Sickle Cell Disease And The Importance Of Donating Blood

GREENWICH, CT — Even though Sickle Cell Disease (SCD) is the most commonly inherited disease in the United States, it doesn't receive a lot of attention. With World Sickle Cell Day set for June 19, Greenwich Hospital is hoping to get the word out about the disease, and the importance of donating blood to help those who are impacted.

SCD affects hemoglobin, the protein that carries oxygen through the body. Normally, red blood cells are disc-shaped and flexible to move easily through blood vessels. But with SCD, the red blood cells are crescent or "sickle" shaped, which causes them to not move around as easily.

As a result, this can block blood flow to the rest of the body, potentially leading to multi-organ failure. Many people can experience strokes, eye problems, pulmonary issues, infections, and pain, or vaso-occlusive crises. That occurs when sickle cells clog blood vessels and create excruciating pain for the patient.

"It does affect about 100,000 Americans. It's most common among African Americans and Hispanics, but it can occur in other subsets of patients like patients of mediterranean origin and caucasians," said Dr. Kisha Mitchel Richards, director of Pathology and Clinical Laboratory at Greenwich Hospital. "With the most commonly inherited disease, even though we can say right now that it mostly affects African Americans, it has the potential to affect so many more."

One single trait for SCD was originally meant to protect people in countries where malaria is endemic. But if a person inherits two of those traits, they then develop SCD.

Mitchell Richards noted that with the transposition of Africans through the slave trade to other countries many years ago, the spread of the disease has been far and wide.

For Mitchell Richards, Sickle Cell Disease is real. As a person of Jamaican descent, her son has the sickle cell trait, as does her husband.

"They don't have the disease, but that just reinforces just how close it is to home for me personally, but also for many other African Americans and Americans in general who often aren't necessarily aware of their complete background or origin," she said. "If you have the trait, you are generally speaking fine throughout the course of your life except for rare instances. But what that means is if you happen to have a child with someone else with the trait, then that child has a one in four chance of having Sickle Cell Disease."

Blood transfusions and bone marrow transplants can help increase oxygen capacity in SCD patients to manage the disease.

Right now, blood supplies are low. Mitchell Richards said the pandemic certainly hasn't helped, as people have stayed home and away from medical offices over the past year and a half. But blood supplies also historically dip around holidays, especially in the summer.

As the head of Greenwich Hospital's blood bank, Mitchell Richards urged residents to get out and donate. To look up the nearest Red Cross site to make an appointment, click here.

"World Sickle Cell Day is upon us on June 19. It really affects a significant amount of Americans, and really your single donation can make a difference in the lifespan and in the way an individual person feels today," she said.