Hem Exam 2 Flashcards
(147 cards)
1
Q
How to manage bleeding
A
identify and manage bleeding source
give blood is Hgb <7 g/dL
reverse offending agent or hold dose
2
Q
What is FFP (fresh frozen plasma)
A
reverses warfarin
frozen within 8 hours
will raise factor levels by 20%
can cause TACO (transfusion associated circulatory overload) 10-20 ml/kg/dose
lower INR 1.6
3
Q
What is recombinant factor VIIa (novoseven, already activated factor)
A
reverse: LMWH, UFH, warfarin
for bleeding associated with hemophilia
(can use for intracranial hemorrhage and refractory bleeding after cardiac surgery)
Boxed warning: serious arterial and venous thrombotic events
activated extrinsic pathway
4
Q
What is Prothrombin complex concentrate (PCC)
A
reverse: warfarin, off-label DOAC, DTIs
feiba: 2, 9, 10, 7
kcentra: 2, 9, 10, 7, C, S (IV with vit K)
5
Q
Boxed warning for PCC and advantages
A
fatal and nonfatal arterial and venous thromboembolic events
lower, volume, low risk of transmission of infection, fast reversal
FACTOR 9
6
Q
What is protamine
A
reveral: heparin, enoxaparin (hypersensitivity in fish)
-1 mg protamine neutralizes 100 units heparin (max dose 50 mg)
will not completely neutralize enoxaparin
BBW: hypotension, CV collapse, pulmonary vasoconstriction
7
Q
Protamine doses (IV UFH)
A
stop drip, 1 mg per 100 units administered in last 3 hrs
max 50 mg
may repeat 0.5 mg for every 100 units in 15 minutes if bleeding or elevated aPTT/aXa
reduce dose by 50% if time (>2 hours) has elapsed since dose given
8
Q
Protamine doses (SQ UFH)
A
only if pt has significant bleeding
1 mg per 100 units heparin, max 50
over 10 min and infuse the rest over 8-16 hours
9
Q
What is Vit K
A
reversal: warfarin
IV or PO
give over 30 min, use PO mainly
0.5-10 mg
takes long time to work
10
Q
What is andexanet alpha (andexXa)
A
reverse: apixaban, rivaroxaban, edoxaban
low dose: 400 mg IV then 4 mg/min for up to 2 hr
-apixaban 5, 10 or any dose given more than 8 hr ago
high dose: 800 mg IV then 8 mg/min for up to 2 hr
-if dose was given within last 8 hours or time unknown
11
Q
What is idarucizumab (praxbind)
A
reverse: dabigatran
w/in minutes
5 g (2.5x2 vials), give first dose over 5-10 minutes, second dose given immediately after
no repeat doses
12
Q
LMWH/fondaparinux TO IV heparin
A
start IV heparin without a bolus 1-2 hours before next dose is due
13
Q
Warfarin TO heparin
A
IV heparin without bolus when INR is around 2
14
Q
DOAC TO heparin
A
start IV without bolus when DOAC next dose is due
15
Q
If on DOAC and need heparin when not urgent vs urgent
A
not: baseline aXa if high check q6h and start drip one level <0.7 units/mL
urgent: baseline aXa and aPTT, if aXa high, then titrate heparin based on aPTT, do not delay drip
16
Q
LMWH/fondaparinux FROM IV heparin
A
stop heparin and start within 1 hour
-if aPTT/aXa subtherapeutic give at same time drip stopped
-if aPTT/aXa supra delay for longer (3-4 hours)
17
Q
Warfarin FROM heparin
A
start warfarin and continue IV heparin until INR os therapeutic 1-2 times
18
Q
DOAC FROM heparin
A
start DOAC when IV heparin is stopped
-if aPTT/aXa is supra consider delaying start time
19
Q
DOAC to LMWH
A
begin LMWH at time next DOAC dose due
20
Q
warfarin to LMWH
A
being LMWH once INR is around 2
21
Q
DOAC from LMWH
A
start DOAC 2 hours prior to next dose of LMWH
22
Q
warfarin from LMWH
A
start warfarin and continue LMWH until INR therapeutic 1-2 times
23
Q
Warfarin to DOAC (target INR)
A
Rivaroxaban: <3
Edoxaban: <2.5
Apixaban: <2
Dabigatran: <2
24
Q
Apixaban and RIvaroxaban to Warfarin
A
stop DOAC then start warfarin on same day and bridge until INR is therapeutic 1-2 times
25
Edoxaban to warfarin
60 mg qd, reduce to 30 mg and begin warfarin at same time
30 mg qd, reduce to 15 mg and begin warfarin at same time
-disc edoxaban once INR >2 1-2 times (get INR right before edoxaban dose
26
Dabigatran to warfarin
stop DOAC then start warfarin on same day, bridge until INE therapeutic 1-2 times
