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The sites most often affected by the blocking or stacking action of sickled cells are found in the lungs, liver, bone, muscles, brain, spleen, penis, eyes, and kidneys.

The immune system of a person with sickled cellsdramatically weakens. People with sickle cells arehighly susceptible to infections from certain forms of bacteria. Some of the most common infections are from flu viruses, pneumonia, and salmonella (a type of bacteria).

Severe pain is the most common of sickle cell disease emergencies (acute sickle cellcrises). A personmay not know what brought on the pain, but one or more of the following situations may have contributed to the start of the painful sickle crisis:

• Dehydration
  
  
• Infection
  
  
• Fever
  
  
• Hypoxia (decrease in oxygen to body tissue)
  
  
• Bleeding
  
  
• Cold exposure
  
  
• Drug and alcohol use
  
  
• Pregnancy and stress

Fourpatterns of an acute sickle cell crisis are now recognizable. They are based on the part of the body where the crisis occurs.

• Bone crisis: An acute or sudden pain in a bone can occur, usually in an arm or leg. The area may be tender. Common bones involved include the large bones in the arm or leg: the humerus, tibia, and femur. The same bone may be affected repeatedly in future episodes of bone crisis.
  
  
• Acute chest syndrome: Sudden acute chest pain with coughing up of blood can occur. Low-grade fevers can be present. The person is usually short of breath. If a cough is present, it often is nonproductive.Acute chest syndrome is common in a young person with sickle cell disease. Chronic (long-term) sickle cell lung disease develops with time because the acute and subacute lung crisis leads to scarred lungs and other problems.
  
  
• Abdominal crisis: The pain associated with the abdominal crisis of sickle cell disease is constant and sudden. It becomes unrelenting. The pain may or maynot be localized to any one area of the abdomen. Nausea, vomiting, and diarrhea may or may not occur.
  
  
• Joint crisis: Acute and painful joint crisis may develop without a significant traumatic history. Its focus iseither ina single jointor in multiple joints. Often the connecting bony parts of the joint are painful. Range of motion is often restricted because of the pain.

Many other organ systems are often injured orimpaired.

• Central nervous system: Two-thirds of all strokes in people with sickle cell disease occur in children, at an average age of8 years. About 10% of people with sickle cell disease have strokes or other brain bleeding when younger than 8-10 years. As the population ages, the incidence of these events also increases. Repeat strokes occur in two-thirds of all survivors within 3 years of the first stroke. Blood clots affect the large vessels in the brain. Bleeding may occur in the small vessels damaged by sickle cell disease.
  
  
• Eyes: The effectofsickle cell disease on the eyes comes from the increased viscosity, or "sludging," of blood and the narrowness of the eye's blood vessels. Retinopathy (disease of the retina in the eye) is common and causes problems with vision. Retinal detachment is frequent. Hyphemas, bleeding in the eye, occur at the same rate as the general population, but complications are more common because of the increased sickling effect that the waterlikefluid in the eye promotes.
  
  
• Kidneys: Some amount of kidney damage occurs in nearly every person with sickle cell disease.
  
  
• Genitals: Priapism (a constant erection of the penis) is common. It affects about 40% of all men with sickle cell disease. Severe episodes are a frequent cause of impotency.
  
  
• Infections: People with sickle cell disease have weakened immune systems and are at increased risk for developing infection, especially in the lungs, kidneys, bones, and central nervous system.
  
  
• Blood problems: People with sickle cell disease can develop anemia-a reduction in the number of red blood cells. Symptoms of anemia are shortness of breath (oxygen is not getting to tissues), lightheadedness, and fatigue.

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