• Hand-foot syndrome
• Pain "episode" or "crisis"
• Anemia
• Infection
• Acute chest syndrome
• Splenic sequestration
• Vision loss
• Leg ulcers
• Stroke
• Other

• Hand-foot syndrome
  This is usually the first symptom of sickle cell disease. Swelling in the hands and feet, often along with a fever, is caused by the sickle cells getting stuck in the blood vessels and blocking the flow of blood in and out of the hands and feet.
  
  Treatment: Pain medicine and fluids, such as water.
  
• Pain "episode" or "crisis"
  This is the most common complication, and the top reason that people with sickle cell disease go to the emergency room or hospital. When sickle cells travel through small blood vessels, they can get stuck and clog the blood flow. This causes pain that can start suddenly, be mild to severe, and can last for any length of time.
  
  Prevention: There are simple steps that people with sickle cell disease can take to help prevent and reduce the number of pain crises:
  
  • Drink plenty of water.
  • Try not to get too hot or too cold.
  • Try to avoid places with high altitudes (flying, mountain climbing, or cities with a high altitude).
  • Try to avoid places or situations with low oxygen (mountain climbing or exercising extremely hard, such as in military boot camp or when training for an athletic competition).
  • Adults with severe sickle cell disease can take a medicine called hydroxyurea to help reduce the number of pain crises.
  • People taking hydroxyurea must be checked often by a doctor because the medicine can cause serious side effects, including an increased risk of dangerous infections.
    
    Treatment: Pain medicine.
    

• Anemia
  This is a very common complication. With sickle cell disease, the red blood cells die early. This means there are not enough healthy red blood cells to carry oxygen throughout the body. When this happens, a person might have:

  Tiredness Irritability Dizziness and lightheadedness Fast heart rate Difficulty breathing Pale skin color Jaundice (yellow color to the skin and whites of the eyes) Slow growth Delayed puberty Treatment: Blood transfusions are used to treat severe anemia.	Fast Fact: Taking iron supplements will not help people with sickle cell disease. This type of anemia is not caused by too little iron in the blood; it's caused by not having enough red blood cells. In fact, taking iron supplements could harm a person with sickle cell disease because the extra iron builds up in the body and can cause damage to the organs.
  A sudden worsening of anemia resulting from infection or enlargement of the spleen is a common reason for a transfusion.

• Infection
  People with sickle cell disease, especially infants and children, are more at risk for harmful infections. Pneumonia is a leading cause of death in infants and young children with sickle cell disease.

  Prevention: Vaccinations can protect against harmful infections. Babies and children with sickle cell disease should have all of the regular childhood vaccinations, plus a few extra. The extra ones are:

  • Flu vaccine (influenza vaccine) every year after 6 months of age.
  • A special pneumococcal vaccine (called 23-valent pneumococcal vaccine) at 2 and 5 years of age.
  • Meningococcal vaccine, if recommended by a doctor.
    
    Click here for the regular childhood vaccination schedule. Don't forget to add the extra vaccines listed previously for children with sickle cell disease.
    
    Click here to find information for parents about vaccinations.
    
    Adults with sickle cell disease should have the flu vaccine every year, as well as the pneumococcal vaccine and any others recommended by a doctor.

  In addition, children with sickle cell disease should receive a daily dose of penicillin, an antibiotic medicine, to help prevent infections. This can begin at 2 months of age and continue until the child is at least 5 years of age.

Treatment: Infections are treated with antibiotic medicines and sometimes blood transfusions. At the first sign of an infection, such as a fever, it is important for people with sickle cell disease to see a doctor. Early treatment of infection can help prevent problems.

Click here for a tip sheet on how to help prevent infection.

• Acute chest syndrome
  This can be life-threatening and should be treated in a hospital. It is similar to pneumonia and symptoms include chest pain, coughing, difficulty breathing, and fever.
  
  Prevention: Adults with severe sickle cell disease can take a medicine called hydroxyurea to help prevent acute chest syndrome. People taking hydroxyurea must be watched closely because the medicine can cause serious side effects, including an increased risk of dangerous infections.
  
  A person who is on bed rest or has recently had surgery can use an incentive spirometer, also called "blow bottle", to help prevent acute chest syndrome.
  
