Sickle Cell Flashcards
sickle cell inheritance
autosomal recessive
how is scd affecting perfusion
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
most severe form of scd
sickle cell anemia
what is sickle cell crisis
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
hgb range of anemia
5-11
hemolytic anemia
Tachycardia, cardiac
murmurs, and
enlarged heart
* Dysrhythmias and
heart failure in
adults
cell death
acute vacoocclusive crisis
Tissue hypoxia
* Inflammation
* Necrosis
* Pain
aplstic crsis
Hgb levels drop
rapidly
* Bone marrow cannot
compensate
sequestration crisis
Other organs pool
sickled cells
* In children: spleen
* In adults: liver and
lungs
diagnosic findings
Presence of the HbS
gene
*Low hematocrit
* Sickled cells on smear
* High WBC
* High Platelets
assessment
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
what is acute chest syndrome
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.
what is pulomnary htn
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
stroke r/t sickle cell
Cognitive dysfunction
* Evidence of ischemia on MRI or Doppler studies
* Transfusion PRBCs to decrease HbS < 30% (decrease
cerebral edema)
reproductive disorders
Males
* Low testosterone; low libido
* Priapism; erectile dysfunction
* Infertility
* Females
* Delayed menarche
* Increased risk for pregnancy complications
medical management
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
pretransfusion
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
transfusion
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
post transfusion
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
febrile nonhemolytic reaction
Reaction by antibodies to
donor leukocytes
* Chills, Fever (>1° Celsius
elevation)
* Prevention: leukocyte
reduction filter, antipyretic
agents
acute hemolytic reaction
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
allergic reaction
Sensitivity reaction to
plasma proteins
* Urticaria, itching, and
flushing
* Prevention: Antihistamines
TACO
can occur if too much blood is transfused too quickly
assess for risk: heart failure, kidney disease, old age, acute mi
s/s of taco
Dyspnea, orthopnea,
increased BP, sudden
anxiety, JVD, crackles in
lung bases, hypoxemia
what to do if taco occurs
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
trali
a potentially fatal
hypersensitivity reaction
* Acute lung injury is abrupt,
occurs within 2- 6 hours of
transfusion
* Underlying pathophysiologic
mechanism is unknown
s/s of trali
Acute SOB
* Hypoxia
* Hypotension
* Fever
* Eventual pulmonary edema
trali treatment
Aggressive supportive therapy
(oxygen, intubation, fluid
support)
