Ask Dr. Kevin: Understanding Sickle Cell Disease

8/2/2017, 4:51 p.m.

In this article, Dr. Kevin answers common questions about sickle cell disease and its impact on the community and provides tips for living with and supporting someone with the disease.

What is sickle cell disease?

Sickle cell disease (SCD) is an inherited genetic disease that affects hemoglobin, the oxygen-carrying protein within red blood cells (RBC). While normal RBCs are flexible and oval-shaped, individuals with SCD have sharp, crescent-shaped RBCs that have trouble passing through the body’s blood vessels, irritating the vessels’ lining. This irritation leads to the production of “sticky” proteins that cause RBCs to clump together, along with other cells in the blood, and creates blockages in blood flow. The reduced blood flow leads to severe pain and organ damage, like the heart, brain, eyes, liver, lungs, and spleen (causing the inability to fight certain infections).

How does someone get sickle cell disease?

SCD is passed from parent to child. Everyone has two hemoglobin genes, one from each parent, and both parents must carry and pass the sickle cell gene to their child. With each pregnancy, the child has a 25% chance of having SCD if both parents have the trait.

Is sickle cell disease contagious?

No. You can only inherit it if your parents carry the sickle cell gene and pass it to you. SCD is a serious, lifelong condition that a person has from birth. You do not “lose” or “outgrow” it over time.

Are African-Americans more likely to have sickle cell disease?

SCD is more common in certain ethnic groups, especially those of African descent. It is estimated that nearly one in 14 African-American individuals carries the sickle trait and SCD occurs in one out of every 500.

What is the most common symptom for people with sickle cell disease?

Pain is the most common and difficult symptom of SCD, as it can be sudden and so severe that people need to go to the emergency room (ER) or be admitted to the hospital. This type of pain is referred to as a “sickle pain crisis” or “vaso-occlusive crisis” (as it is due to blood vessel blockage). Pain can occur anywhere blood flows, but common sites are lower back, arms, chest, stomach, and legs. Certain triggers are known to cause a pain crisis, such as dehydration, extremely hot or cold temperatures, and stress.

How can patients with sickle cell disease prevent pain?

Although you may not prevent every pain episode, avoiding triggers may reduce the occurrence and/or severity of pain crises. It’s important to:

• Stay hydrated to prevent dehydration. Drinking water is best.

• Exercise regularly, but don’t overdo it.

• Avoid very hot or cold temperatures.

• Manage stress to your body and mind.

• Get plenty of rest.

It’s also important to go for regular health checkups and talk to your doctor about managing pain episodes.

Is it possible to die from sickle cell disease?

SCD can cause a lifetime of health issues and complications that may lead to early death. In developed countries, like the United States, people with SCD often live between 40 and 60 years of age. However, in developing countries, like some countries in Africa, 90% of babies born with SCD will die before age 5.