Since she was only 4 months old, Brandy Compton, 31, of Hartford, has been battling sickle cell disease, a painful inherited red blood cell condition impacting the blood’s circulation due to abnormally hook-shaped red blood cells.
“As a baby I wouldn’t stop crying, and nothing my mother did worked to console me. Then they found out I had sickle cell disease,” says Compton. “Growing up was challenging, and I was homeschooled after the third grade since I was missing way too many days of school due to my painful disease.”
Individuals born with the disease have severe pain episodes due to blockages that form in their smaller blood vessels and capillaries when their abnormally shaped cells cannot pass through smoothly. The dangerous blockages can reduce blood flow and may cause blood infections such as sepsis, leading to death. Other issues include profound anemia, which is an unhealthy level of oxygen-rich red blood cells.
This is how Compton vividly describes her lifelong pain episodes: “Sickle cell feels like jagged rocks shredding the inside of your veins, and your bones being crushed.”
Starting at age 12, monthly blood transfusions helped better manage her pain, but Compton still experienced ongoing pain crises.
But thanks to a new drug called Adakveo, her disease and quality of life, especially as a mother of 9-year-old son Jermaine, has become a little easier and more comfortable.
Compton started taking the once-a-month IV medication at the start of the COVID-19 pandemic at UConn Health’s outpatient New England Sickle Cell Institute.
“The medication’s doing real good,” says Compton, whose usual 4-hour monthly blood transfusion of 7 bags of blood has been cut nearly in half to 4 transfused bags — and half the transfusion time.
The medication works by targeting a protein to prevent misshaped red blood cells from becoming sticky, which can cause painful blood vessel blockages. Research shows the drug significantly reduces both sickle cell patient pain crises and the average number of days they spend in the hospital annually.
“UConn Health is helping me be able to keep up with my son, and he sure keeps me on my toes,” says Compton. “My care has been great by Dr. Biree Andemariam and Dr. Genice Nelson – and the entire sickle cell team there at UConn helping me. It’s a very good patient experience and a sickle cell patient unit – all in one place – for our needs.”
For over a decade, Compton has been cared for by UConn Health. At the age of 21. she transitioned from Connecticut Children’s affiliated pediatric sickle cell program’s care to the New England Sickle Cell Institute at UConn John Dempsey Hospital.
“If I need pain management on a given day, I don’t need to go to, or wait in, the Emergency Room. I can go to the Institute for help and care,” says Compton.
She advises others suffering from sickle cell to not give up hope and to seek care. “Keep pushing, there are different care options and treatments available now that can help you too.”
And just like her doctors and care team at UConn Health, she’s looking forward to a cure for the disease someday soon.
“A cure for sickle cell disease would be awesome,” says Compton.
“Sickle cell patients often have a lifetime of pain management and lengthy monthly blood transfusions to minimize their pain and risk of serious disease complications,” says Dr. Genice Nelson of NESCI at UConn Health. “We are so grateful that a new medication is having a positive impact on the health and lives of our sickle cell patients, and hopefully even more promising therapies are on the horizon soon thanks to our ongoing research efforts.”
More than 3.5 million are affected worldwide by sickle cell disease, including approximately 100,000 Americans, and in Connecticut, at least 1,000 adults and 600 children. African-Americans and Latinos are predominantly affected in the U.S., with life expectancy for the majority of people with sickle cell disease is now age 40 or greater thanks to advanced health management.
World Sickle Cell Disease Day is June 19.