n WHAT ARE THE COMPLICATIONS OF SICKLE CELL DISEASE? Because sickle cell disease affects blood, and blood travels to every part of the body, problems can occur almost anywhere in the body. The problems caused by sickle cell disease are a result of the damage caused by the sickle-shaped red blood cells and the chronic anemia caused by hemolysis. These problems can be acute , which means they have a rapid onset and are immediately obvious, or they can be chronic , which means they occur over time and are more subtle. People with sickle cell disease are at an increased risk for life-threatening infections. Sickle- shaped red blood cells can damage the spleen early in life. The spleen is an organ that helps to filter bacteria from the bloodstream and produces some white blood cells. The damaged spleen is no longer able to filter bacteria out of the blood. As a result, bacteria can travel through the blood and, in just a few hours, cause an overwhelming infection. These infections can cause shock and even death. Because of this, every time a person with sickle cell disease has a fever, it must be considered an emergency. You must contact a healthcare provider and seek medical attention immediately if your child has a fever. Your child will need laboratory work, including a blood culture and, possibly, a chest X ray. Your child will be given antibiotics either intravenously or by injection. Your child may be hospitalized or receive antibiotics in the clinic or emergency department. Acute Complications Infection and Fever It is impossible to know just by looking at your child whether their fever is caused by serious infection from bacteria or by another, less serious reason. However, bacteria can quickly and severely affect the body, which is why it must be assumed that the fever is caused by bacteria, and there should be no delay in seeking treatment. It is important to have a thermometer to check your child’s temperature. You must seek immediate medical attention for your child for any temperature greater than 101 °F or 38.3 °C. Splenic Sequestration Oxygen levels in the spleen are low, and the blood moves through it slowly. Because of this, red blood cells tend to sickle in the spleen. In some cases, this can result in a blockage of blood flow out of the spleen, and red blood cells and platelets can become trapped. Normally, the spleen is tucked under the left side of the rib cage. When blood becomes trapped, the spleen swells and there is a rapid fall in hemoglobin and platelets as the amount of blood circulating in the rest of the body decreases. This condition is known as splenic sequestration and is a medical emergency. Sequestration is most common in children with HbSS and HbS-ß0 thal. It can occur in the less severe diseases of HbSC and HbS-ß+ thal. Your child’s hematology team will show you how to recognize a swollen spleen. Your child may appear pale, have decreased energy, or complain of abdominal pain when their spleen becomes larger than normal. Often, splenic sequestration will happen repeatedly. Your child may need their spleen to be removed surgically in an operation called a splenectomy. If this is the case, your hematology team will discuss the risks and benefits a splenectomy.
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