Sickle-Cell Disease

Description

Symptoms

General Symptoms in Infants. In infants, symptoms do not usually appear until late in the baby's first year. Most commonly, they are the following:

• Fever.
• Swelling of the hands and feet.
• Pain in the chest, abdomen, limbs, and joints.
• Infants also may have nosebleeds and frequent upper respiratory infections.

General Symptoms in Childhood. After infancy, children develop the following symptoms:

• Pain is the most common complaint. It can be acute and severe or chronic, usually from orthopedic problems in the legs and low back.
• Anemia.
• Fatigue.
• Irritability.
• Jaundice (yellowish discoloration of the skin and eyes).
• Bedwetting.

Additional Symptoms in Adolescence or Adulthood. Symptoms of childhood continue in adolescence and adulthood. In addition, patients may experience the following:

• Delayed puberty (in young teenagers).
• Severe joint pain.
• Progressive anemia.
• Leg sores.
• Gum disease.

Sickle-Cell Crisis

The hallmark of sickle-cell anemia is a group of devastating symptoms known collectively as a sickle-cell crisis (also sometimes known as a vaso-occlusive crisis). Sickle-cell crises are episodes of pain that occur with varying frequency and severity in different patients and are usually followed by periods of remission. Severe sickle cell pain has been described as equivalent to cancer pain and more severe than postsurgical pain. It most commonly occurs in the lower back, leg, abdomen, and chest, usually in two or more locations. Episodes usually recur in the same areas.

The risk for a sickle-cell crisis is increased by any activity that boosts the body's requirement for oxygen, such as illness, physical stress, or being at high altitudes. In more than half the cases, however, the trigger is unknown. Acute chest syndrome is a particularly serious complication of sickle-cell crisis. It occurs in the lungs and can be extremely serious and even life threatening.