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ID exam 4 > cystic fibrosis > Flashcards

cystic fibrosis Flashcards

1
Q

the basic problem

A

CF is caused by a mutation in a gene that encodes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein - bacteria, DNA and immune cells build up in lungs
The gene is located on chromosome 7
1800 + CFTR mutations have been identified
Most common mutation is ∆F508

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2
Q

newborn screen

A

Blood spot obtained from the infant - Used to test for a variety of diseases including CF
Immunoreactive trypsinogen (IRT)
Positive test not diagnostic - Further testing required for diagnosis
Earlier diagnosis and treatment of CF has increased patient weight and decreased hospitalizations

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3
Q

presenting s/sxs

A

Neonates: Meconium ileus, Prolonged obstructive jaundice
Infants and children: Cough, recurrent URI, wheezing; Failure to thrive (gain weight); Heat intolerance
Adolescents and adults: Delayed sexual maturation; Nasal polyposis

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4
Q

diagnosis of CF

A

One or more sign/symptom + evidence of CFTR dysfunction
Sweat chloride test - Pilocarpine iontophoresis; > 60 mEq/L
Genetic testing
Pancreatic function - Stool fat quantitation; Quantitation of trypsin activity

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5
Q

ivacaftor

A

CFTR potentiator, keeps the gate “open” longer - exact MOA unknown
Class: cystic fibrosis transmembrane conductance regulator (CFTR) potentiator**
Age > 2 years
Dose: 150 mg PO BID for > 6 years; For 2-5 years > 14 kg 75 mg po BID and under 14 kg 50 mg po BID
Take with fatty foods

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6
Q

ivacaftor/lumacaftor

A

Age ≥ 6 years
F508del homozygous
Age ≥ 12 years: Lumcaftor 200 mg and ivacaftor 125 mg = 1 tablet
Age 6-11years: Lumcaftor 100 mg and ivacaftor 125 mg = 1 tablet
Dose: 2 tablets po BID - Take with fatty foods
AST/ALT/Bil q3month for 1 year and then yearly
Birth control drug interaction!
Lumacaftor CYP3A strong inducer
Ivacaftor CYP3A substrate

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7
Q

nasal polyps

A

Occur in an estimated 7 – 56% of patients with CF
Treatment: Sinus surgery, Nasal steroids (Flonase, Nasonex, etc), Saline nasal rinsesl, Gentamicin nasal rinses
Recurrence is high after surgery

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8
Q

allergy sxs

A

Antihistamines - Try to limit

Nasal steroids

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9
Q

psychological

A

Depression
The Cystic Fibrosis Foundation reports occurrence of depression in all ages is increasing - SSRIs, Mirtazapine (Tetracyclic antidepressant, alpha-2 adrenergic; Side effect of increased appetite)
Yearly depression/anxiety screen now recommended
Psycho/social issues

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10
Q

lung disease

A

Cause of ~ 85% of CF deaths
Due to CFTR dysfunction CF patients have thickened mucus
This mucus is hard to clear and creates a good environment for bacteria to grow
Respiratory exacerbations are common
The thickened mucus sets up the lung for cycles of infection and inflammation
Infection and inflammation lead to lung tissue damage
This damage increases overtime and ultimately leads to decreased lung function

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11
Q

airway clearance

A

Recommended for all CF patients
No method proven better than another
Method based on patient: Manual airway clearance techniques (P&PD); Therapy Vest; Flutter, acapella; Huff coughing; Meta neb

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12
Q

dornase alfa

A

maintenance lung treatment
MOA: Cleaves the extracellular DNA from expended neutrophils and other inflammatory cells in the CF mucus, thus reducing viscosity and promoting clearance
Nebulized solution
Generally well tolerated but $$$
Recommended for daily use in CF patients ≥ 6 years old; Can use in select cases under 6 years old
Dose: 2.5 mg inhaled Daily
Sometimes increased to BID - but data does not support

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13
Q

hypertonic saline

A

maintenance lung treatment
MOA: Exact MOA unclear
Proposed MOA: NaCl in the airway creates an osmotic gradient, This osmotic gradient draws water into the airway, Increased water in the airway helps to reduce mucus thickness, Thinner mucus is easier for the patient to expectorate
Hypertonic saline 7% 4 mL nebulized BID
Can reduce the percent of saline if intolerance occurs (3% or 3.5%)
Recommended in all CF patients ≥ 6 years; Can also use in select cases in patients under 6 years old

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14
Q

azithromycin

A

anti-inflammatory
MOA: Immunomodulating effects
Dose: under 40 kg 250 mg MWF, >40 kg 500 mg MWF
May not be tolerated due to GI side effects – dose can be reduced
Recommended in patients with chronic pseudomonas
Consider is patients without chronic pseudomonas

