https://www.yahoo.com/news/sisters-sickle-cell-had-devastating-115609928.html

Gina Kolata
Mon, May 24, 2021, 7:56 AM
Sisters Kami 16, left, and Kyra Crawford, 15, receive a blood transfusion, which helps manage their sickle cell disease, in San Antonio, Texas, Sept. 2, 2020. (Ilana Panich-Linsman/The New York Times)
Sisters Kami 16, left, and Kyra Crawford, 15, receive a blood transfusion, which helps manage their sickle cell disease, in San Antonio, Texas, Sept. 2, 2020. (Ilana Panich-Linsman/The New York Times)
SAN ANTONIO — It was 4 a.m. on a Sunday when Dana Jones heard an ominous sound, barely audible over the whirring of box fans, like someone struggling to breathe. She ran down the hall and found her daughter Kyra, age 12, lying on her back, gasping for air. Terrified, she called 911.

A police officer, the first to arrive, dashed into Kyra’s bedroom, threw the slender girl over his shoulder and laid her on a leather sofa in the living room. He asked her mother, an oral surgery technician, to give her CPR.

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Kyra’s lips were ice-cold. An ambulance whisked the girl to Methodist Children’s Hospital, where staff members swarmed her and put her into a medically induced coma.

Kyra, who has sickle cell, had suffered a devastating stroke — her second — a common complication of this inherited disease, which afflicts 100,000 Americans, most of them Black. She most likely would never have had the strokes if she had been given an annual screening test and treatment proven more than two decades earlier to prevent 9 out of 10 strokes in children with the disease and recommended by the National Institutes of Health. But like countless other children with sickle cell, she was never screened.

This is a paradoxical moment for people who have this painful, deadly disease. For the first time, gene therapies that have advanced through clinical trials offer the real possibility of a cure.

But Dr. Francis Collins, director of the National Institutes of Health, said the lack of attention paid to sickle cell historically “is one more reflection of the fact that we do not have equity in our country.”

Some doctors and researchers believe the national reckoning on race sparked by the pandemic’s devastating impact on people of color, and the Biden administration’s pledge of a broad assault on racial inequities in American medical care, could make this a singular moment for advancing the fight against sickle cell.

Even so, Kyra’s strokes are a striking case study of the broad national failure to provide even the most basic treatments to people with sickle cell. Faulty care and sluggish research are symptoms of what sickle cell specialists say is the deplorable legacy of neglect of Americans with the disease.

One-third as many Americans have cystic fibrosis, a genetic disease that is of comparable seriousness to sickle cell but that primarily affects white children, yet it gets “seven to 11 times the research funding per patient, which results in disparate rates of development of medications,” according to a recent opinion piece in The New England Journal of Medicine. Only four medications are approved by federal regulators for sickle call, and 15 for cystic fibrosis.