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

THE BALTIMORE SUN

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.

The sisters shouldered tasks beyond their years. Gwen cooked. Another sister, Christine, scrubbed floors. When the little boys were hospitalized at the same time, the oldest sister, Carleen, would stay with one while their mother was with the other.

All the siblings found themselves defending their skinny brothers from bullies.

But there was one thing they couldn't take on -- the pain.

It is infamous. In Africa, where researchers believe the disease originated, names for sickle cell translate as "body biting" or "body chewing." Sickle cell anemia hits people of several ethnic backgrounds, but it predominantly affects those of African heritage. One in 400 African-Americans has it, and there are about 80,000 victims nationwide.

When John was 14, the pain nearly drove him to hurl himself from the Lafayette Avenue bridge one night. After making more than a dozen calls, his mother finally reached Dr. Samuel Charache. a Hopkins hematologist whose expertise and commitment earned him the nickname "Sickle Sam."

Charache, now retired, listened to John and believed his complaints. The physician took over his care, and later, Brett's as well.

As they were growing, so was knowledge about the disease. John knew that if anyone would find a cure, it would be Charache. And he was willing to take risks for their faithful physician. In the initial experiment, John was the first patient. Following his older brother, as he did in so many other ways, was Brett, patient No. 3.

"All we did was tell them all the awful things that could happen to them, when we didn't know what might, and what might not, and they were willing to go ahead," said Charache.

Drug treatment

The researcher was attempting to use a drug, hydroxyurea, to halt the sickling process. Red blood cells, which carry oxygen throughout the body, are usually round and soft, like balloons filled with water. But people with sickle cell anemia have abnormal hemoglobin, which makes the red blood cells rigid and shaped like a farm tool, the sickle.

Unlike normal cells, they can't squeeze through narrow blood vessels. Resulting logjams cut off oxygen to tissues and bones. It's like having small heart attacks all over the body.

Eventually, organs accumulate scars and fail. In the early 1970s, many "sicklers," as these patients are sometimes called, died by age 14. Today, the average life span is about 45 years.

The federal government is spending $45 million a year on studies. One trial led by Seattle scientists offered the hope of bone marrow transplants as a cure.

During all these changes, from the mid-1960s until 1995, Charache labored with John Paul and his brother.

"They were good people. There are lots of people who are sick, but most of them are just busy being sick," Charache said. "These two guys did more than that."

John, who wanted to be a doctor but was too ill to reach that goal, became a nurse. He lived in an apartment on Eutaw Place, getting prescriptions or milk for elderly neighbors, taking their blood pressure and even volunteering at Hopkins.

Brett became the mascot at Frederick Douglass Senior High School, strutting around the football field in a duck outfit. His big smile and personality made him popular.

For 15 years, he umpired baseball games down the street for the James Mosher Little League, even when he was taken straight to the hospital afterward.

"I can't play football, I might not be able to play basketball, but I can help with the kids," he said.

Their sister, Gwen VanWright, said her brothers seemed to have an inner strength. "It was inside of them," she said. "It was really amazing."

Gradually, the drug made a difference. John's transfusions, emergency room visits and surgeries dropped. At a news conference two years ago, he stood beside Charache and Dover as they announced that hydroxyurea was effective in reducing the frequency and severity of crises. Patients all over the world now take the drug.

Help too late

For the Paul brothers, the drug came too late to undo years of internal damage. Still, their contribution heartened them.

Family bonds also bolstered them. Charache said those close relationships, as in any illness, enabled them to survive longer.

Their siblings ferried them to doctors and the pain clinic. They all celebrated birthdays with dinner out and a movie of the honoree's choice. Carleen moved home to help with Brett, who always lived with his mother.

Brett never married, saying he didn't want any child to suffer as he did. Siblings Michael and Carleen were found to have the sickle cell gene. To stop the cycle, neither has had children.

When John died suddenly in 1995, Brett lost confidence. He glimpsed his own future.

His crises became more severe. His hip deteriorated so badly that an X-ray showed nothing but shadow. His mother said Brett once confided: "Momma, I hate to hurt you, but sometimes when it hurts this bad, I wish I was dead."

He needed a cane, crutches, then a wheelchair. His sisters were encouraging, reminding him that John had lasted to almost 43. Friends and siblings painted his room late last summer and helped him buy glossy black furniture.

The depression began to break. He set up an appointment with an orthopedic surgeon for late February. Even though he feared it, Brett planned to get a hip replacement like the one his brother had received, hoping to lessen the pain.

"He said he didn't know how he would make out," his mother said, "but he would do the best he could."

But on the first day of this month, in a rush of calling 911, Michael feeling for a heartbeat and medics struggling, Brett died.

"He looked very peaceful," said Michael, savoring the word. "All I could do was just call his name. My heart was breaking. This was my last brother."

At the sickle cell clinic, a gold plaque for Brett will be hung beneath the one already up for John.

After a short church service Thursday, the family laid Brett Paul to rest under sunny skies. They'll contribute to a sickle cell research fund at Hopkins on Brett's birthday, just as they do on John's birthday and at every Christmas. And though their brothers didn't survive, the strong family relationships will.

"We're not going to forget him. Brett's here with us, just like John has never left," said Gwen. "The family is still intact."

Pub Date: 2/09/97

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