Anticoagulants / Hematology Flashcards

Question

*Warfarin: * -Brand -MOA (Remember: "SNOT") -ROA -Onset of Action -Isomers -AVEs

Answer 1

Brand: Coumadin (D/C Brand), Jantoven MOA: competitively inhibits C1 subunit of vitamin K epoxide reductase (VKORC1) enzyme complex, reducing the production of vitamin K-dependent inactive clotting factors (II, VII, IX, and X) and anticoagulants protein C and S -Indirect inhibition of clotting cascade -To remember clotting factors: "SNOT" S: Seven, N: Nine, O: 0 in 10, T: TWO (also the order of the factors in the cascade) --> in first 24 hours see rapid depletion of VII, IX, and protein C and S with slight INR increase ROA: PO Onset of Action: 5 days (to finally see all clotting factors depleted and about 90% of INR reduction) Isomers: racemic mixture (S-warfarin which is 3-5x more potent and is metabolized by CYP2C9; R-warfarin metabolized from CYP3A4) AVEs: BLEEDING/BRUISING (mild to severe), SKIN NECROSIS

Answer 2

Warnings: tissue necrosis/gangrene, HIT (CI if monotherapy in initial TX of active HIT until PLT >150,000 cells/mm3), purple toe syndrome (rare from embolization of cholesterol cyrstals) Pharmacogenomics: presence of CYP2C9*2 or *3 alleles and/or polymorphism of VKORC1 gene --> increases bleed risk CIs: pregnancy (except w/ mechnical heart valves) Monitoring: NARROW THERAPEUTIC INDEX (NTI) drug -INR: if increased, more risk of bleeding; if decreased, risk of clots --> take after initial 2-3 doses then when stable Q4-12 weeks -Goal INR 2-3 (target: 2.5): Afib, bioprosthetic mitral valve, clotting disorder (ex. Factor V Leiden), mechnical aortic valve, VTE -Goal INR 2.5-3.5 (target 3): mechnical mitral valve, 2 mechanical heart valves -Hct, Hgb, s/sx of bleeding Reversal Agent: vitamin K

Answer 3

Warfarin is a racemic mixture where S-warfarin is 3-5x more potent -S-warfarin: metabolized by CYP2C9 -R-warfarin: metabolzied by CYP3A4 and others S-warfarin: -CYP2C9 inducers: decrease INR ("Review Patient Profiles and Counsel Soon): Rifampin, Phenytoin, Phenobarbital, Carbamazepine, St. John's Wort -CYP2C9 inhibitors: increase INR ("AAA": Amiodarone, Azole Antifungals, Anti-infectives --> metronidazole, Bactrim) Warfarin is HIGHLY protein bound --> drugs that are also highly protein bound may displace warfarin (ex. phenytoin, valproic acid)

Answer 4

Additive bleeding risk: NSAIDs, antiplatelets, anticoagulants, SSRIs, SNRIs Increase clotting risk: estrogen, SERMs (avoid use w/ tamoxifen) Supplements with bleed risk: chamomile, chondroitin, dong quai, high doses of fish oils, vitamin E, willow bark (plant salicylate), 5 G's (garlic, ginger, ginkgo, gingseng, glucosamine) Supplement that can decrease efficay: St. John's Wort Dietary Interactions: vitamin K consumption can decrease INR --> STAY CONSISTENT in diet -Foods high in vitamin K: spinach (cooked), broccoli, brussel sprouts, collard greens, kale, turnip greens, swiss chard, parsley, other greens, canola oil, some teas

Answer 5

Available as: "Please Let Greg Brown Bring Peaches to Your Wedding" -P: Pink (1mg), L: Lavender (2mg), G: Green (2.5mg), B: Brown/Tan (3mg), B: Blue (4mg), P: Peach (5mg), T: Teal (6mg), Y: Yellow (7.5mg), W: White (10mg) Initial Doses: 10mg or less for 2 days then adjust per INR -TX of active DVT/PE, start warfarin WHILE on parenteral anticoagulant for at least 5 days on both until INR is 2 or higher for at least 24 hours Lowering Doses: 5mg or less -Elderly, liver diease pt: decreased liver fxn and production of vitamin-K dependent clotting factors -Malnourished: less intake of vitamin K -HF: liver congestion, decreased clotting factor synthesis -CYP inhibitor use: increased warfarin serum levels -Taking select ABXs (PCNs, cephalosporins, quinolones, tetracyhclines): alteration of intestinal flora Maintenance dose: take weekly dose / 7 to see typical average daily dose and consider INR -Consider DDIs, dietary intake, and pt risk factors -Follow institutional protocol of how to adjust (pts can take two different strengths to achieve "one" dose, take different doses each day --> lots of customization)

