A family battles sickle cell Courage: John and Brett Paul volunteered for pioneering medical studies to advance the fight against the painful blood disease.

February 09, 1997|By Diana K. Sugg | Diana K. Sugg,SUN STAFF

She first heard the words in May 1953 at Johns Hopkins Hospital. The doctor pulled her into a small room, away from her 9-month-old son, John, who was squirming in a crib.

Everyone thought he had jaundice. When the doctor told her the diagnosis, Vivian Paul looked up at him. The physician stared back.

Slowly, stressing every syllable, she asked: "What in the name of heaven is 'sickle cell anemia?' "

In the years to come, she would spit out the words "sickle cell" as if they were poison.

Without knowing it, she and her husband would pass the devastating blood disease to their three youngest sons. It can kill tissues throughout the body, scarring organs and making the person susceptible to health problems ranging from infection to stroke.

Each son would endure grinding pain and dreams denied.

One by one, each would die. Craig Sterling Paul at 18 months. John Leroy Paul Jr. at 42 in July 1995. The youngest, conceived in part to help heal the loss of Craig, was buried on Thursday. Donald Brett Paul was 38.

But the brothers, and their Baltimore family, conquered the curse of sickle cell, in ways that helped ease the agony of thousands of other patients.

John and Brett Paul were among the first people in the world to swallow the experimental drug that became the biggest breakthrough for the disease. They volunteered for countless drug trials. In a rare distinction, Hopkins' sickle cell clinic will be named for them.

Their West Baltimore neighborhoods honor a contribution of a different sort: their kindnesses.

Even as the balls of their hips disintegrated, and the pain became so fierce that they twisted and moaned, these men managed to touch others, umpiring at Little League games and carrying groceries for elderly neighbors.

"I call it courage," said Dr. George Dover, who leads sickle cell research at Hopkins and knew the men for years. "They didn't let the disease destroy their lives."

A whole family touched

But sickle cell altered every member of the Paul family, shaping decisions on marriage and children, leaving guilt among the four siblings who were spared, and pulling them all closer.

Even though Vivian Paul was told that John had sickle cell, she wasn't warned that the genetic tragedy could strike again. She gave birth to Craig in 1954. A few months later, the baby landed in Hopkins with the same diagnosis.

Today, newborns in Maryland and other states are screened and put on preventive penicillin, which has reduced childhood mortality. But in the mid-1950s, that wasn't done. At 18 months, Craig developed what was to be the last of many infections. By nightfall on Nov. 9, 1955, he was dead.

The words of the doctor in the cold hallway are burned into the mother's mind: "Mrs. Paul, I tried. God knows, I tried." The doctor began to cry. "We've lost Craig." Vivian Paul fainted.

From then on, the family watched over John, clinging to him, fearing they could lose him in a moment. They mourned Craig. They all wanted another little brother. They got their wish in April 1958.

Donald, called by his middle name, Brett, was pampered from the start. The oldest sister, Carleen, carried him everywhere on her hip. But when he was 2 months old, he became sick. They went back to Hopkins.

"Not him, too?" his mother whispered.

Geneticists now know that, like one in 10 African-Americans, Vivian Paul and her husband, John L. Paul Sr., each carried a gene that was harmless to them. But with every baby there was a 25 percent chance the child would inherit both mutated genes -- and the disease.

"Yes, Mrs. Paul," the doctor said. "Him, too. I'm sorry, but you've got your hands full."

Hopkins became the family's second home.

Both John and Brett had a severe form of sickle cell, suffering attacks that were frequent, unpredictable and painful. Called crises, they would start with a bad nosebleed, or coughing in the night.

Their father was away for months at a time as a cook and steward in the merchant marine. The oldest child, Michael, remembers carrying a limp brother at all hours up the stairs to Hopkins' Broadway entrance, and past the towering statue of Christ.

He and his mother often slept on the hard benches of the emergency room, sometimes staying five days a week. The boys' noses had to be cauterized to stop the bleeding. Cradling her sons calmed them a little, but Vivian Paul was often torn between the hospital and home. She remembers hearing Brett's cries echo as he escaped his bed and toddled down the corridor after her: "Momma! Momma!" She would beseech nurses to hold him until she returned.

The internal damage that sickle cell wreaks is hidden. Physicians often walked out of the examining room to report they'd found nothing. Even Michael began to wonder if his brothers were pretending. A sister, Gwen, sometimes got angry at John and Brett for lying on the couch, frail and sick, on hot summer days; almost as quickly, she'd want to comfort them.

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