Sickle Cell Disease

Sickle cell disease is the most common red blood cell disorder that’s inherited (or passed from parent to child). More than 100,000 people in the United States and 20 million people around the world have SCD. It largely occurs in Black people and those with African ancestry. In fact, more than 90 percent of people in the U.S. with SCD are Black, according to the Centers for Disease Control and Prevention (CDC). SCD affects 1 in every 365 Black or African American babies born. 

In sickle cell disease, affected red blood cells (called sickle cells) contain an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein in red blood cells that delivers oxygen to the body when blood travels through blood vessels.

Hemoglobin S causes healthy, flexible red blood cells to harden, change shape, and clump together. This can lead to blockages in blood vessels that slow or completely stop the flow of blood. The result of these blockages is often severe pain, infections, vision problems, stroke, and other significant health issues. People with SCD have a reduced life expectancy.

Sickle cell disease also causes red blood cells to die much faster than healthy red blood cells. While the lifespan of a healthy red blood cell is around 120 days, sickle cells tend to die after 10 or 20 days. This forces the bone marrow to work harder to keep up with the demand for red blood cells, which places further stress on the body. (Bone marrow is the spongy center of bones where blood cells are made.)

Sickle cell disease is a lifelong condition, but treatment can help you manage SCD and avoid or delay serious complications. For some people, receiving a bone marrow transplant can cure the disease.

New gene therapies that increase production of healthy hemoglobin may also provide a cure for sickle cell disease (more on this below).

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There are multiple types of sickle cell disease. The type a person has depends on the genes they inherited from their parents. Genes are segments of DNA that contain the codes (or instructions) for the production of the body’s cells, including hemoglobin.

The most common types of sickle cell disease include:

Hemoglobin SS (HbSS): The most common type, HbSS makes up 65 percent of sickle cell disease cases. It is a severe form of SCD that occurs when a person inherits one hemoglobin S gene from each parent.

Hemoglobin SC (HbSC): HbSC is typically a milder form of sickle cell disease. It makes up around 25 percent of cases. HbSC occurs when a person inherits a hemoglobin S gene from one parent and a hemoglobin C gene from the other parent. Hemoglobin C is another abnormal type of hemoglobin.

Hemoglobin beta thalassemia (HbS): This type of SCD occurs when a person inherits a hemoglobin S gene from one parent and beta thalassemia from the other. Beta thalassemia is another abnormal type of hemoglobin. There are two subtypes of HbS:

• HbS beta 0: This subtype is typically severe and makes up around 2 percent of SCD cases.
• HbS beta+: This subtype is usually milder and makes up around 8 percent of SCD cases.

Rare types of sickle cell disease

Other types of sickle cell disease are more rare. They include:

• Hemoglobin SE (HbSE)
• Hemoglobin SD (HbSD)
• Hemoglobin SO (HbSO)  

Someone with one of these types of sickle cell disease inherits another abnormal hemoglobin variant (E, D, or O) from one parent and a hemoglobin S gene from the other parent.

What is sickle cell trait?

Sickle cell trait occurs when a person inherits a hemoglobin S gene from one parent and a healthy hemoglobin gene from the other. Around 1 in 13 African American or Black babies is born with sickle cell trait, according to the CDC. The condition is estimated to affect between 1 and 3 million people in the U.S. and 8 to 10 percent of African Americans.

Most people with sickle cell trait lead healthy lives without any related symptoms. Occasionally, the condition may cause blood in urine. In rare cases, factors like intense physical activity, severe dehydration, and high altitude can alter the shape of red blood cells and lead to serious medical complications. These may include:

• Lowered blood supply to the spleen (known as splenic infarction)
• Pressure in the eye (glaucoma) following an eye injury
• Breakdown of muscle (also called rhabdomyolysis)
• A rare type of kidney cancer (known as renal medullary carcinoma)  

If you have sickle cell trait, you’re not at risk of developing sickle cell disease. This is true even if you experience uncommon sickle cell trait complications. However, if you and your partner have sickle cell trait, your children will have a 25 percent chance of being born with SCD.

