HISTORY. Most infants do not develop symptoms during the first 6 months because fetal Hb has a protective effect. The parents or the child may describe a history of lung infections or car-diomegaly (hypertrophy of the heart). Children with the disease may have a history of chronic fatigue, dyspnea, joint pain and swelling, and chest pain.

PHYSICAL EXAMINATION. The extent of the symptoms depends on the amount of Hb S that is present. The general signs are similar to the other types of hemolytic anemia (anemia as a result of destruction of RBCs): malaise, fatigue, pallor, jaundice, and irritability. Children begin to fall below the growth curve in height and weight at around 7 years, and puberty is usually delayed. They are often small for their age and may have narrow shoulders and hips, long extremities, and a curved spine. You may note jaundice and pale skin. Often, the children have heart rates that are faster than normal and heart murmurs; you may find a large liver and spleen. Eventually, all body systems, including the heart, lungs, central nervous system, kidneys, liver, bones and joints, skin, and eyes, are affected. The most severe problem is sickle cell crisis (Table 1).

• TABLE 1 Sickle Cell Crisis


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