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PHA 550 > Cystic Fybrosis > Flashcards

Cystic Fybrosis Flashcards

1
Q

Cystic Fibrosis

A

autosomal recessive

MC life limiting genetic disorder in caucasian population

life expectancy: 40 yrs

salty

later onset chronic bacterial infection of airways

adequate pancreatic exocrine function or pancreatitis

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2
Q

CFTR membrane protein

A

Normal:
transports Cl- ions outside cell –> neg charge outside –> Na+ ions follow to outside–> water leaves cell –> thin mucus

Mutation:

  • no transportation
  • high electrolyte concentration in cell
  • water stays in cell
  • thick mucus –> blocked lung channels –> obstruction, infection, inflammation
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3
Q

Long arm chromosome 7 and CFTR gene

A

88% delta F508

also G551D

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4
Q

CFTR Mutations: Class I

A

defective protein production

worst to have

no synthesis

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5
Q

CFTR Mutations: Class II

A

MC

defective protein processing

includes delta F508

second most detrimental to have

reduced trafficking

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6
Q

CFTR Mutations: Class III

A

defective regulation

includes G551D

gating mutation (impairs opening of the ion channel)

reduced gating

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7
Q

CFTR Mutations: Class IV

A

defective conduction

decreased conductance

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8
Q

CFTR Mutations: Class V

A

reduced number of active CFTR

reduced synthesis

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9
Q

Cystic Fibrosis: treatment goals

A

slow/stop progression of disease

allow for normal growth/development

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10
Q

Cystic Fibrosis: approaches to care

A

good nutrition

pancreatic enzymes and vitamin supplementation

airway clearance and anti-inflammatory therapies

antipseudomonal agents

recognize altered pharmokinetics

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11
Q

Cystic Fibrosis: treatments

A

chest physiotherapy

antibiotics (tobramycin, aztreonam)

pancreatic enzyme supplements

multivitamins (including fat soluble)

anti-obstructives (dornase alpha, hypertonic saline, N-acetylcysteine, bronchodilators)

anti-inflammatory (macrolides-azithromycin, ibuprofen)

CFTR modulators (ivacaftor, lumacaftor, tezacaftor)

vaccinations (influenza, pneumonia) and palivizumab

supplemental oxygen

BiPAP

lung transplant

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12
Q

Chest Physiotherapy

A

postural drainage

chest percussion (cupped hand)

vibration technique device

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13
Q

“Pulmonary Toilet” Regimen

A

6+ yo and concurrent with percussion therapy

  • bronchodilator (opens airways, prevents bronchospasm)
  • hypertonic saline (hydration, facilitates mucociliary function)
  • dornase alpha (dec viscosity of mucus)
  • aerosolized abx
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14
Q

Anti-Obstructives: bronchodilator

A

beta 2 adrenergic agonists (albuterol)

theophylline

anticholinergic

  • ipratropium (short acting)
  • tiotropium (long acting, no significant improvement)
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15
Q

Anti-Obstructives: inhaled hypertonic saline

A

hydrates the mucus (draws water from airway to re-establish aqueous surface layer)

HyperSal

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16
Q

Anti-Obstructives: dornase alpha (rhDNase)

A

Pulmozyme

enzyme mucolytic agent

selectively cleaves DNA

  • reduces mucus viscosity
  • improved airflow
  • dec risk of bacterial infection
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17
Q

Cystic Fibrosis: hallmark

A

abundant, purulent airway secretions composed of highly polymerized DNA from degenerated neutrophils

produces a viscous mucus (dec mucociliary transport and persistent secretions)

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18
Q

Dornase Alpha: ADEs

A 
chest pain
fever
rash
pharyngitis, rhinitis, FVC dec, dyspnea
voice alteration
dyspepsia
conjunctivitis
laryngitis
**dornase alfa serum antibodies (decrease efficacy)**
headache
urticaria
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19
Q

Should we treat patients with CF with chronic oral antibiotics?

