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SCD Flashcards

1
Q

Which chain of hemoglobin is defective in SCD?

A

Beta chain

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2
Q

What are the possible defects in the beta chain in SCD?

A

HbSS
HbSC
SB+-thalassemia (some beta-globulin produced)
SB(null)-thalassemia (no beta-globulin produced)

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3
Q

What are the clinical features of HbSS?

A 
Pain crises
Microvascular disruption of organs
Gallstone
Priapism
Leg ulcers
Anemia
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4
Q

What are the clinical features of HbSC?

A

Painless hematuria
Pain crises less common and occur later in life
Mild anemia

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5
Q

What are SB+-thalassemia clinical features?

A

Rare crises

Less severe than SCD (production of HbA)

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6
Q

What are SB(null)-thalassemia clinical features?

A

Similar severity to HbSS

Anemia

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7
Q

What are the clinical features for sickle cell trait?

A

Generally asx

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8
Q

What element is a sickled RBC lacking?

A

Oxygen

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9
Q

What are the outcomes of sickled cells?

A

Increased viscosity
Adhesion
Occlusion

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10
Q

When do s/sx appear after birth?

A

4-6 months

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11
Q

What are lab findings for SCD?

A

Decreased hgb
Increased retic count
Sickled cells on peripheral blood smear

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12
Q

What is the clinical presentation for SCD?

A 
Acute pain
Dactylitis
Pallor
Weakness
Splenomegaly
Hepatomegaly
Abdominal pain
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13
Q

What infections are we worried about in SCD?

A 
SHiNS
Step pneumoniae
H influenzae type b
Neisseria meningitides
Salmonella
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14
Q

What vaccinations do patients with SCD receive?

A

Pneumococcal
Hib
Meningococcal
Flu

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15
Q

If a patient has HbSS, SB(null) thalassemia, or HbSC, and is 2 months to 3 years old, what penicillin dose do we prophylax with?

A

Oral penicillin VK 125mg PO BID

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16
Q

If a patient has HbSS, SB(null) thalassemia, or HbSC, and 3 years old to 5 years old, what penicillin dose do we prophylax with?

A

Penicillin VK 250 mg PO BID

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17
Q

Which vitamins (and their doses) do we regularly replace?

A

Folic acid 1 mg PO/day

B12 up to 1000mcg PO/day

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18
Q

What are the types of chronic blood transfusion therapy?

A

Simple

Exchange

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19
Q

What are indications for blood transfusions?

A

May reduce stroke risk in children at high risk
Symptomatic severe acute chest syndrome
Acute splenic sequestration and severe anemia

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20
Q

What are risks of chronic blood transfusions?

A

Alloimmunization
Infection transmission
Iron overload

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21
Q

How can we reduce risks in chronic blood transfusions?

A

Blood cross-matching
Immunizations and screening
Iron chelation therapy

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22
Q

When is iron chelation therapy used after chronic transfusions?

A

Consider after a year of transfusions
OR
When ferritin > 1500 mcg/L

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23
Q

What are the types of iron chelation therapies?

A

Deferoxamine
Deferasirox
Deferiprone

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24
Q

Which iron chelation therapies are not oral?

A

Deferoxamine (IV/SQ)

