Organs at Risk in Persons with Sickle Cell Disorder (Part 2)

Organs are at risk in all persons, whatever their Hb genotypes, for reasons that are not always clear. For example I know a 33 year old working lady without sickle cell disorder (SCD), who developed and subsequently died from breast cancer, still has alive, her unaffected mother and grandmother.

Also, while it is true that persons with SCD have more organs at risk than persons without SCD, I know a 72-year old female Medical Doctor, who has sickle cell anaemia (Hb SS), who is still alive, while many other persons, with or without SCD have died at younger ages.

Acute Splenic Sequestration

This term is used to describe the collection of blood cells in a person's spleen. This leads to more severe or fatal anaemia, which often requires blood transfusion. It is most common in young children with sickle cell anaemia (HbSS) usually aged between 4 months and 5 years. It requires urgent blood transfusion and future episodes can be prevented by daily use of oral Hydroxyurea (Hydroxycarbamide). Otherwise, the spleen should be surgically removed (splenectomy). After spenectomy, life-long oral penicillin should be prescribed to prevent future bacterial infection.

Acute Liver [Hepatic) Sequestration

This is fortunately much less common than splenic sequestration, and is commoner in adults with sickle cell anaemia (Hb SS). The liver is enlarged and painful. It should be treated with antibiotics, especially as surgical removal of the liver is inconceivably dangerous. Preventive treatment with hydroxyurea should also be introduced.

The Gall Bladder

The gall bladder is a small organ, located behind the liver. It has several functions, and the formation of gall stones within it, is commoner in persons with SCD. This may lead to infection and to severe pain. Treatment with appropriate antibiotics and even surgical removal of the gall bladder are conceivable.

Bones

Painful bone crises are common in persons with SCD. The definite cause is not well known, but reduction of its frequency can be achieved with daily ingestion of appropriate dose of hydroxyurea.

Bacterial infection in bones (known as osteomyelitis) is also commoner in persons with SCD. It should be identified early for treatment with appropriate antibiotics to be effective. Otherwise, the osteomyelitis may become chronic and difficult to resolve without surgical intervention, which has its own disadvantages.

Some bones, especially around the hips and shoulders, also become chronically painful and less functional owing to inadequate blood supply. This is known as avascular necrosis (AVM).

Leg Ulcers

The common sites of non-traumatic leg ulceration in persons with SCD, particularly Hb SS, are just above their right or left ankles. For unknown reasons these ulcers are much less common in SCD patients in West Africa than those in the West-Indies.

Fairly recently, we discovered that dressing the ulcers with Asmisol (a solution developed in Ghana) heals the ulcers faster than usual.

By Professor Olu Akinyanju, OON