Sickle Cell Disease - Signs, Symptoms, and Complications - Signs, Symptoms, and Complications

If a person has sickle cell disease, it is present at birth. But most newborns do not have any problems from the disease until they are about 5 or 6 months of age.

The symptoms of sickle cell disease can vary from person to person and can change over time. Over time, you may experience symptoms depending on how sickle cell disease affects your health.

Early signs and symptoms

Early symptoms of sickle cell disease may include:

• A yellowish color of the skin, known as jaundice, or whites of the eyes, known as icterus, that occurs when a large number of red cells undergo Hemolysis
• fatigue or fussiness from anemia
• Painful swelling of the hands and feet, known as dactylitis

Complications

Complications of sickle cell disease include:

• Acute chest syndrome. Sickling in blood vessels of the lungs can deprive lungs of oxygen. This can damage lung tissue and cause chest pain, fever, and difficulty breathing. Acute chest syndrome is a medical emergency.
• Acute pain crisis. Also known as sickle cell or vaso-occlusive crisis, this can happen without warning when sickle cells block blood flow. People describe this pain as sharp, intense, stabbing, or throbbing. Pain can strike almost anywhere in the body and in more than one spot at a time. Common areas affected by pain include the abdomen, chest, lower back, or arms and legs. A crisis can be brought on by high altitudes, dehydration, illness, stress, or temperature changes. Often a person does not know what triggers the crisis.
• Chronic pain. Chronic pain is common, but it can be difficult to describe, but it is usually different from crisis pain or the pain that results from organ damage.
• Delayed growth and puberty. Children who have sickle cell disease may grow and develop more slowly than their peers because of anemia. They will reach full sexual maturity, but this may be delayed.
• Eye problems. Sickle cell disease can injure blood vessels in the eye, most often in the retina. Blood vessels in the retina can overgrow, get blocked, or bleed. This can cause the retina to detach, which means it is lifted or pulled from its normal position. These problems can lead to vision loss.
• Gallstones. When red blood cells break down, in a process called hemolysis, they release hemoglobin. Hemoglobin then gets broken down into a substance called bilirubin. Bilirubin can form stones, called gallstones, that get stuck in the gallbladder. The gallbladder is a small sac-shaped organ beneath the liver that helps with digestion.
• Heart problems, including coronary heart disease and pulmonary hypertension. Frequent blood transfusions may also cause heart damage from iron overload.
• Infections. The spleen is important for protection against certain kinds of infections. If you have sickle cell disease, a damaged spleen raises the risk for certain infections, including chlamydia, haemophilus influenzae type B, salmonella, and staphylococcus.
• Joint problems. Sickling in the hip bones and, less commonly, the shoulder joints, knees, and ankles, can decrease oxygen flow and result in a condition called avascular or aseptic necrosis, which severely damages the joints. Symptoms include pain and problems with walking and joint movement. Over time, you may need pain medicines, surgery, or joint replacement.
• Kidney problems. Sickle cell disease may cause the kidneys to have trouble making the urine as concentrated as it should be. This may lead to a need to urinate often and to bedwetting or uncontrolled urination during the night. This often starts in childhood.
• Leg ulcers. Sickle cell ulcers are sores that usually start small and then get larger and larger. Some ulcers will heal quickly, but others may not heal and may last for long periods of time. Some ulcers come back after healing. People who have sickle cell disease usually do not get ulcers until after age 10.
• Liver problems. Sickle cell intrahepatic cholestasis is an uncommon but severe type of liver damage that happens when sickled red cells block blood vessels in the liver. This blockage prevents enough oxygen from reaching liver tissue. These episodes are usually sudden and may happen more than once. Children often recover, but some adults may have chronic problems that lead to liver failure. Frequent blood transfusions can lead to liver damage from iron overload.
• Pregnancy problems. Pregnancy can increase the risk for high blood pressure and blood clots in women who have sickle cell disease. The condition also increases the risk of miscarriage, premature birth, and low birth weight babies. Learn more about pregnancy and sickle cell disease.
• Priapism. Priapism is an unwanted, sometimes prolonged, painful erection. This happens when blood flow out of the erect penis is blocked by sickled cells. Over time, priapism can cause permanent damage to the penis and lead to impotence. Priapism that lasts for more than 4 hours is a medical emergency.
• Severe anemia. People who have sickle cell disease usually have mild to moderate anemia. At times, however, they can have severe anemia, which is life-threatening.
• Stroke or silent brain injury. Silent brain injury, also called silent stroke, is damage to the brain without showing outward signs of stroke. This injury is common and can be detected on magnetic resonance imaging (MRI) scans. Silent brain injury can lead to difficulty in learning, making decisions, or holding down a job.

Severe anemia complications

People who have sickle cell disease also usually have mild to moderate anemia. At times, however, they can have severe anemia. Severe anemia can be life-threatening. Severe anemia in a newborn or child who has sickle cell disease may be a result of:

• Aplastic crisis. Aplastic crisis is usually caused by a parvovirus B19 infection, also called fifth disease or slapped cheek syndrome. Parvovirus B19 is a very common infection, but in sickle cell disease, it can cause the bone marrow to stop producing new red cells for a while, leading to severe anemia.
• Splenic sequestration crisis. Your spleen filters your blood and destroys old blood cells. In people who have sickle cell disease, red blood cells may get trapped in the spleen, making it quickly grow larger than normal. With red blood cells trapped in the spleen, fewer are available to circulate in the blood, and this can lead to severe anemia. A large spleen may also cause pain in the left side of the belly. A parent can usually feel a spleen that is larger than normal in a child’s belly.

Aplastic crisis and splenic sequestration crisis most commonly occur in newborns and children who have sickle cell disease. Adults who have sickle cell disease may also experience episodes of severe anemia, but these usually have other causes. Babies and newborns who have severe anemia may not want to eat and may seem very sluggish.