HEALTHY REASONING: Do You Know Your Sickle Cell Status?

By Allan Bucka Jones
PRIDE Health Columnist

Last Friday, June 19, was a special day for people affected directly and indirectly by sickle cell disease. It is the date designated by the United Nations (UN), as World Sickle Cell Day.

The goal is to raise awareness about this major health issue. The World Health Organization, the health arm of the UN, encourages countries affected by sickle cell disease to establish health programs at the national level and promote access to medical services for people affected by the disease.

So what is sickle cell disease, and what is meant by sickle cell status? Sickle cell disease is an inherited blood disorder affecting red blood cells. It is the most common genetic disorder in Canada. People with sickle cell disease make red cells that contain abnormal hemoglobin.

Hemoglobin is a protein that carries oxygen from the lungs to the rest of the body. When someone has sickle cell disease, their hemoglobin does not work properly, leading to severe pain, a higher chance of serious infections, organ damage, heart attack or stroke. A person with sickle cell disease needs lifelong treatment.

Your sickle cell status indicates whether you have sickle cell disease or just a carrier of the gene for sickle cell disease.

Sickle cell disease causes red blood cells to “sickle”, they change from a circular normal dough-nut shape to a crescent moon shape. Sickled red blood cells can block blood vessels with several associated problems. This is called a sickling crisis.

In Canada, sickle cell disease is largely thought of as a disorder occurring in Black Canadians alone. However it affects other ethnicities, including those of Mediterranean, South American, Caribbean, Southern European, South Asian and those of Middle Eastern descent.

Through the work of Millie Johnson, and the Sickle Cell Association of Ontario, newborn screening for sickle cell disease is now a reality in Ontario. However, most provinces do not screen for sickle cell disease.

Without the screening, the disease might not be detected by doctors for months, or even years. Untreated, sickle cell disease can lead to organ damage and, in extreme cases, death.

Unlike the United States and Britain, Canada does not have a national benchmark for newborn screening.

The result is a nationwide patchwork of testing programs that leaves some newborns with disorders, like sickle cell disease, vulnerable to going undetected before damage is done.

If two individuals who are carriers of the sickle cell gene, decide to have a child, they should be aware that for each pregnancy, there is a one in four (25%) chance that the child can be born with full blown sickle cell disease.

If you are going to take this chance, you should be prepared to deal with the consequences, like several sickle cell crises, and problems with the child growing normally.

However, despite the problems experienced by individuals with sickle cell disease, there is little or no thought given to the disease when individuals decide to have a child. If you are deciding to have a child, check out the sickle cell status of your partner and yourself. This information may cause you to not proceed with the pregnancy.

Talk to your family doctor soon about ordering a sickle cell test to ascertain your sickle cell status. This useful piece of information, if you are of child bearing age, is critical in determining whether you want to have children.

So Canada is nowhere near to fulfilling the directive of the World Health Organization, of encouraging countries affected by sickle cell disease, to establish health programs at the national level, and promote access to medical services for people affected by the disease.

Thankfully, the Sickle Cell Disease Association of Canada is focused on these issues.

A federal election is happening this October, and now is the time to bring healthcare issues, like national newborn screening for sickle cell disease, and a diversity-based approach to Canadian healthcare delivery, to the attention of our politicians.

Please do…let your vote count…support politicians who support your health concerns!

Allan Bucka Jones is a Health Promoter and Broadcaster. You can contact Allan Bucka Jones at allan@jonesandjones.ca.