Sickle Cell Disorder Flashcards
1. Etiology r/t Sickle cell anemia 2. ID clinical manifestations 3. ID direct & indirect causes of sickle cell 4. ID risk factors 5. Formulate priority nursing Dx 6. Create a plan of care for client & family 7. Expected outcomes 8. Discuss therapies 9. Employ evidence-based caring interventions
What is Sickle Cell Anemia?
A severe disease (Chronic). Hemolytic Anemia occuring in persons who are homozygous for the sickle cell gene.
Etiology of sickle cell anemia
- Sickle cell disease is genetically determined, inherited disease.
- Each person inherits 1 gene from each parent, which governs synthesis of Hgb.
Normal parent + Trait parent… What is to be passed on to offspring?
50% Normal
50% Trait
0% Disease
Trait parent + trait parent. What is to be passed on to offspring?
25% Normal
50% Trait
25% DISEASE
Trait parent + Disease parent. What is to be passed on to offspring?
0% Normal
50% Trait
50% Disease
Disease parent + Disease parent. What is to be passed on to offspring?
100% Disease
How is sickle cell anemia an inherited disease though?
- In sickle cell, the animo acid sequence on the DNA chain is altered (replaced) with valine instead of glutamic acid on the 547th amino acid.
- The 547th amino acid makes up the globulin fraction of Hgb.
What does this Amino Acid change do to the Hgb in a sickle cell pt?
- Hgb aggregates into elongated crystals under conditions of low O2 concentration, acidosis & dehydration.
- Aggregation of Hgb molecules causes RBC to assume a crescent(sickle) shape.
- This leads to traffic jamming to increased blood viscosity, vessel occlusion & last, tissue necrosis.
How is this type of Anemia defined?
Anemia results when the rate of Destruction of RBC’s is greater than @ the rate of Production
What are the clinical manifestations specific to sickle cell anemia?
- Hgb 6–9
- Loss of appetite
- Pallor
- Weakness
- Fever–Due to dehydration, fluid in lungs(stasis)
- Irritibility
- Jaundice
Sickle Cell Crisis
Small blood vessels are occluded by the sickle-shaped cells, causing DISTAL ischemia & infarction.
Clinical Manifestation r/t Extremities
- Bony destruction leading to osteoporosis
2. Bone pain; painful swollen large joints
Clinical Manifestations r/t spleen
- Abdominal pain
- Slenomegaly – bc of RBC sequestation (Pulling & holding onto RBC)
- Decreased splenic function increases risk of infection (Most common death in elderly)
Clinical Manifestations r/t CNS
- CVA
- Hemiplegia (paralysis of the arm, leg, and trunk on the same side of the body.)
- Retinal damage leading to blindness
- Seizures
Other Manifestations in other organs include:
- Pulmonary Infarction
- Altered Renal Function
- Impaired Liver Function
Altered Renal Function r/t Sickle Cell Crisis:
- Decreased urine output <30/hr
- Electrolyte imbalance
- Check creatinine lab/BUN as well
Impaired Liver Function r/t Sickle Cell Crisis:
- Increased liver enzymes
- Jaundice
- Peripheral edema
- Albumin pulls smaller molecules (H2O) to itself into intravascular space (3rd spacing)
- Iscites – Fluid accumulation
- Abdominal distention
What Causes a Sickle Cell Crisis?
- Dehydration
- Infection
- Trauma
- Strenuous physical exertion
- Extreme Fatigue
- Cold Exposure
- Hypoxia
- Acidosis
- Acidosis
- Surgery
- Pregnancy
What Will My Assessment Look Like?
- Hx: Possible dehydration, hypoxia, infection or other precipitating events
- Observe for: Pallor, jaundice, changes in vs(Increased Temp, tachycardia, hypotension, tachypnea) Changes in mental status, edema, ulcers, lesions.
- Examine for: Enlarged liver/Spleen, tenderness of hands/feet.
- Hx & characerization of pain
What are some diagnostics to perform for Sickle Cell?
- Sickling test: O2 removed from blood & observed for sickling
- Sickledex: p collection, sickle Hgb indicated if solution turns cloudy
- Hgb Electrophoresis: Dx trait & disease
- Antenatal Dx:
