- reduces viscosity of CF sputum by cleaving extracellular DNA & other proteins in mucus
- lengthen times between acute exacerbations & improves QOL
- dose: 2.5 mg daily or BID |
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Term
| Colonization in Chronic Pulmonary Symptoms of CF pts |
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Definition
Psuedomonas aeruginosa --> predominate organism, may become resistant to some drug therapies;
- acute exacerbations are commonly "overgrowths" of colonized bacteria;
- therapy attempts to limit amount of colonization & may increase time between exacerbations;
Chronic Abx therapy is NOT currently used in practice to control colonization --> resistant strains;
If pt is >= 6 yrs old & having high # of exacerbations (4 in 6 months, 6 in 1 yr): may consider chronic suppressive therapy:
- TOBI (tobramycin) nebulizations 300 mg BID x 28 days on then 28 days off, then repeat; |
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Term
| Oral Corticosteroid Therapy |
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Definition
controversial;
showed positive effects on pulmonary function;
Negative effects on growth & glucose |
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Term
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Definition
MoA: pulls extra water to mucus layer to thin secretions & make them easier to expectorate;
May trigger bronchospasm, administer bronchodilators prior to minimize risk; |
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Term
| Long-term ibuprofen therapy |
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Definition
shown to slow pulmonary deterioration due to its anti-inflammatory effects;
Target Serum Conc: 50-100 mcg/mL;
Dosing: 20-30 mg/kg/dose BID to TID
C/I in peptic ulcer dx |
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Term
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Definition
unofficial standard of care;
increases FEV1 & overall lung function;
does NOT decrease Pseudomonas colonization;
MoA: predominately ANTI-INFLAMMATORY
Dosing:
<40 kg - 250 mg daily on MWF
>=40 kg - 500 mg daily on MWF |
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