Sickle Cell Anemia
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Sickle Cell Anemia: Hemolytic Anemia: Increased destruction of RBCs
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Patho
- Sickle cell disease: formation of abnormal hemoglobin called HbS (more than 40% of RBCs are HbS in sickle cell disease
- HbS cells are sickled in shape, rigid, sticky and clump together, obstructing blood flow and causing hypoxia and ischemia.
- Very painful
- To have the disease, both parents must be carriers
- You can have the sickle cell trait, carry only one gene, and have a very mild form of sickle cell.
- Mostly in african-americans.
- Very painful due to low oxygen from obstructed blood flow.
- Symptos of anemia: Shortnes of breathe and hypoxia, Tachycardia, weakness and pallor.
- Organ damage: renal failure, spleen and liver.
- Sores
- Dactylitis: swelling of hands and feet
- Seizures
What precipitates a sickle cell crisis
- Hypoxia
- Dehydration
- Infections
- Immobility
- pregnancy
- Alcohol
- Extreme temperatures
- Exercise
- Emotional stress
- Anethesia/surgery
- Blood becomes thick, cells clump leading to no blood flow and tissue hypoxia, which causes the pain.
- Hydration makes blood thinner, increasing organ perfusion and decrease pain from hypoxia
- Prevent Infection: handwashing, monitor temp and WBC. May need prophylactic antibiotics to prevent pneumonia and strep.
- Do not elevate HOB > 30 ° to prevent pulmonary embolism (remember that blood is thick and can clot)
- Monitor extremities for poor blood flow (temp, color, CFT, pulses, check pulse ox of fingers and toes if possible)
- Keep room temp moderate
- Hydroxyurea: chemotherapy agent that ⇓ cells from sickling
- RBC transfusion
- Stem cell transplant
- Limit stress
- Prevent infection
- hydrate
- vaccinate
- do not smoke
- avoid high altitudes
- avoid over-exertion
