this does not cause any serious problems. However, the red blood cells of children with sickle cell disease live only a short time, so parvovirus B19 infection can result in severe anemia (a decrease in hemoglobin). Because the bone marrow has stopped producing red blood cells, there are few young red blood cells. Stroke Over time, sickle cell disease may damage the blood vessels of the brain, making them narrower. Sickle-shaped red blood cells can block these damaged vessels and prevent oxygen from getting to the brain tissue, causing a stroke, which can result in permanent brain damage. A child who is having a stroke may have any of these symptoms: severe headache, weakness on one side, inability to speak or understand language, seizures, weakness in facial muscles (this makes one side of the face appear to droop or sag), change in vision, and trouble swallowing. A child may have a transient ischemic attack (TIA), which is like a stroke but with symptoms lasting less than 24 hours. If you suspect your child is having a stroke or TIA, seek immediate medical attention. The sooner medical treatment begins, the less damage the stroke can cause. Approximately 10% of children with HbSS and HbS-ß0 thal are at risk of suffering a serious neurological event (stroke). The average age for experiencing such an event is 8 years old. A child who has suffered one stroke or TIA has an extremely high risk of having another, possibly more serious, stroke or TIA. For this reason, regular red blood cell transfusions are started. The goal of regular red blood cell transfusions is to replace the child’s blood, which can sickle and cause further blockages, with non-sickled red blood cells (Hemoglobin A) from a donor. Transcranial Doppler (TCD) imaging is now used routinely as a screening tool to determine if a child is at risk for stroke. TCDs detect abnormal flow through vessels of the brain that have suffered damage from sickle-shaped red blood cells. An abnormal TCD result is a strong predictor of an increased risk of stroke. Children found to have this should be offered regular transfusions. Annual screening with TCD should begin at 2 years of age for children with HbSS and HbS-ß0 thal. Priapism Sickle-shaped red blood cells can block the blood from flowing out of the penis. The result is priapism , a long-lasting and unwanted erection of the penis that often is extremely painful. Priapism is a medical emergency, especially if it lasts more than 2 hours. It is important that caregivers and health professionals instruct boys to let someone know when they are experiencing priapism. If not treated, priapism may lead to an inability to have a normal erection in the future. Priapism treatments vary but may include intravenous fluids and pain medication. Sometimes blood transfusions and medications that open up blood vessels are needed. If these measures do not work, a surgeon trained in this field, known as a urologist, will use a surgical procedure to release the trapped blood. It is common for priapism to happen again. Preventive measures, such as taking hydroxyurea and medications that dilate, or open, blood vessels may need to be used on a daily basis. Other preventative home measures include drinking plenty of water, taking a warm shower or bath, and taking pain medications.
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