Sickle Cell Disease and Pregnancy
What is sickle cell disease?
This is a condition where the haemoglobin is defective. Haemoglobin is the oxygen-carrying component of the red blood-cells. The defect in this condition, in certain conditions, renders the blood cells susceptible to destruction in the body.
How did this condition acquire its name?
The cells carrying this defective haemoglobin tend to acquire a characteristic "sickle" shape in certain unfavourable conditions, hence the name "sickle" cell.
What are the conditions that lead to this distortion in the shape of the cells?
This is not a simple straightforward affair. However, any condition which reduces oxygen in the bloodstream (such as pneumonia and malaria), high altitude (so mountaineering is not a suitable hobby) and such conditions as dehydration - even body cooling - could lead to. a "sickling" crisis.
Why is sickling" of the cells described as a crisis?
Any person with the condition will tell you that such episodes are very aptly described as crises.
When "sickling" happens, the cells cannot move freely within the bloodstream in the smallest blood vessels, and affected areas are denied oxygen as a result. This could be in bones, joints, kidneys or other organs. This causes severe pain for the sufferer, which can barely be controlled by even the strongest painkillers.
Are any particular measures recommended to prevent the sickling crises from occurring?
It is impossible to completely eliminate the risk of a sickling crisis. This is because some episodes are precipitated by ill-defined causes.
However, it is possible to minimize this risk by a meticulous observation of general measures which prevent the conditions which favour a crisis.
The sufferer has to ensure that she is not short of oxygen (e.g. unduly stressful physical exercises), dehydrated or exposed to very low temperatures for prolonged periods.
Infection is probably the leading trigger of sickling crises. This is worsened by the fact that dehydration often accompanies infections. Early detection and aggressive treatment of infections is therefore one of the principal treatment measures.
There is also a place for preventative steps in the form of antibiotics.
Apart from the crises, what other problems are associated with sickle cell disease ?
People with sickle cell disease (SCD) are chronically anaemic. Their haemoglobin levels hardly ever rise above 9.5 g/dl. Compare this to the normal levels of 12.5-15.0 g/dl for the average woman.
The chronic anaemia is largely due to the recurrent destruction of the blood cells in the body. The cells are vulnerable from carrying the defective haemoglobin. SCD is also known as "sickle cell anaemia" simply because anaemia is virtually a permanent feature.
What about those individuals who are carriers only?
To be affected by sickle cell disease, an individual has to inherit the defective gene from both parents. If only one defective gene is inherited from either parent, the child will be a carrier but will not have the disease. This is known as "sickle cell trait".
These individuals are usually healthy and do not suffer the clinical manifestations of sickle cell disease. However, in very extreme conditions, a sickle cell trait individual may suffer a sickling crisis, usually relatively mild. This is rare.
Are there any special problems suffered by women with sickle cell disease (SCD) in pregnancy?
Pregnancy puts a considerable strain on the body systems of sickle cell disease sufferers. The chronic anaemia and impaired oxygen supply mean these women suffer a higher rate of such complications as miscarriage, preterm labour and even stillbirth.