CF, Sickle Cell Disease, Opthalmic, Thyroid, Smoking Cessation

Cystic fibrosis

Autosomal…disease, mutation in…gene

Diagnosis from…

Presents as…

Early symptoms

• (3)

Pancreas (blockage of pancreatic juices)

• Endocrine →

  • Management → 1

• Exocrine → 1

  • Management → 3

CF resp infections

• >90% chance of colonization by…(could be just colonization or lead to infection). And also…in the 1st decade of life

• Management → 3

• Can use…for long term management to reduce inflammation

Cystic fibrosis

Autosomal recessive disease, mutation in CFTR gene

Diagnosis from sweat gland chloride test >60 (remember salty tasting skin)

Presents as thick, sticky, viscous mucus

Early symptoms

• Failure to gain weight

• Sweaty skin

• Steatorrhea (foul smelling stool with mucus/fats)

Pancreas (blockage of pancreatic juices)

• Endocrine → CF related DM (lack of insulin, pancreatic cells killed)

  • Management → insulin

• Exocrine → malnutrition

  • Management → high calorie diet, Vitamin supplementation (esp ADEK → rickets/osteopenia), pancreatic enzymes

CF resp infections

• >90% chance of colonization by pseudomonas (could be just colonization or lead to infection). And also Staph aureus and Haemophilus in the 1st decade of life

• Management → antipseudomonal Abx (PO, IV, neb), mucolytics, bronchodilators for wheezing

• Can use Azithromycin for long term management to reduce inflammation

Order of tx for cystic fibrosis induced infection:

• (5)

Mucolytic

• Pulmozyme → generic

  • Don’t mix with…

  • Storage

• Hypertonic saline

Formulation, class

• TOBI → generic (frequency)

• Cayston → generic

Order of tx for cystic fibrosis induced infection:

• Albuterol → Hypertonic saline → Dornase alfa → Inhaled Abx → Inhaled Corticosteroid

Mucolytic

• Pulmozyme → Dornase Alfa

  • Don’t mix with other nebs

  • Refrigerated. Protected from light (vs pancreatic enzymes)

• Hypertonic saline

Inhaled Abx

• TOBI → Tobramycin (28 days on, 28 days off)

  • Remember ADE (aminoglycoside → nephro, ototoxicity). Avoid with loops/thiazides (ototoxicity)

• Cayston → Aztreonam

Class

Formulation

-

Creon

Pancreaze

Pancrelipase

Pertzye

Zenpep

Viokace (formulation with what med)

-

Storage

Dont crush/chew. Can be opened over applesauce if…

Pancreatic enzymes (good for fats, carbs, proteins — not needed for simple sugars)

Enteric coated capsules (except viokace) that bypasses stomach to reach the intestines (do not get released in acidic environment)

-

Viokace (tablet with PPI)

-

RT (vs Dornase alfa mucolytic, Tobramycin inhaled Abx)

Dont crush/chew. Can be opened over applesauce if pH <4.5

Class →

Kalydeco → generic, used in patients with…mutation

Trikafta → generic, used in patients with…muation

Class → CFTR modulator

Kalydeco → Ivacaftor, used in patients with G551D mutation

Trikafta → Elexacaftor/Tezacaftor/Ivacaftor and Ivacaftor, used in patients with F508del muation

Sickle Cell Disease Issues

Autosomal…disease

Abnormal…molecules (distorted shape), …disease

Symptoms appearing usually after

Abx prophylaxis started after diagnosis → (…if allergic) until age…

Issues (see picture)

Autosomal recessive

Abnormal Hemoglobin S molecules (distorted shape), HbSS disease

Symptoms appearing usually after 4 months

Abx prophylaxis started after diagnosis → PCN (erythromycin if allergic) until age 5

Vaccines for sickle cell disease

(4)

Vaccines

Pneumococcal Vaccine: both child (Prevnar) and adult (Pneumovax)

Annual influenza

Meningococcal (2 yo with booster Q 5 yo)

Hib (routine and at age 2)

Droxia, Hydrea → generic (used for…(2))

