Sickle cell disease
Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell anemiaSickle cell disease is a disorder passed down through families. The red blood cells that are normally shaped like a disk take on a sickle or crescent shape. Red blood cells carry oxygen throughout the body.
Causes
Sickle cell disease is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.
- Hemoglobin S changes the red blood cells. The red blood cells become fragile and shaped like crescents or sickles.
- The abnormal cells deliver less oxygen to the body's tissues.
- They can also easily get stuck in small blood vessels and break into pieces. This can interrupt healthy blood flow and cut down even more on the amount of oxygen flowing to body tissues.
Sickle cell disease is inherited from both parents. If you get the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell disease.
Sickle cell trait
The sickle cell test looks for the abnormal hemoglobin in the blood that causes the disorder sickle cell disease.
Read Article Now Book Mark ArticleSickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.
Symptoms
Symptoms usually do not occur until after the age of 4 months.
Almost all people with sickle cell disease have painful episodes called crises. These can last from hours to days. Crises can cause pain in the lower back, leg, joints, and chest.
Some people have one episode every few years. Others have many episodes each year. The crises can be severe enough to require a hospital stay.
When sickle cell disease becomes more severe, symptoms may include:
- Fatigue
Fatigue
Fatigue is a feeling of weariness, tiredness, or lack of energy.
Read Article Now Book Mark Article - Paleness
- Rapid heart rate
- Shortness of breath
- Yellowing of the eyes and skin (jaundice)
Jaundice
Jaundice is a yellow color of the skin, mucus membranes, or eyes. The yellow coloring comes from bilirubin, a byproduct of old red blood cells. Jau...
Read Article Now Book Mark Article
Younger children with sickle cell disease have attacks of abdominal pain.
Abdominal pain
Abdominal pain is pain that you feel anywhere between your chest and groin. This is often referred to as the stomach region or belly.
Read Article Now Book Mark ArticleThe following symptoms may occur because small blood vessels become blocked by the abnormal cells:
- Painful and prolonged erection (priapism)
- Poor eyesight or blindness
- Problems with thinking or confusion caused by small strokes
- Ulcers on the lower legs (in adolescents and adults)
Ulcers
An ulcer is a crater-like sore on the skin or mucous membrane. Ulcers form when the top layers of skin or tissue have been removed. They can occur ...
Read Article Now Book Mark Article
Over time, the spleen stops working. As a result, people with sickle cell disease may have symptoms of infections such as:
- Bone infection (osteomyelitis)
Osteomyelitis
Osteomyelitis is a bone infection. It is caused by bacteria or other germs.
Read Article Now Book Mark Article - Gallbladder infection (cholecystitis)
Cholecystitis
Acute cholecystitis is sudden swelling and irritation of the gallbladder. It causes severe belly pain.
Read Article Now Book Mark Article - Lung infection (pneumonia)
Pneumonia
Pneumonia is a lung infection. It can be caused by many different germs, including bacteria, viruses, and fungi. This article discusses pneumonia th...
Read Article Now Book Mark Article - Urinary tract infection
Urinary tract infection
A urinary tract infection, or UTI, is an infection of the urinary tract. The infection can occur at different points in the urinary tract, including...
Read Article Now Book Mark Article
Other signs and symptoms include:
- Delayed growth and puberty
- Painful joints caused by arthritis
- Heart or liver failure due to too much iron (from blood transfusions)
Exams and Tests
Tests commonly done to diagnose and monitor people with sickle cell disease include:
- Bilirubin
Bilirubin
The bilirubin blood test measures the level of bilirubin in the blood. Bilirubin is a yellowish pigment found in bile, a fluid made by the liver. Bi...
Read Article Now Book Mark Article - Blood oxygen saturation
- Complete blood count (CBC)
Complete blood count
A complete blood count (CBC) test measures the following:The number of white blood cells (WBC count)The number of red blood cells (RBC count)The numb...
Read Article Now Book Mark Article - Hemoglobin electrophoresis
Hemoglobin electrophoresis
Hemoglobin is a protein that carries oxygen in the blood. It is present inside the red blood cells. Hemoglobin electrophoresis measures the levels ...
Read Article Now Book Mark Article - Serum creatinine
Serum creatinine
The creatinine blood test measures the level of creatinine in the blood. This test is done to see how well your kidneys are working. Creatinine in t...
