Sickle Cell: One Family’s Remarkable Journey

By, Acacia Cameron RN

Link to article HERE

Keiosha Pinckey’s parents were told their baby was positive for Sickle Cell Disease before she was born. Her parents were even encouraged to consider the difficulty of bringing a baby into the world with Sickle Cell. Keiosha’s story is one that she also shares with her niece, Erykah Weldon. Together they continue to help each other with the daily struggles of living with such a disease, and strive to bring awareness to the importance of blood donation for the survival of those afflicted with sickle cell.

Sickle cell is a genetically inherited disease that affects red blood cells, namely their shape. Normally, red blood cells are rounded and soft. Sickle cell causes these red blood cells to change into a sharp crescent shape, resembling a sickle, hence the name. This condition makes it difficult to bind and transport oxygen around the body and can be very physically painful during a crisis. Those that suffer from these crises are left exhausted and in excruciating pain. Currently, the best way to manage this disease is through frequent blood transfusions, about every 2-4 weeks, for life. 

Keiosha was first told that she would not make it to her 16th birthday. Then she was told she would not make it to 25. Sitting in the lunchroom at age 5, Keiosha remembers not feeling well. “I felt like my body was shutting down…like my back and legs were paralyzed.” She slumped over the lunch table and ended up going to the hospital. This was her first memory of having a sickle cell crisis. 

Today, at 34 years old, Keiosha continues to thrive. She graduated with a degree in sports management with a focus on business, and explored massage therapy. She also plays multiple instruments such as the drums, keyboard, and guitar, and enjoys performing in her church band. By all accounts, Keiosha is living her best life, acting as a source of support and encouragement for her niece, Erykah.

Sickle cell is a disease that affects her daily life. Keiosha must receive blood transfusions every three to four weeks, and it is a lengthy process, often consuming her entire day. “Transfusions help reduce the number of crises per month,” she states. Eager to encourage people to donate blood, Keiosha explains, “We absolutely need (donations). That is how we survive. You are helping a million other people.” 

Erykah Weldon, Keiosha’s niece, was also diagnosed at birth. At age 5 Erykah had a stroke, but bounced back very quickly, with no side effects. She again had a stroke at age 15 and was told that the right side of her brain was not receiving enough oxygen. Erykah underwent surgery to correct the issue and has been doing well since. She also requires blood transfusions every 2-4 weeks to help mitigate the symptoms of sickle cell. Both Keiosha and Erykah have a port that is implanted that allows for much more efficient access for blood transfusions. A port is a means of having permanent IV access. Sickle cell patients tend to have veins that are difficult to find, leading to lots of poking and prodding during treatments. Having the support of family is what helps Keiosha and Erykah moving forward. They each have a unique understanding of what the other is going through. Keiosha states, “I knew it was my task to help (my niece) through this disease… My fight isn’t just for me.”

Needless to say, blood donations are an absolutely vital requirement to those suffering from sickle cell disease. With two groundbreaking gene therapies recently approved by the FDA, blood and platelet donations are critical to these treatments. Sickle cell patients face another challenge that comes with receiving frequent transfusions. Over time, patients may develop an immune response against blood from donors that don’t closely match the blood of the recipient.

To help remedy this, in 2021 the American Red Cross launched the Sickle Cell Initiative to encourage more donations from the Black community, who are in a unique position to help. Black donors are three times more likely to be a match for the blood most commonly needed. This initiative has also provided screening to donors and uncovered that about 40% of donors are sickle cell carriers. With 1 in 13 black babies born with the sickle cell trait, the need for compatible donors is essential. 

“At the end of the day,” says Erykah, “(donations) save lives. Blood transfusions help me to not be in constant pain…it helps create a sense of normalcy. All donations matter.” Keiosh and Erykah continue to show up for each other. They look out for one another and keep each other positive. Keiosha states, “Don’t give up. Always keep fighting.”