‘They know each other’s pain’

The first time D’Yana heard of sickle cell disease, she was pregnant with her first child, a daughter she would name Ari’Yanna. During routine blood tests, her obstetrician informed D’Yana she carried the trait for the genetic blood disorder. Sickle cell disease develops when a child inherits a copy of the sickle cell gene from both parents. She filed the information away as good to know but didn’t grasp the full weight of it until her daughter was diagnosed with sickle cell disease following a newborn screening.

That’s when Ari’Yanna was referred to St. Jude Children’s Research Hospital®, home to one of the largest sickle cell programs in the country. St. Jude treats about 850 patients while continuously refining medical care through research and transformative treatments such as gene therapy and bone marrow transplant. 

“I was crushed, not knowing anything about the disease or anyone with it,” D’Yana recalled. “But at St. Jude, they give you a guideline like what to expect. You never feel alone when you're in St. Jude.”

Her daughter Ari’Yanna became one of approximately 100,000 people in the United States with sickle cell disease, which is predominant among those of African descent. 

Once she arrived at St. Jude, D’Yana learned from doctors how the disease affects red blood cells, causing them to warp from healthy, round and plump to crescent moon shapes. That distortion hinders those cells’ ability to move through blood vessels properly and supply major organs with oxygen. 

Distraught, D’Yana felt like her first few visits were a blur. Doctors told her there is no widely available cure, though bone marrow transplants have been shown to resolve the disease when a suitable donor is available, though not without substantial risk to the patient. Sickle cell disease is a lifelong condition for most afflicted with it, and it leads to a host of health complications, including pain crises, lung disease, heart disease, kidney disease, strokes and frequent infections. 

The care providers at St. Jude were direct but also reassuring. She might have felt out of her depth, but they assured her that St. Jude wasn’t. It had built an impressive body of knowledge around the disease since it got its first research grant for it in 1958 before the hospital even opened.

“They took me step by step every day to get me comfortable to ask, ‘I don't understand this,’ or ‘I don't know this word,’ or ‘I don't know what this means,’” she said. 

When Ari’Yanna was 10 months old, she started taking hydroxyurea, a prescription drug that boosts the production of fetal hemoglobin and helps prevent the sickling of red blood cells so that sickle cell patients have fewer pain crises and other catastrophic health conditions.

That experience came in handy two years later when D’Yana had another daughter, Ariyah, who was also born with sickle cell disease. Ariyah also takes hydroxyurea as part of her care for the condition. 

When they were babies and toddlers, managing her daughters’ sickle cell complications was challenging, D’Yana said, because they were too young to put words to pain. And it manifested so differently in each of them. Ari’Yanna would feel it most in her arms and hands, while Ariyah felt it in her legs. 

When Ari’Yanna grew old enough to summon the words to describe her aches, she told her mother it felt like fire deep in her bones. Her younger sister, Ariyah, had pain crises very early in life, starting at 3 months old, and cried inconsolably. She spiked fevers often, her eyes would swell, and her legs would writhe in pain. By the age of 1, doctors needed to remove her spleen. And now, at 9 years old, Ariyah visits St. Jude monthly to receive transfusions to help prevent sickled cells from blocking blood vessels, which helps reduce her the risk of stroke.     

The sisters advocate for one another. They seek each other out at school, somehow knowing when the other is hurting even when nothing has been said, and alerting teachers for help when necessary. Ari’Yanna, now 11 years old, knows how to help Ariyah wrap her legs in warm blankets when the pain is at its worst, and Ariyah knows how much pressure to apply to her big sister’s arms to help coax the pain away.

“Sometimes it’s overwhelming and I have to remind myself to stay strong when they’re going through their crises,” D’Yana said. “They do a better job of helping each other than I do because they know each other’s pain.” 

To help further research for their disease, Ari’Yanna and Ariyah are both participants in the Sickle Cell Clinical Research and Intervention Program, or SCCRIP, which is a long-term study led by St. Jude that follows people with sickle cell disease over many years to better understand how the condition affects patients throughout their lives.

The sisters don’t let their disease stop them from living a full life. Ari’Yanna plays basketball. Ariyah is a majorette and runs track.  

Sometimes their friends are surprised to hear about their diagnosis and routine visits to St. Jude. “You don’t ‘act sick,’” they say.

That’s when Ari’Yanna, eager to be seen as a person beyond the disease, has a simple response: “I have sickle cell. Sickle cell doesn’t have me.”