Sickle cell disease vs. sickle cell anemia  

The term “sickle cell disease” can be used to describe all forms of the condition. Sickle cell anemia typically refers to sickle cell diseases that are more severe, including hemoglobin SS and hemoglobin beta 0 thalassemia.  

Anemia is a medical condition that occurs when you don’t have enough healthy red blood cells in your body. It’s also a major symptom of sickle cell disease.

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Sickle cell disease can affect various parts of the body and cause a wide range of symptoms. These may differ from person to person and evolve over time. Signs of SCD first develop around 5 or 6 months of age in many cases.

The most common signs and symptoms of sickle cell disease include:

Anemia: A shortage of healthy red blood cells is called anemia. This condition may cause fatigue, shortness of breath, or dizziness. People with lighter skin tones may develop unusually pale skin. Yellowing of the skin or the white parts of the eyes may also occur with some types of anemia. This discoloration is known as jaundice.

Frequent infections: The spleen is a small organ located beneath the left side of your rib cage. It helps filter blood and prevent infections. Sickle cells can clog and damage the spleen, which can lead to more frequent infections. Having a fever is a common sign of an infection.

Swollen feet and hands: Hard sickle cells can cause pain and swelling by disrupting blood circulation around small bones, such as those located in the feet and hands. This problem (known as dactylitis or hand-foot syndrome) mostly occurs in children with SCD.

Trouble with vision: Eyes contain very small blood vessels that may become blocked due to SCD. Poor blood flow to the eyes can damage the retina and impair vision. (The retina is the part of the eye that converts light into electrical signals and creates images.) In severe cases, blindness may occur.

Delayed growth in children: Red blood cells play an important role in development by delivering essential oxygen and nutrients to the body. SCD disrupts healthy blood flow, causing growth delays or even delaying puberty (the life stage when children and teenagers become sexually mature).

What is a sickle cell crisis?

Also called pain crises, sickle cell crises are episodes of intense pain. They’re a frequent reason for hospitalization among people with sickle cell disease. 

A sickle cell crisis occurs when rigid sickle cells collect in small blood vessels and slow or completely block blood flow to a certain area of the body. It can happen anywhere in the body, though pain most often occurs in the chest, back, arms, and legs. This pain can vary in intensity and last for a few hours to a few days at a time. Infants and children with sickle cell disease may experience swollen toes and fingers.

Some people with sickle cell disease have sickle cell crises once or twice a year. Others may experience them monthly or on a more frequent basis. Other possible SCD-related causes of chronic (long-term) pain include ulcers (open sores) and swollen, damaged joints.

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Much of sickle cell disease treatment focuses on preventing complications. These may include:

Acute chest syndrome: This is a life-threatening condition that occurs when sickle cells prevent oxygen-rich blood from reaching the lungs. Acute chest syndrome may also result from a bacterial or viral infection.

Symptoms of acute chest syndrome include:

• Difficulty breathing
• Fever
• Chest pain
• Coughing

Emergency medical care is necessary to treat acute chest syndrome. Call 911 immediately or visit the nearest hospital emergency department (ED).

Stroke: A stroke is a medical emergency that can occur if sickle cells block blood flow to the brain. Some “silent” strokes don’t cause obvious symptoms but can result in brain damage that’s detectable on imaging tests.

A stroke can also cause sudden symptoms, such as:

• Confusion
• Weakness or numbness that may only affect one side of the body
• An intense headache
• Seizures
• Trouble speaking or understanding others
• Vision changes in one or both eyes
• Dizziness, poor balance, difficulty walking, or loss of coordination
• Loss of consciousness

Around 10 percent of children with sickle cell disease will experience a stroke with symptoms, according to the CDC. Call 911 immediately or go to the nearest hospital ED if you or someone around you is displaying signs of a stroke.  

