Sickle Cell Disease Can Effect Anyone, but a Healthy Lifestyle Is Possible

Family traits handed down to us in DNA play a large role in our health and well-being. Genetic changes and traits carried by parents may appear in their offspring, even though the parents don’t have any physical characteristics of those conditions. Some of these are more common than others like heart disease, high cholesterol and Type 2 diabetes.

Some diseases affect the blood and a person’s ability to live a normal life. This group of blood disorders is called sickle cell disease (SCD) and affects the red blood cells. Those “with SCD have abnormal hemoglobin,” a protein that helps our bodies use oxygen, “called hemoglobin S or sickle hemoglobin, in their red blood cells,” according to the National Heart, Lung, and Blood Institute.

When someone has SCD, it means that they’ve inherited two abnormal hemoglobin (red blood cell) genes, one from each parent. The difference in disorders depends on the types of genes that are inherited.

Despite past sweeping statements that SCD is only found in the Black community, SCD can actually affect anyone of any race or ethnicity. 

Normal red blood cells are shaped like donuts and are able to cross through the large and small blood vessels in order to reach the lungs and other parts of the body. So, when someone has SCD the blood cells become crescent or sickle shape, giving it the name of the disease, and are not flexible enough to fit through their route in the body. They can also stick to vessel walls, which may lead to a block in the flow of blood to body tissue, limiting oxygen that the body needs to survive.

Symptoms

Modern medicine has helped to identify this condition before it begins to complicate a child’s daily life. When a child is born with SCD, parents will know before symptoms start to show (at around five to six months in age). In the United States, it is required that all newborns receive screening for SCD. Some children may experience any problems early on; others don’t have problems until later in life.

Early symptoms include:

• Painful swelling of the hands and feet
• Fatigue or fussiness from low iron in the blood
• Yellowing of the skin or of the whites of the eyes, occur when a large number of red blood cells rupture

As every person is different, symptoms may be different for each person, and change over time. Most of the visible signs of SCD are related to complications.

Major Complications of SCD

Acute Pain Crisis

Pain can occur in people with SCD when there is a jam of oxygen delivery. A sickle cell crisis is pain that can last for several hours or even several days. This happens when sickled cells block small blood vessels that carry the blood to the bones. The pain is described as sharp, intense, stabbing or throbbing and can possibly land the person in the hospital for several days. The pain can affect any part of the body and in more than one place at a time. The pain often occurs in the:

• Legs
• Chest
• Arms
• Lower back
• Abdomen

But having this kind of pain doesn’t necessarily mean that it’s the fault of the condition! These symptoms can also be caused by:

• Illness
• Temperature changes
• Stress
• Dehydration
• Being at high altitudes

It’s best to check in with your doctor if you are experiencing unusual symptoms or are concerned about a health issue.

Other issues that may develop are:

• Severe anemia: people with SCD typically have mild to moderate anemia, however severe anemia can also evolve. This type can be life threatening.
• Acute chest syndrome: the effect of SCD on the lungs can deprive one of oxygen. When this happens, tissue is damaged and cannot exchange oxygen properly. This often starts a few days after a painful crisis begins and a lung infection may accompany acute chest syndrome.
• Chronic pain: The cause of chronic pain is not well understood and is usually different from that of crisis pain or organ damage. Patients have stated that the pain is hard to describe.
• Jaundice or Icteris: the yellowing of the skin or eyes from multiple red blood cells rupturing.
• Infections: Sickle cell can damage the spleen early in life. The spleen protects against certain types of germs, however those with a damaged spleen are at risk for serious bacterial infections that can be life-threatening. These infections can develop into blood infections, lung infections, infection of the covering of the brain and spinal cord or bone infections.

Living With Sickle Cell Disease

Education and understanding are always the first steps in living with any kind of chronic illness. If you or your child has SCD, you should learn as much as possible and build a relationship with your doctor so as to feel comfortable to ask questions. Developing a plan to manage your disease and complications can help in many situations—from the less severe to the most.

Babies and children with SCD should be referred to a doctor with experience caring for patients with SCD. Seeing a hematologist, or blood disease specialist, may also be an option for care and insight into the disease. People living with SCD should see their provider regularly, meaning every three to 12 months for preventive measures.

Pursue a Healthy Lifestyle

As with many other conditions, making sure that one maintains a healthy lifestyle can help limit complications. This should include:

• A nourishing diet
• Regular physical activity
• Enough sleep

Making sure to avoid situations in which a crisis can occur is also advised. Taking time to rest and rehydrate, along with avoiding extreme heat or cold can limit situations.

People with SCD also have to be aware of extremely active activities, stop smoking and drink alcohol in moderation with plenty of water to avoid dehydration.

For more information on resources and the SCD visit the Centers for Disease Control and Prevention website or the American Society of Hematology.