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15
Q

ibuprofen

A

MOA: anti-inflammatory NSAID
Dose: 20-30 mg/kg (Max 1600 mg/dose) BID
Check levels: Goal peak > 50 mcg/mL and under 100 mcg/ mL
Not well tolerated due to GI side effects

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16
Q

reactive airway disease

A

Inhaled corticosteriods: Asthma symptoms; Not recommended by current guidelines; Flovent, Asthmanex, etc.
Leukotriene modifiers: Asthma symptoms; Not recommended by current guidelines; Montelukast
Oral corticosteriods: Not recommended by current guidelines

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17
Q

bronchodilators

A

Albuterol
-MOA: Short acting beta agonist
-Used to open up airways prior to airway clearance therapy and to decrease bronchoconstriction prior to inhaled hypertonic saline
-Theoretically increases cilliary beating
-Dosing: Inhaler or nebulized scheduled or prn
Ipratropium: Not recommended by current guidelines

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18
Q

CF exacerbation

A

Not well defined
Typical clinical features: Increased cough, Increased sputum production, Shortness of breath, Chest pain, Loss of appetite, Loss of weight, Decreased lung function

19
Q

pathogens

A 
S. aureus (MSSA, MRSA)
P. aeruginosa
H. influenza, K. pneumonia
E. Coli
Stenotrophomonas maltophilia
Burkholderia cepacia
Aspergillus fumigatus
Achromobacter
20
Q

empiric IV therapy - MRSA

A

bactrim, clinadmycin, vancomycin, tetracycline, linezolid

21
Q

empiric IV therapy - MSSA

A

cefazolin, unasyn, coverage by anti-pseudomonal beta lactam

22
Q

empiric IV therapy - psuedomonas

A

Double coverage
Two different MOA
Piperacillin-tazo, imipenem-cilast, ceftazidime, meropenem, cefepime with aminoglycoside (tobra, amikacin)
Gentamicin no longer recommended
If history of pseudomonas typically cover even if doesn’t grow in current culture

23
Q

zosyn dosing

A

Zosyn 150 mg/kg/dose (dosing based on zosyn) IV q8h infuse over 4 hours (Max dose 6.75 gm IV q8h unless has history of renal issues then 4.5 gm IV q8h)

24
Q

cefepime dosing

A

Cefepime 50 mg/kg/dose IV q8h infuse over 4 hours (Max 2000 mg IV q8h)

25
Q

ceftazidime dosing

A

Ceftazidime 50-100 mg/kg/dose IV q8h infuse over 4 hours (Max 12 grams per day)

26
Q

meropenem

A

Meropenem 40 mg/kg/dose IV q8h infuse over 3 hours (Max 2000 mg IV q8h)

27
Q

tobramycin and gentamicin

A

under 40 kg 12 mg/kg/dose IV q24h, >40 kg 500 mg IV q24h
Random aminoglycoside levels at 2 hours and 6 hours post dose after 1st or 2nd dose
Calculate a peak (at end of infusion time) and trough
Goal: Peak 20-40 mCg/mL (goal 20-30 mCg/mL unless high tobramycin MIC – the target 10 times the MIC), trough undetectable, at least a 6 hour drug free interval.
Follow-up level after PK within goal every 3-5 days check level 18 hours after the start of dose. Goal of undetectable.

28
Q

amikacin

A

30 mg/kg/dose IV q24h
Random aminoglycoside levels at 2 hours and 6 hours post dose after 1st or 2nd dose
Calculate a peak (at end of infusion time) and trough
Goal: Peak 80-120 mCg/mL, trough undetectable under 1 mCg/mL
Follow-up level after PK within goal every 3-5 days check level 18 hours after the start of dose. Goal of undetectable.

29
Q

antibiotics monitoring

A 
Pulmonary function tests
O2 saturation (pulseox)
Serum drug concentrations Q 3-5 days
Kinetic dosage adjustments of aminoglycosides based on peak/trough
Serum creatinine Q 3-5 days
30
Q

inhaled tobramycin

A

Tobramycin (TOBI®) (TOBI, TOBI generic 300 mg/5 mL, Bethkis® 300 mg/4 mL)
-Dose: 300 mg IH BID
-Recommended for initial pseudomonas eradication – One 28 day course
-Suppression therapy in 28 days cycle for patients with chronic pseudomonas**
Tobramycin (TOBI Podhaler) - Dose: 112 mg = 4 capsules IH BID
Administration time 15-20 minutes
300 mg BID Pari LC Plus®
Premade solution