Pulmonary hypertension: This refers to high blood pressure in the lungs. Adults with SCD have an increased risk of pulmonary hypertension, which can cause symptoms like:

• Shortness of breath
• Chest pain or discomfort
• Fatigue
• Dizziness or lightheadedness
• Abdominal swelling or swollen legs or arms

Call 911 immediately or go to the nearest ED if you or someone around you experiences symptoms of pulmonary hypertension.

Avascular necrosis: This condition occurs when small blood vessels that supply bones are blocked by sickle cells. Bones that don’t receive enough blood and oxygen can collapse and eventually die.

Avascular necrosis may occur in any bone, but most often affects the bones that make up the hip joint. The most common symptom of avascular necrosis is worsening pain around the affected area as the disease progresses.

Priapism: Sickle cells can reduce blood flow to the penis and cause painful, prolonged erections, which is known as priapism. Seek emergency medical care if you experience an erection that lasts two or more hours. If left untreated, priapism may lead to erectile dysfunction.

Splenic sequestration: Sickle cells can become lodged in the spleen and block blood flow, causing the organ to enlarge. This is known as splenic sequestration crisis. In addition to painful swelling around the upper left side of the abdomen, symptoms of splenic sequestration may include:

• Rapid heartbeat
• Pale lips
• Sudden weakness
• Rapid breathing

Splenic sequestration typically occurs in young children with sickle cell disease and requires emergency medical attention. If you’re a parent of a child with SCD, ask your healthcare provider (HCP) how you can feel your child’s abdomen to help detect spleen enlargement. 

Deep vein thrombosis: Sickle cell disease increases the risk of blood clots (jelly-like clumps that form when blood cells stick together) in veins deep within the body, such as in the pelvis or leg. This is known as deep vein thrombosis (DVT). Possible signs of DVT include swelling, red or discolored skin, and tenderness. Contact your HCP if these occur.

Sometimes, a blood clot can break free from its original location and become lodged in the lungs, causing a medical emergency called a pulmonary embolism. Call 911 or go to the nearest ED if you or someone around you experiences possible signs of a pulmonary embolism, including: 

• Trouble breathing
• Chest pain or tightness that worsens when coughing or breathing deeply
• Irregular or fast heartbeat
• Low blood pressure or lightheadedness
• A cough, sometimes with blood
• Loss of consciousness

Organ damage: Sickle cells can slow blood flow to and damage various organs throughout the body, such as the heart, lungs, liver, and kidneys. This can lead to the failure of one or multiple organs. Organ failure is a life-threatening issue that requires emergency medical care.

The signs and symptoms of organ damage will depend on what organs are affected, but may include:

• Irregular heartbeat
• Nausea
• Jaundice (yellowing of the skin and whites of eyes)
• Trouble breathing
• Swollen feet and hands

Gallstones: People with sickle cell disease have an increased risk of gallstones. These are pebble-like deposits of bilirubin that can form in the gallbladder (a small organ located below the liver). Bilirubin is a substance that’s released by red blood cells as they break down. If left untreated, gallstones can become a medical emergency.

Gallstones may not cause any noticeable symptoms. When they do, symptoms may include:

• Upper back pain
• Upper-right abdominal pain
• Nausea
• Indigestion
• Fever or chills

Leg ulcers: Sickle cell disease may lead to the formation of painful, open sores called ulcers on the legs.

These are not all the possible complications of sickle cell disease. Contact an HCP if you or your child experiences any symptoms or changes in health that concern you. 

Does sickle cell disease affect pregnancy?

Sickle cell disease can affect a person’s pregnancy. A pregnant person with SCD has an increased risk of blood clots, infections, high blood pressure (preeclampsia), miscarriage, and more frequent sickle cell crises. Anemia may also worsen during pregnancy. Possible risks for the developing baby include premature birth and low birth weight.

