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Therapeutics I > CF > Flashcards

CF Flashcards

1
Q

What is CF?

A

Progressive genetic mutation of the CFTR gene -> multiple organ failure and shortened lifespan

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2
Q

What is the primary cause of death in CF?

A

Lung infection

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3
Q

How is CF diagnosed?

A

Genetic testing and sweat test

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4
Q

What group of people are most susceptible to CF?

A

Caucasians

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5
Q

What is the most common mutation in CF?

A

∆F508

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6
Q

What does the CFTR gene mutation cause?

A
  1. Defective Cl- channel functioning
  2. Altered ion transport and water/salt homeostasis in sweat glands
  3. Increased salt content in sweat
  4. Thick and sticky mucus
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7
Q

What is autosomal recessive inheritance?

A

Both parents are carriers and offspring has a 25% chance of contracting disease

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8
Q

What are the organ systems that are affected by CF?

A
  1. Skin/sinuses
  2. Lungs
  3. Lungs
  4. Liver
  5. Pancreas
  6. GIT
  7. Repro
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9
Q

Skin CF symptoms?

A

Elevated levels of NACl lost in sweat -> electrolyte imbalance

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10
Q

Sinus CF symptoms?

A

Chronic rhosinitis and nasal polyposis

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11
Q

How are lungs affected by CF?

A

Increased mucus viscosity and decreased mucociliary action -> colonization -> chronic lung infection -> destruction

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12
Q

What is the goal of CF therapy?

A
  1. Prevent infection
  2. Maintain lung function
  3. Optimize nutritional status
  4. Achieve normal weight
  5. Promote appropriate growth
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13
Q

What are common bacteria in sputum of CF Patients?

A
  1. Staphylococcus aureus
  2. Haemophilis influenza
  3. Pseudomonas aeruginosa
  4. Burholderia cepacia
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14
Q

What is P. aeruginosa?

A
  1. G-
  2. highly drug resistant
  3. Biofilm protection bacteria from antibiotic penetration
  4. Most CF adults
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15
Q

Which bacteria strain are most prominent in CF children?

A

Staph and Haemo

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16
Q

How is the liver affected by CF?

A
  1. Accumulation of mucus in bile ducts
  2. Cirrhosis and portal HTN
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17
Q

How is unsodiol beneficial for CF related cirrhosis?

A

Increase bile flow and protect liver cells from cytotoxic salts and bile acids

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18
Q

How is the pancreas affected by CF?

A
  1. Accumulation of mucus in ducts -> loss of function and secretion of digestive enzymes
  2. Absence of CFTR limits function of Cl-Bicarb exchanger to secret Bicard -> retention of enzymes and destruction of pancreas
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19
Q

What is the treatment of CF diabetes?

A

Insulin

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20
Q

How do you screen for CF diabetes?

A

Destruction of islets of Lang -> decrease insulin production and secretion -> Increased blood glucose

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21
Q

How do you screen for CFRD?

A
  1. age 10 annually
  2. Pre conception planning
  3. OGTT
  4. A1c monitoring
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22
Q

How is the GI affected by CF?

A

Decrease water secretion -> thickened mucus and constipation

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23
Q

How can the destruction of the pancreas affect the GI?

A
  1. Malnutrition
  2. Decreased absorption of ADEK
  3. Poor growth and caloric loss
  4. Heartburn/GERD
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24
Q

How does CF affect the reproductive system?

A
  1. 97% infertile men
  2. 20% infertile women
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25
Q

What are important decision points to go over when a CF patient wants to conceive?

A
  1. Genetic testing of both patient and partner
  2. Family planning and contraception
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26
Q

What are the clinical presentation of CF?

A
  1. Salty skin
  2. Cough, SOB
  3. DIgital clubbing
  4. Failure to thrive
  5. Weight loss
  6. Malnutrition
  7. Greasy stool
  8. Eleveted glucose levels
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27
Q

What are steps of the multistep process of CF?

A
  1. Newborn screening
  2. Sweat test
  3. Genetic or carrier test
  4. Confirmation of CFTR dysfunction
  5. Clinical eval by specialists
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28
Q

What are the types of CF management techniques?

A
  1. ACT, infection prevention, anti-inflammatory therapies
  2. Nutrition support
  3. Disease modifying therapies: GFTR modulators
  4. Transplants
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29
Q

What are some growth and weight maintenance techniques?

