What Is Sickle Cell Disease?

Sickle cell disease is a genetic disorder affecting the shape and life span of red blood cells. Sickle cell patients' red blood cells can get stuck in small blood vessels and capillaries and block the flow of blood and oxygen to the body's organs.

Overwhelming fatigue. Intense pain. Swollen hands and feet. Kristi Dansby has been battling the effects of sickle cell disease from the day she was born.

Now 42, Dansby has relied on blood transfusions for her entire life – transfusions equalling more than 1,000 units, the Texarkana, Arkansas resident estimates.

“Blood is the only thing that really gets me out of a crisis completely,” Dansby explains.

Dansby is one of roughly 100,000 Americans living with the disease, according to the CDC, and one of millions worldwide.

What Are the Different Types of Sickle Cell Disease?

Sickle cell disease, or SCD, is an inherited disorder that alters the shape of red blood cells, causing them to become stiff, sticky and curved like a sickle. Normally, red blood cells are round and flexible, allowing them to flow smoothly through blood vessels. Sickle cells, however, can clog blood vessels and capillaries, leading to reduced blood flow and oxygen delivery to various parts of the body.

There are several common forms of the disorder:

Hb SS

People who have this form of SCD inherit two genes, one from each parent, that code for hemoglobin “S.” Hemoglobin S is an abnormal form of hemoglobin that causes the red cells to become rigid, and sickle shaped. This is commonly called sickle cell anemia and is usually the most severe form of the disease.

Hc SC

People who have this form of SCD inherit a hemoglobin “S” gene from one parent and a gene for a different type of abnormal hemoglobin called “C” from the other parent. This is usually a milder form of SCD.

Hb S beta-thalassemia

People who have this form of SCD inherit a hemoglobin “S” gene from one parent and a gene for beta-thalassemia, another type of hemoglobin abnormality, from the other parent. There are two types of beta-thalassemia: “zero” (Hb S beta0) and “plus” (Hb S beta+). Those with Hb S beta0-thalassemia usually have a severe form of SCD. People with Hb S beta+-thalassemia tend to have a milder form of SCD.

What Are the Symptoms of Sickle Cell Disease?

The severity of SCD symptoms can vary widely, from individuals not having any symptoms to having severe symptoms, depending on the type of sickle cell gene a person inherits. The most commonly reported symptoms include:

Anemia

Sickle cells have a shorter lifespan than normal red blood cells, leading to a low red cell count. Without a sufficient supply of red blood cells, oxygen can't be effectively distributed throughout the body. This can cause fatigue, shortness of breath, lightheadeness, dizziness and other symptoms.

Pain crises

Vaso-occlusive crises occur when sickle cells block blood flow, causing severe pain in the bones, joints, chest, and/or abdomen. These instances are often referred to as pain crises or sickle cell crises.

Swelling of hands and feet

Blood circulation blockages caused by sickle-shaped red blood cells can lead to painful swelling of a patient's hands and feet.

Organ damage

Over time, the blockage of blood vessels can lead to organ damage, particularly affecting the spleen, liver, kidneys and lungs.

Frequent infections

Sickle cell can weaken the immune system, making those afflicted with the disease more susceptible to life-threatening infections.

Stroke

A blockage of the major blood vessels supplying oxygen to the brain can lead to stroke.

What Are Some Common Treatments for Sickle Cell Disease?

Pain management

Pain crises can be managed with pain medications, heat therapy and fluids to prevent dehydration.

Blood transfusions

Regular blood transfusions can help increase the number of healthy red blood cells in the body. At Our Blood Institute, we collect blood products that patients, including those battling sickle cell disease, receive via blood transfusion.

Hydroxyurea

This medication can stimulate the production of fetal hemoglobin, which can help prevent sickle cell formation.

Bone marrow transplant

In severe cases, a bone marrow transplant from a matched donor can be a potential cure, but it is a risky procedure and not suitable for everyone.

Who Does Sickle Cell Disease Usually Affect?

Sickle cell disease affects people of all ethnicities, but it does occur more often among people from parts of the world where malaria was or is common.

This means that populations from or descending from Africa, the Mediterranean, the Middle East, India or Southeast Asia are disproportionately affected. Of those groups, Africans and those descended from Africa are especially impacted. Around 1 in 500 African Americans living in the United States have SCD.

How Can Donating Blood Help People with Sickle Cell Disease?

One of the best and most direct ways to give those with SCD the highest quality of life is through blood donation. Through this generosity, sickle cell patients are more likely to receive much-needed blood transfusions, which can help stave off severe complications such as anemia, acute chest syndrome and stroke.

Finding compatible blood donors for patients with sickle cell can be incredibly challenging, however, as SCD patients need donors whose red blood cell surface is similar to their own while also having red blood cells that don't sickle. Healthy donors, particularly donors from within the African American community, can make a tremendous difference in the lives of sickle cell patients.

That’s the message Dansby and her loved ones want to spread. Beverly Coleman, Dansby’s nurse and friend for over 30 years, has witnessed the debilitating pain of the disease firsthand.

“The more we give, the more that can be matched,” Coleman says. “It’s worth it, it is so worth it. Just think about it – the little pain you go through – just think about what sickle cell patients go through.”

Like Coleman, Dansby’s boyfriend Dionne Hopson has been by his partner’s side as she’s battled the disease and says he would happily take on her pain if he could.

“I hate needles. I hate them with a passion,” Hopson says. “But if my blood matched hers and it’s going to make her feel better, then doc, get what you need. I’m all in.”

If you would like to help people like Dansby, consider scheduling your blood donation today. Or as Dansby herself puts it: “Please take the time out of your life and give someone else life.”

Schedule your donation