A

No

chronic treatment with ORAL antibiotics is not encouraged

Exceptions:
azithromycin (anti inflammatory)
nebulized tobramycin
nebulized aztreonam
inhaled colistin (MDR)
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20
Q

Cystic Fibrosis: pathogens

A

most prominent: S aureus

PSEUDOMONAS aeruginosa

and others but im too lazy to write them

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21
Q

Antibiotics: Macrolide (Azithromycin)

A

anti inflammatory + antibacterial

cannot kill pseudomonas - reduce ability to produce biofilms

suppress excessive inflammatory response

3x/week (not daily)

indications:

  • airway inflammation
  • chronic cough
  • reduction in FEV1
22
Q

Antibiotics: Aminoglycoside (Nebulized Tobramycin)

A

improves lung function

reduces acute pulmonary exacerbations

interferes with bacterial protein synthesis

slight resistance

alternate with 28 days on/off treatment

23
Q

Inhaled Tobramycin: ADEs

A

SPUTUM DISCOLORATION

FEV1 decreased, RALES, WHEEZING, VOICE ALTERATION, bronchitis, epistaxis, pharyngolaryngeal pain, pulmonary function decreased, rhinitis, tonsillitis

COUGH, dyspnea, oropharyngeal pain, throat irritation, bronchospasm, upper respiratory tract infection

chest discomfort

malaise, pyrexia

abnormal taste, xerostomia, diarrhea

inc RBC sed rate, EOSINOPHILIA

TINNITUS

24
Q

Antibiotics: Inhaled Aztreonam

A

monobactam (beta lactam)(cross allergenicity w/ other beta lactams unlikely)

antipseudomonal

inhibits bacterial ell wall synthesis

alternate with 28 days on/off treatment

DO NOT REPEAT FOR 28 DAYS AFTER COMPLETION

25
Q

Inhaled Aztreonam: ADEs

A

FEVER (MC in children)

COUGH, nasal congestion, pharyngeal pain, wheezing, bronchospasm

chest discomfort

RASH

abdominal pain, vomiting

26
Q

Ibuprofen

A

reversibly inhibits COX 1 and 2 enzymes

  • dec formation of prostaglandin precursors
  • antipyretic
  • analgesic
  • anti-inflammatory
  • inhibits chemotaxis
  • alters lymphocyte activity
  • inhibits neutrophil aggregation/activation
  • dec proinflammatory cytokine levels

must be at greater than 6 months of age

27
Q

High Dose Ibuprofen

A

indication: <18yo w/ FEV1 >60%

20-30mg/kg of body weight twice daily

less decline in pulmonary function

maintain weight

28
Q

Ibuprofen: ADEs

A

EDEMA (Na and H2O retention)

dizziness, HEADACHE, nervousness

rash, itching

FLUID RETENTION

EPIGASTRIC PAIN, heartburn, nausea, abdominal pain/cramps/distress, dec appetite, constipation, diarrhea, dyspepsia, flatulence, vomiting, GI BLEED

TINNITUS

29
Q

CFTR Modulator: Ivacaftor

A

Kalydeco

CFTR potentiator

potentiates/assists in Cl- transport through G551D CFTR protein (inc probability of channel opening)(improves gating abnormality)

improves regulation of salt and water absorption/secretion

interactions:

substrate: CYP3A4 (major)
inhibits: CYP2C8, CYP2C9, CYP3A4, P glycoprotein (weak)

30
Q

Ivacaftor (ADEs)

A

HEADACHE, dizziness

rash, acne

ABDOMINAL PAIN, diarrhea, nausea

oropharyngeal pain, URTI, nasal congestion, NASOPHARYNGITIS, pharyngeal erythema, pleuritic chest pain, rhinitis, sinus congestion, wheezing

HYPERGLYCEMIA

TRANSAMINASES INCREASE

ARTHRALGIA, musculoskeletal chest pain, myalgia

bacteria in sputum

31
Q

Orkambi

A

lumacaftor + ivacaftor combination

potentiates CFTR

Study results:

  • improved lung function
  • reduced pulmonary exacerbactions
  • improved BMI
32
Q