CrCl >50 ml/min: start warfarin 3 days before stopping DOAC
CrCl 30-50: start warfarin 2 days before stopping
CrCl: 15-30: start warfarin 1 say before stopping
27
Argatroban to Warfarin
begin warfarin and stop argatroban once INR >4 (and 5 days minimum overlap if indicated)
-repeat INR in 4-6 hours, if INR is still therapeutic then no argatroban
-INR below goal, resume argatroban drip
Can hold for 2-3 hours and check INR
28
Bival to Warfarin
begin warfarin and stop bival once INR >2 (and 5 days min overlap if indicated)
repeat INR in 2 hours, if INR therapeutic no drip
if INR below goal, resume drip
29
DOAC to DOAC
give are next scheduled dose
30
Low thrombotic risk
C2V <4, no stoke hx
VTE >12 months
MHV: AV bioprosthetic
31
Moderate thrombotic risk
C2V: 5-6 or stroke >3 months ago
VTE past 3-12 months
active cancer
MHV: AV bioprostheitc, one or more risk factors
32
High thrombotic risk
C2V >7, stroke <3 months
VTE <3 months
MHV: any MV prosthesis
33
What does the CHADS2-VASC score indicate
stroke rate %
34
What is the HAS_BLED score for
a fib
major bleeding risk
35
Minimal bleed risk assessment
minor dental
cataract/glaucoma procedure
diagnostic GI procedure
superficial incisions
36
Low bleed risk assessment
colonoscopy
pacemaker implantation
ICD placement
AV nodal ablations
percutaneous coronary intervention
37
High bleed risk assessment
surgery needed neuraxial anesthesia
major intracainal surgery, thoracic, CABG, arthroplasty, bowel resection
38
Dabigatran (CrCl >50 ml/min) and rivaroxaban, apixaban, edoxaban high vs low bleed risk interruption
high: 2 days before until 1 day after
low: 1 day before and day of procedure
39
Dabigatran (CrCl <50 ml/min) high vs low bleed risk interruption
high: 4 days before until 1 day after
low: 3 days before until day of procedure
40
Low thrombotic risk no pt bleed factors: low, high, uncertain procedure bleed risk
minimal/low: do not interrupt
inter/high: likely interrupt/do not bridge
uncertain: likely interrupt/do not bridge
41
Moderate thrombotic risk no pt bleed factors: low, high, uncertain procedure bleed risk
minimal/low: do not interrupt
inter/high: likely interrupt, likely dont bridge (unless recent stroke, TIA, SE)
uncertain: likely interrupt, likely dont bridge (unless recent stroke, TIA, SE)
42
high thrombotic risk no pt bleed factors: low, high, uncertain procedure bleed risk
minimal/low: do not interrupt
inter/high: interrupt and bridge
uncertain: interrupt and bridge
43
low thrombotic risk pt bleed risk factors, all bleed risk factors
likely interrupt/do not bridge
44
moderate thrombotic risk pt bleed risk factors, all bleed risk factors
likely interrupt/do not bridge
45
high thrombotic risk pt bleed risk factors, all bleed risk factors
likely interrupt, likely bridge (unless major bleed or ICH <3 months)
46
Difference between HIT 1 and HIT 2
HIT 1: non-immune mediated, day 1, mild rxn
HIT 2: immune-related, heparin induced (90d), delayed onset 5-14d, life-threatening
47
Risk factors for HIT
post-surgery, trauma
CV > ortho surgery
UFH > LMWH > fondaparinux
duration
female > male
bovine > porcine UFH
48
Pathophysiology of HIT
heparin + PF4
heparin-PF4 complex
IgG antibody production
IgG antibody + heparin/PF4 immunocomplex
binding of complex to platelets
platelet activation and aggregation
49
Mechanism of thrombosis vs thrombocytopenia
thrombosis: arterial or venous, activate platelets and injury endothelial cells
thrombocytopenia: removal of IgG coated platelets by macrophages, consumption of platelets at sites of thrombosis
50
How to calculate 4T:
Thrombocytopenia: 2 (decrease PLT 50%), 1 (30-50)
Timing: 2 (5-10d, or 1 d with heparin), 1 (fall not clear 5-10), 0 (PLT fall <4 d without exposure)
Thrombosis: 2 (new), 1 (progressive)
oTher causes: 2 (none), 1 (possible), 0 (definite)
51
Immunoassays: PF4 EIA serologic test
high sensitivity but low specificity
optical density scoring
test for HIT