A
  1. Pancreatic enzyme replacement
  2. Adequate caloric intake
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30
Q

What are some bone and health supplements?

A
  1. ADEK
  2. Calcium intake
  3. NaCL replacement
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31
Q

What are some pharmocotherapies used for CF?

A
  1. Bronchodilators
  2. Lung surface hydration
  3. Mucolytics
  4. ACT
  5. Antibiotics
  6. Anti-inflammatory (OCS, Azithromycin)
32
Q

How are OCSs used for CF?

A

Slows progression of lung disease in patients with CF (not good for chronic)

33
Q

How is azithromycin used for CF?

A

Anti-inflammatory properties reducing pulmonary exacerbations

34
Q

What is the sequence of CF meds for airway clearance regimen? How often do you do this?

A
  1. Bronchodilator (albuterol) - dilates lungs
  2. Hypertonic saline (hypersal) - hydrates lungs
  3. Dornase alfa (Pulmozyme) - thins mucus
  4. ACT or HFCWO
  5. ABX after ACT if needed

BID

35
Q

What is bronchodilator used for CF?

A

Albuterol - bronchodilator used to open airways prior to clearance

36
Q

How do you hydrate the lungs?

A

Hypertonic saline (Hyper sal) - increases hydration of surface fluid and improves clearance

2YO

37
Q

What do you use for individuals who can’t use Hyper sal? Qualifications? ADR?

A

Inhaled mannitol (Bronchitol) BID
Must pass BTT and premeditated with BD before use
Bronchospasm and hemoptysis

38
Q

What are the mucolytics for CF? Counseling?

A

Dornase (Pulmozyme) that thins out mucuc cleaving DNA in secretion
Less than 5 YO may have ADRs
Do not mix with other nebulizer meds

39
Q

What are the pulmonary effects of using mucolytics?

A
  1. Increased lung function
  2. Decreased sputum viscosity
  3. Decreased pulmonary exacerbations by 28%
40
Q

What are some airway clearance therapies?

A
  1. Percussion and postural drainage (P&PD)
  2. Positive expiratory pressure (PEP) devices
  3. Active cycle of breathing technique (ACBT)
  4. Autogenic drainage (AD)
  5. Oscillatory PEP devices (OPEP)
  6. High-frequency chest compression (HFCC) devices
  7. Exercise
41
Q

What are the different route of antibiotics for CF? What do they do?

A
  1. Inhaled: prevent growth of colonized bacteria -> improve lung function
  2. IV: severe exacerbations from bacteria
  3. Oral: prophylaxis or mild exacerbation and ongoing infection
42
Q

How is P. aureuginosa differ from other bacterial treatment?

A

2 drug combos due to MDR

43
Q

What are the advantage of inhaled ABX?

A
  1. Increased concentration of site of infection
  2. Decreased need for IV and toxicity
44
Q

What are the neb ABX? Brand? Indication?

A
  1. Tobramycin (TOBI) - P. aeruginosa
  2. Aztreonam (Cayston) - P. aeruginosa
  3. Colistin: P. aeruginosa
  4. Amikacin (Arikayce): M. avid complex
45
Q

What does the CF recommendations when handling P. aeruginosa?

A

Routine oropharyngeal cultures to determine if they’re infected opposed to bronchoscopy

Sputum culture is a good predictor of morbidity and mortality

46
Q

How do you dose TOBI? Who would you recommend it for?

A

300 mg BID, 28 days on, 28 days off
Can be alternated with Aztreonam (Cayston) for continuous coverage

<6YO with mild-moderate lung disease and persistent P. ae.
>6 months initial treatment

47
Q

When would you use IV ABX?

A

To treat severe exacerabtions
2 IV ABX to treat P. AER.

48
Q

What are the appropriate IV ABX combos to treat P. aeruginosa?

A
  1. B-lactam + aminoglycoside
  2. B-lactam + fluroquinolone
  3. B-lactam + colistin

Continue inhaled ABX

49
Q

Benefits of chronic azithromycin treatment? Warning?

A
  1. Decrease PA biofilm formation
  2. Anti inflammatory
  3. Improves FEV1

Does not treat P. aeruginosa infection

50
Q

Dosing and recommendations of Azithromycin?