Still, many people with sickle cell disease go on to have healthy pregnancies. It’s essential for any pregnant person to seek and follow regular prenatal care (medical care designed for pregnant people).

If you have SCD and are pregnant or planning on becoming pregnant, reach out to your HCP. They can refer you to an obstetrician (a medical doctor who specializes in pregnancy and childbirth) or a maternal-fetal medicine specialist (an obstetrician who specializes in high-risk pregnancies).  

Can I pass sickle cell disease to my child?

Sickle cell disease is an inherited condition that you can pass to your baby. If you and your partner both have SCD, your baby will have SCD, too. If you and your partner both have sickle cell trait, your baby’s risk of SCD is around 25 percent.

Consider speaking with a genetic counselor if you’re concerned about your baby’s risk of sickle cell disease. A genetic counselor is an HCP who is specially trained in medical genetics (the study of genes and inherited conditions). They can explain how genetics influence health, perform tests to screen for SCD and sickle cell trait in you and your partner, and estimate your baby’s risk of developing sickle cell disorders.  

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Promptly contact a healthcare provider (HCP) if you or your child experience possible signs or symptoms of sickle cell disease. This might include frequent infections, swelling in the feet or hands, or signs of anemia, such as fatigue and cold hands or feet.

Some symptoms and complications of sickle cell disease require emergency medical care. Call 911 or go to the nearest ED if any of the following occurs:

• Chest pain
• Trouble breathing
• An erection that lasts more than two hours  
• Sudden confusion, weakness, changes in vision, difficulty walking or speaking, or numbness on one side of the body
• Irregular or rapid heartbeat
• Dizziness
• An intense headache
• A fever higher than 101.3 degrees Fahrenheit

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Sickle cell disease is a condition you’re born with. It occurs when someone inherits an abnormal hemoglobin gene from each parent. Sickle cell trait happens when someone inherits an abnormal hemoglobin gene from one parent and a healthy hemoglobin gene from the other. People with sickle cell trait are sometimes called sickle cell carriers, meaning they carry the “sickle cell gene.”

An inherited change (mutation) in the HBB gene that provides instructions for creating hemoglobin causes sickle cell disease and sickle cell trait. What exactly triggers this mutation is unclear, though environmental factors (those from one’s experience living in the world) might help provide an explanation.

Sickle cell disorders mostly affect people with ancestry from parts of the world where malaria is or was common. (Malaria is a disease of the blood that’s spread through the bite of an infected mosquito.) Research suggests that the shape of red blood cells in children with sickle cell trait makes it difficult for malaria to spread through the bloodstream. As a result, they’re less likely to experience severe cases of malaria.

The protective effects of sickle cell trait against malaria don’t apply to sickle cell disease, however, as people with SCD have an increased risk of death from malaria.

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Sickle cell disease runs in families. In the U.S., most people with SCD are African American or Black. SCD affects 1 in every 365 Black or African American babies born. Other populations are also affected by sickle cell disease. For example, the condition occurs in around 1 in every 16,300 Hispanic American babies born.

In general, sickle cell disease is most often seen in people whose ancestry can be traced to:

• Sub-Saharan Africa
• Saudi Arabia
• India
• Central America
• South America
• The Caribbean
• Mediterranean countries such as Turkey, Greece, and Italy  

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Sickle cell disease is usually diagnosed through a blood test. If you’re unsure if you carry the sickle cell gene, your HCP can perform a quick blood test or refer you to a genetic counselor for further guidance.

All babies born in the U.S. are tested for sickle cell disease and several other conditions during routine newborn screenings. During screening, the baby’s heel is pricked with a needle and a few drops of blood are collected for a type of lab test called hemoglobin electrophoresis. This blood test measures hemoglobin levels and checks for abnormal types of hemoglobin, including those involved in sickle cell trait and SCD.  

