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What are the signs and symptoms of sickle cell disease?

Anemia is the most common feature of sickle cell disease. Anemia may cause fatigue, paleness, yellowing of the skin and eyes (jaundice), or shortness of breath.

Pain is the most common symptom of sickle cell disease. Some people have few or no severe pain episodes a year while others have 15 or more. Pain may last a few hours to a few days, and, in some cases, it can last for weeks. Some people have chronic, almost daily pain.

The pain occurs in organs or joints and results from tissue damage caused when sickle cells block blood flow. Mild pain episodes may be treated with over-the-counter pain medications such as acetaminophen and ibuprofen. Pain that is severe may require treatment in the hospital with strong pain medicines given intravenously (into a vein).

Complications of sickle cell disease may be acute or or chronic. Acute complications include:

* Infections. Because repeated red cell sickling within the spleen causes it to lose the ability to remove bacteria from the blood, infants and young children are susceptible to blood infections, pneumonia, meningitis, and bone and other serious infections. Young children are given twice-daily penicillin and vaccines to prevent serious infection and death. In regions where malaria exists, it is the leading serious infection in young children and must be prevented with medications and other measures.
* Acute severe anemia. This may include splenic sequestration, transient red cell aplasia and hyperhemolysis. In this condition, the regular anemia becomes even more severe and life-threatening.
* Hand-foot syndrome. Swollen hands or feet may be the first symptom of sickle cell disease in infants and should be evaluated by a doctor immediately.
* Acute chest syndrome. This life-threatening complication of sickle cell disease is caused by trapped sickle cells or infection in the lung. People who have chest pain and fever must be seen by a doctor immediately.
* Stroke. Sickle cells damage and block blood vessels that supply blood in the brain; this may result in a stroke. About 10 percent of children with sickle cell disease develop frank stroke. Another 20 percent develop scars in the brain before or without frank stroke. The damage to brain tissue can cause learning problems and disabilities. Doctors can sometimes identify children who are at increased risk of stroke by using a special type of ultrasound test.
* Priapism. This refers to painful and unwanted erections, which occur because sickled red blood cells cause blood to be trapped in the vessels of the penis. Immediate medical attention is needed when the erection lasts for more than an hour. Recurrent priapism can lead to erectile dysfunction.

Chronic complications include:

* Gallstones. These stones form in the gallbladder when there is too much of a substance called bilrubin in the body; bilirubin is made when red blood cells are destroyed. People with gallstones may have pain episodes in the upper right side of the belly, under the right shoulder or between the shoulder blades. Fatty foods may trigger gallstone attacks.
* Sores (ulcers) on the legs. Leg sores occur more often in males than females and usually appear between ages 10 and 50. These ulcers take a long time to heal and require long-term care.
* Pulmonary hypertension. This is high blood pressure of the main artery to the lungs (pulmonary artery); this artery carries blood from the heart to the lungs where it becomes oxygenated. Pulmonary hyptertension is a common complication of adults with sickle cell disease and has become more widely recognized in children. Early diagnosis and treatment may reverse or prevent progress of this condition.
* Joint disease. Aseptic necrosis (death of tissue not related to infection) of bone is the most common problem of bones and joints related to sickle cell disease. It is caused by sickle cells blocking blood flow. This condition can begin in childhood but is more common later in life. Chronic transfusion may be used to retard progress of joint...

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