Answer 6

INR <4.5, NO bleeding: hold or decrease dose INR 4.5-10:, NO bleeding: hold 1-2 doses of warfarin INR >10, NO bleeding: hold and administer 2.5-5mg PO vitamin K ANY INR with MAJOR bleeding: hold and administer 5-10mg IV vitamink K and 4-PCC (Four Factor Prothrombin Complex Concentrate) Surgery: -Planned surgery: hold dose for 5 days prior (if INR still elevated, consider low dose PO vitamin K) -Planned surgery, HIGH risk for thromboembolism: bridge w/ LMWH (D/C 24 hours before surgery) or UFH (D/C 4-6 hours prior to surgery) -Abrupt surgery: reverse warfarin w/ vitamin K

Answer 7

Brand: Kcentra, Balfaxar MOA: human blood containing factors VII, IX, X, II, Protein C, and Protein S ROA: IV TX: Reversal of warfarin --> only acts for about 8-12 hours so MUST BE GIVEN WITH VITAMIN K Dosing: -do NOT repeat dose -Dosing calculation has different vial concentrations for each factor, BUT dosing is based on factor IX per kg of pt's body weight and INR

Answer 8

Brand: Mephyton ROA: PO or IV TX: warfarin reversal (PO for no bleeding, but INR >10 and IV for major bleed any INR) Administration Considerations: -SQ NOT recommended due to risk of variable absorption -IM NOT recommended due to risk of hematoma -REQUIRES light protection during administration AVEs: anaphylaxis (dilute dose to avoid risk and infuse slowly over at least 20 minutes), flushing, rash, dizziness BBW: severe rxns resembling hypersensitivity rxns after IV

Answer 9

VIIa (another brand called Sevenfact)- BBW for severe thrombotic events

Answer 10

MOA: mixture of proteins derived from fish sperm that combines with heparins to form stable salt complex, neutralizing the anticoagulant activity TX/Dosing: -UFH Reversal: 1mg IV for 100 units of heparin reversal in the last 2-2.5 hours (heparin has very short T1/2), max: 50mg -LMWH Reversal: 1mg IV per 1mg of enoxaparin (LESS effective since shorter chain and reverses what was given in last 8 hours by about 60%) AVEs: hypotension (from rapid IV infusion - administer over 10 minutes), bradycardia, flushing, anaphylaxis BBW: hypersensitivity (due to fish sperm derivation) Monitoring: aPTT, anti-Xa levels, cardiac monitoring (ECG, BP, HR)

Answer 11

Brand: Andexxa MOA: recombinent modified human factor Xa protein ROA: bolus followed by infusion TX: apixiban or rivaroxaban reversal Warnings: THROMBOEMBOLIC RISKS, ischemic events, cardiac arrest, sudden death

Answer 12

Brand: Praxbind MOA/TX: humanized monoclonal antibody fragment that binds and reverses effects of dabigatran ROA: IV Look alike/sound alike: idarubicin Warnings: THROMBOEMBOLIC RISK, serious rxns from sorbitol excipient

Answer 13

Types and Diagnosis: -General diagnosis: D-dimer (protein released to breakdown blood clots) test (low sensitivity) --> if positive, use high sensitivity test to diagnose (ex. ultrasound for DVT and pulmonary CT angiogram for PE) -Deep Vein Thrombosis (DVT): clot in leg, causing unilateral lower extremity swelling and pain -Pulmonary Embolism (PE): clot that has broken off and traveled to lungs causing SOB and chest pain Modifiable risk factors (can go away): acute medical illness, imobility, obesity, pregnancy/postpartum period, recent surgery/trauma -MEDICATIONS: estrogen-containing, SERMs, erythropoiesis-stimulating agents (ESAs) Non-modifiable risk factors: increasing age, cancer/chemotherapy, previous VTE, certain disease states (HF, nephrotic syndrome, respiratory failure), inherited or acquired thrombophilia (antithrombin deficiency, factor V Leiden, anti-phospholipid syndrome, protein C or S deficiency)

