Journey Williams: A Sickle Cell Story
DeShawna Williams describes her daughter Journey as “one of a kind,” and it’s not hard to see why.
Journey was DeShawna’s “surprise child” born 17 years after her other daughter—an unexpected but very welcome blessing. Currently thriving in the fourth grade, ten-year-old Journey is lively, outdoorsy, and bright, and attends one of Cincinnati's most lauded schools, the School for Creative and Performing Arts.
Journey Williams is unique in another way: She is one of the 100,000 Americans living with Sickle Cell Disease.
DeShawna remembered vividly the moment she learned that her newborn had Sickle Cell Disease. “Journey was first diagnosed with Sickle Cell Anemia- SS Type while undergoing all of the tests that take place at birth. I did not find out prior to leaving the hospital,” she recalls. “On her 3rd day home from the hospital, I received a call saying, “Hello, I’m calling from Children’s Hospital. They told you that your baby has Sickle Cell and I’m calling to schedule an appointment... "
“I lost it at that point and burst out crying,” she continues. “I had limited knowledge of Sickle Cell and the advancements in treatments. My only association with Sickle Cell was death at a young age and that is what I thought of... That is how I found out.”
Sickle cell disease is an inherited red blood cell disorder. For most people, healthy red blood cells are round, and they move through small blood vessels to carry oxygen to all parts of the body. But for someone with Sickle Cell Disease, their red blood cells become hard and sickle-shaped, inhibited normal blood flow and causing symptoms like pain, infections, and even strokes.
Living with sickle cell is no easy feat, and for Journey, her early years were filled with hospital stays to treat pain crises, experiment with new treatments, and ward off potentially life-threatening complications. “We spent lots of time at the hospital,” says DeShawna. “Symptoms that were typical of illnesses that children her age catch like a common cold or having a fever were indications of something life threatening for Journey.”
In those early years, Journey’s physician team had tried treatments like Hydroxyurea to help manage her disease, but nothing had worked the way they wanted. The countless hospital visits were still a regular occurrence for years, until Journey became seriously ill.
“In April of 2015, Journey became so ill that it ended up charting a new path for her treatment,” DeShawna says. “She had a double crisis that I could tell was extremely concerning for her Hematology team, and it was clear that her Sickle Cell was advancing and not responding as they would like. At this point, she got a mediport and began ‘traditional’ blood transfusions.”
Receiving blood from volunteer donors can often be a successful treatment for Sickle Cell crises, but unfortunately, Journey’s condition continued to decline. Her medical team decided to advance the treatment to apheresis blood transfusions, in which Journey’s blood would be drawn and separated out into the different components, with the problematic cells being removed and the remaining plasma and other healthy cells being returned to her body with specially-matched red blood cells from a local donor.
“Although it took some time—years!—for her to get used to the treatments (she would scream, cry and sometimes try to run), we FINALLY found something that works for Journey’s Sickle Cell, for which we are very thankful,” says DeShawna. “Thanks to her blood transfusions every four weeks on Thursdays, she is able to lead a relatively normal life.”
Journey’s ability to learn, play, and live like any other child is important to DeShawna and her family. “While I try to limit her exposure, I do deliberately work to make sure that she has as much of a ‘normal’ life as possible…because when you are the parent of a child with Sickle Cell, you always know that any day could be her last,” she says. “Journey loves the outdoors and loves jumping on the trampoline in our back yard. We love doing ‘girls’ nights’ together, and we sometimes include my oldest daughter, who teaches 7th Grade in Texas, via FaceTime.”
However, there’s no denying that Journey’s life is much more complicated than that of other children her age. Sickle Cell Disease means a lifetime of looking out for symptoms of pain crises, strokes, and other life-threatening conditions—a scary and draining thing for a ten-year-old to contend with.
