Sickle cell anemia - Sickle cell disease

Article
2021-02-03

Also known as sickle cell anemia, hemoglobin S or SS disease, sickle disease due to hemoglobin S

Sickle cell anemia is a group of inherited disorders of the red blood cells that affect hemoglobin, the protein that carries oxygen around the body. Normally, red blood cells are disc-shaped and flexible to move easily through the blood vessels. If you have sickle cell disease, your red blood cells are crescent or sickle-shaped. These cells do not bend and move easily and can block blood flow to the rest of your body.

The blocked flow of blood throughout the body can lead to serious problems, including stroke, eye problems, infections and episodes of pain called pain crises. Having sickle cell disease also increases your risk of serious illness from COVID-19. Learn the steps you can take to prevent infection external link from the Centers for Disease Control and Prevention.

Sickle cell disease is a lifelong disease. A blood and bone marrow transplant is currently the only cure for sickle cell disease, but there are effective treatments that can reduce symptoms and extend life.

Your healthcare team will work with you on a treatment plan to reduce your symptoms and manage the condition. The NHLBI leads and supports research and clinical trials to find a cure for sickle cell disease.

Symptoms

If a person has sickle cell disease, it is present at birth. But most newborns have no problems with the disease until they are about 5 or 6 months old.

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

Early signs and symptoms

Early symptoms of sickle cell disease can include:

A yellowish color of the skin, known as jaundice, or whites of the eyes, known as jaundice, which occurs when a large number of red blood cells undergo hemolysis
Fatigue or agitation from anemia
Painful swelling of the hands and feet known as dactylitis

Complications

Complications from sickle cell disease include:

Acute breast syndrome. Sickling in the blood vessels of the lungs can deprive the lungs of oxygen. This can damage lung tissue and cause chest pain, fever, and difficulty breathing. Acute breast 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 place at the same time. Common areas of pain include the abdomen, chest, lower back, or arms and legs. A crisis can be triggered by high altitude, dehydration, illness, stress, or temperature changes. Often someone does not know what is causing the crisis.
Chronic pain. Chronic pain is common but can be difficult to describe, but it usually differs from crisis pain or the pain that results from organ damage.
Delayed growth and puberty. Children with sickle cell disease can grow from anemia and develop more slowly than their peers. They will reach full sexual maturity, but this can be delayed.
Eye problems. Sickle cell disease can damage blood vessels in the eye, usually in the retina. Blood vessels in the retina can overgrow, become blocked or bleed. This can cause the retina to detach, meaning it is lifted or pulled out of 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 is then broken down into a substance called bilirubin. Bilirubin can form stones called gallstones that become trapped in the gallbladder. The gallbladder is a small bag-shaped organ under the liver that aids in digestion.
Heart problems, including coronary artery disease and pulmonary hypertension. Frequent blood transfusions can also cause heart damage from iron overload.
Infections. The spleen is important for infectionprotection against certain types of infections. If you have sickle cell disease, a damaged spleen increases your risk of certain infections, including chlamydia, haemophilus influenzae type B, salmonella, and staph.
Joint problems. Disease in the hipbones and, less commonly, the shoulder joints, knees and ankles, can reduce oxygen supply and result in a condition called avascular or aseptic necrosis that seriously damages the joints. Symptoms include pain and problems with walking and joint movement. Over time, you may need pain medication, surgery, or joint replacement.
Kidney problems. Sickle cell disease can cause the kidneys to struggle to make urine as concentrated as possible. This can cause you to urinate often and to bedwetting or uncontrolled urination at night. This often starts in childhood.
Leg ulcers. Sickle cell ulcers are sores that usually start small and then get bigger and bigger. Some sores heal quickly, but others may not heal and can last for a long time. Some sores come back after healing. People with sickle cell disease usually don't develop an ulcer until after the age of 10.
Liver problems. Sickle cell intrahepatic cholestasis is an uncommon but serious form of liver damage that occurs when sickle-shaped red blood cells block blood vessels in the liver. This blockage prevents enough oxygen from reaching the liver tissue. These episodes are usually sudden and can occur more than once. Children often recover, but some adults can 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 of high blood pressure and blood clots in women with sickle cell anemia. The condition also increases the risk of miscarriage, premature birth, and low birth weight babies. Read more about pregnancy and sickle cell anemia.
Priapism. Priapism is an unwanted, sometimes long-lasting, painful erection. This happens when the blood from the erect penis is blocked by sickle cells. Over time, priapism can cause permanent damage to the penis and lead to impotence. Priapism that lasts longer than 4 hours is a medical emergency.
Severe anemia. People with sickle cell anemia usually have mild to moderate anemia. However, sometimes 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 any outward signs of a stroke. This injury is common and can be detected on Magnetic Resonance Imaging (MRI) scans. Silent brain injuries can lead to difficulties in learning, making decisions, or holding on to a job.

Serious anemia complications

People with sickle cell anemia usually also have mild to moderate anemia. However, sometimes they can have severe anemia. Severe anemia can be life-threatening. Severe anemia in newborns or children with sickle cell anemia can result from:

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 making new red blood cells for a while, which can lead to severe anemia.
Spleen storage crisis. Your spleen filters your blood and destroys old blood cells. In people with sickle cell anemia, red blood cells can become trapped in the spleen, making it larger than normal quickly. With red blood cells stuck in the spleen, there are fewer available to circulate in the blood, and this can lead to severe anemia. A large spleen can also cause pain on the left side of the abdomen. A parent can usually feel a spleen that is larger than normal in a child's abdomen.

Aplastic crisis and splenic sequestration crisis are most common in newborns and children with sickle cell disease. Adults with sickle cell disease can also experience episodes of severe anemia, but these usually have other causes

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