Sickle cell disease

Newborns have no visible symptoms of sickle cell disease. Symptoms may begin to appear at the age of 6 months.

The frequency and severity of symptoms vary extensively from one individual to another depending on the type of sickle cell disease.

These symptoms must be observed carefully for timely intervention.

Quick identification of the symptoms of a person with sickle cell disease plays an essential role in treatment of the disorder and can help reduce the risks of complications. The earlier health issues are detected, the easier it will be to treat them.

The most common symptoms to monitor include:

• Fever, chills or deterioration of general condition (signs of bacterial infection)
• Pain that varies in intensity, localization and duration and often lasts several days
  • Joint pain (arms, legs, shoulders, thighs, etc.)
  • Chest or stomach pain
• Swollen stomach (enlarged spleen)
• Difficulty breathing  
• Headache
• Painful and prolonged erection (priapism)
• Convulsions
• Paralysis, weakness or swelling of the arms or legs
• Vision problems
• Limping or pain with walking
• New fatigue or pallor

When these symptoms appear, you must consult a doctor quickly. Call your health care team as soon as possible and take your child to the hematology clinic or Emergency. Remember to inform the emergency team that your child has sickle cell disease.

A disorder leading to complications

Sickle cell disease can lead to many complications. Some of these, such as pain crises and infections, are easily recognized. Others, however, require tests that will be prescribed and recommended by your health care team. These tests will help detect the development of complications prior to any tragic consequences, such as:  

• abnormalities in cerebral blood vessels prior to a cerebrovascular accident (CVA) or stroke
• rupture of small blood vessels in the eye prior to vision loss
• etc.

One of the most frequent complications of the disorder is vaso-occlusive crisis. Commonly referred to as pain crises, vaso-occlusive crises are caused by the blockage of small blood vessels by clumps of deformed red blood cells cells.This cuts off the flow of blood and oxygen to the affected area causing pain.

​Consult your health care team promptly or go to Emergency as soon as your child develops a fever. Refer to the information pamphlet on fever and sickle cell disease (in French only) as well as the Action plan for the relief of pain from sickle cell disease and follow the recommended steps.

How can pain crises caused by sickle cell disease be prevented?

Pain caused by sickle cell disease can arise spontaneously and unpredictably. However, certain factors can cause pain, such as:

• Temperature changes and extreme temperatures (e.g., bathing in cold water, exposure to intense heat or cold)
• Fever and infections
• Dehydration (e.g., due to vomiting or diarrhea, or after intense physical exercise without sufficient hydration)
• Fatigue and stress
• Lack of oxygen (e.g., scuba diving or high-altitude trekking)