Complications and Treatments

People with sickle cell disease (SCD) start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each person and can range from mild to severe.

The reason that infants don’t show symptoms at birth is because baby or fetal hemoglobin protects the red blood cells from sickling. When the infant if around 4 to 5 months of age, the baby or fetal hemoglobin is replaced by adult hemoglobin and the cells begin to sickle.

There is no single best treatment for all people with SCD. Treatment options are different for each person depending on the symptoms.

 

Hand-Foot Syndrome

 

Swelling in the hands and feet usually is the first symptom of SCD. This swelling, 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

The most common treatments for swelling in the hands and the feet are pain medicine and an increase in fluids, such as water.

 

Pain "Episode" or "Crisis"

Pain is the most common complication of SCD, and the top reason that people with SCD 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 SCD 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 or situations that expose you to high altitudes (for example, flying, mountain climbing, or cities with a high altitude).
• Try to avoid places or situations that expose you to low oxygen levels (for example, mountain climbing or exercising extremely hard, such as in military boot camp or when training for an athletic competition).
• Adults with severe SCD 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

Most pain related to SCD can be treated with over the counter pain medications such as ibuprofen and aspirin.  Some people who have severe crises are given opioid (i.e. morphine) medications daily, along with additional pain medication.

 

Anemia

Anemia is a very common complication of SCD. With SCD, 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
  
  
• A 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. A sudden worsening of anemia resulting from infection or enlargement of the spleen is a common reason for a transfusion.

 

Infection

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

Prevention

Vaccines can protect against harmful infections.

• Babies and children with SCD should have all of the regular childhood vaccines, 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.

• In addition, children with SCD 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.
  
  
• Adults with SCD should have the flu vaccine every year, as well as the pneumococcal vaccine and any others recommended by a doctor.

See a tip sheet on how to help prevent infection »

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 SCD to see a doctor. Early treatment of infection can help prevent problems.

 

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 SCD 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 SCD 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 SCD 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.