52
Functional Assay: serotonin release assay (SRA)
gold standard for HIT
detects platelet-activating properties
53
What is the chronic phase of HIT
subacute HIT A: period of PLT recovery, functional assay and immunoassay positive
subacute HIT B: functional assay becomes negative, immunoassay remains positive
54
What to do if HIT suspected then 4T score is high vs low
high: disc heparin -> immunoassay -> negative HIT unlikely, positive treat tx or ppx -> if functional assay positive start HIT management
low: HIT unlikely, no lab testing, continue heparin if needed
55
Acute Phase HIT treatment
Argatroban (critically ill, avoid in hepatic dysfunction)
Bival (critically ill, avoid in renal)
DOAC (stable, avoid in complicated, threatening emboli)
Warfarin (NOOOOO)
56
Chronic Phase HIT treatment
DOAC over warfarin
3-6 months care if thrombus
cont tx until PLT recovery
avoid tx in >3 months isolated HIT
Warfarin is PLT >150, Edoxaban/dabigatran with 5 d overlap
57
Monitoring for HIT
renal/hepatic
CBC
PT/INR (warfarin)
aPTT (agatroban/bival)
S/S of thrombosis
S/S of bleeding
58
Goals of HIT therapy
halt platelet activation as rapidly as possible
provide therapeutic-dose anticoag with non-heparin
prevent thrombosis
prevent mortality
avoid heparin products in future
59
hemoglobin criteria for men and women
men: <13
women: <12
anemia is a disorder resulting in reduced oxygen-carrying capacity of the blood
60
Stimulation of erthropoiesis
feedback loop:
decrease tissue oxygen concentration
hormone EPO gets produced in kidneys
EPO gets released into plasma
stimulate production of RBC in bone marrow
stimulated maturation of RBCs
(RBCs maruration is 1 wk)
61
What does transferrin do
delivers iron to bone marrow to be incorporated into the Hb
extra iron stored as ferritin
62
Red blood cell destruction
spleen breaks down RBC
average life span 120 days
63
Clinical presentation symptoms of anemia
fatigue, headache, yellow skin, irregular heartbeat, chest pain, cold hands, dizziness, leg cramps, insomnia
(due to lack of oxygen to tissues)
64
Clinical presentation signs of anemia
pallor (iron deficiency)
abnormal gait (B12 deficiency)
Decreased mental acuity
tachycardia
65
Common causes of anemia
deficiency of iron, B12, folic acid
cancer
chronic diseases: kidney, HF, liver, inflammation
blood loss: GI bleed, surgery, injury, menstruation
66
CBC components: hemoglobin, hematocrit, RBC
hemoglobin: amount of hemoglobin per volume of whole blood
hematocrit: volume of RBCs in a unit volume of whole blood (%)
RBC: total count of RBCs per unit of blood
67
mean cell volume, meant cell hemoglobin, mean cell hemoglobin concentration
MCV: average volume of RBCs
MCH: average amount of Hb in a RBC
MCHC: concentration of Hb per volume of cells
68
Microcytic, normocytic, macrocytic
Microcytic: MCV < 80 (iron deficiency, chronic disease)
Normocytic: 80-100 (acute blood loss, bone marrow failure
Macrocytic: >100 (alcoholism, liver, hypothyroidism, folic acid deficiency, Vit B12 deficiency
69
Total reticulocyte count, red blood cell distribution width (RDW)
Total reticulocyte count: measures of new RBC production (immature cells), low = impaired production, high = acute blood loss
Red blood cell distribution width (RDW): distribution of RBC size, higher = more variable sizes, harmful for anemias
70
Iron studies: serum iron, total iron binding capacity, transferrin saturation, serum ferritin
serum iron: concentration of iron bound to transferrin (diurnal)
TIBC: indirect measurement of iron-binding capacity of serum transferrin (high=bad)
TSAT: ratio of serum iron to TIBC (low=bad)
Serum Ferritin: total iron storage (low=iron def)
-general population ferritin <30 (greater than or equal to)
71
Folic acid, B12, homocysteine, methylmalonic acid (MMA)
folic acid: <4 decreased serum indicates deficiency
B12: <200 is deficiency
homocysteine: increased levels could mean either
MMA: increased levels mean B12
