Sickle Cell

Question 1

sickle cell inheritance

Answer 1

autosomal recessive

Question 2

how is scd affecting perfusion

Answer 2

Inheritance of sickle hemoglobin gene (HbS)
Defective hemoglobin molecule
low o2 tension
The erythrocyte containing HbS becomes dehydrated, rigid, and sickle shaped
long, rigid erythrocytes adhere to endothelium of small vessels and to each other
Blood flow to a region or an organ is reduced

Question 3

most severe form of scd

Answer 3

sickle cell anemia

Question 4

what is sickle cell crisis

Answer 4

When blood flow
is significantly reduced, ischemia
or infarction of tissue can cause
severe pain, swelling, and fever.
-Can be triggered by cold,
dehydration, stress, infection, high
altitude

Question 5

hgb range of anemia

Answer 5

5-11

Question 6

hemolytic anemia

Answer 6

Tachycardia, cardiac
murmurs, and
enlarged heart
* Dysrhythmias and
heart failure in
adults
cell death

Question 7

acute vacoocclusive crisis

Answer 7

Tissue hypoxia
* Inflammation
* Necrosis
* Pain

Question 8

aplstic crsis

Answer 8

Hgb levels drop
rapidly
* Bone marrow cannot
compensate

Question 9

sequestration crisis

Answer 9

Other organs pool
sickled cells
* In children: spleen
* In adults: liver and
lungs

Question 10

diagnosic findings

Answer 10

Presence of the HbS
gene
*Low hematocrit
* Sickled cells on smear
* High WBC
* High Platelets

Question 11

assessment

Answer 11

History- Factors that precipitated crisis, measures to
prevent/manage, medical care, treatments
Assess for pain, swelling, fever, fatigue, dehydration
Thorough assessment of all
body systems
Presence of infection
Degree of anemia

Question 12

what is acute chest syndrome

Answer 12

Caused by infection with atypical bacteria, pulmonary
embolism, pulmonary infarction
* S&S: fever; respiratory distress (tachypnea, cough and
wheezing)
* Infiltrates on CXR
* Medical management: blood transfusion, antibiotics,
bronchodilators, inhaled nitric oxide, mechanical
ventilation.

Question 13

what is pulomnary htn

Answer 13

Early diagnosis difficult, symptoms presentation
delayed/irreversible damage
* S&S: fatigue, dyspnea on exertion, dizziness, chest pain,
or syncope
* CT of chest- may see microvascular pulmonary occlusion

Question 14

stroke r/t sickle cell

Answer 14

Cognitive dysfunction
* Evidence of ischemia on MRI or Doppler studies
* Transfusion PRBCs to decrease HbS < 30% (decrease
cerebral edema)

Question 15

reproductive disorders

Answer 15

Males
* Low testosterone; low libido
* Priapism; erectile dysfunction
* Infertility
* Females
* Delayed menarche
* Increased risk for pregnancy complications

Question 16

medical management

Answer 16

Hematopoietic stem cell transplant
* Hydroxyurea
* Increases fetal hemoglobin
* Decreases formation of sickled cells
* Folic acid supplementation
* Helps make new RBCs
* Transfusion therapy
*Supportive therapy
* Pain management
* Hydration- oral /IV
* Oxygen

Question 17

pretransfusion

Answer 17

Ensure type & crossmatch is
completed
Verify written consent
Educate S&S transfusion reaction
obtain vs
Assess for fluid overload, request
order for diuretic if needed
Assess gauge and patency of IV
prime with only NS

Question 18

transfusion

Answer 18

Double-check (RN-RN)
Confirm patient ID
Check ABO group & Rh type with
compatibility record
Start transfusion within 30 minutes of
removal blood bank refrigerator
For the first 15 minutes, run transfusion
slow 5 mL/min
Monitor closely for 15-30 minutes
Routine monitoring of VS & observations
for signs of reactions. If reaction is
suspected STOP IMMEDIATELY, notify
provider, and follow institutional policy
Administer time may not exceed 4 hours
Change tubing after every other unit

Question 19

post transfusion

Answer 19

obtain vs
Assess for fluid overload,
request order for diuretic if
needed
Dispose of materials properly
document procedure
Monitor for response to and
effectiveness of procedure

Question 20

febrile nonhemolytic reaction

Answer 20

Reaction by antibodies to
donor leukocytes
* Chills, Fever (>1° Celsius
elevation)
* Prevention: leukocyte
reduction filter, antipyretic
agents

Question 21

acute hemolytic reaction

Answer 21

Most dangerous, potentially
life threatening
* Donor blood is incompatible
with the recipient’s
* Rapid hemolysis with ABO
incompatibility, Rh
incompatibility – less sever
reaction
* Fever, chills, low back pain,
nausea, chest tightness,
dyspnea, anxiety
* Prevention: double checks,
bar coding methods

Question 22

allergic reaction

Answer 22

Sensitivity reaction to
plasma proteins
* Urticaria, itching, and
flushing
* Prevention: Antihistamines

Question 23

TACO

Answer 23

can occur if too much blood is transfused too quickly
assess for risk: heart failure, kidney disease, old age, acute mi

Question 24

s/s of taco

Answer 24

Dyspnea, orthopnea,
increased BP, sudden
anxiety, JVD, crackles in
lung bases, hypoxemia

Question 25

what to do if taco occurs

Answer 25

If reaction is mild: slow
transfusion
* If reaction is severe: stop
transfusion, sit upright with
legs in dependent position,
notify provider, treat S&S as
ordered

Question 26

trali

Answer 26

a potentially fatal
hypersensitivity reaction
* Acute lung injury is abrupt,
occurs within 2- 6 hours of
transfusion
* Underlying pathophysiologic
mechanism is unknown

Question 27

s/s of trali

Answer 27

Acute SOB
* Hypoxia
* Hypotension
* Fever
* Eventual pulmonary edema

Question 28

trali treatment

Answer 28

Aggressive supportive therapy
(oxygen, intubation, fluid
support)