What is sickle cell anemia?
Sickle cell disease (SCD) is a group of inherited bleeding disorders affecting red blood cells, which contain hemoglobin that carries oxygen throughout the body. Healthy red blood cells are shaped like a donut or disc, but in people with sickle cell disease, the shape changes to a crescent, or "C" shape, called a "sickle." This causes the red blood cells to become stickier, which can clog blood flow and cause pain and serious health problems. In addition, sickle cells die much faster than healthy red blood cells, leading to a shortage of red blood cells and causing severe anemia. While sickle cell anemia is a form of sickle cell disease, the terms are often used interchangeably.
Sickle Cell Anemia
Sickle cell disease is a blood disorder that is inherited from both parents.
Symptoms and risk factors
What are the signs of sickle cell disease?
The symptoms of sickle cell disease and sickle cell anemia may begin to appear around five months of age, although they may vary by individual and over time. These may include:
- Fatigue, caused by anemia
- Pain crises, or intense pain lasting hours to days
- Swollen hands and feet due to insufficient blood circulation
- Frequent infections
- Delayed puberty
- Vision problems
Screening and diagnosis
How is sickle cell disease detected and diagnosed?
Because sickle cell disease and sickle cell anemia are genetic conditions passed down from parent to child, most babies are tested at birth using a simple blood test. Other babies may be diagnosed while in the womb using certain genetic tests by taking a sample of the placenta (chorionic villus sampling) or amniotic fluid (amniocentesis). Older children and adults can also undergo the blood test, which screens for sickle cells.
If you are diagnosed with sickle cell disease, you may be referred to one of our board-certified genetic counselors who can help you and your family understand the benefits and drawbacks of genetic testing.
Types of sickle cell disease
What are the most common types of sickle cell disease?
Depending on the inherited gene mutation, individuals with this disease may have one of the following types :
Hemoglobin SS disease (Sickle cell anemia)
Known as HbSS, this is the most severe form of the disease and the most common, occurring when someone inherits the hemoglobin S gene from both parents.
Hemoglobin SC disease
This type occurs in people who inherit the HbC gene from one parent and the Hb S gene from the other. It is often milder than HbSS.
Hemoglobin SB (beta) thalassemia
People with this type inherit the HbS gene from one parent and beta thalassemia (another hemoglobin abnormality) from the other. There are two variations of this: zero or HbS beta0 (more severe) and plus or HbS beta+ (milder).
What is the sickle cell trait (HbAS)?
It's possible to inherit a hemoglobin S gene from one parent and a normal hemoglobin gene from the other (A). In this instance, you would be carrying the sickle cell trait but may not have any signs of the disease. However, you can still pass on the abnormal S gene to your children.
Treatments for sickle cell disease
How is sickle cell disease treated and managed?
For many people, sickle cell disease is a lifelong disorder. However, we've made tremendous advances in care for patients living with SCD over the past decade, helping to prevent complications and improve their quality of life.
Medications
Many patients with sickle cell disease undergo treatment designed to minimize pain episodes, alleviate symptoms, and prevent serious health problems. There are several FDA-approved medications that may reduce how often someone with SCD experiences a pain crisis, such as hydroxyurea, L-glutamine, Crizanlizumab, and others. Other medications may also help to lower the risk of anemia by improving blood flow throughout the body.
Preventing infection with vaccinations and antibiotics
Most babies diagnosed at birth will receive antibiotics, such as penicillin, up until at least the age of five. This can help to prevent life-threatening infections, such as pneumonia. Adults may also benefit from taking penicillin in various instances, such as if they've undergone surgery to remove the spleen (an organ that stores red blood cells).
In addition, staying up-to-date with vaccinations is essential to preventing disease and infection-related complications in both children and adults with sickle cell anemia.
Blood transfusions
Some people with sickle cell disease may undergo blood transfusions which can help treat severe anemia, prevent stroke, and improve chronic pain. During a blood transfusion, an individual's red blood cells are replaced with donor red blood cells. Because transfusions can lead to other serious side effects, it's important to seek care from an experienced team of blood doctors who will closely monitor the body's response and minimize the risk of iron overload.
