Anemia & Sickle Cell Anemia

Front 1

Anemia

Back 1

Below normal concentration of hemoglobin in the body

Hb < 13g/dL in men
Hb < 12 g/dL in women

Front 2

Anemias are classified

Back 2

1. morphology
2. etiology
3. pathophysiology

Front 3

Morphology - Macrocytic

Back 3

1. Vitamin B12 or Folic Acid Deficiency
2. Cells are larger

Front 4

Morphology - Microcytic

Back 4

1. Iron Deficiency
2. Cells are small, Hb down

Front 5

Morphology - Normocytic

Back 5

1. Recent blood loss
2. Cells are normal, Hb normal

Front 6

Etiology of Anemia

Back 6

1. Deficiency (iron, Vit B12, Folic Acid)
2. Central (impaired bone marrow function)
3. Peripheral (bleeding, hemolysis)

Front 7

Pathophysiology of Anemia

Back 7

1. Excessive blood loss (hemorrhage, trauma, peptic ulcer)
2. Chronic Hemorrhage (vaginal bleeding, peptic ulcer)
3. Excessive RBC destruction
4. Inadequate production of RBCs

Front 8

Life span of Red Blood cells

Back 8

120 days

Front 9

Entire process of formation of Red blood cells

Back 9

1 week

Front 10

Erythropoeitin

Back 10

1. produced mainly in the kidneys
2. initiates and stimulates the production of RBCs

MOA: prevents apoptosis of erythroid precursor cells to allow for proliferation and susequent maturation

3. decreased tissue oxygen signals to kidney to increase production

Front 11

Clinical presentation of Acute Onset Anemias

Back 11

1. Tachycardia
2. Lightheadedness
3. Palpitations
4. Hypotension
5. Angina
6. Dyspnea

Front 12

Clinical Presentation of Chronic Anemia

Back 12

*May present as asymptomatic at first*

1. Weakness/lethargy/fatigue
2. headache
3. vertigo
4. faintness
5. edema
6. SOB
7. Sensitivity to cold
8. Hunger for ice, starch, clay

Front 13

Goal of Anemia Therapy

Back 13

1. improve RBC oxygen carrying capacity
2. Alleviate symptoms
3. Prevent anemia complications

Front 14

Sources of Dietary Iron

Back 14

1. Seafood (oysters, tuna, salmon)
2. Meats (liver, lean red meat)
3. Grains (fortified cereals, whole grains)
4. Veggies (spinach, broccoli, asparagus)

Front 15

Iron is best absorbed in what form

Back 15

Ferrous form

Front 16

Findings in Iron Deficiency Anemia

Back 16

1. Decreased Serum Iron
2. Decreased Serum Ferritin
3. Decreased TSAT%
4. Increased TIBC
5. Decreased Hb & Hct
6. Peripheral smear: microcytosis, hypochromia

Front 17

Signs and Symptoms of Iron Deficient Anemia (Microcytic)

Back 17

1. Hb <9g/dL
2. Headache, Cardiovascular, SOB (general)
3. Smooth, sore tongue
4. Pica - compulsive eating of nonfood items
5. spoon shape fingernails

Front 18

Treatment of Iron Deficiency Anemia

Back 18

1. Treat Underlying cause
2. Replenish iron stores (200mg of elemental iron daily)

Front 19

Recommended dose of Iron for Anemia

Back 19

200mg elemental iron daily taken in 2-3 divided doses on an empty stomach

Front 20

Ferrous Sulfate for Anemia

Back 20

*Formulation of choice for iron deficiency anemia
1. Effective and Low Cost
2. 60-65mg of elemental iron per 325mg tablet
3. take 1 tablet TID
4. Should not be administered 1 hour before or 2 hours after a meal

Front 21

When should response to iron therapy be seen

Back 21

1. within 7-10 days
2. Hb should rise 2-4g/dL every 3 weeks

Front 22

ADRs of Iron supplement

Back 22

*primarily GI*
1. Dark discoloration of feces
2. Constipation
3. Ab cramping

Front 23

Drugs that decrease Iron Absorption

Back 23

1. Al-, Mg, Ca containing antacids
2. H2 antagonists
3. PPIs
4. Caffiene

Front 24

Drus that are affected by iron

Back 24

1. Decrease levothyroxine
2. Decrease fluoroquinolones
3. Decrease Methyldopa

Front 25

Counseling points for Iron

Back 25

1. Keep out of reach of children
2. take on an empty stomach
3. take 2 hours before or after antacids, PPIs, H2RAs

Front 26

How long should treatment be given for Anemia

Back 26

3-6 months after the anemia is resolved

Front 27

Which parenteral Iron needs to have a test dose

Back 27

Iron Dextran (0.5mL = 25mg)

