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PHRM 865: Theraputics V > Lecture 33: Cystic Fibrosis > Flashcards

Lecture 33: Cystic Fibrosis Flashcards

1
Q

What is some of the Epidemology Cystic Fibrosis?

A
  • Lethal Genetic Disease
  • 1 in 3000 people have it
  • Autosomal Rescessive [mother/father have 1 gene for it]
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2
Q

What is Cystic Fibrosis mainly caused by and whats is most common mutation?

A
  • Mutations within the CFTR
  • Most common is F508del
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3
Q

Breifly explain what happens when there is a CFTR Channel that is absent or reduced within the Lungs?

A
  • Normal: CFTR moves Cl into mucus and H20/Na into cell
  • Abnormal: Cl cant move out [NO CFTR]; alot of H20/Na move in helping bacteria get in
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4
Q

What is the important Newborn Screening Test that should be done with ALL babies?

A
  • Blood Spot [from heel]
  • Immunoreactive Trypsinogen [IRT]
  • Sweat Test [+ = >60mEq/L]

if you have 1 (+) = sweat test; 2 (+) = CF testing

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5
Q

What are some of the presenting signs and symptoms for the different age groups?

Neonates? Infants/Childern? Teens/Adults?

A
  • Neonates: Meconium Ileus, Jandice
  • Infants/Children: Cough, URI, Wheezing, Weight Gain..
  • Teens/Adults: Delayed sexual maturation, Nasal polyposis
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6
Q

What happens when there is a nonsense mutations within CFTR?

A
  • NO CFTR is made = so Cl going anywhere
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7
Q

What happens when there is a F508del mutation with CFTR?

A
  • Barely any CFTR = so not a lot of Cl moving around
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8
Q

What happens when there is a G551D mutation of CFTR?

A
  • Normal amount of CFTR BUT they aint working
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9
Q

What is Kalydeco, and what is in good in?

MOA? Age that its good in? CYP?

A
  • MOA: Helps “open the door” for G551D [CFTR Potentiator]
  • Good from 1m to 6y and older
  • 3A4 substrate
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10
Q

What are some of the important things to note when taking Kayldeco?

A
  • Take with fatty foods
  • Check LFTs [ALTs and ASTs]
  • Eye Exams
  • Dose adjust for Hepatic Issues
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11
Q

What is Ivacaftor/Lumacaftor [Orkambi], and what is it good with?

A
  • MOA: F508del
  • Good in age > 1
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12
Q

What are some of the important things to note when taking Orkambi?

A
  • Take with fatty foods
  • AST/ALT checked
  • Eye Exams
  • Dose adjust in Hepatic issues
  • Birth control interaction
  • SE: SOB & chest tightness

Lumacaftor = 3A inducer & Ivacftor is 3A substrate

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13
Q

What is Symdeko [tezacaftor/ivacaftor] and what is it good for?

A
  • Approved for F508del
  • Age > 6 yo
  • Has a tablet in the AM and in PM
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14
Q

What are some of the important things to note when taking Symdeko?

A
  • Take with Fatty Foods
  • AST/ALT increased
  • Eye Exam
  • Hepatic liver adjustment
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15
Q

What is Trikafta [Elexacaftor/Tezacaftor/Ivacaftor] and what is good to know about it?

Missed dose?

A
  • MOA: has 2 agents that “move the door to the wall” and opens it [for both F508del Homo or Herto]
  • For Age > 2yo
  • Miss dose by 6 hour; take when you remember and skip that evening dose
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16
Q

What are some of the important things to note when taking Trikafta?

A
  • Take with fatty foods
  • AST/ALT exams
  • Eye exam
  • Dosage adjust in LIVER disease
17
Q

What is the website to see if you qualify?

A
  • www.vertextreatmentshcp.com
18
Q

What is some of the pathophysiology for the Head?

A
  • Nasal Polyps - 7-56% have [treat: Surgery, Steroids, Nasal Rinses]
  • Allergies: Preferred treatment = Nasal Steroids
  • Psychological: Depression is common [Treat: SSRIs or Mirtazapine
19
Q

What is some of the pathophysiology of CF-Lung Disease?

A
  • Can cause ~85% of CF deaths
  • Caused by CFTR dyfunctions with thickened mucus
  • Exacerbation is VERY common
20
Q

What are som eof the methods that can help with Airways Clearences?

