Sickle cell disease (SCD) is an inherited health condition that slows the flow of red blood cells in your body. For people with SCD, hemoglobin S (a mutated protein that transports oxygen) causes red blood cells to become curved (sickle-shaped) and stiff. This disrupts how easily red blood cells and the oxygen they carry can move through your blood vessels.¹

In SCD, sickle-shaped cells clump together and block small blood vessels, leading to organ damage and other health complications. Common symptoms include severe pain, fatigue, and frequent infections. There is no cure for sickle cell disease, but there are treatment options that can reduce complications.²

This type of blood disorder disproportionally affects people of African descent. In the United States, about one in 365 African American children is born with SCD.³ People of Hispanic, Middle Eastern, and Indian descent also have higher rates of SCD.⁴

Types

The type of SCD you have depends on which genes you inherited from one or both of your parents.^5 1

• Hemoglobin SS (HbSS): Also known as sickle cell anemia, this type of SCD comes from inheriting the hemoglobin S gene (or sickle cell gene) from both parents.
• Hemoglobin SC (HbSC): This type involves having two different mutated hemoglobin genes, where one parent passes on the hemoglobin S gene and the other passes on the hemoglobin C gene (a type of mutated hemoglobin). HbSC is usually less severe than HbSS.
• HbS beta thalassemia: This type combines hemoglobin S with another blood condition called beta thalassemia. HbS comes in two forms—beta zero and beta plus. In beta zero, the body does not make healthy hemoglobin, which leads to more serious complications. Beta plus allows some healthy hemoglobin production, so symptoms are usually milder.

HbSS is the most common and severe form of sickle cell disease. Rarer types of sickle cell disease include HbSD, HbSE, and HbSO. They occur when a person inherits the hemoglobin S gene from one parent and a gene for another unusual type of hemoglobin, such as D, E, or O, from the other parent.¹

Symptoms

SCD symptoms vary depending on age, the type of SCD you have, and the frequency of treatment. Symptoms of SCD may be noticeable in infants as young as five to six months old. Still, symptoms aren't the same for everyone. Some people might experience severe symptoms requiring frequent hospitalizations, while others might have milder symptoms.⁶

Sickle cell disease symptoms may include:^6 7

• Pain in your joints, bones, and abdomen 
• Fatigue 
• Anemia (low red blood cell count) 
• Yellowing of the skin or eyes, known as jaundice
• Swollen hands and feet (dactylitis)
• Priapism (persistent and often painful erections)
• Frequent infections as a result of a damaged spleen
• Delayed growth in children

What Causes Sickle Cell Disease?

Sickle cell disease is an inherited condition typically passed down from parents to their children through genes. To have sickle cell disease, a person must be born with two sickle cell genes. The severity of the disease depends on how it is inherited.¹

Getting the trait from both parents ensures you will have sickle cell disease. However, if one parent passes down the hemoglobin S gene and the other does not, the child will only carry the sickle cell trait. Although carrying the sickle cell trait does not mean you have sickle cell disease, you could still pass it on to your children.¹

Risk Factors

Sickle cell disease can affect anyone, but people of African, Hispanic, and Middle Eastern descent have the highest risk of developing sickle cell disease. Sickle cell trait is also common in people from parts of Asia and India. Having two parents with the sickle cell trait increases your risk of having some form of SCD.⁷

How Is Sickle Cell Disease Diagnosed?

Sickle cell disease is generally diagnosed through a blood test. In the United States, newborns are routinely screened for SCD.⁸

To do this test, a healthcare provider takes a tiny drop of blood from the baby's heel. They send the blood to a lab to check for sickle cell trait and other potential preexisting conditions. They may perform another blood test when the baby is 3-6 months old.⁸

If you have symptoms or a family history of SCD, you can request that your primary healthcare provider perform a blood test to check for sickle-shaped cells. They may also use genetic testing to look for the specific gene that causes the condition. These tests can determine whether you have the disease or the trait and help identify the type of SCD.^8 9

Treatment

Although there's currently no widely available cure for sickle cell disease, a hematologist can help ease symptoms through different treatment options. A bone marrow transplant is a cure that works for some people, but is not as accessible for everyone. Some healthcare providers may use a red blood cell apheresis exchange to get rid of harmful red blood cells using a machine.^10 11

SCD is a condition that often becomes more severe over time. The main treatment goals are alleviating pain, reducing blood cells containing HbSS, minimizing organ damage, and preventing complications.^12 13

Medications

A few medications can help treat different aspects of sickle cell disease. For example, hydroxyurea (Hydrea) is a commonly used treatment that can help your body make more healthy red blood cells and reduce bouts of severe pain.¹⁴ Other U.S. Food and Drug Administration (FDA) approved medications to treat sickle cell disease include Oxbryta (voxelotor), Endari (L-glutamine), and Adakveo (crizanlizumab).¹⁵

