NU 448 Sickle Cell Anemia Summary

Pathol Priy Ierto
The HgBs gene is inherited and associated with African American (FARMS)
heritage. When these red blood cells are exposed to poor oxygen ❖ Fluids to reverse the sickling proce
/Fever
Nurg esn tension they crystalize and take on a sickle shape. They can clump
together in the circulation causing tissue hypoxia, ischemia, ❖
❖
Air
Rest
Pain, Swelling of joints ,Fever, Scleral jaundice necrosis, and inadequate perfusion to vital organs. These patients
❖ Medications
,Enlarged facial bones, tachycardia, murmurs, may experience multi organ dysfunction issues such as:
❖ Cardiomegaly ❖ Situations: flying, extreme cold,
cardiomegaly, renal failure, Impotence, acute
❖ Increased risk of stroke illness ect …...
chest syndrome, pulmonary hypertension

P: Pulmonary hypertension
❖
❖
Renal failure
Myocardial infarction Medin
A: Acute chest syndrome ❖ Acute chest syndrome ❖ Folic Acid daily
I: Intense pain and Increased swelling of joints ❖ Pulmonary hypertension. ❖ Pain management- heat preferred
N: No sex ( impotence) ❖ Hydroxyurea: Decreases the
F: Fast heart rate/ Murmurs ❖ formation of sickling cells.
❖ Aspirin
U: Under worked Kidneys ( renal failure)
L: Large heart
Pat Eduon ❖ Arginine : A pulmonary vasodilator
❖ Avoid the cold that inhibits sickle cell formation.
❖ Stay hydrated ❖ Vasopressin/desmopressin
❖ Educate on ❖ NSAIDs
proper use of ❖ Oxygen
Hydroxyurea and ❖ RBC transfusion to decrease the
Arginine. amount of sickling cells. Maintain
❖ Pain management Hemoglobin at 11 or less to preven
the blood from becoming too thick
❖ SCT




! EL
IMTO !
Do not give a sickle cell patient
demerol because of risk for
normeperidine induced seizures



Sic c cis
A painful episode that can be precipitated by the cold. It Nurg Ce
occurs when the sickle-shaped red blood cells (RBCs) block ❖ Pain Management
blood vessels, causing decreased perfusion and pain. Pain ❖ Psychological support
occurs in the joints, back, hip, and extremities ❖ Blood transfusions
❖ Electrolyte imbalances
Tye f Cis ❖ Fluid imbalances- daily weights
❖ Acute Vaso Occlusive Crisis: Clumps of ❖ If Splenic sequestration is
reoccurring- splenectomy can be a
sickled cells gather in the microcirculation such
as capillaries causing hypoxia, necrosis and Lab surgical intervention
inflammation. Oxidation occurs causing vessel ❖ Sickledex (fingerstick)
damage. Each crisis is worse than the last ❖ Hemoglobin electrophoresis
❖ Aplastic Crisis: A rapid fall in hemoglobin ❖ CBC (retic count, Hgb, Plt , WBC),
precipitated by the Human Parvovirus. Chemistry, UA, Urine protein,
❖ Sequestration Crisis: Clumping of sickled cells Ferritin
in the organs. Most common organs include the ❖ Hemoglobin & Hematocrit are
spleen if the crisis occurs in a child or Liver and monitored frequently.
lungs for adults. ❖ Blood smear shows RBCs with a
sickle shape.