72
Low MCV (<80) meaning if serum ferritin is normal/high or low
normal/high: low TIBC = anemia of chronic disease, high = further work up
low (<30): iron deficiency anemia
73
Normal MCV (80-100) meaning for low or high reticulocyte count
low: check WBC/platelets -> low = bone marrow failure, high=infection
74
High MCV (>100) meaning if B12 level is normal and folate is decreased
normal B12, normal folate: hepatic, hypothyroidism, drug anemia, reticulocytosis
decreased B12, normal folate: Vit B12 deficiency
75
What regulates intestinal iron absorption
hepcidin (normal iron content: 3-4g)
76
Increased risk for anemia
children <2
adolescents
pregnant females (30 mg/d)
>65 yo
77
Specific symptoms with IDA
glossal pain
smooth tongue
pica (compulsive eating of nonfood items)
pagophagia (compulsive ice eating)
78
IDA lab finding
low serum iron
low ferritin levels (<30)
high TIBC**
Hg, Hct, RBC normal at first then drop
79
Pharm Tx for IDA
ferrous gluconate: 38 mg elemental iron
ferrous sulfate: 65 mg (syrup, elixir)
ferrous fumarate: 106 mg
PIC (come in liquid)
80
Adverse effects of oral iron supplements
dark colored feces
ab pain
indigestion
constipation
n/v
81
Monitoring parameters of oral iron supplements
CBC (Hg/Hct)
RBC indices
Iron
(peak 4 weeks, repeat lab 1-3 months, cont supply for 3-6 months after labs normalize
82
Clinical pearls of iron supplements
start low and slow
food decrease absorption (take with acidic drinks)
1 hr before or 2 hours after food
fatal to children/pets
add stool softener to prevent constipation
83
What drugs decrease Iron supplements
antacids
tetracyclines
H2RA
PPI
cholestyramine
84
What drugs does iron supplements decrease their absorption
levodopa
methyldopa
levothyroxine
penicillin
fluoroquinolones
tetracyclines
mycophenolate
85
Treatment failure for oral iron supplements
poor patient adherence
poor absorption
incorrect diagnosis
continued bleeding
concurrent inflammatory process limiting therapeutic response
86
When to use IV iron over oral
not tolerating orals
malabsorption
non-adherence
significant blood loss
87
Parental Iron Products
None: Iron dextran
CKD: Ferric Carboxymaltos
CKD and HD:
Sodium Ferric gluconate
Iron Sucrose
ferumoxytol
88
Adverse effects of IV iron products
infection reactions
arthralgia/myalgias
hypotension
tachycardia
chest pain
peripheral edema
pruritis
89
Monitoring parameters for IV iron products
REACTIONS (during/post infection)
CBC (Hb/Hct)
RBC indices
Iron studies
1-3 months
90
Etiology of B12 deficiency
inadequate intake
Malabsorption
inadequate utilization (low gastric acid production, metformin, H2RA, PPI >2 years of use)
91
B12 deficiency symptoms
bilateral paraesthesia
deficits in proprioception and vibration
ataxia
dementia-like symptoms
psychosis
vision loss
92
B12 deficiency lab findings
Macrocytic anemia >100
leukopenia/thrombocytopenia
low reticulocyte count
low serum B12 <200
Low Hct
elevated MMA/homocysteine
93
B12 treatment
cereal, fish, liver, milk, clams, yogurt
oral: 1000-2000 mcg qd
SQ/IM: 1000 mcg inj d x7d then weekly x1 month then monthly
ALWAYS give if presenting neurologic
Intranasal: 500 mcg (1 spray) qw for maintenance
94
What is the active form of folic acid
tetrahydrofolate
works with Vit B12 as a cofactor
95
Folic Acid etiology: inadequate intake and increased folate requirements
inadequate intake: elderly, teens, alcoholics
increased folate requirements: pregnancy, infancy, malignancy, inflammatory disease, burn pt, dialysis
96
Folic acid medications that decreased absorption
methotrexate
trimethoprim
phenytoin
phenobarbital
97
Folic Acid deficiency lab findings
normal B12 and normal MMA (B12 deficiency)
Serum folate: <3
RBC folate: <150
homocysteine elevated
98
Treatment for folic acid
oral: 0.4, 0.8, 1 mg qd
IV: rare
beef liver, lentils, leafy veggies, cereals, OJ, rice
99
Adverse effects of folic acid
IV: pruritus, skin rash, flushing, bronchospasms
100
Efficacy for folic acid