  Treatment: Depending on the cause, treatment might include oxygen, medicine to treat an infection, medicine to open up blood vessels to improve blood flow, and blood transfusions.
  
• Splenic sequestration
  This can be life-threatening and should be treated in a hospital. It happens when a large number of sickle cells get trapped in the spleen and cause it to suddenly get large. Symptoms include sudden weakness, pale lips, fast breathing, extreme thirst, abdominal (belly) pain on the left side of body, and fast heart beat.
  
  Parents of a child with sickle cell disease should learn how to feel and measure the size of their child's spleen.
  
  Prevention: For those who have had a very severe, life-threatening episode of splenic sequestration or who have had many episodes in the past, it might be necessary to have regular blood transfusions or the spleen can be removed (called splenectomy) to stop it from happening again.
  
  Treatment: Treatment typically is a blood transfusion.
  
• Vision loss
  Vision loss, including blindness, can occur when blood vessels in the eye become blocked with sickle cells and the retina (the thin layer of tissue inside the back of the eye) gets damaged.
  
  Prevention: People with sickle cell disease should have their eyes checked every year to look for damage to the retina. If possible, this should be done by an eye doctor who specializes in diseases of the retina.
  
  Treatment: If the retina is damaged, laser treatment often can prevent further vision loss.
  
• Leg ulcers
  This usually occurs on the lower part of the leg. They happen more often in males than in females and usually appear from 10 through 50 years of age. The cause of leg ulcers is unclear.
  
  Treatment: Leg ulcers can be treated with medicated creams and ointments. Leg ulcers can be painful, and patients can be given strong pain medicine. Bed rest and keeping the leg (or legs) raised to reduce swelling is helpful, although not always possible.
  
• Stroke
  A stroke can happen if sickle cells get stuck in a blood vessel and clog blood flow to the brain. About 10% of children with sickle cell disease will have a stroke. Stroke can cause lifelong disabilities and learning problems.
  
  Prevention: Doctors can sometimes identify children who are at risk for stroke using a special type of exam called, "transcranial Doppler ultrasound." In some cases, a doctor might recommend frequent blood transfusions to help prevent a stroke. People who have frequent blood transfusions must be watched closely because there are serious side effects. For example, too much iron can build up in the body, causing life-threatening damage to the organs.
  
• Other possible complications
  
• Damage to body organs, tissues, or bones because not enough blood is flowing to the affected area(s).
• Gallstones.
• Painful erection of the penis, called priapism, that can last less than 2 hours or more than 4 hours. If it lasts more than 4 hours, the person should get urgent medical help. It can lead to impotence.
  
  
  

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Q Is there a cure?

A The only cure for SCD is bone marrow/stem cell transplant.

Bone marrow is a soft, fatty tissue inside the center of the bones where blood cells are made. A bone marrow/stem cell transplant is a procedure that takes healthy cells that form blood from one person - the donor - and puts them into someone whose bone marrow is not working properly.

Bone marrow/stem cell transplants are very risky, and can have serious side effects, including death. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister. Bone marrow/stem cell transplants are used only in cases of severe sickle cell disease for children who have minimal organ damage from the disease.

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Q Can a person "catch" sickle cell disease from someone who has it?

A No, a person cannot "catch" sickle cell disease from another person. Sickle cell disease is a genetic condition that is inherited when a child receives two sickle cell genes - one from each parent.

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Q Can a woman with sickle cell disease have a healthy pregnancy?

A Yes; however, women with sickle cell disease are more likely to have problems during pregnancy that can affect their health and the health of their unborn baby. During pregnancy, the disease can become more severe and pain episodes can occur more frequently. A pregnant woman with sickle cell disease is at a higher risk of preterm labor and of having a low birthweight baby. However, with early prenatal care and careful monitoring throughout pregnancy, women with sickle cell disease can have a healthy pregnancy.

During pregnancy, there is a test to find out if the baby will have sickle cell disease, sickle cell trait, or neither one. The test is usually done after the second month of pregnancy.

Women with sickle cell disease might want to see a genetic counselor to find information about the disease and the chances that sickle cell disease will be passed to the baby.