“One of the most difficult things about Sickle Cell and the requirements to keep Journey safe is that her outward appearance doesn’t indicate that there is anything seriously wrong with her---she looks like any other 10 year old,” DeShawna notes. “However, if it’s too cold, too hot, or if she’s too wet or too fatigued, it could cause a pain crisis. A belly ache can indicate a splenic sequestration crisis, or a fever can indicate an infection that could be life threatening.
Even keeping Journey healthy is a somewhat gargantuan task. Apart from regular blood transfusions, a Sickle Cell diagnosis “requires tons of visits to what seems like every clinic,” according to DeShawna. “Sickle Cell affects everywhere the blood flows—so Journey has seen Neuropsychology, Cardiology, Optometry, Pulmonology, Neurology, and more. Plus, she’s had two surgeries….one for her medi-port to be placed on one side of her chest, and a second for her aphaeresis port to be placed in her chest. As a result, she has missed lots of school which impacts her ability to keep up, despite being gifted. And all of the hospitalizations, doctors’ visits and transfusions have made it difficult to maintain employment.”
Despite the myriad challenges and hurdles that her daughter, and the rest of the family, have faced in recent years, DeShawna feels grateful that Journey is able to receive the treatment she needs, and that their family lives so close to an amazing team of physicians and nurses. More than that, she feels enormous gratitude to the strangers in her community who donate blood on a regular basis to flood her daughter’s body with healthy cells.
“What I feel when watching her transfusions is difficult to put into words,” she says. “Since she turned five years old, Journey has been receiving transfusions every four weeks. Initially, it was difficult to explain to a kindergartner why she needs blood from others to keep her healthy and alive. I tried to describe what was going on in terms that she could understand—I told her that her blood gets sick and it can’t do what it’s supposed to for her body, but that the transfusions gave her STRONG blood to keep her healthy.”
It’s true that the blood that Journey receives is strong—in fact, it is specially matched to be as close to her unique blood antigens as possible. Those healthy red blood cells glide smoothly through her veins, ensuring that her organs receive adequate oxygen, unlike the sickled cells that cause so many problems.
“Usually, she requires at least 2 large bags of blood, sometimes more, so she has received tons of blood over the years,” according to DeShawna. “The transfusions make her feel better almost immediately.”
Journey herself adds that receiving blood from local donors means a lot to her. “I want to thank the donors for their blood, because it makes me healthier...and it helps to make sure that I don’t feel icky all of the time. And I really appreciate it, and that it helps other kids like me as well, because I know I’m not the only one that people help.”
“Without these transfusions of blood that is her exact type, I know that my daughter would not be here,” DeShawna continues. “I’ve worried about whether Journey will be able to get what needs at the time that she needs it to keep her alive, so I’m extremely thankful that this lifesaving treatment is available to us.”
DeShawna also wants to send her thanks to the teams at Cincinnati Children’s Hospital and the therapeutics team at Hoxworth for their hard work and dedication.
“All of them are amazing and you definitely tell that it is a team effort and a good partnership!” she says. “We love Kelly Klapp, our Hematology NP, because she is personable, knowledgeable, caring and diligently works to make sure Journey is able to have a childhood that allows her to do what other children are doing. And Carole at Hoxworth is AMAZING with my daughter! She’s able to calm her down when she’s fearful after seeing the large needle used for transfusions when they are accessing both ports. She’s reassuring, nurturing, knowledgeable and is somehow able to make my daughters blood transfusions a pleasurable experience. When Journey sees her, she’s said to me while smiling, ‘My friend is here today!’"
Sickle Cell Disease is a lifelong journey, and DeShawna knows that there will be ups and downs in the years to come. But for now, she’s thankful for the support she and Journey have received—and that her one-of-a-kind daughter has been able to grow and thrive, in spite of the challenges she’s faced, because of local blood donors.
“I know that my baby is only alive because of the kindness of strangers give their time and, most importantly, a part of themselves so that I can continue to enjoy every moment with my daughter until they develop a cure for Sickle Cell Anemia,” she says. “Thank you!”