12 Things You Should Know About Sickle Cell Disease

You Don't Have to Be Black to Have Sickle Cell

It's true that sickle cell disease has long been associated with people of African descent, but it can be found in people of many races.

Other groups more likely to have sickle cell anemia include Hispanic-Americans of Central and South American descent and people of Middle Eastern, Asian, Indian, and Mediterranean heritage.

While white people don't rank amongst those most likely to have sickle cell, they can develop it as well.

Since it can affect people of any background, all babies born in the United States are tested for sickle cell anemia. 

It's an Inherited Disease

Sickle cell disease is not contagious like a cold. People are either born with it or they are not. If you are born with sickle cell disease both of your parents have sickle cell trait (or one parent with sickle cell trait and the other with another hemoglobin trait).

A person with sickle cell trait cannot develop sickle cell disease but can pass the gene on to their children.

It Is Diagnosed at Birth

In the United States, every baby is tested for sickle cell disease. This is part of the newborn screen performed shortly after birth. Identifying children with sickle cell disease in infancy can prevent serious complications. 

It Has a Connection to Malaria

People with sickle cell trait can be found most heavily in areas of the world that have malaria. This is because sickle cell trait can protect a person from becoming infected with malaria.

This doesn't mean a person with sickle cell trait cannot be infected with malaria, but it is less common than a person without sickle cell trait. 

Not All Types of Sickle Cell Are Created Equal

There are different types of sickle cell disease that vary in severity. Hemoglobin SS (also the most common type) and sickle beta zero thalassemias are the most severe followed by hemoglobin SC and sickle beta plus thalassemia.

Sickle Cell Causes More Than Just Pain

There is a lot more to sickle cell disease than just painful crises. Sickle cell disease is a disorder of the red blood cells, which supply oxygen to all the organs.

Because sickle cell disease occurs in the blood, every organ in the body can be affected. Patients with SCD are at risk for stroke, eye disease, gallstones, serious bacterial infections, and anemia, to name a few.  

Children With Sickle Cell Are at Risk for Stroke

Although all people with sickle cell disease are at risk for stroke, children with sickle cell disease have a much higher risk than the children without sickle cell disease.

Because of this risk, physicians who treat children with sickle cell disease use an ultrasound of the brain to screen and determine who is at highest risk of stroke and start treatment to prevent this complication. 

Antibiotic Changes Life Expectancy

The antibiotic penicillin is life-saving. People with sickle cell disease are at increased risk of serious bacterial infections.

Starting penicillin twice a day for the first five years of life has changed the course of this condition from something only seen in children into a condition people live into adulthood with.

It Can Affect Periods

Teens with sickle cell disease may not start their periods at the average age of their peers. People who menstruate and have sickle cell disease may also have shorter cycles and abnormal bleeding.

Sometimes, when a person with sickle cell disease has a period, it can trigger an attack of sickle cell pain (acute vaso-occlusive pain).

However, studies have found that most people who menstruate and have sickle cell can tell the difference between pain from their period and pain from a sickle cell crisis.

Fertility Challenges Are Common

Sickle cell disease can make it challenging for people to get pregnant. Some of the issues have to do with the disease itself, while others are related to the treatments for sickle cell.

People who have sickle cell may not be able to get pregnant or carry a pregnancy for a few reasons:

• Chronic inflammation and oxidative stress in their bodies
• Damage to reproductive organs from the disease and/or its treatments
• Blood disorders related to getting transfusions (hemochromatosis)
• They are taking medications like hydroxyurea that would be harmful to a developing fetus

People with sickle cell disease who want to have children might be able to use assisted reproduction or surrogacy.

Treatments Are Available

There is more than pain medication for treating sickle cell disease. Today, blood transfusions and a medicine called hydroxyurea are changing the lives of people with sickle cell. 

These therapies are allowing people with sickle cell disease to live longer lives with fewer complications. Multiple research studies are ongoing to find additional treatment options.

Sickle Cell Can Be Cured

Bone marrow (also called stem cell) transplantation is the only cure. The best success has come from donors who are siblings whose genetic makeup matches the person with sickle cell disease.

Sometimes types of donors, like unrelated individuals or parents, are used but mostly in clinical research studies. In the coming years, gene therapy looks like a promising treatment.  

If you or your family member has sickle cell disease, it is important to have regular follow-ups with a physician to ensure up-to-date care is provided.