Read Article Now Book Mark Article - Serum potassium
- Sickle cell test
Sickle cell test
The sickle cell test looks for the abnormal hemoglobin in the blood that causes the disorder sickle cell disease.
Read Article Now Book Mark Article
Treatment
The goal of treatment is to manage and control symptoms, and to limit the number of crises. People with sickle cell disease need ongoing treatment, even when not having a crisis.
People with this condition should take folic acid supplements. Folic acid helps make new red blood cells.
Treatment for a sickle cell crisis includes:
- Blood transfusions (may also be given regularly to prevent stroke)
- Pain medicines
- Plenty of fluids
Other treatments for sickle cell disease may include:
- Hydroxyurea (Hydrea), which helps reduce the number of pain episodes (including breathing problems and risk for stroke) in some people
- Antibiotics, which help prevent bacterial infections that are common in children with sickle cell disease
- Medicines that reduce the amount of iron in the body
- Newer therapies to reduce the frequency and severity of pain crises have been approved
Treatments that may be needed to manage complications of sickle cell disease include:
-
Dialysis or kidney transplant for kidney disease
Kidney transplant
A kidney transplant is surgery to place a healthy kidney into a person with kidney failure.
Read Article Now Book Mark Article - Counseling for psychological complications
- Gallbladder removal in people with gallstone disease
Gallbladder removal
Laparoscopic gallbladder removal is surgery to remove the gallbladder using a medical device called a laparoscope. The gallbladder is an organ that s...
Read Article Now Book Mark Article - Hip replacement for avascular necrosis of the hip
Hip replacement
Hip joint replacement is surgery to replace all or part of the hip joint with a man-made joint. The artificial joint is called a prosthesis....
Read Article Now Book Mark ArticleAvascular necrosis
Osteonecrosis is bone death caused by poor blood supply. It is most common in the hip and shoulder but can affect other large joints such as the kne...
Read Article Now Book Mark Article - Surgery for eye problems
- Treatment for overuse or abuse of narcotic pain medicines
- Wound care for leg ulcers
Bone marrow or stem cell transplants can cure sickle cell disease, but this treatment is not an option for most people. People with sickle cell disease often cannot find well-matched stem cell donors.
Stem cell transplants
A bone marrow transplant is a procedure to replace damaged or diseased bone marrow with healthy bone marrow stem cells. Bone marrow is the soft, fatt...
Read Article Now Book Mark ArticlePeople with sickle cell disease should have the following vaccinations to lower the risk for infection:
- Haemophilus influenzae vaccine (Hib)
Haemophilus influenzae vaccine
All content below is taken in its entirety from the CDC Hib (Haemophilus Influenzae Type b) Vaccine Information Statement (VIS): www. cdc. gov/vaccin...
Read Article Now Book Mark Article - Pneumococcal conjugate vaccine (PCV)
Pneumococcal conjugate vaccine
Content below is taken in its entirety from the CDC Information Statement (VIS): www. cdc. gov/vaccines/hcp/vis/vis-statements/pcv. html
Read Article Now Book Mark Article - Pneumococcal polysaccharide vaccine (PPSV)
Pneumococcal polysaccharide vaccine
All content below is taken in its entirety from the CDC Pneumococcal Polysaccharide Vaccine Information Statement (VIS): www. cdc. gov/vaccines/hcp/v...
Read Article Now Book Mark Article
Support Groups
Joining a support group where members share common issues can relieve the stress of a chronic disease.
Support group
The following organizations are good resources for information on sickle cell anemia:American Sickle Cell Anemia Association -- ascaa. orgCenters for...
Read Article Now Book Mark ArticleOutlook (Prognosis)
In the past, people with sickle cell disease often died between ages 20 and 40. Thanks to modern care, people now can live to the age of 50 and beyond.
Causes of death include organ failure and infection.
When to Contact a Medical Professional
Contact your health care provider if you have:
- Any symptoms of infection (fever, body aches, headache, fatigue)
- Pain crises
- Painful and long-term erection (in men)
References
Howard J. Sickle cell disease and other hemoglobinopathies. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 149.
Meier ER. Treatment options for sickle cell disease. Pediatr Clin North Am. 2018;65(3)427-443. PMID: 29803275 pubmed.ncbi.nlm.nih.gov/29803275/.