Bone marrow or stem cell transplants
Currently, bone marrow or stem cell transplantation is the only potentially curative treatment option. This involves replacing bone marrow affected by abnormal hemoglobin with healthy bone marrow from a donor. Because it can be difficult to find a donor match for every patient and it comes with significant risks, transplantation may not be an option for everyone. If your doctor thinks you are a candidate, they will discuss the advantages and disadvantages so you can make an informed decision about your treatment.
Lifestyle habits
While you may not be able to prevent sickle cell disease symptoms, the following behaviors can help to reduce the frequency of pain crises and minimize other health complications:
- Drink plenty of water
- Avoid extreme temperatures
- Avoid high altitudes
- Take precautions against harmful infections, such as practicing frequent hand-washing
- Get yearly vision exams to screen for any retina damage
Looking for expert care?
With multiple locations throughout the region, patients have access to many of the nation’s renowned cancer specialists offering high-quality care, second opinions, and a chance for better outcomes close to where they live and work. Georgetown Lombardi Comprehensive Cancer Center, one of the nation’s comprehensive cancer centers designated by the National Cancer Institute (NCI), serves as the research engine allowing patients access to clinical trials that often lead to breakthroughs in cancer care.
Our providers
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Pedro E Alcedo Andrade, MD
Hematology
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Ravi Krishnan Anandakrishnan, MBBS
Medical Oncology
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Catherine Meyers Broome, MD
Benign Hematology & Hematology
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Kevin Y. Chen, MD
Gastrointestinal Medical Oncology, Medical Oncology & Hematology Oncology
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Julia Cunningham, MD
Benign Hematology & Hematology Oncology
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Rumaisa Hameed, MD
Hematology Oncology
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Craig M Kessler, MD
Benign Hematology & Hematology
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Salem Kim, MD
Hematology Oncology
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Shweta Kurian, MD
Hematology Oncology & Medical Oncology
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Hira Latif, MD
Hematology Oncology
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Kamyar Nader, MD
Myeloma, Lymphoma, Leukemia, Hematology & Hematologic Malignancy
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Aarthi Ganesh Shenoy, MD
Hematology
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Hedy P. Smith, MD, PhD
Hematology
Our locations
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MedStar Georgetown Cancer Institute at MedStar Georgetown University Hospital
3800 Reservoir Rd. NW Washington, DC 20007
MedStar Georgetown Cancer Institute at MedStar Franklin Square Medical Center
9103 Franklin Square Dr. The Harry and Jeanette Weinberg Cancer Institute Suite 220 Baltimore, MD 21237
MedStar Georgetown Cancer Institute at MedStar Washington Hospital Center
110 Irving Street, NW Washington, D.C., 20010
MedStar Franklin Square Cancer Center at Loch Raven Campus
5601 Loch Raven Blvd. Russell Morgan Building First Floor Baltimore, MD 21239
MedStar Georgetown Cancer Institute at MedStar Health Bel Air Medical Campus
12 MedStar Blvd. Ste. 180 Bel Air, MD 21015
Why choose us
If you or your child has sickle cell disease, you need a comprehensive team of specialists who understand the challenges you face and can help address the full range of complications associated with the condition. At MedStar Health, your care involves input from several specialists who can help determine what will be most effective in managing your unique case, with advanced treatment options backed by the latest research.
Here you'll find:
- A team approach to care: Our world-class hematologists (blood doctors) work closely with several other specialists to ensure you receive comprehensive care. Your treatment team may also include pain management specialists, neurologists, psychologists, social workers, and others who can help you live a full, healthy life.
- Expertise in pediatric hematology: Through our specialized pediatric, adolescent, and young adult hematology program designed for birth through age 39, we're helping even the youngest patients thrive while living with blood disorders.
- State-of-the-art treatment options: In addition to a full range of emerging FDA-approved medications, we also offer the latest in blood transfusions and stem cell transplants, which can help to improve your quality of life or potentially even offer hope for a cure.
- Holistic support for patients and families: Living with SCD can be difficult for both patients and their loved ones, which is why we offer a variety of integrative medicine services and supportive care for coping and navigating life with a hematologic diagnosis.
Clinical trials and research
Clinical trials
Clinical trials are studies that help blood doctors and researchers better understand different diseases and disorders, including the effectiveness of medications and therapies to prevent or reduce the severity of bleeding episodes.
Research
Through our research engine, the Georgetown Lombardi Comprehensive Cancer Center, we're proud to apply the latest advances in research, ensuring our patients receive the most up-to-date, evidence-based care available.