Developing fetuses can also be tested for sickle cell disease as early as the eighth or tenth week of pregnancy. During prenatal screening, a sample of tissue from the placenta or amniotic fluid is collected for testing. (The placenta is a temporary organ that connects the fetus to the womb. Amniotic fluid is the protective liquid that surrounds an embryo or fetus.) Rather than checking for abnormal hemoglobin, prenatal screening tests for the sickle cell gene itself.

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If you or your child has sickle cell disease, share your questions and concerns with an HCP. This can help you and your family make confident, informed decisions. Some common questions you may want to ask about SCD include:

• What type of sickle cell disease do I have?
• Why does sickle cell disease cause chronic pain?
• What is the cause of my symptoms? Are there any other possible causes?  
• What are my treatment options? Can you explain the risks and benefits of each?   
• Am I a candidate for a bone marrow transplant?
• What activities should I avoid or restrict?
• Should I make changes to my diet? What foods should I avoid or eat more of?  
• What healthy lifestyle choices can help me manage my condition?
• Am I at risk for sickle cell disease complications? If so, how can I lower my risk?
• How can I manage sickle cell disease with other health conditions?
• What is the outlook for my condition?  
• I’d like to become pregnant. Can you refer me to a genetic counselor?
• Can you suggest a sickle cell disease support group?
• Should I consider speaking with a licensed mental health provider?  

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Sickle cell disease can be cured with a bone marrow transplant, but most people with the condition aren’t candidates for this treatment. For many, the goals of SCD treatment are to:  

• Manage the disease
• Relieve symptoms
• Avoid or reduce the severity of sickle cell crises
• Prevent complications 

Sickle cell disease is primarily treated by hematologists, or medical doctors who specialize in disorders that affect blood and bone marrow. They often collaborate with other HCPs to provide comprehensive care. These HCPs include:  

• Primary care physicians (medical doctors who provide preventive health care and treat routine conditions)
• Pediatricians (medical doctors who specialize in children’s health)
• Cardiologists (medical doctors who treat heart and blood vessel disorders)
• Pulmonologists (medical doctors who specialize in lung health) 

Most sickle cell disease treatment plans include a combination of approaches. Depending on your age, symptoms, overall health, and other factors, your SCD treatment may involve:  

Medication

Several medications may be used to help control sickle cell disease symptoms and improve blood flow through the body.

Hydroxyurea: This daily oral medicine can lower the frequency of sickle cell crises, acute chest syndrome episodes, and episodes of dactylitis (swollen feet and hands). It may also help improve anemia and prevent hospitalizations in children and adults.

Possible side effects of hydroxyurea include low white blood cell count and increased infections. (White blood cells are immune system cells that help prevent illness and infection.) Pregnant people should not take hydroxyurea.

Voxelotor: This oral medicine helps improve blood flow to organs by preventing red blood cells from changing shape and sticking together. It also reduces the risk of anemia. Voxelotor is used in adults and children ages 4 years and older. Possible side effects include diarrhea, headache, fever, nausea, fatigue, and abdominal pain.

Crizanlizumab-tmca: This medicine is given intravenously (through an IV directly into a vein). It helps decrease the frequency of sickle cell crises. Used in adults and children ages 16 and older, crizanlizumab-tmca works by preventing blood cells from sticking to the inside of blood vessels and clogging them. Possible side effects include back pain, joint pain, fever, and nausea.

L-glutamine: Available as a powder that can be mixed into foods and drinks, L-glutamine helps decrease the frequency of sickle cell crises and hospitalizations. It’s used in people ages 5 and older. Possible side effects of L-glutamine include chest pain, fatigue, nausea, and pain in muscles or bones. 

Penicillin: This is an antibiotic that can help prevent serious infections in young children when taken twice daily. Penicillin is available in liquid and tablet form.

Many HCPs recommend children with sickle cell disease take penicillin until around age 5 or throughout their lives, depending on their health. People who have had a previous pneumococcus infection or a splenectomy (surgical removal of the spleen) should also continue taking penicillin. Possible side effects of penicillin include nausea, diarrhea, abdominal pain, and skin rash.  