Answer 14

Non-pharmacologic VTE prophylaxis: intermittent pneumatic compression (IPC) devices, graduated compression stockings (higher pressure at bottom to help blood flow and avoid DVT, use w/ 15-30mmHg), frequent ambulatoin, calf-muscle exercises VTE Treatment: -D/C: estrogen-containing medications, SERMs -Provoked VTE (surgery or reversible risk factor): 3 months of TX -Unprovoked VTE (unknown cause): extended phase therapy (potentially 6 months or more) using apixiban or rivaroxaban unless high risk of bleeding (3 months) -Two unprovoked VTE episodes: extended TX phase -If unprovoked VTE and D/C anticoagulant, recommended to continue ASA if no CIs Drug choice in VTE TX: -W/o cancer: DOAC (oral factor Xa inhibitors or dabigatran) for first 3 months > warfarin (sometimes used w/ longer duration for cost) -With cancer: oral factor Xa inhibitors preferred (others: oral anticoagulants, LMWHs)

Answer 15

Warfarin depletes factors II, VII, IX, and X which are procagulants AND protein C and S which are ANTI-coagulants. Warfarin depletes protein C and S FASTER than the clotting factors which means in the first few days patients are still at a potential risk for clotting until after 5-7 days have passed where warfarin depletes more protein C and S. During the first few days up to 5 days or at least until INR has stabilized for >24 hours, LMWH or UFH is used to close this gab of procoagulation risk.

Answer 16

Preferred: LMWH, intermittent pneumatic compresion devices -Monitoring with anti-Xa levels recommended Warfarin: teratogenic, but can be used in mechanical heart valve high risk or inherted thrombophilias --> consider converting from LMWH back to warfarin after 13th week of pregnancy and when close to delivery switch to LMWH since warfarin takes longer to D/C NOT recommended: oral factor Xa inhibitors and direct thrombin inhibitors (not been adequately studied)

Answer 17

Reason for anticoagulant: heart is NOT adequately pumping and blood pools which can lead to clots, potentially causing cardioembolic stroke Anticoagulation Selection: -Nonvalvular Afib: DOAC > warfarin -Mechanical heart valve: HIGHEST RISK --> warfarin (DOACs have NOT been approved) Duration: usually for life Cardioversion (attempt to convert Afib to normal sinus rhythm w/ electricty or medications): -Have risk of blood clot dislodging during transition -If pt Afib 48 hours, initiate anticoagulant for 3 weeks PRIOR to cardioversion then 4 weeks after cardioversion

Answer 18

Risk factors: -1 point for: CHF, HTN, DM, Vascular disease (prior MI, PAD, aortic plaque), age 65-74 yo, female -2 points for: age >/=75 yo, prior stroke/TIA/thromboembolism Recommendations based on score: -No anticoagulant if 0 points in men or 1 point in women -Oral anticoagulant considered if 1 point in men or 2 points in women -Oral anticoagulant recommended (DOACs > warfarin): 2 or more points in men, 3 or more points in women

Answer 19

Assess bleeding risk in AF pts on anticoagulants H: HTN (SBP >160mmHg) 1 point A: Abnormal liver or kidney functoin: 1-2 points S: Stroke 1 point B: Bleeding tendency or predisposition: 1 point L: Labile INR (if on warfarin): 1 point E: Elderly (>65 yo): 1 point D: Drugs (ASA, NSAIDs), excess alcohol use: 1-2 points **May NOT need to know this entire scoring system, but in general, more points = more bleeding risk

Answer 20

Anemia: decrease in RBCs, hemoglobin, and/or hematocrit Causes: -Impaired RBC or Hgb production (nutritional deficiencies - iron, folate, vitamin B12), complications of a medical disorder (CKD, malignancy) -Increased RBC destruction (hemolysis, mechanical such as valves) or blood loss -Genetic predispositions (sickle cell disease) S/Sx: mild can be asymptomatic -Reduced oxygen delivery: fatigue, weakness, SOB, exercise intolerance, HA, dizziness, pallor (pale skin) -Chronic anemia: tachycardia (to compensate for reduced oxygen) which can lead to ventricular hypertrophy -Sudden blood loss: chest pain, syncope, palpitations, tachycardia

Answer 21

MCV (Meancorpuscular volume): size or average volume of RBCs Microcytic MCV: low MCV <80fl -Likely from iron deficiency (use iron studies to further investigate) Normocytic MCV: 80-100 fl -Likely from acute blood loss (reticulocytes, immature RBCs, would be high), malignancy, CKD, bone marrow failure (aplastic anemia), or hemolysis Macrocytic MCV: high MCV >100 fl -Likely from vitamin B12 or folate deficiency (reticulocyte, immature RBCs would be low in these deficiencies w/ bone marrow suppression)

