Sickle cell anemia is not cancer, but it is also not a routine disease. Every person’s disease is different, with individually unique genes and molecules driving that sickle cell anemia.
At the OSUCCC – James, our subspecialists are world-renowned blood disorder experts who focus solely on sickle cell anemia and who reach across medical disciplines (hematologists, surgeons, radiologists, pharmacists and more) to design the very best treatment plan and therapies to target each patient’s sickle cell anemia.
And by offering access to the country’s most advanced clinical trials right here at the OSUCCC – James, patients know that additional options, when needed, are always available for their treatment and care.
Sickle cell anemia is a benign (non-cancerous) blood disease where an abnormal hemoglobin is produced that causes cells to change from a round shape to that of a sickle, or a crescent.
It’s an inherited disorder that affects 70,000 – 100,000 Americans, mainly African Americans who have ancestors from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece and Italy), India and Saudi Arabia.
Sickle cell disease is inherited when someone inherits mutated sickle cell genes from both parents. If, however, a person inherits a sickle cell gene from one parent and a normal gene from the other parent, it results in a condition called sickle cell trait (more than 2 million Americans have sickle cell trait). People with sickle cell trait do not have sickle cell anemia, but they are at high risk for passing along the gene to their children.
Sickle cell anemia results from a gene mutation with production of an abnormal hemoglobin — the protein in red blood cells that carries oxygen to the body’s cells and tissues and carries away waste products from the blood.
The abnormal sickle shape makes the cells stiff and sticky, preventing them from moving normally through the bloodstream. These abnormal cells can block blood flow, causing pain, organ damage and an increased risk for infection.
Sickle cells can lead to a below-normal amount of red blood cells, creating anemic effects like extreme fatigue. This is because sickle cells usually die faster than normal blood cells, and the bone marrow (the spongy, inside layer of large bones) cannot make enough cells to replace the loss.
Sickle cell anemia is also known as:
- HbS disease
- Hemoglobin S disease
- Hemoglobin SS disease
- Sickle cell disease (a broad term that includes sickle cell anemia)
- Sickle cell disorders (a broad group of conditions that includes sickle cell anemia)
- Sickling disorder due to hemoglobin S
(Source: National Institutes of Health)
Sickle Cell Anemia Symptoms
Sickle cell anemia can produce varied symptoms ranging from mild to severe, with the most severe symptoms requiring in-patient hospital care for treatment.
The most common symptom of sickle cell anemia is fatigue. If sickle cell anemia is detected at birth, symptoms may not show up until after 4 months of age.
Other symptoms include:
- Shortness of breath
- Coldness in the hands and feet
- Paler than normal skin or mucous membranes (the tissue that lines the nose, mouth, and other organs and body cavities)
- Jaundice (a yellowish color of the skin or whites of the eyes)
Signs & Symptoms Related to Pain
When sickle cells block blood flow to limbs and organs, it can cause sudden, severe pain and even organ damage. When this kind of pain occurs, it’s called sickle cell crisis. These episodes of pain can be infrequent or can occur once a month or more.
Repeated episodes can cause damage to the organs and bones. Research experts continue to study what makes these sickle cell crises happen, but it’s possible that dehydration may increase the risk of a crisis.
Pain from sickle cell anemia is sudden. It can last from a few hours to a week or more, and many people with the disease also experience chronic (persistent) pain, especially in their bones.
Having these symptoms does not necessarily mean you have sickle cell anemia. But if you have symptoms, especially if they last longer than a few weeks, talk to your doctor about it.
If you’ve been diagnosed with sickle cell anemia, would like a second opinion or would like to speak with a blood disorder specialist, please call The James Line at 800-293-5066 or 614-293-5066 to make an appointment.
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Sickle cell disease patients sometimes forced to wait for pain treatment can find faster relief at the OSUCCC – James Sickle Cell Day Hospital. Read More