Elle Cole says she and her husband were excited. Doctors told the couple they were going to have twins. But something happened that she didn’t expect weeks into the pregnancy. The doctor called them both in to discuss an issue.
“I felt like both my husband, and I had been blindsided,” Cole said. “She told us we were both carriers of the sickle cell trait. Now prior to that neither of us had heard of the sickle cell trait or knew that we were carriers. So that appointment she said you have one in four chance of having a child with sickle cell disease.”
They were fraternal twins. And after birth, a test determined one had sickle cell trait like mom and dad, while the other had sickle cell disease. That was more than 14 years ago.
“It was something that I really felt isolated with,” Cole said. “I have a parent that has sickle cell trait, but I did not know that prior to the time. And so, prior to that doctor’s appointment someone should have tested me and my husband and told us.”
Cole’s story is not unique. Prior to 2006, screening for sickle cell trait was not mandatory. According to the Centers for Disease Control and Prevention (CDC), all 50 states and the District of Columbia now screen newborns for sickle cell status as part of the Newborn Screening Program. Sickle Cell disease is a genetic disorder that affects red blood cells, causing the hemoglobin to clump together, which can deform the cells into a crescent or banana shape, as described by Sicklecellspeaks.com.
The CDC estimates that Sickle Cell disease affects about 100,000 people in the United States and around 14,000 in the United Kingdom. Most of those affected have African ancestry. Sickled red blood cells are stiff and sticky, which prevents them from moving freely through blood vessels, hindering the transport of oxygen to organs. The CDC reports those with Sickle Cell trait inherit one sickle cell gene and one normal gene. They usually do not exhibit the painful symptoms of Sickle Cell disease, but they can pass the trait on to their children.
Dr. Sophie Lanzkron, Director of the Division of Hematology at Thomas Jefferson University Hospital, explained how some people miss being warned about carrying the trait.
“If you’re born in this country now, you should be diagnosed at birth. But …lots of people weren’t born in this country or were born after reporting or were identified to have trait when they were born and the parents were told…and nobody shared that information,” Lanzkron explained. So, the populations that are most at risk are people of the African descent, Middle Eastern descent, East Asian descent and Hispanic descent.
Cole revealed that most don’t understand the kind of pain patients experience. As an ambassador for St. Jude Children’s Research Hospital, she recalls her daughter’s first painful episode at 5-years old.
“I’m actually in Maryland and it was extremely cold,” Cole said. “I homeschooled my kids at the time and we were going to the Maryland Science Museum, which was one of our favorite places. And on the way I started to have some car trouble, and I called AAA…I wasn’t able to pull over in a place where there was a building for us to go and warm up, and so we were outside waiting for a while. And so, when we got home. She was in pain and she was in pain for days. I could see just her body just throbbing in pain. And she cried. And it was really — just this scream. I felt so helpless.”
Following that incident, Cole felt it was crucial to explain the disease to both of her daughters, even though they were young. This inspired her to create educational materials that her girls could understand during their homeschooling sessions. That eventually led her to write several children’s books on the subject including “The ABCs of Sickle Cell Disease.”
Rev. Zemoria Brandon is the director of the Sickle Cell Disease Association of America, Philadelphia/Delaware Valley Chapter. She says didn’t know much about the disease until she met a man during a job training in Chicago back in the early 1970s.
“I applied for a job as a residential summer camp counselor. I went to the orientation. This man, Walter Brandon, saw me walk in. And he pulled the chair out next to him, like that was to be my chair. Which normally I wouldn’t respond to that. But I sat in that chair,” Zemoria said.
It started a relationship that soon blossomed into love. And then one day Walter popped the question.
“When he proposed to me, he said, ‘you know, I’ve never shared this with you, but I have sickle cell disease,'” Zemoria shared. “Well, I knew the word sickle cell. I didn’t know much about it. And so I said, why would you tell me this? You know, now you’re proposing to me? He said, because I wanted to make sure that you cared about me as a person and that you didn’t feel sorry for me because I have a disease.”
Walter told Zemoria his father, twin sisters and brothers all had sickle cell disease.
These days she advocates for sickle cell patients — many who need strong drugs to deal with painful episodes.
“Children are on morphine for pain, children,” explained Zemoria. “So this is not something you decided you want to take. This is your prescription throughout your life that you’re going to have to take this pain medication. So that’s a sore issue. And the other issue that we don’t even talk about and people don’t know about is those that are incarcerated, that have sickle cell disease. In the prison system, you don’t get opioids.”
The ordained interfaith minister says she has had to advocate for many patients who report that when they have pain episodes they simply aren’t believed. She says often medical personnel assume the person is asking for heavy drugs unnecessarily.
“We try to maybe call their doctor or the social worker of the hospital, but they are not believed, and this is not just in Philadelphia,” Zemoria said. “This is all over the United States and certainly we know in African countries.”
Zemoria explained that it’s estimated that one out of every 10 to 12 African Americans is a sickle cell trait carrier. And because there are very minor symptoms, many people don’t know that they carry the trait. Couples who both carry the trait have a higher chance of having a child with sickle cell disease.
She says right now any person of color who donates blood with the American Red Cross can ask to be screened for sickle cell trait. The results will be sent to them.
Brandon believes people should apply the phrase “Check Your Status” usually associated with HIV, to sickle cell, especially with families with African ancestry.
“If you go to a blood drive and you are a person of color, Black or brown person, you can be screened for sickle cell trait,” said Brandon.