cont at least 4 months to correct folate deficient RBC in circulation
may need long-term in chronic conditions
repeat CBC, reticulocyte count, folic acid level in 4 months
101
Anemia of Inflammation (AI)
ACD: anemia of chronic disease, develop over mon to yr
ACI: anemia of critical illness, rapid onset to tissue damage and acute inflammation
102
Etiology of AI
multifactorial, usually a diagnosis of exclusion
infection, inflammation, tissue injury, proinflam cytokine
shorter RBC lifespan
disturbance of iron hemostasis
aspirin therapy, NSAID, anticoag, corticosteriods
103
Examples of ACD
chronic infections: TB, HIV, Osteo, endocarditis
chronic inflammation: RA, SLE, IBD, gout
Malignancies: carcinoma, lymphoma, leukemia, multiple myeloma
Other: alcohol liver disease, HF, COPD
104
Underlying causes in ACI
sepsis and increased metabolic demands
frequent blood sampling
surgical blood loss
immune-mediated functional iron deficiency
decreased production of EPO
reduced RBC life span
nutritional deficits
105
AI lab findings
no definitive test to confirm
ACD mild-moderate (Hb >8-9.5)
can coexist with other anemias
normocytic
106
IDA vs AI lab findings: Iron, TIBC/transferrin, TSAT, ferritin
AI: increased ferritin, others decreased/normal
IDA: increased transferrin, others all decreased
107
Treatment for AI (iron supplementation only effective if iron deficiency is also present)
Erythropoietin Stimulating Agents (ESAs)
No HgB >10
for CKD, HIV, malignancy
products are not interchangeable
reserved for severe anemia (HgB <7-8)
108
Epoetin Alfa (epogen, procrit) MOA
glycoprotein that stims RBC production
stims division and differentiation of progenitors in bone marrow
endogenous EPO production feedback loop regulation
109
Epoetin Alfa (epogen, procrit) indications and contraindications
anemia in cancer pt due to chemo, CKD, HIV
not: serious allergic rxn to drug, uncontrolled HTN, pure red cell aplasia (PRCA)
110
Epoetin alfa adverse drug reactions
hypersensitivity, headache, pruritus, n/v, injection site pain, fever, arthralgias
increased risk for seizures, pure red cell aplasia
111
Epoetin alfa BBW
CV events: risk of MI, stroke, VTE, HTN
increased risk of death, CV event, stroke when Hb >11
increased tumor progression in cancer
112
Epoetin alfa dosing
50-150 u/kg sq 3 times weekly
if Hb increase by >1 in 2 wks or >2 on 4 wks, decrease dose by 25-50%
if no increase by 1 after 4 wks increase dose by 25%
Hb target: 10-11.5
disc therapy if no response 4-12 wks
113
Darbepoetin Alfa (aranesp): MOA, tx, CI
stim division of erythroid progenitors in bone marrow
longer half life
for anemia in cancer and CKD
CI in serious allergic rxn, PRCA
114
Darbepoetin alfa ADE and BBW
hypersensitivity, edema, ab pain, dyspnea, cough, increased risk of seizures, PRCA
CV events (same as epoetin alfa)
115
Darbepoetin alfa dosing
0.45-2.25 qw, 0.75 q2w, 500 mcg q3w
>1 inc in 2 w or >2 inc in 4 w, dec dose by 25-50%
Hb no inc by >1 in 4 wk inc dose by 25%
HB target (10-11.5)
no response in 4-12 wk disc
116
ESA risk/benefits
risk: increase thrombotic events, decrease survival, increased time to tumor progression
benefit: transfusion avoidance, gradual improvement s/sx
117
RBC transfusion risk/benefit
risk: increase thrombotic events, decrease survival, transfusion rxn, TACO, virus transmission, bacterial contamination, iron/vol overload
benefit: rapid increased Hb/Hct, improved s/sx
118
Goals of therapy
return hematologic parameter to normal
QOL
prevent long-term complications
treat underlying disorder
correct reversible causes of anemia
B12: prevent/resolve neurologic sx
119
Sickle cell syndromes
SCT: sickle cell trait
HbAS
SCD: sickle cell disease
HbSS (sickle cell anemia)
120
What is the point mutation in HbS polymerization
glutamic acid to valine
-alters RBC shape, density, membrane, adhesion, ability to deform into sickle shapes
121
Pathophysiology of sickle cell anemia
HbS sickles leading to RBC hemolysis
