Sickle Cell Disease

What is Sickle Cell Disease?

Sickle Cell disease is a genetic blood disorder characterized by sickling of the red blood cell instead of the normal agile round/disk red blood cell shape.It is mostly found in people of African and Mediterranean ancestry.

What the chances of having a child with sickle cell?

A couple who have each a sickle cell trait can have a child who has sickle cell disease.There are chances that the same couple may have a child with no trait nor disease.Having the trait does not mean that you have the disease.

The normal morphology of a red blood cell is a nice and round disk which makes it possible to pass through even the smallest of blood vessels like arterioles and venules thus transporting oxygen to to all parts of the body,the sickle cell condition makes these red blood cells to form rods that take on the form of a sickle making it impossible to pass through the smallest blood vessels leading to damming and vaso-occlussion.

The sickled red blood cells tend to agglutinize (clump) together blocking the blood flow to some parts of the body and hence stopping the oxygen supply to the parts.Oxygen is an important component needed by all body cells to carry out metabolism.It is this lack of oxygen that causes the hallmark of sickle cell "Pain".Ischemia also cause complications in many vital organs of the body.

Mzee Wamala celebrating the new year with USCRF president and SCAU chairperson and other sickle cell patients in Uganda.

The Sickle Cell Association of Uganda celebrating the new year ,Jan 2008 at Bat Valley Kampala

Complications of Sickle Cell disease anemia

The vaso-occlusive crisis in microcirculation causing ischemia and infarctions.pain episodes, enlargement of the spleen,the heart and the liver (due to pooling and stasis of blood in these organs), massive destruction of red blood cells causing jaundice(yellowing of the skin especially the eye scelera).there is also retinal damage that may cause blindness.In men there are painful erections(priapism).Chronic leg ulcers are also very common.There is an increased risk of cerebral vascular accident (CVA) due to brain hemorrhage.

Management of sickle cell disease

This includes pain management using narcotic based analgesics e.g morphine, delaudid and non narcotic analgesics such as motrin.Adequate hydration, oxygenation, blood transfusions and exchanges, spleenctomy.Hydroxyurea which is said to increase fetal hemoglobin levels and significantly reduce hemolysis has been reported to reduce crises inpatients.

Other Therapies

The most promising of these with evidence of curing sickle cell disease is bone marrow transplant. Further genetic research is still going on, giving hope to millions of sickle cell disease sufferers and their families.Our hope is that these advances are made available to patients in developing countries like Uganda.

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