Front 28

What is the dose for the parenteral Irons (Sodium Ferric Gluconate, Iron Sucrose, Ferumoxytol)

Back 28

1 gram given over several days

Front 29

Must rule out Vitamin B12 deficiency before treating what

Back 29

Folic Acid Deficiency must be treated first

Front 30

Signs and Symptoms of Vitamin B12 Deficient Anemia

Back 30

1. Physical (Glossitis, Anorexia/weight loss, Beefy red tongue)
2. Neurological (peripheral neuropathy)
3. Psychiatric (irritability, memory impairment, depression)

Front 31

Laboratory Findins for Vitamin B12 Defiency Anemia (Macrocytic)

Back 31

1. Decreased Hct
2. Decreased reticulocyte count
3. Decreased Vit B12 level
4. Increased MCV & MCH
SMEAR: macrocytosis

Front 32

Treatment for Vitamin B12 Deficient Anemia

Back 32

1. Cyanocobalamin Oral
2. Cyanocobalamin Parenteral (preferred for Neurological symptoms)
3. Cyanocobalamin Intranasal Spray

Front 33

ADRs of Cyanocobalamin

Back 33

*RARE - well tolerated*
1. Hypokalemia
2. Injection site pain
3. Pruritis
4. Rash

Front 34

Causes of Folic Acid Deficiency Anemia

Back 34

1. Inadequate Intake
2. Malabsorptive Disease
3. Medication Use
4. Inadequate utilization
5. Hyperutilization

Front 35

Signs and Symptoms of Folic Acid Deficiency Anemia

Back 35

SAME AS Vitamin B12 Deficiency Anemia EXCEPT no Neurological symptoms

Front 36

Laboratory Findings for Folic Acid Deficiency Anemia

Back 36

1. Decreased Hct
2. Decreased Reticulocyte count
3. Decreased Serum Folic Acid
4. Increased MCV & MCH
SMEAR: macrocytosis

Front 37

Treatment for Folic Acid Deficiency Anemia

Back 37

Supplementation (OTC or RX)

Front 38

ADRs of Folic Acid Supplementation

Back 38

1. Bitter/Bad taste
2. GI upset (nausea, flatulence, anorexia)

Front 39

Ingestion of what interferes with the absorption of folate

Back 39

Alcohol

Front 40

Factors responsible for Anemia of CKD

Back 40

1. Decreased erythropoietin production (MAJOR)
2. shorter life span of RBCs
3. blood loss during dialysis

Front 41

New Recommendation with erythropoiesis stimulating agents (ESAs)

Back 41

*NO TARGET Hb RANGE*
increased mortality, MI, Stroke, thromboembolism when using ESAs to target Hb > 11g/dL

Front 42

2 agents available as erythropoiesis stimulating agents (ESAs)

Back 42

1. Epoetin-a (Epogen, Procrit)
2. Darbepoetin a (Aranesp)

Front 43

Starting dose of Epoetin a

Back 43

50-100 units/kg/dose IV or SC 2-3 times/week

Front 44

Starting dose of Darbepoetin-a

Back 44

Dialysis: 0.45mcg/kg IV once weekly
No Dialysis: 0.45mcg/kg IV or SC once every 4 weeks

Front 45

ADRs of Epoetin-a and Darbepoetin-a

Back 45

1. Hypertension (most common) - do not start med if BP >180/100
2. seizures
3. edema
4. allergic reactions

Front 46

Evaluation of Therapeutic Outcomes with ESAs

Back 46

1. Hb levels monitored every week, then every 2-4 weeks when stable
2. upward dose adjustments made every 4 weeks
3. patients dont respond after 8 weeks, do not continue

Front 47

Change in dosing of Epoetin-a and Darbepoetin-a

Back 47

*made in 25% intervals

1. change in Hb < 1g/dL in 4 weeks - increase dose by 25%
2. change in Hb >1g/dL in 2 weeks - decrease dose by 25%

Front 48

Common causes of inadequate response to ESAs

Back 48

1. Iron deficiency (Most Common)
2. infection/inflammation
3. chronic blood loss
4. folate or B12 deficiency
5. malnutrition

Front 49

ESAs will not be effective if

Back 49

Iron stores are not adequate

Front 50

Most patients with CKD require

Back 50

an Iron Supplement

Front 51

Oral Iron therapy is not effective for patients...

