What Are the Signs and Symptoms of Sickle Cell Anemia?

The signs and symptoms of sickle cell anemia vary. Some people have mild symptoms. Others have very severe symptoms and often are hospitalized for treatment.

Sickle cell anemia is present at birth, but 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 the disease's complications.

Signs and Symptoms Related to Anemia

The most common symptom of anemia is fatigue (feeling tired or weak). Other signs and symptoms of anemia include:

• Shortness of breath
• Dizziness
• Headache
• Coldness in the hands and feet
• Pale skin
• Chest pain

Signs and Symptoms Related to Pain

Sudden pain throughout the body is a common symptom of sickle cell anemia. This pain is called a "sickle cell crisis." Sickle cell crises often affect the bones, lungs, abdomen, and joints.

A sickle cell crisis occurs when sickled red blood cells form clumps in the bloodstream. (Other cells also may play a role in this clumping process.) These clumps of cells block blood flow through the small blood vessels in the limbs and organs. This can cause pain and organ damage.

The pain from sickle cell crises can be acute or chronic, but acute pain is more common. Acute pain is sudden and can range from mild to very severe. The pain usually lasts from hours to a few days. Chronic pain often lasts for weeks to months. Chronic pain can be hard to bear and mentally draining. This pain may severely limit your daily activities.

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

Many factors can play a role in a sickle cell crisis. Often, more than one factor is involved and the exact cause isn't known. You can control some factors. For example, your risk for a sickle cell crisis increases if you're dehydrated (your body doesn't have enough fluid). Drinking plenty of fluids can lower your risk for a painful crisis. Other factors, such as an infection, you can't control.

Painful crises are the leading cause of emergency room visits and hospitalizations of people who have sickle cell anemia.

Complications of Sickle Cell Anemia

The effects of sickle cell crises on different parts of the body can cause a number of complications.

Hand-Foot Syndrome

Sickle cells can block the small blood vessels in the hands or feet. This condition is called hand-foot syndrome. It can lead to pain, swelling, and fever. One or both hands and/or feet may be affected at the same time.

You may feel the pain in the many bones of the hands and feet. Swelling often occurs on the back of the hands and feet and moves into the fingers and toes. Hand-foot syndrome may be the first sign of sickle cell anemia in infants.

Splenic Crisis

The spleen is an organ in the abdomen. Normally, it filters out abnormal red blood cells and helps fight infection. In some cases, the spleen may trap cells that should be in the bloodstream. This causes the spleen to grow large and leads to anemia.

If the spleen gets too clogged with sickle cells, it won't work right. This can cause the spleen to shrink. If this happens, you may need blood transfusions until your body can make more cells and recover.

Infections

Both children and adults who have sickle cell anemia have a hard time fighting infections. This is because sickle cell anemia can damage the spleen, an organ that helps fight infections.

Infants and young children who have damaged spleens 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 who have sickle cell anemia.

Acute Chest Syndrome

Acute chest syndrome is a life-threatening condition linked to sickle cell anemia. It's similar to pneumonia. The condition is caused by an infection or sickle cells trapped in the lungs.

People who have this condition usually have chest pain and fever. They also often have abnormal chest x ray results. Over time, lung damage from acute chest syndrome may lead to pulmonary arterial hypertension (PAH).

Pulmonary Arterial Hypertension

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 rise.

Increased blood pressure in the lungs is called pulmonary arterial hypertension, or PAH. Shortness of breath and problems with breathing are the main symptoms of PAH.

Delayed Growth and Puberty in Children

Children who have sickle cell anemia often grow more slowly than other children. They also reach puberty later. A shortage of red blood cells causes the slow growth rate. Adults who have sickle cell anemia often are slender or smaller in size than other adults.

Stroke

Two forms of stroke can occur in people who have sickle cell anemia. One form occurs when a blood vessel in the brain is blocked. The other form occurs when a blood vessel in the brain bursts.

A stroke can cause learning disabilities and/or lasting brain damage, long-term disability, paralysis (an inability to move), or death.

Eye Problems

Sickle cells also can clog the small blood vessels that deliver oxygen-rich blood to your eyes. This can damage the retinas-thin layers of tissue at the back of your eyes. The retinas take the images you see and send them to your brain.

Without enough blood, the retinas will weaken. This can cause serious problems, including blindness.

Priapism

Males who have sickle cell anemia may have painful and unwanted erections. This condition is called priapism (PRI-a-pizm). It happens because the sickle cells block 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. The body breaks down this protein into a compound called bilirubin. Too much bilirubin in the body can cause stones to form in the gallbladder.

Gallstones may 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 who have gallstones may have nausea (feeling sick to the stomach), vomiting, fever, sweating, chills, clay-colored stools, or jaundice (a 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. These sores usually appear between the ages of 10 and 50.

The cause of sickle cell ulcers isn't clear. The number of ulcers can vary from one to many. Some heal quickly, but others persist for years or come back after healing.

Multiple Organ Failure

Multiple organ failure is rare, but serious. It happens if you have a sickle cell crisis that causes two out of three major organs (lungs, liver, or kidney) to fail.

Symptoms of this complication are a fever and changes in mental status, such as sudden tiredness and loss of interest in your surroundings.