A

Patient >6YO with chronic PA colonization

Patient <6 YO initial to delay PA colonization

Dose: ≥40 kg: 500 mg three times a week

51
Q

How do you monitor Azithromycin treatment?

A

Acid fast bacteria smear prior to starting then check smear and culture every 6-12 months

52
Q

When is OCS used for CF?

A

First line for allergy brochopulmonary aspergillosis (ABPA)

53
Q

When is ICS used for CF?

A

Unless they also have asthma

54
Q

What is the benefit of using ibuprofen for CF?

A
  1. Recommended in 6-17 YO with >60%
  2. Mild PK monitoring
  3. No evidence for improvement in adults
54
Q
A
55
Q

What kind of vaccines are recommended for CF patients? Kids? Adults?

A

Kids: Flu (not FluMist), Synagis, COVID
Adults: Boosters and seasonal vaccines

56
Q

Are CR patients excluded from vaccines?

A

No, they are not more susceptible to viral infections but outcomes are worse

57
Q

What kind of diet should CF patients have?

A

High caloric and fat
110-200% energy intake
Supplemental nutrition

58
Q

What is the weight goal for CF patients? 20+? 2-20?

A

Women BMI: >22
Men BMI: >23

Kids: maintain 50th percentile

59
Q

What are PERTs? Brands?

A

Pancreatic enzyme replacement therapy that may require H2 antagonist or PPI for alkaline environments

Creon, Prancreaze, Viokase

60
Q

What is the dosage form of PERTs? Administration/Counseling? ADRs?

A

Enteric coated
Take before meals, snacks, and ADEKs
Don’t crush (capsules may be sprinkled)

ADR: age <12 years, doses >6000 lipase units/kg/meal for more than 6 months, history of bowel obstruction or IBD

61
Q

How do you treat PERT OD?

A

Reduce dose, oral or enema laxatives, surgery

62
Q

What may occur after excessive dosing of PERTs?

A

Fibrosing colonopathy

63
Q

What may you need to supplement in CF patients? Fluids?

A

ADEK
Salt tablets and fluid containing salt and electrolytes

64
Q

What supplements improves bone health and osteoporosis prophylaxis?

A

D, K and calcium

65
Q

Why do you vitamin D3 or D2?

A

Better absorption

66
Q

What are the disease modifying therapies for CF?

A
  1. Ivacaftor (Kalydeco)
  2. Lumacaftor/Ivacaftor (Orkambi)
  3. Tezacaftor/Ivacaftor (Symdeko)
  4. Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)
67
Q

Ivacaftor

A

Kalydeco
Potentiator
1month
At least 1 mutation in CFTR gene responsive to therapy

68
Q

Ivacaftor+Luacafotr

A

Orkambi
Potentiator/Corrector
1 year
2 copies of F508del mutation

69
Q

Ivacaftor+Tezacaftor

A

Symdeko
Potentiator/Corrector
6 YO
2 copies of F508del mutation
OR
At least 1 mutation in the CFTR gene responsive to therapy

70
Q

Ivacaftor+Tezecaftor+Elexacafotr

A

Tricafta
Potentiaor/Correctors
2 YO
At least 1 copy of the F508del mutation

71
Q

How do you administer CFTR modulators?

A
  1. Taken Q12H
  2. Mised dose, wait 6 hours between doses
  3. Take with fat-containing food
  4. Do not take with grapefruit juice or Seville oranges
72
Q

DDIs of CFTR modulators?

A

CYP3A4 (inducers and inhibitors)

73
Q

ADRs of CFTR modulators? Monitoring?

A

Nausea, abdominal pain, increased hepatic enzymes, HA, URTI, blurred vision, chest discomfort, dyspnea

BP, hepatic enzymes, liver toxicity, cataracts

74
Q

When do you require lung transplant for CF?

A

Severe disease
FEV1<30%, gender, nutritional status, diabetic, sputum, number of exacerbations

Must replace both lungs

75
Q

What does the daily CF management look like?

A
  1. ACR
  2. Nutrition support
  3. Personal fitness routine
  4. ABX and other meds for acute conditions
76
Q

How should you monitor CF therapy?

A
  1. Adherence
  2. Adequate nutrition, weight, bone health
  3. ADRs
  4. Disease and med complications
  5. Patient and caregiver educations

Therapeutics I (32 decks)

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