One hundred years of sickle cell

A century after the disease was discovered, the focus is still on finding a cure

BY: Tonya Facey

This year marks the 100th anniversary of the discovery of sickle cell disease (SCD) by Dr. James B. Herrick. In the century since SCD was first chronicled in medical journals, scientific research has produced treatments for those suffering from the blood disorder. However, for the millions of SCD patients around the globe, there's still no cure.

Sickle cell anemia is a genetic blood disorder that causes red blood cells to become hard and sickle-shaped, which makes it difficult for the blood cells to travel through blood vessels. Complications from this disorder can include severe pain, damage to vital organs and premature death.

The black community is most affected by SCD, as it is commonly found among people of African descent. It is also found among Middle Eastern, Mediterranean, Asian and Hispanic populations. SCD is passed onto children from parents who share the sickle cell trait, which is why it is important for adults to know whether or not they carry the trait and assess the risk of having a child. In Canada, one in 10 people are carriers.

Lanre Tunji-Ajayi, president and CEO of the Sickle Cell Awareness Group of Ontario (SCAGO), stresses the importance of not only knowing your status, but also your partner's, in order to alleviate the burden of SCD on future generations.

"Sickle cell disease is so deadly and painful that if you could prevent one child from being born with it, that would do a lot of good," she says.

Tunji-Ajayi is passionate about raising awareness of SCD because she watched her late brother suffer from the disorder for years. She now works within the community raising awareness, educating patients and raising funds for scholarships and grants.

One of those community members is Busola Arimoro, who has been living with SCD for more than 30 years and finds herself in the hospital about once a month with extreme, widespread pain.

"Everywhere I have bone in my body, I have pain," she says. "When I have a crisis, it is from the top of my head to the bottoms of my feet."

According Dr. Richard Ward, a hematologist with the Red Blood Cells Disorder Program at Toronto General Hospital, a viable cure for SCD has yet to be found. However, a bone marrow transplant is considered very close to a cure. The challenge with a bone marrow transplant is finding a match, particularly within the black community. Other treatment options include blood transfusions and the use of the drug hydroxyurea. Researchers are also looking closely at genetics as a possibility for a cure. Gene therapy has proven promising in some laboratory studies, but still requires a lot of research before a viable treatment is developed.

Until there is a cure, the key to slowing the spread of SCD is to know whether or not you are a carrier and, if you are, to understand the risks involved. Fundraising is also critical for research. That's why the SCAGO hosts events such as the Hope Gala and Awards (May 15, 2010) to raise funds that will be directed toward increasing the focus on sickle cell research.

- To learn more about the Sickle Cell Awareness Group of Ontario or to purchase tickets to the Hope Gala & Awards, visit sicklecellanemia.ca