Lumacaftor: MOA

A

partially corrects CFTR misfolding by F508del mutation

33
Q

Orkambi: ADEs

A 
MC: 
nausea, diarrhea
NASOPHARYNGITIS
change in respiration
chest discomfort
dyspnea
less common:
FATIGUE
rash
MENSTRUAL DISEASE
flatulence
influenza
INC CREATINE PHOSPHOKINASE
URTI
RHINORRHEA
34
Q

Symdeko

A

tezacaftor + ivacaftor

ages 12+

take with fat containing food

reduce dose in pts with mod-sev hepatic impairment

reduce dose when co-administered with mod-strong CYP3A4 inhibitors

35
Q

Tezacaftor: MOA

A

moves the defective CFTR protein to the proper place in the airway cell surface

36
Q

Tezacaftor and CYP3A enzyme

A

strong inducer: decreases efficacy of tezacaftor
(co-administration not recommended)
eg: rifampin, st john’s wort

mod-strong inhibitor: inhibition of metabolism prolongs clearance and delays half life
eg: ketoconazole, fluconazole

avoid grapefruit, seville oranges

37
Q

Symdeko: ADEs

A

headache
nausea
sinus congestion
dizziness

38
Q

Symdeko: monitoring parameters

A

ALT and AST

cataracts (non-congenital lens opacities) in pediatrics

39
Q

Cystic Fibrosis: oxygen treatment

A

indicated in progressive CF with worsening hypoxemia

40
Q

Cystic Fibrosis: BiPAP treatment

A

indicated in advanced CF and hypercapnia

41
Q

Cystic Fibrosis: lung transplantation

A

FEV1<30% or rapid decline in FEV1 (esp young F patients)

inc frequency of exacerbations requiring abx

refractory/recurrent pneumothorax

recurrent hemoptysis not controlled by embolization

42
Q

Cystic Fibrosis: GI treatments

A

GERD

pancreatic enzyme supplementation

vitamin supplementation

prevention and treatment of cirrhosis

43
Q

GERD: proton pump inhibitors

A

irreversibly block the gastric proton pump (H+K+ATPase) of the parietal cells

omeprazole
lansoprazole
dexlansoprazole
esomeprazole
pantoprazole
rabeprazole

put pts at risk for pneumonia

44
Q

Omeprazole: ADEs

A

headache, dizziness

rash

abdominal pain, diarrhea, N/V, flatulence, ACID REGURGITATION, CONSTIPATION

BACK PAIN, WEAKNESS

COUGH, uri

45
Q

Pancreatic Enzyme Supplementation

A

backbone of GI therapy

lipase+amylase+protease

utilized any time they eat

dosing calculated using the lipase units

eg: pancreaze, creon, zenpep, ultresa, viokace, pertzye

46
Q

Pancreatic Enzyme Supplementation: ADEs

A

ulcers (w/ prolonged contact with oral mucosa)

  • administer w/ food
  • rinse mouth after

excessive dose:
-fibrosing colonopathy (inflammation, strictures)

47
Q

Vitamin Supplementation

A

fat malabsorption –> fat soluble vitamin deficiencies (A,D,E,K)

dec Ca absorption –> bone loss

48
Q

Vitamin supplementation is based on …

A

age

49
Q

Ursodeoxycholic Acid (UCDA)

A

gallstone dissolution agent

dec cholesterol content of bile and bile stones

reduces secretion of cholesterol from the liver and reabsorption of cholesterol in the intestines

50
Q

Cystic Fibrosis: Related Liver Disease: ADEs (i actually think this might be able UCDA but who knows)

A

headache, dizziness

diarrhea, constipation, dyspepsia, N/V, flatulence, peptic ulcer

back pain, arthritis, myalgia

urti, pharyngitis, bronchitis, cough

ALOPECIA, rash

hyperglycemia

uti

LEUKOPENIA, THROMBOCYTOPENIA

cholecystitis

INC SERUM CREATININE

PHA 550 (26 decks)

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