Over-the-counter medications such as ibuprofen and acetaminophen may also be used to help control mild to moderate pain from sickle cell disease. Be sure to speak with your HCP before combining over-the-counter products with prescription medications.

Vaccines

If you have sickle cell disease, receiving routine vaccinations can help prevent serious infections and complications. Common infections such as influenza (the flu) and pneumonia can become life-threatening in people with SCD, especially children.  

Ask your HCP about what vaccines are recommended for you or your child. These will likely include vaccines that protect against:  

• Meningitis
• Hepatitis B
• Influenza
• Pneumonia  
• COVID-19

Your HCP will also advise you to take other steps to help prevent infections, such as thoroughly washing your hands, practicing safe food handling, and avoiding people who are sick.

Blood transfusion

Blood transfusions can help treat and prevent complications (including stroke) and relieve symptoms in people with sickle cell disease. During a transfusion, red blood cells from a healthy donor are given through an IV in the arm. This helps increase the amount of healthy red blood cells and dilutes the volume of sickle cells in the body. Treatment usually takes one to four hours, though emergency blood transfusions can be given more rapidly if needed.

Blood transfusions may be given on a routine basis to help prevent issues or in response to sudden complications related to sickle cell disease, such as acute chest syndrome or severe anemia. They’re also usually done before surgery to lower the risk of complications related to anesthesia in people with SCD. 

Risks associated with blood transfusions include infections and an unwanted immune response to donor blood. (When this happens, your body may reject blood provided by another person.) Iron overload (a buildup of iron in the blood) may also occur and damage organs if it’s left untreated.

Bone marrow transplant

A bone marrow transplant (BMT), also known as a stem cell transplant, can cure sickle cell disease in people who are candidates for treatment. Stem cells are cells that make blood cells. They are made in marrow, the soft, spongy tissue located in the center of bones.  

A bone marrow transplant involves replacing unhealthy stem cells with healthy stem cells from a carefully matched donor. People with sickle cell disease who are candidates for bone marrow transplantation are usually children with severe symptoms or complications.

Before a bone marrow transplant, chemotherapy is administered to destroy stem cells in a person’s body that produce abnormal hemoglobin. (Chemotherapy, or “chemo,” uses potent drugs to destroy certain types of cells in the body.) Healthy donor stem cells are then fed into a person’s body through an IV in the arm, similar to a blood transfusion. These stem cells travel to the bone marrow and work to create an adequate supply of healthy blood cells.

Finding a well-matched donor is key to a successful bone marrow transplant. Patients and donors must have the same type of human leukocyte antigen (HLA). HLA is a protein or “marker” found on most cells.  There are many types of HLA, and finding an exact match can be a complex process. In most cases, successful donors are siblings or other family members of patients. Just around 18 percent of people with sickle cell disease have matching donors.   

The process of receiving a bone marrow transplant can take several weeks or months and is followed by a few months of recovery. Treatment for sickle cell disease using a bone marrow transplant is successful in around 85 percent of cases when a genetically related donor with a matching HLA type is used.  

Bone marrow transplants come with several risks, including infections, seizures, and graft-versus-host disease. This is a serious condition in which transplanted stem cells attack the patient’s organs. Death has occurred in around 5 percent of bone marrow transplant patients.

Most bone marrow transplant-related complications occur in the first two years after treatment. The 10-year survival rate for bone marrow transplant patients who survive the first two years is 96 percent, according to a 2022 study published in Transplantation and Cellular Therapy.   

Researchers are working to improve the safety and accessibility of bone marrow transplant options for people with sickle cell disease. For example, some HCPs offer bone marrow transplants with gene therapy. In this treatment, stem cells are taken from an SCD patient who does not have a compatible donor. The stem cells are injected with a gene in a lab that creates healthy hemoglobin, then given back to the patient. The altered stem cells travel to the bone marrow, where they can begin to create healthy red blood cells.  