• MOA →

• ADE →

Droxia, Hydrea → Hydroxyurea (used for cancer and sickle cell anemia)

• MOA → increases production of fetal hemoglobin (dont have problems) to create healthy hemoglobin

• ADE → bone marrow suppression

Generic

• Viagra

• Revatio → indication

Class

• MOA →

• CI with…(med)

• DDI → CYP

Sildenafil

• Viagra

• Revatio → PHTN

ED Drug

• MOA → PDE5 inhibition

• CI with nitrates (include Bidil or Hydralazine + Isosorbide dinitrate)

• DDI → CYP3A4 substrate

Sildenafil (Viagra, Revatio for PHTN), Tadalafil (Cialis for ED and BPH, Adcirca for PHTN)

Vardenafil (Levitra, Staxyn ODT), Avanafil (Stendra)

Generic

• Cialis → indication (2)

• Adcirca → indication (1)

Class

• MOA →

• CI with…(med)

• DDI → CYP

Tadalafil

• Cialis → ED, BPH

• Adcirca → PHTN

ED Drug

• MOA → PDE5 inhibition

• CI with nitrates (include Bidil or Hydralazine + Isosorbide dinitrate)

• DDI → CYP3A4 substrate

Unique → Works for 36 hrs → called the “weekender”

-

Sildenafil (Viagra, Revatio for PHTN), Tadalafil (Cialis for ED and BPH, Adcirca for PHTN)

Vardenafil (Levitra, Staxyn ODT), Avanafil (Stendra)

Generic

• Levitra

• Staxyn → formulation

Class

• MOA →

• CI with…

• DDI → CYP

Unique →

Vardenafil

• Levitra

• Staxyn → ODT

ED Drug

• MOA → PDE5 inhibition

• CI with nitrates (include Bidil or Hydralazine + Isosorbide dinitrate)

• DDI → CYP3A4 substrate

Unique → increased risk of seizures

Stendra

Class

• MOA →

• CI with…(med)

• DDI → CYP

Avanafil

ED Drug

• MOA → PDE5 inhibition

• CI with nitrates (include Bidil or Hydralazine + Isosorbide dinitrate)

• DDI → CYP3A4 substrate

Sildenafil (Viagra, Revatio for PHTN), Tadalafil (Cialis for ED and BPH, Adcirca for PHTN)

Vardenafil (Levitra, Staxyn ODT), Avanafil (Stendra)

Generic (class)

• MUSE → formulation, directions

• Caverject → formulation, directions

Alprostadil (prostaglandin PGE-1)

• MUSE → thin tube that has pellet inserted into the urethra

• Caverject → Injection that goes into spongy tissue of penis (corpus cavernosum)

Pulmonary HTN cause by…

Meds Summary

Pulmonary HTN cause by…narrowing of pulmonary arterioles → right heart pushing against that pressure to the lungs → right heart enlarged

Class for Indication

• Tracleer→ generic

• Letairis → generic

-

Formulation →

ADE →

Endothelin receptor antagonist for PHTN

• Tracleer→ Bosentan

• Letairis → Ambrisentan

-

Formulation → PO

ADE → teratogenicity

Class for Indication

• Flolan → generic, formulation

• Tyvaso → generic, formulation

• Ventavis → generic, formulation

Prostacyclin analogs for PHTN

• Flolan → Epoprostenol — IV

• Tyvaso → Treprostinil — IV/SQ, PO, inhalation

• Ventavis → Iloprost — inhalation

Multiple Sclerosis pathophys

For an acute attack → treat with

Multiple Sclerosis is an autoimmune disease where the body’s immune system attacks the myelin (axon insulation) in the central nervous system and prevents axons from communicating to each other

For an acute attack → treat with IV methylprednisolone

Indication →

Formulation → all…except for …

Storage → all… (except for…)

• Copaxone, Glatopa → generic, preferred med in…

---

• Generic

  • Avonex

  • Rebif

• Generic

  • Plegridy

• Generic

  • Betaseron

  • Extavia

• ADE →

Indication → MS

Formulation → all SQ except for Avonex (IV)

Storage → all in the fridge (except for interferon beta-1b until reconstituted)