Children with SCD may be prescribed antibiotics such as penicillin to reduce their risk of infections. Pain relievers like Tylenol (acetaminophen) and Advil (ibuprofen) can help manage mild to moderate pain. A healthcare provider may prescribe stronger prescription medicines if you are experiencing severe pain.¹²

Blood Transfusions

This treatment involves receiving donor blood to increase the amount of healthy red blood cells in your body. These healthy cells can carry oxygen more effectively, which helps prevent severe anemia. Blood transfusions can also lower the percentage of sickle-shaped cells in the blood, reducing the risk of other health complications like stroke.¹²

Bone Marrow Transplant

This procedure replaces damaged bone marrow—the spongy tissue inside bones where blood cells are made—with healthy marrow from a donor. It helps your body produce healthy red blood cells. However, the process of receiving a transplant can be long and complicated. It requires a well-matched donor (often a family member) and involves risks such as your body not reacting well to the transplant or infection.¹²

Gene Therapy

In December 2023, the FDA approved the first gene therapy to treat sickle cell disease. This treatment involves taking a person’s blood or bone marrow to a lab and altering red blood cells. The modified cells are then put back into the person's body. While this is an exciting breakthrough, it's still very new, and access is limited.¹⁶

Prevention

Since sickle cell disease is inherited, you cannot prevent yourself from getting it. If you’re planning on becoming a parent, you and your partner can choose to take a genetic test to see if you are carriers of any mutated genes that can lead to SCD. This can help determine the chances that you may pass on the condition to your child or have a child who’s a carrier.¹⁷

An internal medicine doctor or hematologist (a doctor specializing in blood disorders) can determine whether or not your child is likely to develop SCD even before birth. This involves genetic tests like examining the amniotic fluid (the fluid surrounding the fetus in the womb) or a small placenta sample. This test can be performed as early as eight to 10 weeks into the pregnancy.⁹

While there is not yet a cure for sickle cell disease, there are ways to reduce the severity of some symptoms. Steps you can take to alleviate pain, reduce infections, and the risk of other health conditions include the following:^17 11

• Stay hydrated
• Be mindful of hydration and protection in extreme heat or cold
• Keep up with vaccinations
• Follow a nutritious eating plan
• Exercise regularly
• Take medications as recommended by your healthcare provider
• Visit your hematologist and other specialists on your healthcare team for regular screenings
• Avoid high altitude environments (flying, mountain climbing, and more) when possible

Complications

As sickle-shaped cells move through your bloodstream, they can damage organs and tissues in your body.

Organ and tissue damage from SCD can cause serious complications, including:^2 18

• Acute chest syndrome: This is a severe lung complication where sickle cells block blood flow in the lungs, causing chest pain and trouble breathing.
• Avascular necrosis: When this occurs, bone tissue dies due to a lack of blood supply. It most often affects the hip joint.
• Blood clots: Sickle cells can cause blood to clot more quickly, blocking blood flow to different parts of your body. This can lead to deep vein thrombosis (DVT), when blood clots form in the large veins, usually in your legs.
• Kidney problems: Sickle cells can limit blood flow to your kidneys. This can lead to kidney damage or chronic kidney disease (CKD), making it difficult for your kidneys to clean your blood effectively.
• Liver issues: Your liver may become damaged from working overtime to replace sickled cells.
• Pulmonary hypertension: High blood pressure can occur due to blood vessel damage, making it hard for your heart to pump blood through your lungs. Pulmonary hypertension is a potentially life-threatening condition. 
• Splenic sequestration: Sickle cells can get trapped in the spleen, causing it to enlarge.  
• Stroke: Sickle cells block blood vessels and cut off oxygen to your brain.
• Vision loss: Sickle cells can damage blood vessels in the eye, leading to vision problems or blindness.

Living With Sickle Cell Disease

Sickle cell disease is a blood disorder where the altered protein hemoglobin S changes the shape of red blood cells into a sickle shape. This makes it more difficult for red blood cells to move throughout your body. This disruption in blood flow can cause intense pain, anemia, fatigue, and shortness of breath, among other symptoms. More severe cases may result in organ damage and blurry vision.

Every person who carries the sickle cell trait will not automatically develop SCD. However, if both of your parents carry a gene for SCD, you are more likely to develop the disease. A hematologist can help you come up with a treatment plan for living with SCD. Although there is no widely available cure for SCD, there are various treatments and medications available to help manage symptoms.

Frequently Asked Questions

How long can someone with sickle cell disease live?

Many people with sickle cell disease live into their 50s, with some living even longer.¹⁹ However, on average, people with sickle cell disease typically live about 20 years less than the general population.²⁰

Does sickle cell disease get worse with age?

