16/09/2025
SICKLE CELL DISEASE (SCD) 🩸
Definition
Sickle cell disease (SCD) is an inherited blood disorder where red blood cells, which are normally round and flexible, become rigid and shaped like a sickle (crescent moon) due to an abnormal type of hemoglobin called hemoglobin S.
These sickle-shaped cells break down easily (hemolysis) and block blood flow, leading to anemia, pain, organ damage, and increased risk of infections.
*Normal Physiology of Red Blood Cells*
Red blood cells (RBCs) contain hemoglobin, which carries oxygen from the lungs to the rest of the body.
Normal RBCs are round, smooth, and flexible, allowing them to move easily through blood vessels.
They live for about 120 days before being replaced by new cells.
*Pathophysiology of Sickle Cell Disease*
1. A genetic mutation leads to production of hemoglobin S instead of normal hemoglobin A.
2. When oxygen is low, hemoglobin S molecules stick together, causing RBCs to change into a sickle shape.
3. These sickle cells are:
Fragile → break down quickly → chronic anemia.
Sticky → block small blood vessels → pain crises and organ damage.
4. Lifespan of sickle cells is only 10–20 days, compared to 120 days for normal cells.
*Causes & Risk Factors*
Genetic inheritance:
A child must inherit the sickle gene from both parents (SS) to have the disease.
If only one parent passes the gene, the child has sickle cell trait (AS), which is usually symptomless but can be passed to offspring.
Risk is highest when both parents are carriers (AS + AS).
*Symptoms*
Anemia: Fatigue, weakness, pale skin.
Pain crises: Sudden episodes of severe pain (bones, chest, abdomen).
Swelling: Hands and feet (dactylitis).
Frequent infections: Due to spleen damage.
Delayed growth & puberty in children.
Vision problems due to blocked eye blood vessels.
Jaundice: Yellowing of eyes and skin (due to rapid RBC breakdown).
*Types of Sickle Cell Disorders*
1. HbSS: Most common and severe form.
2. HbSC: Milder form (one sickle gene + one C gene).
3. HbAS (Sickle cell trait): Usually no symptoms, but carrier.
4. HbSβ-thalassemia: Combination of sickle gene and thalassemia gene.
Complications
Stroke.
Pulmonary hypertension (high blood pressure in lungs).
Organ damage (kidneys, liver, spleen).
Leg ulcers.
Gallstones (from breakdown of RBCs).
Death from severe infection or organ failure.
*Treatment*
Pain management: NSAIDs, opioids during crises.
Hydroxyurea: Increases fetal hemoglobin, reducing sickling and crises.
Blood transfusions: Reduce risk of stroke and anemia.
Bone marrow/stem cell transplant: The only potential cure, but rarely available in Africa due to cost.
Antibiotics & vaccines: Prevent infections (especially in children).
Folic acid supplementation: Helps new RBC production.
Prevention & Clinical Advice
Genetic counseling: Couples should know their genotype (AA, AS, SS) before marriage.
If both partners are AS, there is a 25% chance of having a child with sickle cell disease.
Drink plenty of water and avoid extreme temperatures (cold/heat).
Manage stress and avoid strenuous activity that may trigger crises.
Children should receive routine immunizations and extra vaccines against pneumonia, meningitis, and influenza.
Regular hospital follow-up is essential.
⚠️ Takeaway: Sickle cell disease is preventable through genotype testing before marriage. For those already living with SCD, proper care, good nutrition, and medical follow-up can greatly improve quality of life and life expectancy.
Note:If you're suffering from Sickle cells Disease.
Visit hospital nearest to you consistently!!!
Consult a Pharmacist close to you for proper and appropriate use of medicine and lifestyle..
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Yours FOFANAH 🥼🩺