• Copaxone, Glatopa → Glatiramer, preferred agent in pregnancy

---

• Interferon beta-1A

  • Avonex

  • Rebif

• Pegylated Interferon beta-1A

  • Plegridy

• Interferon beta-1B

  • Betaseron

  • Extavia

• ADE → flu like symptoms

Indication →

Formulation → all…

Monoclonal antibodies

• Tysarbi → generic

  • ADE

  • Must be enrolled in the…program

• Lemtrada → generic

• Ocrevus → generic

Mitoxantrone → antimetabolite also for…(indication), (max dose) due to…ADE

Indication → MS

Formulation → all IV

Monoclonal antibodies

• Tysarbi → Natalizumab

  • ADE → progressive multifocal leukoencephalopathy (PML)

  • Must be enrolled in the TOUCH program

• Lemtrada → Alemtuzumab

• Ocrevus → Ocrelizumab

Mitoxantrone → antimetabolite also for MS, max 140 mg/m2 due to cardiac ADE

Formulation for Indication

Tecfidera → generic

• MOA →

• ADE→

PO med for MS

• dimethyl fumarate (tecfidera), teriflunomide (aubagio), cladribine (mavenclad)

• fingolimod (gilenya caps), siponimod (mayzent), dalfampridine (ampyra)

---

Tecfidera → Dimethyl fumarate

• MOA → activates antioxidant pathways

• ADE→ flushing

Formulation for Indication

Aubagio → generic

• Prodrug stuff

  • Same ADE→ (2)

Mavenclad → generic

• ADE → (1)

PO med for MS

• dimethyl fumarate (tecfidera), teriflunomide (aubagio), cladribine (mavenclad)

• fingolimod (gilenya caps), siponimod (mayzent), dalfampridine (ampyra)

---

Aubagio → Teriflunamide

• Leflunomide (Arava) for non-biologic RA is prodrug of Teriflunomide

  • Same ADE→ Liver toxicity, Pregnancy Category X

Mavenclad → Cladribine

• ADE → risk of teratogenicity

Formulation for Indication

Gilenya → generic, formulation

Mayzent → generic

Observe both meds for the first…hrs due to…(ADE)

PO med for MS

• dimethyl fumarate (tecfidera), teriflunomide (aubagio), cladribine (mavenclad)

• fingolimod (gilenya caps), siponimod (mayzent), dalfampridine (ampyra)

---

Gilenya → Fingolimod, capsules

Mayzent → Siponimod

Observe both meds for the first 6 hrs due to bradycardia (MI, stroke, HF, AV block — ABCD med)

Formulation for Indication

Ampyra → generic

MOA →

May improve…

PO med for MS

• dimethyl fumarate (tecfidera), teriflunomide (aubagio), cladribine (mavenclad)

• fingolimod (gilenya caps), siponimod (mayzent), dalfampridine (ampyra)

---

Ampyra → Dalfampridine

MOA → K channel blocker

May improve walking speed

Class for Indication

• Lumigan → generic

  • Latisse → indication

• Xalatan → generic

• Travatan Z → generic

MOA →

Dose timing →

ADE →

Prostaglandin analogs for Glaucoma

• Lumigan → Bimatoprost

  • Latisse → Bimatoprost for eyelash growth

• Xalatan → Latanoprost

• Travatan Z → Travoprost

MOA → increase humor outflow

• prostaglandin analogs, cholinergics, alpha 2 agonist, rho kinase inhibitor

Dose timing → QHS

ADE → darkening of the iris and under the eyes

Class for Indication

Timoptic, Timoptic-XE, Istalol → generic

• Cosopt → generic

  • Cosopt PF → formulation note

• Combigan → generic

Betoptic S →

MOA →

ADE →

Beta blockers for glaucoma

Timoptic, Timoptic-XE, Istalol → Timolol 0.25% and 0.5% (non selective BB)

• Cosopt → Timolol + Dorzolamide (CAI)

  • Cosopt PF → preservative free

• Combigan → Timolol + Brimonidine (alpha 2 agonist)