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25
What is the common dose of deferoxamine?
20-50 mg/kg/d over 8-24 hours
26
What is the common dose for deferasirox?
Exjade: 20-40 mg/kg/d Jadenu: 14-28 mg/kg/d
27
What is the common dose for deferiprone?
75-99 mg/kg/d
28
What is the frequency of deferoxamine dosing?
5-7 days/week
29
What is the frequency of deferasirox dosing?
Daily
30
What is the frequency of deferiprone dosing?
TID
31
What are the AEs of deferoxamine?
``` Injection site reactions Arthralgias Ocular and ototoxicities Growth retardation Renal disturbances ```
32
What are the AEs of deferasirox?
Renal and hepatic failure Agranulocytosis Gastrointestinal hemorrhage
33
What are the AEs of deferiprone?
QT prolongation GI upset Elevated LFT Agranulocytosis
34
What is the rationale for use of hydroxyurea of SCD?
Increased HbF levels Increased HbF-containing reticulocytes and intracellular HbF Ribonucleotide reductase inhibitor Antioxidant properties
35
What are the baseline labs?
CBC, reticulocyte count, WBC differential HbF measurement Renal and liver function tests Pregnancy test
36
What is the dosing for hydroxyurea?
15 mg/kg PO QD | Max = 35 mg/kg
37
How can hydroxyurea be increased/
5 mg/kg/day every 12 weeks
38
What is the dosing of the liquid hydroxyurea suspensions?
100mg/ml
39
How do we monitor for hydroxyurea?
Efficacy: less pain episodes and hospitalizations, improved well being HbF: Every 3 months x 2, then every 6 months CBC w/diff Periodic renal and liver function tests
40
What are the toxicity cut offs for hydroxyurea?
``` ANV < 2,000 Plt < 80,000 Hgb < 5 or 20% reduction SCr > 50% above baseline ALT 100% increase ```
41
If there is a toxicity with hydroxyurea, what is the course of action?
Stop for 1-2 weeks and resume (if normal counts) at 5 mg/kg/d less than previous dose If toxicity does not recur after 12 weeks on lower dose, the dose may be increased
42
What is the counseling for hydroxyurea?
Bleeding risk Infections Status regarding pregnancy Wash hands before and after handling
43
What oral powder was approved to reduce severe complications associated with SCD in > age 5?
L-glutamine
44
What is the MOA of Crizanlizumab?
Monoclonal antibody binds to P-selectin and blocks interaction with PSGL-1 (may prevent vasoocclusion by inhibiting adhesion of erythrocytes to vessel walls
45
What SCD affect does crizanlizumab work on?
Significantly lower rate of pain crises
46
What are the strategies to modify disease outcomes?
Hydroxyurea L-glutamine Crizanlizumab HSCT
47
What causes vasoocclusive crisis or acute pain crisis?
Bone or muscle infarction d/t vasoconstriction
48
What is the most common reason for hospitalization?
Vasoocclusive crisis or acute pain crisis
49
What is the treatment for vasoocclusive crisis or acute pain crisis?
Hydration and pain management
50
What are acute complications of SCD?
Vasoocclusive crises/acute pain crisis Fever and acute infection Acute chest syndrome
51
Compared to normal, how must we increase hydration in an acute pain crisis or vanoocclusive crisis?
1.5 times maintenance
52
What is the regular hydration maintenance requirement for adults?
30-35 ml/kg
53
What is the regular hydration maintenance requirement for children?
For the first 10kg: 100ml/kg per day For the 2nd 10kg: 1000ml + 50Ml/kg per day For more than 20kg: 1500mL + 20 ml/kg per day for each kg over 20 kg
54
Why do we monitor fluid status during hydration?
Volume overload can lead to acute chest syndrome
55
What are chronic complications of SCD?
``` Retinopathy Anemia, leukocytosis OSA Pulmonary hypertension Indirect hyperbilirubinemia Cardiomegaly Chronic renal failure Functional asplenia Avascular necrosis Delayed puberty Skin ulcers ```
56
What are acute complications of SCD?
``` Stroke, meningitis Post-hyperma glaucoma, retinal infarction Acute chest syndrome Sickle hepatopathy Splenic sequestration, splenic infarction Cholelithiasis Priapism Bone marrow infarction, osteomyelitis ```
57
Does Fetal Hgb (HgF) sickle?
No
58
What are non pharm approaches for pain management in SCD?
Hydration Heating pads Ect
59
What are pharm options for pain management of SCD?
Opioids 1st line for mod-sev pain Morphine sulfate or hydromorphone most common PCA with basal and on demand options NSAIDs in combo with opioids - esp for bone pain Bowel regimen Antihistamines for itching Antiemetics for n/v
60
Which medications are used for bowel regimens?
Senna-colace | Polyethylene glycol
61
Why are fever and acute infection increased in SCD?
Loss of splenic function
62
What are the manifestations of fever and acute infection in SCD?
Pneumonia Osteomyelitis Meningitis Bacteremia
63
What are the likely organisms that occur in SCD?
SHiNS Mycoplasma pneumonia Viral influenza
64
How do we diagnose infections and fever in SCD?
``` Fever WBC Cultures Imaging Lumbar punctures ```
65
What leads to acute chest syndromes?
Vasoocclusive crisis of pulmonary vasculature
66
What are the etiologies of acute chest syndrome?
Infection Pulmonary vascular occlusion Pulmonary edema
67
What is the presentation of acute chest syndrome?
``` Acute fever Respiratory symptoms New infiltrates on chest x-ray Hypoxia Pain Wheezing ```
68
What other condition is radiologically indistinguishable from acute chest syndrome?
Pneumonia
69
What are the antimicrobials used in acute chest syndromes?
``` Ceftriaxone Or Cefotaxime + Azith Or Moxifloxacin ```
70
What is the management for acute chest syndrome?
``` Bronchodilators Hydration Pain management O2 etc ```

Heme/Onc (20 decks)

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