A
  • Manual Airway Clearence Techniques
  • Vest [shakes out the mucus]
  • Flutter or Acapella [device that viberates lungs when breathing]
  • Huff Coughing [inhale, hold, force exhale]
  • Meta Neb
21
Q

What is the way that Dornase Alfa helps with Maintenance of Lung Treatment?

A
  • MOA: Chops up the mucus to get it out [Cleaves extracellular DNA from neutrophils in mucus]
  • Can start daily in those >6 yo
  • Daily –> BID
22
Q

What is the way that Hypertonic Saline helps with the Maintenance of Lung Treatment?

A
  • MOA: Helps water move into the mucus; thinning it out
  • Recommended for ALL CFs but can cause broncospams
23
Q

What is the way that Inhaled Mannitol [Bronchitol] helps with the Maintenance of Lung Treatment?

A
  • MOA: Pulls water into the mucus; thinning it out
  • For 18 and older
  • Dry power BID [10 capsules]
  • Could cause bronchospams so may want to give albuterol
24
Q

How can Azithromycin be helpful with Maintenance of the Lungs?

MOA? Side Effects? When to use?

A
  • MOA: Immunomodulating Effects [Anti-inflammatory]
  • GI side effects
  • Recommended with chronic Pseudo
25
Q

What is the way that Albuterol is able to help with Lung Maintenance?

A
  • MOA: Short acting beta agonist = opens up the airways [decreases bronchoconstriction]
  • Used before saline becuase of the bronchospamsa
26
Q

What are the clinical features of a CF exacerbation?

A
  • Increased Cough
  • Increased Sputum Production
  • SOB
  • Chest Pain
  • Loss of appetite
  • Loss of weight
  • Decreased Lung Function Test
27
Q

For antibiotic therapy in CF, what are some of the pathogens that are needed to be covered?

A
  • S. Aureus [MSSA or MRSA]
  • Pseudo
  • H. Flu
  • K. Pneumonia
  • E. Coli, Streno
  • Aspergillus
  • Achromobacter
28
Q

What is the Empiric IV Therpay for someone that has MSSA and/or MRSA?

A
  • MRSA: give bactim, clinadmycin, Vancomycin, Tetrecycilne, Linezoild
  • MSSA: Cafazolin, Unsyn…
29
Q

What is the IV therpay for someone with Pseudomonas?

A
  • Double coverage
  • Pip/tazo, Meropenem, Cefepime + [Tobra or Amikacin]
30
Q

When might using the Inhaled Tobramycin might be good in your patients that has CF?

A
  • Used for Eradication of Pseudo in lungs [Sick or Not]
  • +3 cultures = Chronic Suppression of Pseudo [28d on and 28d off]
31
Q

When might the use of Aztreonam be used?

A
  • eflow Nebulizer
  • During the off months of Inhaled Tobra [used for chronic pseudo and when patients can not tolerate tobra alone]
32
Q

What is the reasoning behind using Nebulizations?

A
  • Reduced Systemic Exposure
  • Decreased Systemic Side Effects
  • Chronic Administration for Pseudo WITHOUT IV
  • Higher Drug concentrations in sites
33
Q

What is important to know about the Pancreas in CF?

A
  • Insufficieny is 85%
  • Treat with giving Pancreatic Enzymes [500-2500 lipase/kg per meal]
  • Creon is most commonly used
34
Q

What is importnat to know about the stoamach during CF?

A
  • Stomach Pain and Indigestion is normal
  • Take a PPI [-prazoles] or H2RA [Famotidine]

Acid suppression therapy may help with pancreatic anzyme activity BUT may also decrease Ca absorption

35
Q

What are the Vitamins that are used and what is the important monitoring for each?

A
  • Vit A: Monitor the levels
  • Vit E: Monitor the levels
  • Vit D: 25-OH – goal > 30 [give cholecalciferol]
  • Vit K: PT/INR
36
Q

Is cystric Fibrosis Related Diabetes the same this a like Type I or Type II Diabetes?

A
  • NO; not the same as Type I or Type II
  • A1c is NOT reliable for diagnosis
  • Ketoacidosis is rare
37
Q

what is important to know about the effectice of CF on Reproduction?

A
  • 95% of male are sterile because of the Vas Defernes
  • Contraception is encouraged
38
Q

What is important to know about the Bones during CF?

A
  • Ostroporosis/Ostropenia can happen
  • Give Calcium, Vit D or Bisphosphonates

PHRM 865: Theraputics V (42 decks)

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