Some sickle cell disease complications may become more common or severe with age, but the disease can affect people differently.²¹

What organ does sickle cell disease damage?

Sickle cell disease can affect many organs in the body. The most commonly affected organs include the lungs, heart, kidneys, and liver. Sickle cells can block blood flow to these organs, causing damage over time.²²

SOURCES

1. Sickle Cell Disease Association of America. What is sickle cell disease (SCD)?.
2. Centers for Disease Control and Prevention. Complications of sickle cell disease. 
3. Thomson, Azalea M. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: A systematic analysis from the Global Burden of Disease Study 2021. Lancet Haematol. 2023;10(8):e585-e599. doi:10.1016/S2352-3026(23)00118-7
4. MedlinePlus. Sickle cell disease. 
5. Centers for Disease Control and Prevention. About sickle cell disease. 
6. National Heart, Lung, and Blood Institute. Sickle cell disease symptoms. 
7. American Society of Hematology. Sickle cell disease. 
8. National Heart, Lung, and Blood Institute. Sickle cell disease diagnosis. 
9. Merck Manual: Professional Version. Sickle cell disease. 
10. Children's Healthcare of Atlanta. Sickle cell disease: red blood cell exchange (apheresis).
11. National Heart, Lung, and Blood Institute. Living with sickle cell disease.  
12. National Heart, Lung, and Blood Institute. Sickle cell disease treatment. 
13. Sickle Cell Disease Association of America. Complications and treatments. 
14. MedlinePlus. Hydroxyurea. 
15. Pace BS, Starlard-Davenport A, Kutlar A. Sickle cell disease: Progress towards combination drug therapy. Br J Haematol. 2021;194(2):240-251. doi:10.1111/bjh.17312
16. U.S. Food & Drug Administration. FDA approves first gene therapies to treat patients with sickle cell disease. 
17. Centers for Disease Control and Prevention. Prevention and treatment of SCD complications.
18. Brandow AM, Liem RI. Advances in the diagnosis and treatment of sickle cell disease. J Hematol Oncol. 2022;15(1):20. doi:10.1186/s13045-022-01237-z
19. Jiao B, Johnson KM, Ramsey SD, Bender MA, Devine B, Basu A. Long-term survival with sickle cell disease: A nationwide cohort study of Medicare and Medicaid beneficiaries. Blood Adv. 2023;7(13):3276-3283. doi:10.1182/bloodadvances.2022009202
20. Kavanagh PL, Fasipe TA, Wun T. Sickle cell disease: A review. JAMA. 2022;328(1):57-68. doi:10.1001/jama.2022.10233
21. Idris IM, Botchwey EA, Hyacinth HI. Sickle cell disease as an accelerated aging syndrome. Exp Biol Med (Maywood). 2022;247(4):368-374. doi:10.1177/15353702211068522
22. National Heart, Lung, and Blood Institute. How sickle cell disease may affect your health.  

• Sickle cell disease causes your red blood cells to change into a crescent shape, which blocks blood flow.
• You can develop symptoms like anemia, pain, fatigue, jaundice, an increased risk of infections, and swelling in your hands and feet.
• Seek immediate medical attention if you have chest tightness, difficulty breathing, or a high fever.

Sickle cell disease (SCD) is a group of inherited red blood cell disorders that causes symptoms such as anemia, pain, fatigue, jaundice, an increased risk of infections, and swelling in your hands and feet. In some cases, this disease can worsen and cause more serious symptoms like high fever, problems with vision, and organ damage.¹²

This condition occurs due to a mutation in your hemoglobin. Hemoglobin is a type of protein in your red blood cells that carries oxygen through your body. Healthy red blood cells with normal hemoglobin are shaped like discs and are flexible enough to move through your blood vessels. In people with sickle cell disease, the hemoglobin doesn’t function properly, causing their red blood cells to change into a crescent-like shape and block the normal flow of blood.³

About 20 million people have SCD globally.³ People who inherit the sickle cell gene from both parents develop symptoms of the condition, while those who inherit the gene from just one parent have the sickle cell trait and do not develop the condition. Knowing the signs of this condition can help you understand when to seek care from your healthcare provider and get started on treatment sooner.³

Common Symptoms

You are most at risk of developing sickle cell disease if both of your parents carry the sickle cell trait. Most people with this condition are born with the disease. However, infants do not show symptoms until they are five or six months of age.⁴

The severity of symptoms varies from person to person. Some people may have mild symptoms like body aches or swelling, while others experience more severe symptoms that may require hospitalization. 

