CF/SCD/ED/PHTN/MS Flashcards

(51 cards)

1
Q

How to diagnose CF?

Early symptoms?

A

Sweat gland chloride test >60 mmol/L

Failure to gain weight, salty tasting skin, steatorrhea, episodes of pneumonia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What type of heriditary disease is CF?

A

Autosomal recessuve

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the two major organ systems affected in CF?

A

Pancreas
-endocrine issue leased to CF related diabetes
-Exocrine issues leads to malnutrition

Lungs
-Causes bronchitis and pneumonia to occur more frequently

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How do you manage malnutrition in CF?

A

High calorie diet
Vitamins like ADEK
Pancreatic enzymes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What organisms are common in CF patients?

A

PSA, MSSA, MRSA, h.influenza

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the options for chronic PSA infections in CF patients?

A

Inhaled tobramycin BID 28 days on 28 days off

Inhaled aztreona e TID 28d on 28d off

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Dornase alfa

Brand
ROA
Indication
Storage

A

Pulmozyme

Inhaled

Mucolytic for CF

Refrigerated and protected from light

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are your pancreatic enzyme options for CF?

Where to store?

When to take?

What to avoid?

A

Creon, pancrease, pancrelipase, pertyze, viokace

At room temp

Before meals

Avoid antacids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Antiinflammatory options for CF?

A

Ibuprofen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Ivacaftor

Brand
MOA
Indication/age

A

Kalydeco

CFTR modulator therapy

CF >4 months old with G551D mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Tezacaftor - ivacaftor

Brand
MOA
Indication

A

Symdeko

CFTR modulator

Cf >6y.o with F508del mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Lumacaftor-ivacaftor

Brand
MOA
Indication

A

Orkambi

CFTR modulator therapy

> 2y.o with F508del mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Trikafta

Generic
MOA
Indication

A

Elexacaftor-tezacaftor-ivacaftor + ivacaftor

CFTR modulator therapy

> 6y.o with F508del mutation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What kind of hereditary disease is sickle cell disease?

A

Autosomal recessive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

When do symptoms start for SCD and what tests are there?

A

4 months old

Newborn screening, Hgb electrophoresis, high performance liquid chromatography

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What labs change when sicked RBC’s breakdown prematurely?

A

Anemia
High bilirubin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are the five types of sickle cell crisis?

A

Vasoocclusive - hydration, transfusion, pain management

Aplastic crisis

Hemolytic crisis

Splenic sequestration crisis

Acute chest pain

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What vaccines do you give for asplenia?

A

Pneumoncocca
Hib
meningococcal
influenza

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

When do you give PCN prophylaxis for SCD and what do you give?

A

First 5 years - PenVK 125mg PO BID for 1st 3 years then increase to 250mg PO BID

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Hydroxyurea

Brand
Indication
MOA
ADE

A

Hydrea, droxia, siklos

SCD

reactivating fetal hemoglobin

BMS, GI, rash, hepatitis, pulmonary fibrosis , pregnancy category D

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Voxelotor

Brand
Indication
MOA

A

Oxbryta

SCD

Hbs polymerization inhibitor to prevent sickling

22
Q

Crizanlizumab

Brand
Indication
MOA

A

Adakveo

SCD

IV mAb to reduce frequency of vaso-occlusive episodes

23
Q

How does inhaled hypertonic saline work for CF? ADE?

A

It’s a mucolytic that is recommended alongside dornase alfa to help draw out water from the cells so it can mix with the mucus and expel it

CoughingE

24
Q

Exa-cel

Brand
Indication
MOA

A

Casgevy

SCD

Gene editing to increase fetal Hgb and reduce vaso-occlusive crisis

25
Sildenafil Brand Indication Can it be cut in half? Duration
Viagra - Tabs Revatio - tabs, suspension, injection ED (viagra) Pulmonary HTN (revatio) Yes, even though it is not scored. 3-4 hours
26
Tadalafil Brand Indication Duration
Cialis, adcirca, tadliq Pulmonary HTN and ED 36 hours
27
Vardenafil Brand / dosage Indication ADE
Levitra - tabs Staxyyn ODT ED Sudden hearing loss, seizures
28
Avanafil Brand Indication
Stendra ED
29
Alprostadil Brand MOA ROA Max dose
Caverject MUSE (pellet inserted into urethra) PGE1 analgoe Intracavernosally 3x/week
30
What is DOC for pulm.HTN? What is conventional therapy?
Nifedipine Diuretics, O2, anticoag
31
What if you fail DOC for pulm.HTN? Class 2-4
Class II: ambrisentan + tadalafil Class III: Epoprostenol , iloprost,, treprostitnil Class IV: Epoprostenol
32
What is multiple sclerosis?
Autoimmune disease that attacks myelin sheath which prevents communication between brain and spinal cord.
33
How do you treat acute multiple sclerosis attacks?
IV methylprednisone x3-5 days then use oral to taper off
34
What are the types of MS classifications?
Relapsing - what the drugs are for Primary progressive - only one drug approved - ocrelizumab (ocrevus)G
35
Glatiramer Brand Indication ROA Storage
Copaxone, Glatopa MS SQ Fridge
36
Interferon beta 1A Brand Indication ROA
Avonex, rebif MS Avonex- IM Rebif - SQ
37
Interferon beta-1B Brand Indication ROA Storage
Betaseron, extavia MS SQ Room temp
38
Pegylated interferon beta-1A Brand Indication ROA
Plegridy MS SQ
39
Natalizumab Brand ADE Indication ROA Program?
Tysabri MS Progressive multifocal leukoencephalopathy IV monthly Patient and prescriber must be enrolled in TOUCH
40
Alemtuzumab Brand Indication ROA
Lemtrada MS IV
41
Ocrelizumab Brand Indication ROA
Ocrevus Primary progressive MS IV every 6 months
42
Dimethyl fumarate Brand Indication ROA ADE
Tecfidera MS 240mg PO BID Nausea, flushing
43
Monomethyl fumarate Brand Indication ROA
Bafiertam MS PO
44
Teriflunomide Brand Indication ROA MOA ADE
Aubagio MS PO Pyrimidine synthesisi inhibitor Preganacy category x, hepatotox
45
Cladribine Brand Indication ROA
Mavenclad MS PO
46
Fingolimod Brand MOA Indication ROA ADE
Gilenya Sphingosine-1- phosphate receptor modulator MS PO Bradycardia, PML
47
Siponimod Brand Indication ROA
Mayzent MS PO
48
Ozanimod Brand Indication ROA
Zeposia MS PO
49
Ponesimod Brand Indication ROA
Ponvory MS PO
50
Dalfampridine Brand Indication ROA
Ampyra MS PO
51
What do you monitor with interferon therapy?
CBC Depression Thyroid CVD LFT Seizures Flu-like symptoms