Diagnosis:
Sickle cell-hemoglobin SS disease (disorder)
Spanish:
Haemoglobin disease SS-sickle cell disease (disorder)
Description
An inherited disease in which red blood cells become sickle-shaped. These deformed red blood cells die prematurely, causing a shortage of functioning red blood cells (anaemia), and can also obstruct the blood supply and cause painful crises (sickle cell crises).
Explanation
Normally, the round, flexible red blood cells move easily through the blood vessels. In sickle cell disease, the red blood cells are sickle-shaped or crescent-shaped. These stiff, sticky cells can get stuck in small blood vessels, which can slow or block the flow of blood and oxygen to different parts of the body.
Signs and symptoms of sickle cell disease usually appear around 5 months of age. They vary from person to person and change over time. Signs and symptoms may include the following:
- Anaemia. Sickle cells break down easily and die, leaving you with very few red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die within 10 to 20 days, resulting in a shortage of red blood cells (anaemia). Without enough red blood cells, your body cannot get enough oxygen, and this causes fatigue.
- Episodes of pain. Periodic episodes of pain, called pain crises, are an important symptom of sickle cell disease. Pain occurs when sickled red blood cells block blood flow in small blood vessels in the chest, abdomen and joints. Bone pain may also be present.
The pain varies in intensity and can last from a few hours to a few weeks. Some people have only a few pain crises a year. Others have a dozen pain crises a year, or more. A severe pain attack requires hospital admission.
Some adolescents and adults with sickle cell disease also have chronic pain, which can result from bone and joint damage, ulcers, and other causes. - Swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
- Frequent infections. Sickle cell can damage the spleen and make you more vulnerable to infections. Doctors usually give vaccines and antibiotics to babies and children with sickle cell disease to prevent life-threatening infections, such as pneumonia.
- Delayed growth or puberty. Red blood cells provide your body with oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children, and delay the onset of puberty in adolescents.
- Vision problems. The tiny blood vessels that supply the eyes can become clogged with sickle cells. This can damage the retina, the part of the eye that processes visual images, and cause vision problems.
Other acceptable terms
- Haemoglobin disease SS-sickle cell disease
- Sickle cell anaemia
- Sickle cell anaemia
- Sickle cell disease
- Sickle cell disease
- Haemoglobin S-S disease
- Homozygous hereditary haemoglobinopathy homozygous for haemoglobin S
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