Menapace LA, Thein SL. Clinical aspects of sickle cell disease. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 43.
National Heart Lung and Blood Institute website. Evidence-based management of sickle cell disease: expert panel report, 2014. www.nhlbi.nih.gov/health-topics/evidence-based-management-sickle-cell-disease. Published September 2014. Accessed April 22, 2024.
Smith-Whitley K, Kwiatkowski JL. Hemoglobinopathies. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 511.
Red blood cells, sickle cell - illustration
Sickle cell anemia is an inherited blood disease in which the red blood cells produce abnormal pigment (hemoglobin). The abnormal hemoglobin causes deformity of the red blood cells into crescent or sickle-shapes, as seen in this photomicrograph.
Red blood cells, sickle cell
illustration
Red blood cells - normal - illustration
This photomicrograph shows normal red blood cells (RBCs) as seen in the microscope after staining.
Red blood cells - normal
illustration
Red blood cells - multiple sickle cells - illustration
Sickle cell anemia is an inherited disorder in which abnormal hemoglobin (the red pigment inside red blood cells) is produced. The abnormal hemoglobin causes red blood cells to assume a sickle shape, like the ones seen in this photomicrograph.
Red blood cells - multiple sickle cells
illustration
Red blood cells - sickle cells - illustration
These crescent or sickle-shaped red blood cells (RBCs) are present with Sickle cell anemia, and stand out clearly against the normal round RBCs. These abnormally shaped cells may become entangled and block blood flow in the small blood vessels (capillaries).
Red blood cells - sickle cells
illustration
Red blood cells - sickle and Pappenheimer - illustration
This photomicrograph of red blood cells (RBCs) shows both sickle-shaped and Pappenheimer bodies.
Red blood cells - sickle and Pappenheimer
illustration
Formed elements of blood - illustration
Blood transports oxygen and nutrients to body tissues and returns waste and carbon dioxide. Blood distributes nearly everything that is carried from one area in the body to another place within the body. For example, blood transports hormones from endocrine organs to their target organs and tissues. Blood helps maintain body temperature and normal pH levels in body tissues. The protective functions of blood include clot formation and the prevention of infection.
Formed elements of blood
illustration
Blood cells - illustration
Blood is comprised of red blood cells, platelets, and various white blood cells.
Blood cells
illustration
Red blood cells, sickle cell - illustration
Sickle cell anemia is an inherited blood disease in which the red blood cells produce abnormal pigment (hemoglobin). The abnormal hemoglobin causes deformity of the red blood cells into crescent or sickle-shapes, as seen in this photomicrograph.
Red blood cells, sickle cell
illustration
Red blood cells - normal - illustration
This photomicrograph shows normal red blood cells (RBCs) as seen in the microscope after staining.
Red blood cells - normal
illustration
Red blood cells - multiple sickle cells - illustration
Sickle cell anemia is an inherited disorder in which abnormal hemoglobin (the red pigment inside red blood cells) is produced. The abnormal hemoglobin causes red blood cells to assume a sickle shape, like the ones seen in this photomicrograph.
Red blood cells - multiple sickle cells
illustration
Red blood cells - sickle cells - illustration
These crescent or sickle-shaped red blood cells (RBCs) are present with Sickle cell anemia, and stand out clearly against the normal round RBCs. These abnormally shaped cells may become entangled and block blood flow in the small blood vessels (capillaries).
Red blood cells - sickle cells
illustration
Red blood cells - sickle and Pappenheimer - illustration
This photomicrograph of red blood cells (RBCs) shows both sickle-shaped and Pappenheimer bodies.
Red blood cells - sickle and Pappenheimer
illustration
Formed elements of blood - illustration
Blood transports oxygen and nutrients to body tissues and returns waste and carbon dioxide. Blood distributes nearly everything that is carried from one area in the body to another place within the body. For example, blood transports hormones from endocrine organs to their target organs and tissues. Blood helps maintain body temperature and normal pH levels in body tissues. The protective functions of blood include clot formation and the prevention of infection.
Formed elements of blood
illustration
Blood cells - illustration
Blood is comprised of red blood cells, platelets, and various white blood cells.
Blood cells
illustration
- Sickle cell disease - InDepth(In-Depth)
Review Date: 3/31/2024
Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.