Back 51

for CKD patients on Dialysis (USE IV THERAPY)

Front 52

oral Iron supplementation indicated for patients who do not have IV access

Back 52

1. Stages 3 and 4 CKD
2. Receiving peritoneal dialysis
3. Switch to IV if unable to manage

Front 53

Shape of Sickled Cells

Back 53

1. Oxygen is unloaded into tissues - HbS solubility decreases
2. deoxygenated Hgb becomes semi-solid gel
3. lose biconcave shape, become elongated and stiff with curved ends

Front 54

Pathophys of Sickle Cell Anemia

Back 54

1. Sickled Cells increase blood viscosity and cause sludging = tissue hypoxia
2. cell membrane damage, loss of membrane flexibility = vasoocclusion
3. Damage to cell membranes - dehydrated, dense sickle cells
4. loss of potassium/water
RESULT: impaired circulation, RBC destruction, stasis of blood flow

Front 55

Clinical presentation of Sickle Cell Anemia

Back 55

1. Painful Vasoocclusive Crisis (Hallmark)
2. Dactylitis (sausage toe/finger)
3. Infaction of spleen, liver, bone marrow
4. Gallstones
5. Priapism
6. Weakness/fatigue

Front 56

Signs of Sickle Cell Anemia

Back 56

1. Chronic Hemolytic Anemia
2. Enlargement of Heart or spleen

Front 57

Non-Pharmacological Therapy of Sickle Cell Anemia

Back 57

1. HYDRATION - decrease blood viscosity
2. Pain control

Front 58

Immunization Schedule for Sickle Cell Anemia

Back 58

1. Annual Flu Vaccine
2. Pneumococcal Vaccine (6wks-4months = 4 shots; >2 years = 1 shot booster)
3. Meningococcal ( >2 years)

Front 59

MOA of Hydroxurea for Sickle Cell Anemia

Back 59

1. stimulates the production of HbF levels
2. decreases hemolysis
3. Vasodilation

Front 60

Hydroxyurea (Droxia, Hydrea) is indicated for

Back 60

patients with 3+ vasoocclusive pain crisis and/or ACS per year or severe symptomatic anemia

Front 61

Dosing for Hydroxyurea (Droxia, Hydrea) in Sickle Cell Anemia

Back 61

10-15 mg/kg/day (up to 35mg/kg/day) and adjust by 5 mg/kg after 8-12 hours

Front 62

Most common ADRs of Hydroxyurea

Back 62

1. MYELOSUPPRESSION
2. nausea/vomiting
3. constipation
4. confusion
5. peripheral neuropathy

Front 63

MOnitoring of Hydroxyurea

Back 63

Baseline: CBC, MCV, Hgb (goal increase 15-20%), SCr

CBC - every 2 weeks then every 4 weeks once stable
HbF - every 3 months

Front 64

What do you do if there is a toxicity with Hydroxyurea

Back 64

stop use for at least one week then restart at 2.5mg/kg/day less than previous dose

Front 65

Deferasirox

Back 65

USE: chelating agent for iron overdose
INDICATION: after 1 year of chronic transfusion or serum ferritin > 1500-2000
ADRs: SCr increase, fever, headache

Front 66

Deferoxamine

Back 66

USE: IV chelating agent for hemosiderosis
INDICATION: after 1 year of chronic transfusion or serum ferritin > 1500-2000
*supplement with Ascorbic acid
ADRs: associated growth failure

Front 67

Deferiprone

Back 67

USE: oral chelating agent (LAST LINE)
INDICATION: treatment of transfusion iron overload due to thalessemia syndromes
BLACK BOX: agranulocytosis

Front 68

Acute Complications with 'iron chelators' in sickle cell anemia

Back 68

1. Fever and infection (empirical antibiotics)
2. Neurologic
3. Acute Chest Syndrome
4. Priapism

Front 69

Sickle Cell Crisis problems

Back 69

1. Vasoocclusion Pain
2. Aplastic Crisis
3. Acute Splenic Sequestration Crisis

Front 70

Therapeutic regiment for Mild-to-Moderate Pain with Sickle Cell

Back 70

non-opioid with/without weak opioid

Front 71

Therapeutic Regimen for Moderate-to-Severe pain with Sickle Cell

Back 71

weak opioid OR low dose of strong opioid with/without nonopioid

Front 72

Therapeutic regimen for Severe pain with Sickle Cell

Back 72

strong opioid with nonopioid

Front 73

Weak Opioids used in Sickle Cell

Back 73

1. APAP w/ Codeine
2. Hydrocodone w/ APAP

Front 74

Strong Opioids used in Sickle Cell

Back 74

1. Morphine
2. Hydromorphone
3. Fentanyl
4. Methadone

Front 75

Non-Opioids used in Sickle Cell

Back 75

1. Naproxen
2. Ibuprofen
3. Ketorlac (IV)