Answer 22

S/Sx: glossitis (inflammed sore tongue), kolionychia (thin, concave, spoon-shaped nails), pica (craving and eating non-foods such as ice or clay) -Labs: decreased Hgb, MCV (<80), reticulocytes, serum iron (iron in blood), ferritin (iron stores), and TSAT (% transferrin bound to iron); increased TIBC (total iron binding capacity: available amount of transferrin available to bind to iron) Heme iron: from meat and seafood; READILY MORE ABSORBED Non-heme iron: from nuts, benas, vegetables, fortified grains; LESS absorable (which is why vegan/vegetarian diet is more suspectable to anemia) Causes: -Low iron intake from diet -Blood loss: hemorrhage, heavy menses, PUD, IBD, drug-induced (NSAIDs, antiplatelets, anticoagulants) -Decreased absorption: high gastric pH (PPIs), GI disorders (celiac disease, IBD), gastric bypass -Increased iron requirements: pregnancy, lactation, infants, adolescents

Answer 23

Gluconate: 12% Sulfate: 20%; dried: 30% Fumarate: 33% Ferric maltol: 100%

Answer 24

Preferred since there is no differences in effiacy between other ROAs, cheap (OTC), and less side effects Dosing: 1 tablet QD or QOD (QOD still shows same Hgb increases with less AVEs) -Ferrous sulfate: 325mg (65mg elemental iron) AVEs: GI in up to 70% (constipation; dark, tarry stools; nausea/stomach upset) BBW: accidental overdose of especially fatal in <6 yo -Child if taken iron and asymptomatic should go to ED or call poison control -Antidote: deferoxamine (Desferal)

Answer 25

1. Take OES (improves absorption), but can be taken CF if GI AVEs occur 2. SR or EC NOT recommended due to poor absorption (advertised for less GI AVEs) 3. If child ingests (risk of overdose), ED or call poison center DDIs: -Avoid H2RAs and PPIs, separate 2 hours before or 4 hours after antacids: decrease absorption due to making environment less acidic -Vitamin C: may increase absorption by increasing acidic environment -Iron is a polyvalent ion that will decrease absorption of other drugs through chelation, separate from: Quinolone and tetracycline ABXs: 2 hours before or 4-8 hours after Bisphosphonates: >/=60 minutes away from PO ibandronate or >/= 30 minutes away from risedronate or alendronate Levothyroxine: separate by 4 hours Integrase strand transfer inhibitors (INSTIs) **For NAPLEX, more likely to know whichs drugs to seperate than exact timing

Answer 26

When to use: typically restricted to pts that need faster rates and potentially with single dose, but has more cost: -CKD on hemodialysis and/or receiving ESAs -Unable to tolerate iron from GI AVEs (ex. H, pylori, celiac disease, IBD) -Severe anemia (also pts who do NOT accept blood transfusions) Types: iron sucrose (Venofor), ferumoxytol (Faraheme), iron dextran complex (Infed) AVEs: N/V/D, hypotension, dizziness, dyspnea, chest pain, muscle aches, peripheral edema, injection site rxns BBW: iron dextran and ferumoxytol can cause serious and sometimes fata anaphylatic rxns (dextran REQUIRES test dose ) -Warning for anaphylaxis/hypersensitivity for others --> sometimes premedicate to help (Medrol, Benadryl) AND SLOW INFUSION TO HELP

Answer 27

Pernicious Anemia: autoimmune condition caused by antibodies binding to intrinsic facot which is needed for vitamin B12 absorption Other causes: low intake, alcohol use disorder, GI disease or surgery, drug-induced (metformin, PPIs, H2RAs) S/Sx: neurologic dysfunction (cognitive impairment and peripheral neuropathy - can be IRREVERSABLE after 3 months of no TX), visual disturbances, psychiatric symptoms -Labs: MCV >100; decreased reticulocytes, serum vitamin B12, and serum folate; elevated homocysteine (metabolized by vitamin B12 and folate) and methylmalonic acid (metabolized by vitamin B12) TX: severe or neurologic symptoms - vitamin B12 injections

Answer 28

Causes: low intake, alcohol use disorder, GI disease or surgery, drug-induced (methotrexate) S/Sx: ulcerations of tongue/oral mucosa, hair and fingernail pigmentation -Labs: MCV >100; decreased reticulocytes, serum vitamin B12, and serum folate; elevated homocysteine (metabolized by vitamin B12 and folate) TX: PO folic acid usually sufficient

