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OVERVIEW

What is sickle cell disease?

Sickle cell disease (SCD) is a genetic condition you have at birth. It runs in families and is more common in African Canadians.

SCD refers to a group of red blood cell disorders. Normal red blood cells are round and flexible. They contain hemoglobin (a protein), which carries oxygen in your blood. With SCD, some red blood cells are misshapen and look like sickles (a C-shaped farm tool). These sickle cells become hard and sticky. They can get stuck—or even burst and die—when traveling through small blood vessels. This slows or blocks blood and oxygen to parts of your body. Sickle cells also contain an abnormal type of hemoglobin. This can also result in cells dying.

There are several forms of SCD. They vary based on the sickle cell and hemoglobin genes you inherit from your parents. HbSS is the most common form. It is known as sickle cell anemia. It also is the most severe form of SCD. People who have HbSS get 2 sickle cell genes, 1 from each parent.

Another form of SCD is sickle cell trait. People who have this get 1 sickle cell gene from a parent and 1 normal gene from the other parent. Typically, they don’t have SCD symptoms. However, they can pass the sickle cell gene on to their kids.

SYMPTOMS

Anemia is a common symptom of SCD. It occurs because your body has a hard time creating enough new cells to replace the ones that die early. Side effects of anemia include:

• Fatigue
• Headaches
• Dizziness
• Shortness of breath
• Coldness in your hands and fee

Other symptoms of SCD include:

• Swollen hands and feet
• Jaundice (the yellowing of the skin and the whites of the eyes)
• Severe chronic pain
• Severe infections
• Organ damage

CAUSES

What causes sickle cell disease?

SCD is a condition you inherit from your parents at birth.

DIAGNOSIS & TESTS

 How is sickle cell disease diagnosed?

 SCD is diagnosed with a simple blood test. It often is included in routine newborn screening tests at birth in the hospital. It’s important to test babies for SCD. Early diagnosis and treatment can help prevent and manage symptoms of SCD. If you are at risk, ask your healthcare provider to check you and your sexual partner for sickle cell trait or disease before you get pregnant. Then you will know if you might have a child with sickle cell disease. Your healthcare provider might want you to get genetic testing. If you are already pregnant, you might get testing for your fetus. In Nova Scotia and Prince Edward Island, sickle cell disease is screened in newborns by the Maritime Newborn Screening Program.

TREATMENT

Treatment for SCD varies for each person. It’s based on your form of SCD, your symptoms, and your overall health. Most treatment options aim to reduce symptoms and prevent complications.

Pain crises are a common symptom of SCD. These painful attacks occur quickly. They can last for a few hours to a few days. They happen when sickle cells get stuck in your blood vessels. This reduces the oxygen that gets to your tissues. You may feel pain in your back, knees, legs, arms, chest, or stomach. Crises are hard to predict. However, there are things you can do to help prevent them.

• Visit your healthcare provider for regular check-ups.
• Don’t smoke. If you do smoke, quit.
• Eat a balanced diet.
• Limit how much alcohol you drink.
• Exercise regularly but not so much that you become really tired. When you exercise, drink lots of fluids.
• Drink at least 8 glasses of water a day, especially during warm weather.
• Reduce or avoid stress. Contact your healthcare provider if you’re depressed or have problems at home or at work.
• Contact your doctor if you have a fever or possible infection. . Illnesses should be treated as soon as possible.
• Try not to get too hot, too cold or too tired. Dress properly for the weather. Get rest when needed.
• Tell your healthcare provider if you think you might have a sleep problem, such as snoring or sleep apnea.  This is when you stop breathing for short periods of time while you sleep.
• Manage other health issues you have, such as diabetes. Talk to your doctor if you’re pregnant or plan to become pregnant. They can tell you about genetic counseling and get you started with prenatal care early on.
• Only travel in commercial airplanes. If you have to travel in an unpressurized aircraft, contact your healthcare provider about extra precautions.

New treatment options have been introduced in recent years. These include bone marrow and stem cell transplants. These two treatments can cure SCD when successful. Transplants require a donor and are risky. They typically only are approved for severe cases. They involve taking bone marrow or stem cells from the donor and implanting them into your body. Transplants have a lot of side effects and can result in death when not successful.

Living with sickle cell disease

SCD is a life-long condition. For most people, there is no cure. A managed lifestyle is the best way to reduce symptoms and complications and prolong life.

FOR MORE INFORMATION

Sickle Cell Disease Association of Atlantic Canada

https://sicklecellatlanticcanada.ca/

Sickle Cell Disease Association of Canada

https://sicklecelldiseasecanada.com/