Krystal Tames the Hurricane of Sickle Cell

Pamela, whose daughter receives sickle cell disease care at La Rabida Children’s Hospital, has weathered the storm of the disorder yet keeps a vigilant eye out for the next strong wind.

“It’s like living in a hurricane zone and not knowing when the hurricane is going to strike,” said Pamela, whose 15-year-old Krystal suffers with the potentially devastating disease. “You don’t know where it’s going to land or how much damage it’s going to do. It can go to the brain; it can go from the head to the toe and stop the circulation anywhere along the way. That’s why kids have strokes.”

If this emergency room nurse and mother of four sounds like an expert, it’s because she has had first-hand experience: five family members, including Krystal, have been touched or killed by sickle cell.

The disease is inherited and characterized by crescent-shaped blood cells.  Normal blood cells are round.  The sickled cells hinder the flow of flood, especially through small blood vessels. The poor flow results in a lack of oxygen and can cause extreme pain and catastrophic tissue damage.

African Americans -- one in 500 -- are disproportionately affected by the disease, for which there is only a high-risk cure of bone marrow transplant. There are promising drug treatment therapies, however.

As one of the state’s main referral centers for babies with sickle cell disease, La Rabida is an excellent resource for affected families.

That reputation lured the family back to the Chicago area in 1998. They had been living in southern Illinois, but couldn’t find adequate care for young Krystal. She said going to La Rabida was like returning to an extended family where the nurses treated Krystal like a niece and the Child Life specialists were like older cousins, coloring and working out puzzles during her long in-patient stays.

“They love the kids. They say, ‘Hey, you back again.’ They hug her like she’s family. And, I can talk with (Sickle Cell Specialist) Patricia (Bailey, MSN,RN) and she’ll see that I get what I need.”

For Pamela, that personal touch is worth the 20 minute drive when other hospitals are closer to home and the cost of gas is high. She recalls a pain crisis earlier this year, “It was like someone hit her with a bat in the stomach, and the pain radiated out to her extremities.’’ She took Krystal to a nearby hospital, but the staff didn’t help much. When Krystal’s condition worsened, mom picked up the phone in frustration and secured a bed almost immediately at La Rabida.

“When we came into the urgent care unit, they let us go straight back. There’s a standing protocol. They started an (intravenous feed) and pain medication right away.”

That was the first of four inpatient stays, ranging up to four days, for Krystal this year.

Pamela describes her daughter as a “good student with a spirit to match.” “I’m proud of her. She has high hopes and aspirations and she doesn’t let the condition stop her. She never says, ‘Why me, Ma?'  She inspires me.”

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