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| |    Sickle Cell Disease: The Basics by Laura Jana, M.D., F.A.A.P. reviewed by Lewis Hsu, M.D., Ph.D. If you are the parent of a child with sickle cell disease (or have it yourself), you know firsthand about the far-reaching and sometimes devastating effects of this inherited disorder. All aspects of a child's health and environment are involved, from the food he eats to the sports he plays, from how he avoids infections to how he manages the pain. But many--perhaps even most--people know little about this disease, believing that it only strikes people of African descent, for example, or is simply a form of anemia that can be managed with iron supplements and a careful diet. The truth, however, is that sickle cell disease is a complex disorder, and the first step in understanding it is to learn a little about certain genetic factors and the role of red blood cells in the body. The red blood cell Red blood cells play a very important role as the agents responsible for delivering oxygen to every part of the body. Normal red blood cells are flexible and rounded, which is crucial to their ability both to carry oxygen and to pass through all the small blood vessels. In large part, their structure is determined by one of their key components, known as hemoglobin. However, in the case of sickle cell disease, a very small alteration in the structure of hemoglobin makes the red blood cell much more prone to becoming deformed and assuming the shape of a sickle-hence the name sickle cell disease. The potential for cells to sickle is inherited Every person's genetic make-up is determined by the genes he receives from each of his parents. In order for his body to produce hemoglobin, it requires a blueprint-in the form of a hemoglobin gene-from each of his biological parents. In the case of sickle cell trait, however, only one of the two genes gives incorrect instructions, resulting in the production of normal and sickle hemoglobin (commonly referred to as HbS). In the most common type of sickle cell disease, both inherited genes are responsible for the production of sickle hemoglobin (referred to as HbSS). There also are other forms of the disease in which a child has received the sickle cell gene from one parent while the other has passed on a gene for another type of anemia, such as thalassemia; the combination results in sickle cell disease. When cells sickle When the red blood cells of children (and adults) begin to sickle-an event that is fundamentally triggered by low oxygen conditions-they become rigid and sticky. They no longer can pass smoothly through small blood vessels and can quickly cause blockages that result in poor blood flow to many areas of the body. Seemingly harmless situations of everyday life, such as mild dehydration, simple over exertion, and even fatigue or stress, can suddenly bring on sickling. If you take a step back and consider how important it is for every part of the body to receive oxygen, you'll understand why this sickling of red blood cells is more than just an inconvenience. Living with sickle cell disease Along with the diagnosis of sickle cell disease comes a long list of potential complications, not the least of which are significant episodes of pain, potential kidney damage, increased risk of infection, anemia, and stroke. Fortunately, there are things that people living with sickle cell disease can do to improve their quality of life and limit the likelihood of complications. The following set of articles has been created to offer you a better understanding of sickle cell disease and help children and parents alike live happy, productive, and healthy lives in the face of this disease.
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