Gene therapy

Gene therapy is an emerging and promising treatment for sickle cell disease that works by changing or “editing” the DNA in a person’s stem cells. The goal is to produce healthy red blood cells, either by adding a healthy gene or correcting a faulty gene.

In December 2023, the Food and Drug Administration (FDA) approved two breakthrough gene therapies—Casgevy (exagamglogene autotemcel) and Lyfgenia (lovotibeglogene autotemcel)—for use in people 12 years and older with sickle cell disease. Clinical trials on gene therapies are ongoing, but research thus far shows that, when successful, these treatments can eliminate SCD symptoms and may cure the disease. Experts are working to make gene therapy for SCD more widely available.

Clinical trials

A clinical trial is a research study with voluntary human participation. Many clinical trials are underway with the goal of improving outcomes for people with sickle cell disease. Gene therapy and bone marrow transplantation are two major areas of focus.

Speak with your HCP to learn if you’re a candidate for a sickle cell disease clinical trial. They can help you weigh the pros and cons of clinical trial participation, which often involves evaluating new and unproved treatments. You can also browse active clinical trials on ClinicalTrials.gov or the National Heart, Lung, and Blood Institute website.

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Sickle cell disease is an inherited condition that can’t be prevented. If you’re pregnant or are planning on becoming pregnant, consider speaking with a genetic counselor about your risk of passing SCD to your child.

In addition to explaining how genetics influence health, a genetic counselor can also outline other reproductive options you may have, such as adoption or pursuing in vitro fertilization (IVF) with an egg or sperm from a donor. (IVF involves combining an egg and sperm in a lab, then implanting the resulting embryo directly into the uterus.)

IVF with pre-implantation genetic diagnosis (PGD) is another option for people with sickle cell disease or sickle cell trait who wish to have a child. With this method, embryos that have been prepared through IVF are tested for the gene associated with SCD before being implanted into the uterus.

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Sickle cell disease is a long-term condition that reduces life expectancy. Even for those who receive a bone marrow transplant (which often cures SCD), organ damage sustained before treatment and complications associated with the procedure can result in health challenges. Common causes of death among people with SCD include heart disease, stroke, liver disease, and infection.

A 2023 study published in Blood Advances found that the average life expectancy of patients with sickle cell disease was 52.6 years. (Specifically, these were patients who had public health insurance and who did not receive bone marrow transplants.) By comparison, the average life expectancy in the U.S. is 77.5 years. Another study, published in 2020 in Annals of Emergency Medicine, found the median age at death for Black people with SCD to be 43 years.

It's important to note that these figures reflect outcomes of people who died several years ago. Sickle cell disease outcomes vary according to the type of disease and treatment regimen, among other individualized factors. It’s very possible for someone with SCD to live beyond their 50s if they manage the disease well. Your HCP can provide you with a more accurate prognosis (an estimate of the course of the disease) and suggest ways to improve your health and quality of life.

Medical advances are steadily improving outcomes for people with sickle cell disease. The median age at death for Black people with SCD has risen 15 years since 1979. And between 1999 and 2002, SCD-related deaths among Black children younger than 4 years dropped 42 percent. This is likely due to the introduction in 2000 of a vaccine that protects against deadly pneumococcal infections, such as meningitis and sepsis. Overall, death rates among Black children with SCD younger than 5 fell 158 percent between 1979 and 2017.  

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It’s possible to enjoy a full, active life with sickle cell disease. Living well with SCD starts with managing the disease along with your overall health. Visiting your HCP regularly for preventive health checkups can allow them to identify potential issues early and provide appropriate treatment.

How often you should visit your HCP depends on your age, what type of sickle cell disease you have, and other health factors. Experts generally recommend the following preventive health schedule:

• Babies from birth to 1 year of age should visit an HCP every two to three months.
• Children between 1 and 2 years of age should visit an HCP every 3 months, at least.
• Children and adults older than 2 should visit an HCP once every year, at least.   