Betoptic S → Betaxolol (selective BB → less asthma/COPD issues)

MOA → reduce humor production (anti fight/flight)

• BB, CAI, alpha 2 agonist

ADE → avoid in asthma/COPD, HF, bradycardia, hypotension, heart block

Class for Indication

Azopt → generic

• Symbrinza → generic

Trusopt → generic

• Cosopt → generic

MOA →

Avoid in…allergy

Carbonic anhydrase inhibitors (CAIs) for Glaucoma

Azopt → Brinzolamide

• Symbrinza → Brinzolamide + Brimonidine (alpha 2 agonist)

Trusopt → Dorzolamide

• Cosopt → Dorzolamide + Timolol (BB)

MOA → reduce humor production

• BB, CAI, alpha 2 agonist

Avoid in sulfa allergy

CAI not for glaucoma → Acetazolamide (Diamox), Methazolamide

Diamox → generic

Methazolamide

-

Class →

Indication →

Avoid in…allergy

Diamox → Acetazolamide

Methazolamide

-

Class → Carbonic anhydrase inhibitors

Severe systemic ADE→ not often used for glaucoma, used for acute mountain sickness or acute glaucoma crises

• CAI for glaucoma: brinzolamide (azopt), dorzolamide (trusopt)

Avoid in sulfa allergy

Alphagan P → generic

• Combigan → generic

• Simbrinza → generic

Class for Indication

MOA →

• (2)

Alphagan P → Brimonidine

• Combigan → Brimonidine + Timolol

• Simbrinza → Brimonidine + Brinzolamide

Alpha 2 agonist for Glaucoma

MOA →

• Increase humor outflow (just like prostaglandin, cholinergic, rho kinase inhibitor)

• Reduce humor production (just like BB, CAI)

Rhopressa → generic

Class for Indication

MOA →

Rhopressa → Netarsudil

Rho kinase inhibitor for Glaucoma

MOA → increase humor outflow (just like prostaglandin analogs, cholinergic, alpha 2 agonist, rho kinase inhibitor)

Pilocarpine

Carbochol

Class for Indication

MOA →

ADE →

Cholinergic (Miotics) for Glaucoma

MOA → increase humor outflow (just like prostaglandin analogs, cholinergic, alpha 2 agonist, rho kinase inhibitor)

ADE → pupil constriction, poor night vision

Indication →

Class →

• Vigamox, Moxeza → generic

• Ocuflox → generic

• Ciloxan → generic

Class →

• Erythromycin

• Azithromycin → storage

Class →

• Tobradex → generic

Indication → infective bacterial conjunctivitis

• viral conjunctivitis has no treatment

FQ

• Vigamox, Moxeza → Moxifloxacin

• Ocuflox → Ofloxacin

• Ciloxan → Ciprofloxacin

Macrolide →

• Erythromycin

• Azithromycin → fridge before dispensing, RT for 14 days after opening

Aminoglycosides →

• Tobradex → Tobramycin + Dexamethasone

Class for Indication

Restasis → generic, formulation

Cequa → generic, formulation

Verkazia → generic, formulation

Vevye → generic, formulation

Calcineurin inhibitors for Dry eyes

Restasis → Cyclosporine 0.05% preservative free emulsion

Cequa → Cyclosporine 0.09% solution

Verkazia → Cyclosporine 0.1% emulsion

Vevye → Cyclosporine 0.1% oral solution

Indication → dry eyes

Xildra → generic, class

Tyrvaya → generic, class → formulation

• Chantix → indication

Eysuvia → generic, class → up to…(time) of use

Miebo →

Xildra → Lifitegrast 5%, integrin antagonist

Tyrvaya → Varenicline, cholinergic nicotinic receptor agonist → nasal spray

• Chantix → smoking cessation, not for dry eyes

Eysuvia → Loteprednol, steroid → up to 2 weeks of use

Miebo → Perfluorohxyloctane, unknown MOA

Nicorette → generic

Commit → generic

OTC/Rx

Indication →

What dose to choose →

• (2)

Weeks

• 3 steps

Avoid gum in patients with…

Nicorette → Nicotine gum

Commit → Nicotine lozenge

OTC (just like patch)

