1. Sickle Cell anemia

2. What is Sickle Cell?
People who have Sickle Cell have sickle shaped red blood cells, which causes complications because the blood cells are not able to reach certain parts of the body.

3. Hb S Valine replaces glutamic, in the B polypeptide chain.
This alters the secondary and thirtiary 3 ry structures of B chain giving rise to Hb S molecules which clump together, causing the “sickling” of the cells.

4. Pathogenesis There are 4 protein subunits of Hemoglobin A Hemoglobin A
Alpha
Beta

5. A carrier for Sickle Cell Will have…
An S mutation in one copy of the hemoglobin beta gene.
Half of the beta subunits are replaced with Beta S.
This person has the Sickle Cell trait.
Hemoglobin
Alpha
Beta
Beta S

6. Sickle Cell Disease: HbSS
This results when both copies of the hemoglobin beta gene have an S mutation.
All of this person’s beta subunits are replaced by S.
Hemoglobin
Alpha
Beta S

7. inheritance

8. Red blood cells Going through Vessels
Sickle cells tend to block the small blood vessels
This gives rise to micro infarcts
Abnormal RBC being destroyed from the circulation gives rise to anemia

9. Symptoms of Sickle Cell
Anemia- pallor,breathlessness,fatigue
Jaundice
Delayed growth and puberty
Abdominal and bone/joint pain
Greater risk for infection
Adolescents and adults can develop ulcers on their legs
Chest pain
Excessive thirst
Poor eyesight, blindness
Auto splenectomy-due to micro infarcts in the spleen

10. An x-ray of a hand swollen from dactylitis
Crisis
Acute chest syndrome
Aplastic crisis
Dactylitis – swelling of the hands and feet
Painful crises: really painful episodes when blood cells are blocked from going to certain parts of the body – pain can occur anywhere, but it is usually in the chest, arms, and legs
Acute Enlarged spleen – sickle cells pool in the spleen, causing abdominal pain
Stroke
An x-ray of a hand swollen from dactylitis

11. Treatment Options For Sickle Cell Anemia

12. There is no known cure for sickle cell anemia.
Blood Transfusions
Drug Treatment-hydroxyurea/penicillin prophylaxis as spleen not functioning
Blood and Marrow Stem Cell Transplantation
Gene Therapy
These main treatment options for the painful crisis involves analgesics and oral and intravenous fluids.

13. Thank you…