Answer 29

Brands/ROA: -Nascobal (nasal spray): spray in ONE nostril Qweek -Physcians EZ Use B-12 (injection): IM or deep SQ QD or Qweek or Qmonth -Others: lozenge, tablet, SL liquid AVEs: injection site rxns, polycthemia vera, pulmonary edema -All side effects besides injection pain are RARE Warnings/CIs: -Warning: parenteral products may contain aluminum which can accumulate and cause CNS/bone toxicity if renal fxn impaired or benzyl alcohol which can cause fatal toxicity and "gasping syndrome" in neonates -CI: allergy to cobalt or vitamin B12 (intradermal test for any suspected sensitivity prior to intranasal or injection recommended)

Answer 30

Brand: FA-8 ROA: PO, injection AVEs: bronchospasm, flushing, rash, pruritus, malaise (ALL RARE) Warnings: parenteral products may contain aluminum which can accumulate and cause CNS/bone toxicity if renal fxn impaired or benzyl alcohol which can cause fatal toxicity and "gasping syndrome" in neonates

Answer 31

Erythropoietin (EPO): hormone produced from kidneys that stimulate bone marrow to produce RBCs Causes of normocytic anemia: CKD, malignancy General TX Recommendations: -Kidney Disease Improving Global Outcomes (KDIGO): all iron therapy in CKD if TSAT IV iron preferred, but PO iron can be used if NOT on HD (if other causes have been corrected and Hgb <10g/dL, ESA) -Erythropoeisis-stimualting agents (ESAs): require iron stores to function, recommended to decrease dose or D/C when Hgb approaches or exceeds >11 g/dL due to RISK OF DEATH AND THROMBOSIS

Answer 32

Drugs: epoetin alfa (Epogen, Procrit, biosimilar: Retacrit), darbepoetin (Aranesp) ROA: IV of SQ (IV recommended in HD) -Epoetin: given three times weekly -Darbopoetin: given weekly in HD MOA: act like natural hormone erythropoietin, increasing RBC production in bone marrow -does NOT stimulate production of erythropoietin Administration considerations: -IV recommended in HD -Store in refrigerator: discard vial after 21 days -do NOT shake (prevent degradation of protein) -Darbopoetin half life is 3x longer than epoetin, allowing weekly administration

Answer 33

AVEs: arthralgia/bone pain (from direct stimulation of bone marrow) Warnings: HTN -Epoetin alfa: contains albumin from human blood (remote risk for transmission of viral diseases) BBW: increased risk of death, MI, stroke, or thrombosis -CKD: further increased risk of death if Hgb >11 g/dL -Cancer: decreased survival - NOT intended to cure (palliative care is an option) -Use lowest effective dose Monitoring: Hgb, Hct, TSAT, serum ferritin, BP

Answer 34

Immune-mediated hemolysis: medication binds to RBC surface and triggers antibody development, identified w/ (+) direct Coombs test -Drugs: PCNs, cephalosporins, isoniazid,, rifampin, sulfonamides, levodopa/methyldopa, quinidine/quinine Hemolysis in glucose-6-phosphate dehydrogenase (G6PD) deficiency: inherited disorder where G6PD is deficient - enzyme that protects RBCs from oxidation -Drugs: primaquine/quinidine/quinine, sulfonamides, dapsone, nitrofurantoin, methylene blue, pegloticase, rasburicase S/Sx of hemolysis: jaundice, dark urine, splenomegaly Actions: -D/C causative drug -Immune-mediated: list causative drug as an allergy -G6PD deficiency: avoid ALL potential causative drugs, note pt is G6PD deficient on chart

Answer 35

SCD: group of inherited (autosomal recessive) RBC disorders where RBCs contain sickle hemoglobin (HgbS) which is misshapen with rigid, concacer shape (shorted life span of 10-20 days, unable to carry oxygen, and can stick together) S/Sx: do NOT usually occur until 2-3 months after birth due to having fetal hemoglobin (HgbF) which is protective -Acute: vasoocclusive crises (VOC: ischemic pain): ACUTE CHEST SYNDROME (leading cause of death in SCD), anemia, infections, priapism, stroke, acute pain criss, cholecystitis -Chronic: avascular necrosis (bone death), leg ulcers, gallstones, pain, pregnancy complications, pulmonary HTN, renal impairment, retinopathy, priapism Population more likely to have SCD: African Americans

