Chronic Complications Eye Problems
Bilirubin is a yellow substance found inside red blood cells. Because sickle-shaped red blood cells are fragile, they can break open easily and release bilirubin into the bloodstream, causing a yellow tint to the eyes that often is seen in people with sickle cell disease. The retina is the thin lining on the inside of the eye that receives images the eye sees and changes them into messages the brain can read. The blood vessels
of the retina are extremely tiny. Over time, the small blood vessels of the retina can become blocked by sickle-shaped red blood cells. To help the blood flow, the body creates new blood vessels to carry blood to the retina. However, these new vessels are weak and tend to break open, causing bleeding and damage to the retina, which is called sickle cell retinopathy . Sickle cell retinopathy has no symptoms in its early stages, which is when it is most easily treated by laser surgery. For this reason, children who have sickle cell disease should have yearly eye exams, beginning in kindergarten. Sickle cell retinopathy is most common in people with HbSC and in older adolescents and adults. Heart and Lung Complications The heart is a muscle. Its job is to pump blood to the lungs, which is where the blood picks up oxygen and carries it to all the tissues in the body. Because people with sickle cell disease have lower hemoglobin levels (anemia) and less oxygenated blood, their hearts must work harder. As a result, it is very common for them to have cardiomegaly , which is an enlarged heart. Individuals with sickle cell disease often are told that their hearts have a murmur , or unusual sound. These murmurs are most often the result of chronic anemia. Children with sickle cell disease can develop a condition known as pulmonary hypertension , once thought to only affect adults with sickle cell disease. Pulmonary hypertension is high blood pressure in the artery that supplies blood to the lung from the heart. Pressure builds up as this artery becomes damaged by sickle-shaped red blood cells and becomes narrower. This increased pressure makes the heart’s job much more difficult. The right side of the heart, which is the side that pumps blood into the lungs, can become enlarged and weak. When this happens, patients can experience shortness of breath and dizziness. These symptoms often are not felt until pulmonary hypertension has progressed. Fortunately, pulmonary hypertension can be detected early by a simple test called an echocardiogram . A visit to a heart specialist (cardiologist) may be needed to check for pulmonary hypertension in children and adults who have sickle cell disease. Conditions that affect the lungs and the amount of oxygen that comes into the body, such as asthma and sleep apnea, must be controlled in a person living with sickle cell disease to decrease long-term problems. If your child is diagnosed with one of these conditions, it is very important that he or she is under the care of the appropriate specialist (pulmonologist) and that all of the treatment advice is followed.
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