Sickle Cell Anemia
What is it?
Genetic blood disorder that causes the body to produce abnormal hemoglobin. The abnormal hemoglobin causes the red blood cells to become shaped like a sickle or crescent, which can block small blood vessels, prevent oxygen from reaching tissues, and cause pain and organ damage. Mostly affects people of African descent, but it also occurs in people of Hispanic, Middle Eastern, and Mediterranean ancestry.
- 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.Â
- 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 disease.
- Mostly in African-Americans
Symptoms
Fatigue & weakness
and shortness of breath. The client is anemic due to the sicklig of red blood cells.
Strokes
Sickled cells clog vessels reducing blood flow/oxygen to brain
Organ Damage
Sickled cells prevent oxygen/nutrients from reaching organs. Give Hydroxyurea (prevents cells from sickling)
Increased risk for infections
Damage to the spleen which helps remove bacteria/viruses from body
Delayed Growth
Slow growth and development in children
What precipitates a sickle cell crisis
- Hypoxia
- Dehydration
- Infections
- Immobility
- Pregnancy
- Alcohol
- Extreme temperatures
- Exercise
- Emotional stress
- Anesthesia/Surgery
- Blood becomes thick, cells clump leading to no blood flow and tissue hypoxia, which causes the pain.Â
Nursing Interventions
- Hydration makes blood thinner, increasing organ perfusion and decreasing pain from hypoxia
- Prevent Infection: handwashing, monitor temp and WBC.Â
- May need prophylactic antibiotics to prevent pneumonia and strep infections.
- Monitor extremities for poor blood flow (temp, color, CFT, pulses, check pulse oximeter of fingers and toes if possible).
- Monitor for ISCHEMIC stroke: neurological symptoms such as slurred speech, unilateral weakness, headache, or blurred vision.Â
- Keep room temperature moderate
- Hydroxyurea: Chemotherapy agent that ⇓ cells from sickling
- RBC transfusion
- Stem cell transplant
Crisis Prevention
- Limit stress
- Prevent infection
- Hydrate
- Vaccinate to prevent illnesses
- Do not smoke
- Avoid high altitudes (causes hypoxia)
- Avoid over-exertion
Treatment
H
O
P
Hydration
Oxygen
Pain Management
Acute chest syndrome
- Sickled red blood cells block blood vessels in the lungs → hypoxia and lung damage
- Symptoms: chest discomfort/pain, cough, shortness of breath, fever, and rapid breathing
- Leads to respiratory failure/death
- A vaso-occlusive crisis in sickle cell anemia is a painful episode characterized by the obstruction of small blood vessels (vaso-occlusion) by abnormally shaped, rigid red blood cells.
- Clumping of cells and blockage of blood flow in the microcirculation.
- The impaired blood flow can result in severe pain and tissue damage.Â
- Triggered by factors such as dehydration, infection, stress, or low oxygen levels.
- Treatment is pain management and hydration.Â
