1. Sickle Cell Anemia Roxbury Community College ADN 253 Honors Presentation Adanna Uwandu, Shadia Laurent, Salwa Said 05/01/07

2. Objective To apply the nursing process to care for patients and families with sickle cell anemia. To learn the community resources available to patients and families with sickle cell anemia and sickle cell trait. To provide care to: -individuals with the disease -individuals planning to have a family.

3. Definition of Sickle Cell Anemia It is an autosomal recessive inherited disorder affecting the beta chain of hemoglobin It is a congenital hematological disease. Hemoglobin: -iron-containing portion of RBC -allows RBC to carry O 2 from the lungs to tissues -normal levels in g/100mL (p.1383): *Birth; 13.7- 20.1 *3 mths 9.5-14.5 *adult levels 11-16

4. Pathophysiology Erythrocytes (RBC) become elongated and crescent-shaped (sickled) when they are submitted to -low O 2 tension/levels (< 60%-70%) -a low blood pH (acidosis) -increased blood viscosity (thick blood) Dehydration and hypoxia can trigger these effects on the blood Sickle cells can accumulate in capillaries and smaller blood vessels causing occlusions, impair normal circ., tissue infarctions (tissue death), swelling and anoxic changes. Defective hemoglobin molecule is produced called Hemoglobin S.

5. Differences in Red Blood Cells

6. Pathophysiology Sickled RBC’s are thick and clump together. RBC’s do not move freely through the blood vessels causing stasis and further sickling to occur. Blood flow stops and the tissue distal to the blockage becomes ischemic thus causing: acute pain, cell destruction.

7. Fetal Hemoglobin Is present until about six months and the child with sickle cell anemia will not usually have clinical symptoms before such time. Fetal hemoglobin changes to adult hemoglobin around 6 mths. Can be diagnosed in utero by Child with sickle cell disease has hemoglobin SS, and produces no normal hemoglobin and thus shows characteristic symptoms of the disease.

8. Sickle Cell Trait 25-50% of the hemoglobin produced is abnormal. Patients who are carriers (heterozygous), can pass the gene to their offspring. It is the benign type of SC disease. Their normal hemoglobin outnumber the abnormal hemoglobin thus; they have

9. Assessment Hemoglobin electrophoresis: few drops of blood that have converted to their adult form Show signs ~ 6mths of disease - fever, anemia, stasis of blood and infarction > local disease. Other signs incl: -hand-foot syndrome -slight built/thin, long arms & legs -protruding abdomen, atrophic spleen in adolescence > inc risk for infection, prophylactic antibx given

10. Assessment -chest syndrome ~ pneumonia occurs, enlarged liver > cirrhosis from infarctions and scarring tissue. -dec kidney function, yellowed sclerae -dec vision in children, from small retinal occlusions, regular eye exams. -priapism

11. Sickle Cell Crisis Definition: Sudden, severe onset of sickling. Types: sequestration crisis, aplastic crisis, megaloblastic crisis Symptoms result from: -vaso-occlusive crisis (pooling > tissue hypoxia past the blockage point. Triggers include: - dehydration, respiratory infection, lowered O2 exchange, dec arterial O2 level

12. Sickle Cell Trait