Some common symptoms of SCD include:^4 5

• Anemia: Sickle cell disease can cause your red blood cells to break down prematurely, which can cause you to have fewer red blood cells than your body needs to function. As a result of anemia, you can also experience fatigue, weakness, and shortness of breath.
• Pain: Sickle-shaped red blood cells become stiff, which can make it difficult for blood to flow normally through small blood vessels in the abdomen and bones. This can cause chronic (or long-term) pain. 
• Jaundice: The rapid breakdown of a large number of red blood cells can lead to yellowing of the skin and eyes.
• Infections: SCD can increase your risk of other infections, such as Salmonella osteomyelitis (bone infection), meningitis (inflammation of the brain), and pneumococcal septicemia (blood poisoning). Without proper treatment, these infections can cause serious complications, such as death.
• Edema: This condition may also cause painful swelling in your hands and feet.

Severe Symptoms

Sickle cell disease can lead to various severe symptoms or complications that may arise at any age. More serious symptoms may include:¹

• High fever: Fever may be the first sign of sickle cell disease-related complications. A fever of 38.5 degrees Celsius or 101.3 degrees Fahrenheit or higher requires immediate attention from a healthcare provider.
• Acute chest syndrome: This condition occurs when sickle cells block the small blood vessels in the lungs, leading to a lack of blood flow to the lungs. The symptoms of acute chest syndrome are similar to pneumonia and include chest pain, difficulty in breathing, and cough.
• Spleen sequestration: The spleen is an organ that helps your body fight infections. SCD can cause your spleen to become enlarged, painful, and filled with blood. Spleen sequestration can affect anyone with sickle cell disease, but it is most common in children.
• Priapism: If the red blood cells in your penis become affected, you may also experience prolonged and painful erections.
• Stroke: This disease can interrupt blood flow to your brain and increase your risk of stroke.
• Avascular necrosis: A lack of blood supply due to sickle cell disease can lead to the death of your bone tissue, leading to pain in your joints and bones.
• Vision problems: Sickle cell disease can affect the blood vessels in your eyes, which may lead to problems such as sickled red blood cells blocking blood vessels of the eye, causing blurred vision, floaters, and sometimes blindness.
• Organ damage: Because SCD causes a lack of blood flow to your major organs, your body may experience heart, kidney, or lung failure in severe cases.

Symptoms in Children

Approximately 275,000 children are born with sickle cell disease each year. A few common symptoms of SCD in children are:^6 7

• Slow developmental growth
• Delayed puberty
• Irritability
• Crying persistently
• Swollen hands and feet
• Severe infections  

Children can also experience more serious symptoms similar to adults, such as spleen sequestration, acute chest syndrome, and stroke.⁸

When To Contact a Healthcare Provider

If you or your child experiences any symptoms of SCD or has the trait for the condition, it’s good practice to see your healthcare provider for proper testing. Experts recommend seeing a healthcare provider before eight weeks of age if your infant was born with SCD.⁹

It’s important to seek care from your provider urgently if you or your child is experiencing any of the following symptoms:¹⁰

• Chest tightness
• Difficulty breathing
• A fever above 101 degrees Fahrenheit 
• Abdominal swelling
• Sudden weakness
• Loss of movement
• Seizures
• Body aches

Frequently Asked Questions

What is the life expectancy of a person with sickle cell disease?

The average life expectancy of a person with sickle cell disease is 52.6 years. People assigned male at birth have a life expectancy of 49.3 years, and those assigned female at birth have a life expectancy of 55 years.

Do symptoms of sickle cell disease get worse as you age?

Yes, symptoms of sickle cell disease may worsen with age. You may experience additional complications such as pulmonary hypertension (high blood pressure in the lungs), leg sores, gallstones, and kidney damage.

What organs does sickle cell disease damage?

Sickle cell disease can damage all major organs of the body, including the heart, lungs, kidneys, spleen, liver, and brain.

SOURCES

1. Centers for Disease Control and Prevention. Complications of sickle cell disease.
2. MedlinePlus. Sickle cell disease.
3. Centers for Disease Control and Prevention. What is sickle cell disease?
4. National Heart, Lung, and Blood Institute. Sickle cell disease - symptoms.
5. MedlinePlus. Sickle cell disease.
6. Ajinkpang S, Anim-Boamah O, Bimpong KA, Kanton FJ, Pwavra JBP, Abdul-Mumin A. Sickle cell disease in children: knowledge and home-based management strategies among caregivers at a tertiary facility in northern ghana. Biomed Res Int. 2022;2022:3384813. doi:10.1155/2022/3384813.
7. National Organization for Rare Disorders. Sickle cell disease.
8. American Academy of Physical Medicine and Rehabilitation. Pediatric sickle cell disease.
9. National Heart, Lung, and Blood Institute. Sickle cell disease - treatment.
10. Centers for Disease Control and Prevention. Sickle cell disease emergency guide.