Therapeutics I > CF > Flashcards

CF Flashcards

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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
What are important decision points to go over when a CF patient wants to conceive?
1. Genetic testing of both patient and partner 2. Family planning and contraception
26
What are the clinical presentation of CF?
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
27
What are steps of the multistep process of CF?
1. Newborn screening 2. Sweat test 3. Genetic or carrier test 4. Confirmation of CFTR dysfunction 5. Clinical eval by specialists
28
What are the types of CF management techniques?
1. ACT, infection prevention, anti-inflammatory therapies 2. Nutrition support 3. Disease modifying therapies: GFTR modulators 4. Transplants
29
What are some growth and weight maintenance techniques?
1. Pancreatic enzyme replacement 2. Adequate caloric intake
30
What are some bone and health supplements?
1. ADEK 2. Calcium intake 3. NaCL replacement
31
What are some pharmocotherapies used for CF?
1. Bronchodilators 2. Lung surface hydration 3. Mucolytics 4. ACT 5. Antibiotics 6. Anti-inflammatory (OCS, Azithromycin)
32
How are OCSs used for CF?
Slows progression of lung disease in patients with CF (not good for chronic)
33
How is azithromycin used for CF?
Anti-inflammatory properties reducing pulmonary exacerbations
34
What is the sequence of CF meds for airway clearance regimen? How often do you do this?
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
What is bronchodilator used for CF?
Albuterol - bronchodilator used to open airways prior to clearance
36
How do you hydrate the lungs?
Hypertonic saline (Hyper sal) - increases hydration of surface fluid and improves clearance 2YO
37
What do you use for individuals who can't use Hyper sal? Qualifications? ADR?
Inhaled mannitol (Bronchitol) BID Must pass BTT and premeditated with BD before use Bronchospasm and hemoptysis
38
What are the mucolytics for CF? Counseling?
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
What are the pulmonary effects of using mucolytics?
1. Increased lung function 2. Decreased sputum viscosity 3. Decreased pulmonary exacerbations by 28%
40
What are some airway clearance therapies?
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
What are the different route of antibiotics for CF? What do they do?
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
How is P. aureuginosa differ from other bacterial treatment?
2 drug combos due to MDR
43
What are the advantage of inhaled ABX?
1. Increased concentration of site of infection 2. Decreased need for IV and toxicity
44
What are the neb ABX? Brand? Indication?
1. Tobramycin (TOBI) - P. aeruginosa 2. Aztreonam (Cayston) - P. aeruginosa 3. Colistin: P. aeruginosa 4. Amikacin (Arikayce): M. avid complex
45
What does the CF recommendations when handling P. aeruginosa?
Routine oropharyngeal cultures to determine if they're infected opposed to bronchoscopy Sputum culture is a good predictor of morbidity and mortality
46
How do you dose TOBI? Who would you recommend it for?
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
When would you use IV ABX?
To treat severe exacerabtions 2 IV ABX to treat P. AER.
48
What are the appropriate IV ABX combos to treat P. aeruginosa?
1. B-lactam + aminoglycoside 2. B-lactam + fluroquinolone 3. B-lactam + colistin Continue inhaled ABX
49
Benefits of chronic azithromycin treatment? Warning?
1. Decrease PA biofilm formation 2. Anti inflammatory 3. Improves FEV1 Does not treat P. aeruginosa infection
50
Dosing and recommendations of Azithromycin?
Patient >6YO with chronic PA colonization Patient <6 YO initial to delay PA colonization Dose: ≥40 kg: 500 mg three times a week
51
How do you monitor Azithromycin treatment?
Acid fast bacteria smear prior to starting then check smear and culture every 6-12 months
52
When is OCS used for CF?
First line for allergy brochopulmonary aspergillosis (ABPA)
53
When is ICS used for CF?
Unless they also have asthma
54
What is the benefit of using ibuprofen for CF?
1. Recommended in 6-17 YO with >60% 2. Mild PK monitoring 3. No evidence for improvement in adults
54
55
What kind of vaccines are recommended for CF patients? Kids? Adults?
Kids: Flu (not FluMist), Synagis, COVID Adults: Boosters and seasonal vaccines
56
Are CR patients excluded from vaccines?
No, they are not more susceptible to viral infections but outcomes are worse
57
What kind of diet should CF patients have?
High caloric and fat 110-200% energy intake Supplemental nutrition
58
What is the weight goal for CF patients? 20+? 2-20?
Women BMI: >22 Men BMI: >23 Kids: maintain 50th percentile
59
What are PERTs? Brands?
Pancreatic enzyme replacement therapy that may require H2 antagonist or PPI for alkaline environments Creon, Prancreaze, Viokase
60
What is the dosage form of PERTs? Administration/Counseling? ADRs?
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
How do you treat PERT OD?
Reduce dose, oral or enema laxatives, surgery
62
What may occur after excessive dosing of PERTs?
Fibrosing colonopathy
63
What may you need to supplement in CF patients? Fluids?
ADEK Salt tablets and fluid containing salt and electrolytes
64
What supplements improves bone health and osteoporosis prophylaxis?
D, K and calcium
65
Why do you vitamin D3 or D2?
Better absorption
66
What are the disease modifying therapies for CF?
1. Ivacaftor (Kalydeco) 2. Lumacaftor/Ivacaftor (Orkambi) 3. Tezacaftor/Ivacaftor (Symdeko) 4. Elexacaftor/Tezacaftor/Ivacaftor (Trikafta)
67
Ivacaftor
Kalydeco Potentiator 1month At least 1 mutation in CFTR gene responsive to therapy
68
Ivacaftor+Luacafotr
Orkambi Potentiator/Corrector 1 year 2 copies of F508del mutation
69
Ivacaftor+Tezacaftor
Symdeko Potentiator/Corrector 6 YO 2 copies of F508del mutation OR At least 1 mutation in the CFTR gene responsive to therapy
70
Ivacaftor+Tezecaftor+Elexacafotr
Tricafta Potentiaor/Correctors 2 YO At least 1 copy of the F508del mutation
71
How do you administer CFTR modulators?
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
DDIs of CFTR modulators?
CYP3A4 (inducers and inhibitors)
73
ADRs of CFTR modulators? Monitoring?
Nausea, abdominal pain, increased hepatic enzymes, HA, URTI, blurred vision, chest discomfort, dyspnea BP, hepatic enzymes, liver toxicity, cataracts
74
When do you require lung transplant for CF?
Severe disease FEV1<30%, gender, nutritional status, diabetic, sputum, number of exacerbations Must replace both lungs
75
What does the daily CF management look like?
1. ACR 2. Nutrition support 3. Personal fitness routine 4. ABX and other meds for acute conditions
76
How should you monitor CF therapy?
1. Adherence 2. Adequate nutrition, weight, bone health 3. ADRs 4. Disease and med complications 5. Patient and caregiver educations
Therapeutics I
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