Sickle-cell is a form of anemia, meaning that there are not enough red blood cells to properly carry needed oxygen around the body. This results in a variety of symptoms, which typically start to appear around four months.
These symptoms can vary in severity, intensity, and frequency from person to person. While some sufferers have only mild symptoms, in other cases, they can be excruciating or debilitating. They will almost always cause quite a bit of distress to young children with the illness.
The earliest symptom is called hand-foot syndrome. When the oddly shaped cells block a child’s small blood vessels, this causes pain and swelling, most commonly in the extremities. It is usually accompanied by fever. This is often the first visible sign of sickle cell, appearing in infants who are not yet manifesting the disease in any other way.
As with other types of anemia, sickle-cell patients tend to be pale and tired, and get winded easily. Anemia can seriously impact a person’s energy level. In addition, the unusually accelerated breakdown of red blood cells can cause jaundice, or a yellowing of eyes and skin.
The red blood cell shortage can also cause developmental problems. Though cognitive ability is generally unaffected, children with sickle-cell disorder grow more slowly and reach puberty later than others.
Most people with sickle-cell anemia report pain that occurs randomly and unpredictably throughout the body. In severe cases, someone can have more than 15 of these episodes, called crises, in a year, and a sickle-cell crisis can last anywhere from a few hours to a few weeks. This is the main symptom of the illness, and can require hospital care if the pain is severe or goes on especially long.
In the long term, sickle-cell patients are more prone to infections. The malformed cells damage the spleen so it can’t respond to infectious bacteria in the bloodstream. Infants and small children are particularly vulnerable; physicians often administer preventative antibiotics to infants known to have sickle-cell anemia. Children with sickle-cell are often prone to stroke, because their smaller blood vessels are particularly liable to be blocked by accumulations of sickled red blood cells.
Although symptoms are often clear in the early months of life, sickle-cell disease is particularly dangerous in those months, so it’s important to be on the alert. If possible, you and your partner should be tested before you have children. If your child is at risk, he or she should be tested soon after birth so that immediate preventative measures can be taken.