It’s important to attend all scheduled medical appointments and promptly report any changes in your health to your HCP. Your healthcare team is your best source for guidance on how to manage sickle cell disease and achieve your best health.

Other general tips for living well with sickle cell disease include:

Prepare for sickle cell crises

It helps to establish a game plan for handling sickle cell crises (pain crises) with your HCP before they occur. If one is just starting, take these steps:   

• Take an over-the-counter nonsteroidal anti-inflammatory drug (NSAID) such as ibuprofen or another pain reliever such as acetaminophen according to package directions. (Acetaminophen may be the better choice if you have kidney issues.)
• Drink plenty of water.
• Place a heating pad or warm compress on the affected area.
• If pain becomes unmanageable, contact your HCP or visit an outpatient clinic to receive stronger pain medications or IV fluids. In a severe sickle cell crisis, you may need to go to a hospital ED.  

Adopt healthy habits

Adopting a healthy lifestyle is important for everyone, especially those with chronic conditions like sickle cell disease. With your HCP’s guidance, try implementing these healthy habits:  

• Prioritize sleep. Aim to get between 7 and 9 hours of sleep daily. 
• Quit smoking or vaping. Don’t hesitate to ask your HCP for assistance with quitting smoking or vaping if you need it. If you don’t smoke or vape, don’t start.  
• Practice stress management techniques. Some simple ways to better manage stress include limiting your social media intake, meditating daily, doing yoga, and practicing deep breathing.  
• Stay physically active. According to the CDC, most adults need at least 150 minutes of moderate-intensity aerobic activity every week. That’s 30 minutes of exercise five days per week. (That can include exercises such as brisk walking or even pushing a lawn mower). Ask your HCP about what level and types of exercise would be best for your health needs.
• Eat for heart health. Add more vegetables, fruits, and whole grains into your diet while limiting saturated fats, added sugars, salt, and alcohol. Some popular heart-healthy eating plans you may want to try are the DASH (Dietary Approach to Stop Hypertension) diet and the Mediterranean diet.

Find support  

Coping with pain and other stressors related to sickle cell disease can lead to mental health conditions like depression and anxiety. Research suggests that 20 to 57 percent of people with SCD experience depression, while 6 to 29 percent deal with anxiety. Moreover, depression and anxiety may lead to more frequent hospitalizations and worse outcomes for people with SCD.

It’s important to stay connected with others and share your thoughts and feelings with loved ones if you’re living with sickle cell disease. There may be times when you prefer to be alone, but try to avoid isolating yourself as much as possible. You may also find it helpful to speak with a mental health provider (such as a counselor or psychologist) or join an online or in-person sickle cell disease support group. In settings like these, you can connect with others who face similar challenges.  

Take preventive steps

If you or your child has sickle cell disease, you may be able to reduce the frequency of sickle cell crises or help prevent complications by taking these steps:  

• Stay hydrated.
• Avoid intense heat or cold and sudden changes in temperature, such as what you may experience if you jump into a swimming pool.
• Feel your child’s abdomen daily for signs of splenic sequestration (your HCP can show you how). If your child is sick, check their abdomen several times a day. Promptly contact your HCP if the upper left part of their abdomen is swollen.
• Don’t fly on a plane with an unpressurized cabin. (These include smaller planes that fly short distances.)
• If your child attends daycare or school, inform your child’s teachers of their condition and what signs may signal a medical emergency.  

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Speak with your HCP if you have questions about sickle cell disease, such as how to manage SCD, prevent complications, or care for a loved one with the condition. You can also browse helpful resources from organizations such as the Centers for Disease Control and Prevention and the Sickle Cell Disease Association of America.

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• How Does Sickle Cell Disease Affect the Body?  
• Racism: America’s Longest-Running Epidemic  
• CRISPR Gene Editing Offers Hope for People With Sickle Cell Disease