Indication → smoking cessation

What dose to choose →

• Within 30 min → 2 mg

• > 30 min → 4 mg

Weeks

• 1 gum/lozenge Q1-2 hrs x6 weeks

• 1 gum/lozenge Q2-4 hrs x3 weeks

• 1 gum/lozenge Q4-8 hrs x3 weeks

Avoid gum in patients with significant dental work or have dentures

4 mg gum/lozenge can reduce/delay weight gain

NicoDerm CQ

Generic, OTC/Rx

Dosing

• 2 groups

Avoid in patients with…

Don’t… Remove before…

Nicotine Patch (OTC just like lozenge/gum)

Dosing

• If >10 cigs/day (half pack) →

  • 21 mg patch x 6 weeks (step 1)

  • 14 mg patch x 2 weeks (step 2)

  • 7 mg patch x 2 weeks (step 3)

• If <10 cigs/day →

  • 14 mg patch x 6 weeks (step 2)

  • 7 mg patch x 2 weeks (step 3)

Avoid in patients with derm issues

Don’t cut. Remove before MRI

Nicotrol → formulation

Nicotrol NS → formulation, avoid in…

OTC/Rx

Indication →

Nicotrol → inhaler

Nicotrol NS → nasal spray, avoid in asthma/COPD

Rx

Indication → smoking cessation

Zyban →

• MOA →

• ADE →

• Avoid….(2 conditions)

• Beneficial in patients with…(2 conditions)

Chantix →

• MOA →

• ADE →

  • 3

• Avoid in…(2 conditions)

Zyban → Bupropion SR

• MOA → DNRI

• ADE → dry mouth, insomnia

• Avoid seizures and eating disorders

• Beneficial in patients with depression or CV disease

• Remember Wellbutrin for depression

Chantix → Varenicline

• MOA → cholinergic nicotinic receptor agonist

• ADE →

  • Vivid dreams/insomnia → take dose earlier than bedtime

  • Nausea → take after eating with full glass of water

  • Suicidal ideation

• Avoid in seizures and psych disorders

HYPO vs HYPER thyroidism

S/S

Labs

• HYPO →

• HYPER →

Normal free T4 range →

Normal TSH range →

Recheck levels every… For hypothyroidism, TSH every…during the 1st half of pregnancy

Labs

• HYPO → low T4, high TSH

• HYPER → high T4, low TSH

Normal free T4 range → 0.9 - 2.3 ng/dL

Normal TSH range → 0.4 - 4 mIU/L

Recheck levels every 6-8 weeks. For hypothyroidism, TSH every 4 weeks during the 1st half of pregnancy

Med that cause HYPO/HYPERthyroidism

Key Drugs Causing HYPOthyroidism

*Can also cause HYPERthyroidism

Synthroid, Levoxyl, Unithroid →

• IV to PO →

Armour Thyroid →

Cytomel →

---

Indication →

ADE →

Timing, regards to food →

DDI →

Synthroid, Levoxyl, Unithroid → Levothyroxine (T4)

• IV to PO → 0.75 to 1

Armour Thyroid → Thyroid USP (T3/T4)

Cytomel → Liothyronine (T3)

---

Indication → HYPOthyroidism tx

ADE → nervousness, insomnia, diarrhea, weight loss

Timing, regards to food → morning on empty stomach

DDI → antacids and polyvalent cations (including iron, Ca, Al, Mag), multivitamins, cholestyramine, sevelamer and sucralfate

Propylthiouracil (PTU)

Methimazole

MOA → …, PTU also…

ADE → 3

PTU BBW →

Indication →

• which is preferred when

MOA → inhibit synthesis of thyroid hormones, PTU also inhibit conversion of T4 to T3

ADE → hepatoxicity, agranulocytosis, DILE

PTU BBW → hepatotoxicity

Indication → HYPERthyroidism

• Methimazole preferred over PTU (bc has less liver ADE) except when…

  • PTU is preferred in thyroid storm and 1st trimester in pregnancy (switch back to methimazole in 2nd/3rd trimester)