Sickle cell disease is a type of blood disorder that affects the red blood cells. Normally, red blood cells are round and move around the body through small tubes delivering oxygen. In sickle cell disease the red blood cells become rigid and gluey and take on the shape of sickles. This happens because of a material in the red blood cells, which actually carries the oxygen.
A small change in this matter results in the hemoglobin forming long rods in the cell when distributing oxygen. These rods cause the sickle shape. They cause an obstruction in the blood flow so body tissues and organs are deprived of oxygen. This can cause terrible pain, which is known as the sickle cell crisis.
In the long run it causes a lot of damage to the body. The sickle cells have a shorter life span and though the body produces new red blood cells every day the sickle cells are destroyed so quickly that the body cannot keep up so the count drops. This leads to anemia.
With sickle cell disease there is oxygen deprivation and so an increased number of such cells are produced, which leads to acidity and thereafter dehydration of cells. There is a deficiency of nitric oxide, which is important for clotting the blood and maintaining the flexibility of blood vessels, and this adds to the pain.
This disease affects all races and newborns can be screened for it. Sickle cell disease is genetic as children inherit the sickle cell hemoglobin from their parents who may be carriers or actually suffer from the disease. If one sickle cell is inherited it is referred to as a trait while if two are inherited the person has the disease itself.
The symptoms manifest themselves as acute or chronic pain, anemia, fever, jaundice, leg ulcers, bone damage, and gallstones.
Hemoglobin electrophoresis is a simple blood test that will determine if you have the trait or the disease. If this is detected at birth the doctor can start treatment immediately by putting the baby on a course of penicillin and monitoring the effects. The doctor will have to rule out other infections such as bone infections before diagnosing sickle cell disease. There is no cure as such for this disease though research is ongoing on transplantational procedures.
When patients suffer from a sickle cell crisis they are in severe pain and this pain has to be managed. The crisis occurs because of some activity that increases the body’s need for oxygen but more often than not the cause is not known. Opioids, anti-inflammatory drugs, anesthesia etc can be given. Watch out for symptoms such as chest pain, fever, unexplained and increased fatigue, sudden weaknesses, and recurring pain. The patient will then need critical medical evaluation.
Caregivers should ensure that the patient takes medication as prescribed. Plenty of water and rest is vital. Extreme temperatures should be avoided and stress should be minimized. Regular medical checkups are mandatory to keep the condition under control. This will lead to a better prognosis.
