time. Fever is the first sign of infection. Because of this, fever is a lifelong medical emergency for people who have had their spleen surgically removed (splenectomy). If your child develops a fever after having a splenec- tomy, contact your child’s healthcare provider immediately. TRANSFUSION REACTIONS The immune system’s function is to fight off what it thinks is foreign. Although uncommon, transfusion reactions can happen when the body’s immune system considers the transfused blood to be foreign. These reactions are often mild, causing a rise in body temperature or hives. The reaction can be very severe, causing trouble breath- ing, a drop in blood pressure, and bleeding. A rare but serious reaction that can occur is a delayed transfusion reaction. The symptoms of this include back pain, jaundice (yellowing of the skin and eyes), and dark urine. A delayed transfusion reaction may occur 2 days to 2 weeks after transfusion. IRON OVERLOAD Iron overload occurs when too much iron accumulates in the body. Blood transfusions contain iron. With each blood transfusion, extra iron is added to the body. The extra iron is stored in the body’s organs, where it can cause damage to the tissue. Many of the complications of thalassemia are a result of the organ damage resulting from iron overload. The impact of this damage will be discussed in upcoming sections.
ENDOCRINE SYSTEM PROBLEMS The endocrine system includes special organs called glands. Glands produce hormones that are needed for different func- tions of the body, including growth and puberty. The endocrine system is very sensitive to the effects of iron overload. Chronic anemia and iron overload may lead to slow growth rates, and children with thalassemia major rarely reach normal adult height. Puberty may also be delayed in children with thalas- semia.
Damage from iron deposits can affect the pancreas, an organ of the endocrine system that releases the hormone insulin. Insulin manages the glucose, or sugar, in the body. Diabetes can develop when the pancreas is damaged from iron over- load. Diabetes results in the body being unable to produce insulin and manage glucose. Hormones released by organs of the endocrine system help form and maintain healthy bones. Along with bone marrow expansion, damage to these organs can lead to weakened bones. HEART PROBLEMS Many patients with thalassemia have heart-related problems that result from both the anemia and iron overload. Anemia causes the blood to have less oxygen, and the heart then has to work harder, which can lead to an en- larged heart. Iron overload can cause iron deposits in the heart that result in arrhythmias (abnormal heartbeats) and congestive heart failure (a decreased ability of the heart to pump blood). Pulmonary hypertension is a rare condition in which there is a gradual narrowing of the blood vessels in the lungs and those carrying blood from the heart to the lungs. This condition may occur in people with thalassemia as a result of chronic anemia and abnormal red blood cells. Pulmonary hypertension is more concerning in children with very low hemoglobin, especially if a splenectomy was done.
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