SICKLE CELL ANEMIA Flashcards
is an inherited blood disorder that affects hemoglobin, the protein that carries oxygen through the body.
Sickle Cell Anemia
An autosomal recessive disorder with mishaped RBC – characterized as SSS
sticky
stiff
sickle
normal lifespan of RBC;
abn lifespan
normal -120 days
abn- 20 days
Risk Factors - S-I-C-K-L-E
Significannt blood loss
Illness
Climbing/flying high altitudes
Keeping stress
Low fluid intake
Elevated temperature
Manifestation of Sickle Cell CRISIS
Vaso-occlusive
Hyperhemolytic
Nonfunctional Spleen
Anemia
Priority mngt for sickle cell ANEMIA
oxygen
Priority mngt for sickle cell CRISIS
fluids
to help with the vaso-occlusiven
Dx. test
- Dithionite
- Hemoglobin Electropharesis
DX TEST that will assess for abnormal hemoglobin on the red blood cell, but will not differentiate between sickle cell disease and sickle cell trait.
Dithionite test
the patient will need to have what other test to determine differentiate between sickle cell disease and sickle cell trait
CONFIRMATORY TEST to differentiate HGB
Hemoglobin Electropharesis
Which type of hemoglobin is present in a patient who has sickle cell anemia?
Hemoglobin SS
SS is homozygous and the patient must have two abnormal alleles present to have sickle cell anemia. The patient receives each abnormal allele for each parent (hence one from each parent which is Hemoglobin SS). If a patient has Hemoglobin AS (normal allele (A) and abnormal allele (S)) this is known as sickle cell trait, which most patients with this don’t present with signs and symptoms of the disease…it’s rare because they usually have just enough hemoglobin A to prevent the RBCs from sickling.
Which type of hemoglobin is present in a patient who has sickle cell TRAIT?
Hemoglobin AS
Sickle cell TRAIT is heterozygous, which means the patient has one NORMAL allele (which is Hemoglobin A…this is NORMAL hemoglobin) and one ABNORMAL allele (which is Hemoglobin S)…..this is the abnormal hemoglobin that leads to the abnormal construction of the RBC). However, most patients with sickle cell trait don’t show signs and symptoms related to sickle cell anemia because they have just enough of the normal hemoglobin A to prevent sickling of the RBC.
s/sx
- Dactilytis- hard feet syndrome
- Pain- sickle cell deposit in joints
- Anemia- RBC dying too fast
- Infection- 20 days goes to spleen
- Gallstone- RBC dies- bilirubin accumulation= gallstone
- Stroke- sickle cell blockage = in brain = stroke
- Eye problem
- Abn. clotting
- Leg ulcers- low part occlusive
- Acute chest syndrome- pneumonia, embolism
s/sx
- Dactilytis
- Pain
- Anemia
- Infection
- Gallstone
- Stroke
- Eye problem
- Abn. clotting
- Leg ulcers
- Acute chest syndrome
N MNGT
- O2
- Bed rest
- Risk factors avoidance
- Warm compress @ joint
- Elevated extremities
- Remove restrictive clothing
