What Are the Signs and Symptoms of Sickle Cell Anemia?

The signs and symptoms of sickle cell anemia are different in each person. Some people have mild symptoms. Others have very severe symptoms and are often hospitalized for treatment. Although sickle cell anemia is present at birth, many infants don’t show any signs until after 4 months of age.

The most common signs and symptoms are linked to anemia and pain. Other signs and symptoms are linked to some of the complications of the condition.

Anemia

The general signs and symptoms of anemia are fatigue (tiredness), pale skin and nail beds, jaundice (yellowing of the skin and eyes), and shortness of breath.

Pain (Sickle Cell Crisis)

Sudden episodes of pain throughout the body are a common symptom of sickle cell anemia and are often referred to as “sickle cell crises.”

A sickle cell crisis occurs when the red blood cells sickle (become “C” shaped) and stick together in clumps. The clumps block the flow of blood through the small blood vessels (capillaries) in the limbs and organs.

Sickle crises can cause acute or chronic pain. Acute pain is the most common type. This is sudden pain that can range from mild to very severe. The pain usually lasts from hours to a few days. Chronic pain usually lasts for weeks to months. Chronic pain can be hard to bear and mentally draining. This pain may severely limit daily activities.

Almost all people with sickle cell anemia have painful crises at some point in their lives. Some have a crisis less than once a year. Others may have 15 or more crises in a year.

Many factors can contribute to a sickle cell crisis. Often, more than one factor is involved and the exact cause can’t be identified. Factors that occur in your body and aren’t under your control can cause a sickle cell crisis, such as an infection.

Factors that you can control also can affect whether you have a sickle cell crisis. For example, dehydration (when your body doesn’t have enough fluid) can increase your chances of having a sickle cell crisis. Drinking plenty of fluids so your body is hydrated can often help decrease the chance of a crisis.

The most common sites affected by sickle cell crises are the bones, lungs, abdomen, and joints. The blocked blood flow can cause pain and organ damage.

Complications of Sickle Cell Anemia

Complications of sickle cell anemia come from the effects of sickle cell crises on different parts of the body.

Hand-Foot Syndrome

When sickle cells block the small blood vessels in the hands or feet, pain and swelling along with fever can occur. One or both hands and/or feet may be affected at the same time. Pain may be felt in the many bones of the hands and feet. Swelling usually occurs on the back of the hands and feet and moves into the fingers and toes. This may be the first sign of sickle cell anemia in infants.

Splenic (Sequestration) Crisis

The spleen is an organ in the abdomen that filters out abnormal red blood cells and helps fight infection. Sometimes, the spleen traps many cells that should be in the bloodstream and it grows large. This causes anemia. Blood transfusions may be needed until the body can make more cells and recover. If the spleen becomes too clogged with sickle cells, it can’t work normally. It begins to shrink and stop working.

Infections

Both children and adults with sickle cell anemia have a hard time fighting infections. Sickle cell anemia can damage the spleen.

Infants and young children with a damaged spleen are more likely to get infections that can kill them within hours or days. Pneumonia is the most common cause of death in young children who have sickle cell anemia. Meningitis, influenza, and hepatitis are other infections that are common in people with sickle cell anemia.

Acute Chest Syndrome

Acute chest syndrome is a life-threatening condition linked to sickle cell anemia. It's similar to pneumonia and is caused by an infection or by sickle cells trapped in the lungs. People with this condition usually have chest pain, fever, and an abnormal chest x ray. Over time, lung damage may lead to pulmonary arterial hypertension (see below).

Delayed Growth and Puberty in Children

Children with sickle cell anemia often grow more slowly and reach puberty later than other children. A shortage of red blood cells (anemia) causes the slow growth rate. Adults with sickle cell anemia often are slender or small in size.

Stroke

Sickle-shaped red blood cells may stick to the walls of the tiny blood vessels in the brain. This can cause a stroke. This type of stroke occurs mainly in children. The stroke can cause learning disabilities or more severe problems.

Eye Problems

The retina, a thin layer of tissue at the back of the eye, takes the images you see and sends them to your brain. When the retina doesn’t get enough blood, it can weaken. A weak retina can cause serious problems, including blindness.

Priapism

Males with sickle cell anemia may have painful and unwanted erections called priapism (PRI-a-pizm). This happens because the sickle cells stop blood flow out of an erect penis. Over time, priapism can damage the penis and lead to impotence.

Gallstones

When red blood cells die, they release their hemoglobin, which the body breaks down into a compound called bilirubin. When there is too much bilirubin in the body, stones can form in the gallbladder.

Gallstones can cause steady pain that lasts for 30 minutes or more in the upper right side of the belly, under the right shoulder, or between the shoulder blades. The pain may happen after eating fatty meals. People with gallstones may have nausea, vomiting, fever, sweating, chills, clay-colored stool, or jaundice (yellowish color of the skin or whites of the eyes).

Ulcers on the Legs

Sickle cell ulcers (sores) usually begin as small, raised, crusted sores on the lower third of the leg. Leg sores occur more often in males than in females and usually appear between the ages of 10 and 50. The cause of leg ulcers is not clear. The number of ulcers can vary from one to many. Some heal rapidly, but others persist for years or come back after healing.

Pulmonary Arterial Hypertension (High Blood Pressure)

Damage to the small blood vessels in the lungs makes it hard for the heart to pump blood through the lungs. This causes blood pressure in the lungs to increase. This condition is called pulmonary arterial hypertension. Excessive shortness of breath is an important symptom linked to this problem.

Multiple Organ Failure

Multiple organ failure is rare, but serious. It happens when a person has a sickle cell crisis that causes two out of three major organs (lungs, liver, or kidney) to fail. Symptoms linked to this complication are a fever and changes in mental status such as sudden tiredness and loss of interest in your surroundings.