Sickle Cell Disease

What is Sickle Cell Disease?

Sickle cell disease is a group of inherited blood disorders that affect red blood cells. In healthy people, red blood cells are round and flexible, moving easily through blood vessels. People with sickle cell disease have red blood cells that become hard, sticky, and shaped like a crescent or sickle.

These sickled cells die early, causing a constant shortage of red blood cells. They also get stuck in small blood vessels, blocking blood flow and oxygen to parts of the body. This blockage causes pain and can damage organs over time. Sickle cell anemia is the most common and often most severe form of the disease.

The condition is present from birth and lasts a lifetime. It affects millions of people worldwide, especially those with African, Mediterranean, Middle Eastern, and Indian ancestry. Understanding your sickle cell status helps you manage symptoms and prevent complications before they become serious.

Sickle Cell Test

Detect sickle cell disease early
Clarify unexplained pain or fatigue
Confirm your genetic health status

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Zinc

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Complete Blood Count / CBC (includes Differential and Platelets)

Reveal underlying infections or inflammations effortlessly
Detect anemia or other blood disorders early
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Symptoms

Painful episodes called crises that can last hours to days
Swelling in hands and feet, especially in young children
Frequent infections due to spleen damage
Delayed growth and puberty in children and teens
Vision problems from blocked blood vessels in the eyes
Fatigue and weakness from anemia
Pale skin, gums, or nail beds
Yellowing of skin and eyes from bilirubin buildup
Shortness of breath during physical activity
Chest pain or sudden severe headaches

Some infants with sickle cell disease may not show symptoms until after 4 months of age. Early testing helps identify the condition before serious complications develop.

Causes and risk factors

Sickle cell disease is caused by inheriting two copies of a mutated gene, one from each parent. This gene controls hemoglobin production, the protein in red blood cells that carries oxygen. The mutation causes hemoglobin to form abnormal strands that make red blood cells rigid and sickle-shaped. Parents who each carry one copy of the gene have sickle cell trait but usually do not have symptoms. When both parents have the trait, each child has a 25% chance of inheriting sickle cell disease.

Risk factors include family history and ancestry from regions where malaria is or was common. The sickle cell gene provides some protection against malaria, which explains why it persists in certain populations. Triggers for pain crises include dehydration, extreme temperatures, stress, high altitudes, and infections. Knowing your genetic status before having children helps you understand the risks and plan accordingly.

How it's diagnosed

Sickle cell disease is diagnosed through blood tests that detect abnormal hemoglobin. The sickle cell screen identifies the presence of sickle hemoglobin in your blood. Additional tests measure hematocrit to check red blood cell levels, bilirubin to assess hemolysis or red blood cell breakdown, and red cell distribution width to evaluate cell size variation. Urine urobilinogen levels can indicate chronic hemolysis, a hallmark of the disease. Zinc levels are also monitored because people with sickle cell disease often have zinc deficiency.

Rite Aid offers testing for sickle cell disease through our flagship panel. You can get tested at over 2,000 Quest Diagnostics locations with results reviewed by our medical team. Early diagnosis allows for timely treatment and helps prevent serious complications like stroke, organ damage, and severe infections.

Treatment options

Stay well hydrated by drinking 8 to 10 glasses of water daily
Avoid extreme temperatures, high altitudes, and situations that trigger crises
Take folic acid supplements to help produce new red blood cells
Consider zinc supplementation to address common deficiencies
Get all recommended vaccines to prevent infections
Use pain medications during crises as directed by your doctor
Hydroxyurea medication can reduce pain episodes and complications
Blood transfusions for severe anemia or to prevent stroke
Newer medications like voxelotor and crizanlizumab target disease mechanisms
Bone marrow or stem cell transplant may cure the disease in some cases

Frequently asked questions

Sickle cell disease occurs when you inherit two copies of the sickle cell gene, one from each parent. Sickle cell trait means you inherited only one copy of the gene and typically have no symptoms. People with the trait can pass the gene to their children but usually live normal, healthy lives.

Most doctors recommend blood tests every 3 to 6 months to monitor your condition. Regular testing tracks red blood cell levels, bilirubin from cell breakdown, and organ function. More frequent testing may be needed during pregnancy, before surgery, or when starting new medications.

Bone marrow or stem cell transplant is currently the only cure for sickle cell disease. This procedure replaces diseased cells with healthy ones from a matched donor. Because transplants carry serious risks, they are usually reserved for people with severe disease, especially children with matched sibling donors.

Pain crises can be triggered by dehydration, infections, extreme cold or heat, stress, and high altitude. Overexertion, sudden temperature changes, and alcohol use can also trigger episodes. Avoiding these triggers and staying well hydrated can reduce the frequency of painful crises.

Sickled red blood cells die much faster than normal cells, breaking down in 10 to 20 days instead of 120 days. This rapid breakdown releases bilirubin, a yellow pigment, into the blood. Elevated bilirubin causes yellowing of the skin and eyes and can increase further during pain crises.

While sickle cell disease is most common in people of African ancestry, it also affects those from Mediterranean, Middle Eastern, Indian, and Latin American backgrounds. The gene evolved in regions where malaria is common because sickle cell trait provides some malaria protection. Anyone with family history from these regions should consider testing.

Many people with sickle cell disease live full, active lives with proper management. Following your treatment plan, staying hydrated, avoiding triggers, and getting regular medical care can minimize complications. Advances in treatment have significantly increased life expectancy and quality of life over the past few decades.

People with sickle cell disease often have low zinc levels due to increased zinc loss and poor absorption. Zinc deficiency can worsen symptoms, slow wound healing, and weaken immune function. Studies show that zinc supplementation may reduce infection rates and improve growth in children with the disease.

Hydroxyurea is a medication that increases fetal hemoglobin production, which prevents red blood cells from sickling. It can reduce pain crises by up to 50%, lower the need for blood transfusions, and prevent organ damage. Most doctors recommend it for both adults and children with frequent pain episodes.

Yes, genetic counseling is highly recommended if you have sickle cell disease or trait and plan to have children. A counselor can explain the chances of passing the condition to your children and discuss testing options. If both parents carry the gene, each child has a 25% chance of inheriting the disease.