31
Q

inhaled aztreonam

A 
Aztronam (Cayston®)
-Dose: 75 mg IH TID
-eflow® nebulizer
Used in patient with chronic pseudomonas
Patients that can’t tolerate tobramycin
Can be used in off months
Treatment time: 5 minutes per treatment X 3 = 15 minutes per day
Cleaning time: 15 minutes per day (depending on number of hand sets)
Administration time 2-3 minutes
Bronchodilator pretreatment
Extensive cleaning required
75 mg TID
32
Q

other inhaled ABs

A

Amikacin 500 mg inhaled BID

Colistin 75-150 mg IH BID

33
Q

why nebulize?

A

Delivery of the drug to the site of infection
Reduced systemic exposure
Decreased risk of systemic side effects
Route allows for chronic administration of antipseudomonal antibiotics without IV
Ability to get higher concentrations of the drug to the site

34
Q

ABPA

A

Aspergillus→ allergic bronchopulmonary aspergillosis (ABPA); specific IgE titers

  • Prednisone burst and then taper
  • Voriconazole, Itraconazole, Posaconazole
  • If using antifungals – check levels
35
Q

liver

A 
Focal biliary cirrhosis
-Biliary obstruction
-Progressive periportal fibrosis
Liver steatosis
Liver failure
Treatment options for biliary slugging - Ursodiol 20-30 mg/kg/day divided BID
36
Q

pancreas

A

Exocrine insufficiency 85%
-Mucus obstructs exocrine ducts
-Decreased enzymes (amylase, lipase, protease) and HCO3 output
Pancreatic enzymes
-500 – 2,500 units of lipase/kg per meal
-Typically start with 1,000 units of lipase/kg/meal
-Do not exceed 10,000 units of lipase/kg/day
Pancreatic enzymes are adjusted based on number of stools per day, fat content of stools, and growth/weight

37
Q

stomach

A

Stomach pain and indigestion
-PPIs: Omeprazole, Lansoprazole, Esomeprazole, Pantoprazole
H2RAs: Ranitidine, Famotidine
Acid suppression therapy helps with pancreatic enzyme activity but may also decrease calcium absorption

38
Q

supplements

A 
Vitamin A – 10,000 IU
Vitamin E – 200-400 IU
Vitamin D – 400-800 IU
Vitamin K – 0.3-0.5 mg
Calcium - 1000 mg po daily
Iron - Monitor Hgb/Hct
Zinc
Sodium - Loose sodium through sweat; Require diets high in sodium or supplemental sodium
39
Q

vitamin monitoring

A

Vitamin D: 25-OH – goal > 30; preferred supplement cholecalciferol (vitamin D3)
Vitamin A Level
Vitamin E Level
Vitamin K: PT/INR

40
Q

CF related diabetes

A

CFRD Incidence: 2% children, 19% adolescents, 40-50% adults
Diagnosis
-Fasting plasma glucose ≥ 126 mg/dL
-2 hour plasma glucose ≥ 200 mg/dL
Screening
-OGTT annually in CF patients > 10 years
-HgbA1c – not reliable for diagnosis
Ketoacidosis rare
Macrovascular effects rare
Meal coverage
-Rapid acting insulin 0.5 – 1 unit for every 15 grams of carbs
-Adjusted based on 2-hr post-prandial
Correction dose: Rapid acting insulin 1 unit for every 50 mg/dL above 150 mg/dL
Basal insulin: Long acting insulin 0.25 units/kg – starting dose
Coverage of overnight feeding

41
Q

small intestine

A

Small bowel obstruction - Intestinal obstruction in 10% of older children
Meconium ileus - 10-15% of newborns
Treatment: 5% N-acetylcystine; Gastrograffin

42
Q

large intestine

A

Steatorrhea - Adjust pancreatic enzymes
Rectal prolapse
Distal intestinal Obstruction (DIOS)

43
Q

reproductive system

A

95% of males are sterile because of absence of the vas deferens - Surgical repair sometimes successful
Women with adequate nutritional and pulmonary reserve can have successful pregnancies
-Pregnancy is difficult on lung function due to volume status and pressure on lungs
-Pregnancy can lead to a decrease in lung function
-Increased nutrition is necessary 120-150% of baseline energy requirements is recommended
Contraception use is encouraged

44
Q

bone

A 
Osteoporosis/Osteopenia
-Calcium
-Vitamin D
-Bisphosphonates
Regular monitoring of calcium and vitamin D status is recommended
-Osteoporosis screening is encouraged

ID exam 4 (9 decks)

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