She added that it’s important that more people of color donate blood.
The American Red Cross reports that the national blood product supply has fallen by more than 25 percent since July 1, 2024. And for sickle cell patients the blood supply issue is extremely serious. Many patients have to undergo multiple blood transfusions in their lifetime. The reasons are to avoid complications like stroke.
“Donating blood is a major, major issue and people can go and donate blood now,” Brandon explained. “And designate that it goes to the sickle cell program, whether it be pediatric or what have you.” She explained that in Pennsylvania and across the country, the American Red Cross puts a special blue tag on that blood to make sure it goes to the right place.
The American Red Cross shares a story of a nurse on their website named Kelly who suffered a stroke at four-years-old. Since that time, she received monthly blood transfusions throughout her childhood and since then, red cell exchanges every seven to eight weeks. That means she has received thousands of units of blood over her lifetime. The agency notes the need for a diverse blood supply. So there has been an increased effort to encourage more Black and Brown people to donate blood.
As of now there are several treatments for sickle cell but no universal cure. This is because the types of sickle cell disease vary and so do the complications of individual patients. Dispite this, parents like Elle Cole say it’s exciting to see that researchers are making progress.
NBC News reported in 2023, the therapy, called Casgevy, from Vertex Pharmaceuticals and CRISPR Therapeutics, is the first medicine to be approved in the United States that uses the gene-editing tool CRISPR. This won its inventors the Nobel Prize in chemistry in 2020. The FDA also approved a second treatment for sickle cell disease, called Lyfgenia, a gene therapy from drugmaker Bluebird Bio. Both treatments work by genetically modifying a patient’s own stem cells.
“The gene therapy treatment that’s FDA approved is groundbreaking,” explained Elle Cole. “It’s amazing and it’s exciting, but only a few patients who are living with sickle cell may qualify, and so in my daughter’s case, she isn’t because she doesn’t have a lot of severe pain crises. She doesn’t qualify.”
Cole says she’s hopeful there will be something her daughter will qualify for in the future.
When asked how the stress of the disease has affected her and her family, Cole said she feels fortunate because her daughter hasn’t had as many severe episodes as she’s seen other patients on this journey.
“I think it’s always a struggle if I’m being very honest, because I think when you have a child with a chronic illness. You’re always thinking about what could be, and so you don’t necessarily get to relax mentally in the same way that other parents do.”
She practices self-care and goes to a therapist.
“Someone who is objective… who can look at my situation and not really be a part of it, but who can help me navigate the journey in a way that I think is healthy. And I also try to carve out some time where I feel like I am giving back to my community in some way because I feel blessed that my daughter isn’t sick and always in the hospital. So that does afford me some opportunities to really be active.”
One of the ways the Maryland area mom does this is by hosting a Sickle Cell Caregivers Summit in November.
“(It’s) to really help educate other caregivers and really create a space where we have fun together. And we play games and win prizes.”
Elle knows she’s fortunate because many Sickle Cell patients have a short life expectancy of about 54 years old according to a 2019 study by the National Institute of Health.
That is unfortunately the case for Zemoria Brandon’s husband. He passed away in 1998 at 59 years old. She says although she did not carry the sickle cell trait, her husband’s Sickle Cell disease journey in some ways encouraged her to do more research on her family blood line. And so she took a test with African Ancestry which revealed Zemoria was part of a line of Ghanian African royalty.
“My DNA showed that I was my mother’s ancestral line goes back to the Asere people in Accra, Ghana,” Zemoria explained.
Somehow the information got to one of her neighbors across the street, named Dr. Samuel Quartey. The podiatrist reached out to her by phone explaining he was a USA Chief of the Asere Royal Family.
“He says — I’d like to nominate you to be enstooled as one of our queens. And I said, why would you want me? He said because I’ve watched your work. I’ve watched your walk. And I watched how you have been so attentive to your husband with sickle cell.”
So in October, 2011 the Kingmaker, Nii Amarkai, III, of the Asere Royal Family came to Philadelphia, Pennsylvania and enstooled four people. She was one of the four.
“I am an enstooled Queen through the Asere Royal Family located in Accra, Ghana. My royal name is Naa Dede Ota I.”
Zemoria is queen of the village Adjenkotoku. She says it’s something she knows — had her mother been still alive — she would be shocked at, but yet proud.
“It’s so interesting…my mother said when my sister and I we were growing up. I have two brothers, she would say. I am raising you girls so that you can walk among kings and queens. So get it together. And unfortunately, my mom passed two years before I was enstooled. So she never (saw it). But she planted the seed,” Brandon said.
She says finding out this secret made a lot of her personal life make sense. For years, she’s collected African art and clothing.
“And it’s so funny because my mother, when she would come to visit me…and she’d say, what is all this African stuff? What these African clothes you’re wearing? What’s all this African art? And I said, Mommy, it speaks to me.”
The African queen continues daily with a royal assignment of shining the light on the challenges of Sickle Cell Disease. Brandon says her organization helped usher in World Sickle Cell Awareness Day celebrated every year on June 19th. She’s partnered with dozens of organizations around the country to get tall buildings to illuminate their roofs in red on that day as a reminder for others to acknowledge the disease and hopefully change how others see it.
Credits:
Family Photo includes:
Elle Cole
David Cavins
Maya Cavins – 15
Laila Cavins – 15
The photo copyright is owned by Elle Cole.
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