Answer 36

Infection risk: healthy spleen removes old and damaged RBCs, stores WBCs and helps immune function, and clears bacteria from body (S. pneumoniae, H. influenzae, N. meningitidis) -Asplenia: RBC sickling causes infarction of spleen, spleen shrinks and becomes fibrotic w/ increased infection risk -Encourage pt to seek help ASAP w/ fever >101.3F Immunizations: goal to prevent sepsis and pnuemonia -Routine Childhood Series: Haemophilus influenzae type B (Hib), pneuomococcal conjugate (PCV13 or Prevnar 13) -Additional vaccines for Asplenia: meningococcal conjugate series + routine boosters, meningooccal serogroup B (Bexsero, Trumenba), pneuomococcal (Prevnar 20 x1 OR PCV15 x1 then PPSV23 x1 >/= 8 weeks later) Antibiotics: oral PCN VK BID until age 5 yo (if spleen removed, may be longer)

Answer 37

Blood transfusions: helps supply HgbA, BUT risk of iron overload (can damage organs)--> maintain serum Hgb at 10 g/dL or less -Chelation therapy can be used to remove exces iron stores (PO options: deferasirox - Exjade, Jadenu; deferiprone - Ferroprox) Only cure for SCD: bone marrow transplantation (can be untolerable and COSTLY) Pain control: IV opioids (PCA - pain-controlled analgesia) -Avoid seeing multiple providers for pain control to avoid overdose Disease-modifiying therapy: PRIMARILY hydroxyurea -Others: L-glutamine, voxelotor, crizanlizumab (alone or in combo w/ hydroxyurea)

Answer 38

Brand: Droxia, Hydrea, Siklos MOA: stimulates production of hemoglobin F (HgbF) ROA: PO TX: sickle cell disease -ALL pts w/ 1 or more moderate-pain crisis or acute chest pain syndrome in one year or in chronic pain -ALL pediatric pts between 9 months and 18 yo regardless of disease severity Clinical response: can take 3-6 months AVEs: increased LFTs, uric acid, BUN, and SCr; N/V/D, alopecia, hyperpigmentation or atrophy of skin and nails. low sperm counts (men)

Answer 39

Warnings: -FETAL TOXICITY: MUST use contraception during TX and for 6 (females) or 12 (males) months after D/C, do NOT breastfeed during TX) -AVOID LIVE VACCINES -Hazardous drug: teratogenic, chemotherapy-like drug (handle gloves when handling, wash hands) -Folic acid supplementation may be needed for macrocytosis -Others: skin ulcers, pulmonary toxicity, pancreatitis risk, hepatotoxicity, peripheral neuropathy BBW: MYLEOSUPPRESSION (decreased WBCs and PLTs), malignancy (leukemia, skin cancer) -Additive risk when used w/ other drugs that cause myloesuppression -Can increase risk of infection Monitoring: HgbF, LFTs, uric acid, renal function, baseline pregnancy test -CBC w/ differential Q2-4 weeks after initiation and titration then Q2-3 months after once stable dose achieved -If ANC <2,000 cells/mm3 or PLTs <80,0000 cells/mm3: HOLD dose until recovery

Answer 40

Brand: Endari MOA: amion acid that is thought to have antioxidant properties ROA: PO TX: sickle cell disease (reduces acute complications: pain crises, hospitalizations, occurance of acute chest pain syndrome) -Approved for 5 yo and older AVEs: constipation, flatulence, HA, nausea, pain (abdominal, extremeties, back, chest, cough) -Safer profile than hydroxyurea Administration: mix each dose (5 grams of powder per packet) with 8 oz of cold or room temperature beverage (water, milk, apple juice) or 4-6 oz of food (applesauce, yogurt) -does NOT need to completely dissolve before administration

Answer 41

Brand: Oxbryta MOA: inhibits hemoglobin S (HgbS) polymerization, which prevents RBC sickling ROA: PO TX: sickle cell disease AVEs: HA, fatigue, abdominal pain, diarrhea, nausea Warnings: hypersensitivity rxns (lab test interference w/ measurement of Hgb subtypes - A, F, and S) Administration: swallow tablets whole (do NOT cut, crush, or chew) ***Manufacturer voluntarily wtihdrew from market saying recent data does NOT show a benefit over risk, causing higher rates of voco-occlusive crisis (severe pain from sickle cell RBCs blocking blood flow and oxygen delivery to tissues)

Answer 42

Brand: Adakveo MOA: MAB that binds and inhibits P-selectin, involved in adhesion of sickled erythrocytes to vessels ROA: injection TX: sickle cell disease - reduces frequency of vaso-occlusions AVEs: nausea, fever, arthralgias Warnings: infusion-related rxns, lab test interference w/ PLT counts