Sickle Cell Disease (SCD) – Basic Notes-and latest MOH GUIDELINES

 

Sickle Cell Disease (SCD) – Basic Notes

Definition

Sickle Cell Disease (SCD) is an inherited blood disorder caused by abnormal hemoglobin called Hemoglobin S (HbS). Red blood cells become sickle-shaped leading to blockage of blood vessels and destruction of red blood cells.

Genetics / Inheritance

It is an autosomal recessive disorder. A person develops SCD when they inherit one abnormal gene from each parent.

Genotypes

HbAA – Normal
HbAS – Sickle cell trait
HbSS – Sickle cell disease
HbSC – Variant form of SCD

Pathophysiology

HbS polymerizes during low oxygen states causing red blood cells to become rigid and sickle-shaped. These cells block blood vessels leading to ischemia, pain, organ damage, and hemolytic anemia.

Triggers of Sickling

Infection, dehydration, cold exposure, stress, hypoxia, and acidosis.

Clinical Features

Recurrent body pains, easy fatigability, pallor, jaundice, fever, delayed growth, anemia, yellow eyes, splenomegaly, leg ulcers, and bone tenderness.

Common Crises

1. Vaso-occlusive crisis – severe pain crisis.
2. Aplastic crisis – sudden fall in hemoglobin.
3. Sequestration crisis – pooling of blood in spleen.
4. Hemolytic crisis – increased destruction of RBCs.

Complications

Stroke, acute chest syndrome, osteomyelitis, gallstones, leg ulcers, priapism, kidney damage, and heart failure.

Investigations

Full blood count (FBC), peripheral blood film, reticulocyte count, sickling test, and hemoglobin electrophoresis.

Management

Hydration, nutrition, folic acid, infection prevention, oxygen, IV fluids, analgesics, antibiotics, blood transfusion, and hydroxyurea.

Prevention

Genetic counseling, premarital screening, newborn diagnosis, vaccination, and regular clinic follow-up.

Key Exam Points

SCD is inherited autosomal recessively. HbSS is the severe form. Vaso-occlusive crisis is the commonest crisis. Electrophoresis confirms diagnosis. Hydroxyurea increases HbF.

download new guidlines here:sickle cell desease guidelines

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May 2, 2026 at 6:02 AM
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