they interact with neutrophils and PLTs leading to vaso-occlusion
HbF (fetal) is immune to sickling
occurs when RBCs are deoxygenated
122
Symptoms of SCA
fever
arthralgia
ab pain
weakness and fatigue
weight loss
hematuria
pain and swelling in hands and feet
123
Signs of SCA
chronic anemia/pallor
anorexia
hematuria
delayed growth in children
enlargement of liver, spleen, heart
increased reticulocytes, lactate dehydrogenase, PLT count, WBC count
normal MCV
sickle RBSs on peripheral smear
signs of VTE
124
Diagnosis of SCA
heel stick test preformed w/in 24-48 hrs of birth
positive screening should be confirmed by 2nd test before 2 months of age
125
Acute SCA complications
acute pain crisis
acute chest syndrome
stroke
venous thromboembolism
cholecystitis
priapism
infection
splenic sequestration
vaso-occlusive crisis
126
Chronic SCA complications
chronic pain
iron overload
osteonecrosis
retinopathy
nephropathy
HF
pulmonary HTN
leg ulcers
127
Hydroxyurea (gold standard for SCA) reduces the symptoms of what
frequency of acute pain crises
episodes of acute chest syndrome
blood transfusions
hospital stays
prevents/slow organ damage
128
Hydroxyurea MOA
inhibits DNA synthesis
129
Hydroxyurea indication
>9 months old with SCD
3 or more moderate-to-severe pain crises per year
h/o severe or recuttent acute chest syndrome
130
Hydroxyurea boxed warnings and ADE
BBW: myelosuppression, malignancy
ADE: increased lft, uric acid, BUN, SCr, mouth ulcers, N/V/D, alopecia, hyperpigmentation or atrophy, low sperm count
131
Hydroxyurea dosing
15 mg/kg/d adults
20 mg/kg/d children
maintenance: titrated based on CBC, 20-35 mg/kg/d
max: 2500 mg qd
(comes in 50 mg increments)
132
Hydroxyurea monitoring
CBC with diff
HbF
uric acid
renal function/LFTs
baseline pregnancy test
133
Hydroxyurea treatment goals
less pain and ACS episodes
increase HgF
increase HgB
ANC 2000-4000
134
Hydroxyurea toxicity
myelosuppresion
-ANC <2000
-PLT <80,000
-Reticulocyte <80,000 if Hgb <9
-HgB <5 or more than 20% at baseline
Increase SCr 50% above baseline
Increase ALT 100% above baseline
135
Hydroxyurea titration
clinical response 3-6 months
CBC with diff q2-4wk
increase 5 mg/kg/d q8-12w
if toxicity then hold
restart 2.5-5 mg/kg/d
CBC q2-3 months once stable dose achieved
136
L-glutamine MOA
precursor for nicotinamide adenine dinucleotide (NAD+) synthesis
137
L-glutamine indication, monitoring, ADE
5 years and older
monitor: renal and hepatic function
ADE: constipation, ab pain, nausea
138
Crizanlizumab MOA
binds and inhibits P-selectin
P-selectin helps sickle cells to adhere to vessels
139
Crizanlizumab indication, monitoring, and ADE
16 years and older
monitor: signs and symptoms of infusion-related rxns
infusion-related rxn, headache, arthraliga, diarrhea, pruritus, vomiting, chest pain
140
Non-pharm SCA
blood transfusions
bone marrow transplant
gene therapy
hematopoietic stem cell transplant
141
Acute SCA fever and infection
functional asplenia w/in 1st year of life
increased sick for serious infections
Strep pneumoniae, n. meningitis, h. influenzae
fever = bad = sepsis (>38.5C/101.3F)
142
When to use penicillin in SCA
all infants screened positive at birth
continue until 5 yo unless invasive pneumococcal infection or splenectomy
143
SCA pain crises
most common complication
leading cause ED visits
assess pain and admin med w/in 1 hr of presentation
144
SCA pain crises management
hydration: 1-1.5x maintenance fluid requirement
Mild: NSAID/APAP (codeine or hydrocodone if worse)
Severe: morphine, methadone, ketamine
145
Acute chest syndrome
new pulmonary infiltrate associated with fever/respiratory symptoms
pulmonary vascular occlusion and infection
Abx: macrolide/quinolone
146
Acute chest syndrome care
avoid analgesic-induced hypoventilation
steroids for inflammation
oxygen for supportive care
147
Goals of SCA therapy
reduce hospital
reduce acute and chronic complications
improve qol
prevent death
