Signs of sickle cell anemia (Hb SS) usually begin a few months after birth. In some cases, signs may not appear until childhood.
In the United States sickle cell screening is done for all babies at birth. However this is not always accurate because in babies the fetal hemoglobin (HbF) will predominate but a small amount of sickle hemoglobin (HbS) may still be detected. A full work up should be done after the child reaches 6 months of age. This includes a hemoglobin electrophoresis to measure the different types of hemoglobin in the blood.
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Sickle cell anemia symptoms and the complications experienced will vary greatly from person to person, even within the same family.
Early signs of sickle cell include:
- Sleeping longer or more often
- Tiredness
- Difficulty breathing
- Pain or swelling in the hands or feet
- Cold hands or feet
- Pale skin
These signs can occur if the baby has a low number of red blood cells or if the sickle-shaped red blood cells stick together and block a blood vessel.
If your baby shows any of these signs, be sure to contact your baby’s doctor immediately.
Symptoms
Anemia. Sickle cells break apart easily and die, leaving patients without enough red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anemia).
Without enough red blood cells, the body can’t get the oxygen it needs to feel energized, causing fatigue.
Episodes of pain. Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain is caused when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. Pain can also occur in the bones.
The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain episodes. Others have a dozen or more crises a year. If a crisis is severe enough, there may be need for hospitalization.
Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers and other causes.
Painful 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 cells can damage an organ that fights infection (spleen), leaving patients more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
Delayed growth. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers. This is why sometimes sickle cell patients are noticeably smaller than their peers.
Vision problems. Tiny blood vessels that supply the eyes may become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images, leading to vision problems.
How is sickle cell anemia treated?
A number of different treatments are available for SCD. Treatment usually depends on the symptoms being experienced at the time.
- Rehydration with intravenous fluids helps red blood cells return to a normal state. The red blood cells are more likely to deform and assume the sickle shape when a patient is dehydrated.
- Treating underlying or associated infections is an important part of managing the crisis, as the stress of an infection can result in a sickle cell crisis. An infection may also result as a complication of a crisis.
- Blood transfusions improve transport of oxygen and nutrients as needed.
- Supplemental oxygen makes breathing easier and improves oxygen levels in the blood.
- Pain medication is used to relieve the pain during a sickle crisis. There may be need for over-the-counter drugs or strong prescription pain medication like morphine.
- Hydroxyurea (Droxia, Hydrea) helps to increase production of fetal hemoglobin. It may reduce the number of blood transfusions.
- Immunizations can help prevent infections. Sickle cell patients tend to have lower immunity.
- Bone marrow transplant has been used to treat sickle cell anemia. This treatment is highly specialized and comes with a lot of risk .
- Children younger than 16 years of age who have severe complications and have a matching donor are the best candidates.
Home care
There are things you can do at home to help with sickle cell symptoms:
- Use heating pads for pain relief.
- Take folic acid supplements.
- Eat an adequate amount of fruits, vegetables, and whole-wheat grains. This can help the body make more RBCs.
- Drink more water to reduce the chances of sickle cell crises.
- Exercise regularly and reduce stress to reduce crises, too.
- Contact your doctor immediately if you think you have any type of infection. Early treatment of an infection may prevent a full-blown crisis.
