Stories

The Times-Picayune
May 15, 2005

Keith O'Brien
Staff Writer

METAIRIE, La. -- Slow songs always made the little girl sad. The Wards were never sure why. Amanda couldn’t tell them. She couldn’t talk. By the end, she couldn’t walk either, or feed herself, or go to the bathroom. Simple tasks were crippling. A regular school life impossible. Many times, even breathing was a problem.

Jim Ward would wake in the middle of the night and pad down the carpeted hallway of their modest Metairie home to check on Amanda. He wanted to make sure her tiny body still rose and fell with each rattling breath. In the doorway, he would pause, wary of what he might find.

He and his wife, Missy, knew how the story ended. They knew there was no beating this disease, that one day Niemann-Pick Type C would kill Amanda, the youngest of their three children, just as it had killed their oldest, Adam, in 2000. There was nothing love or science could do about it. Nothing they could learn from one death to stop another. It was cruel, and it was real, and it was coming.

"Look at her," Missy said to her husband.

It was Christmas Day. They were at Tulane Hospital. Amanda, 10, was having trouble breathing again. And this time, Missy refused to let doctors put her on a ventilator. She had seen them do this just six months earlier. And although it had worked, Missy couldn’t bear to watch her daughter, blond and stubborn, fight the tubes and doctors again.

Jim Ward wasn’t so sure. How do you let go of something when you have held on so tight for so long? How do you release the disease when the disease has become you, changed everything? When the disease is your child?

He wavered. They cried. Missy walked to a window. Outside, on the streets of New Orleans, it was snowing, a Christmas miracle of sorts, and Missy, like so many others that day, watched it fall from the heavens.

This, however, is not a story of simple miracles. There will be a funeral, and a eulogy, and an epitaph. There will be tears. And then the girl, and her disease, will be gone. Some things cannot be beaten.

But in their darkest moments, as they mourned one child and prepared to mourn another, the Wards stumbled onto a secret most will never know and a peace most will never find.

They discovered themselves.

. . . . . . .

It began in December 1985, when Jim Ward bought a new Dodge Omni and decided to spend $20 extra on car insurance just so he could talk to Missy Webre one more time.

It was a decision that went against his nature. Jim is a computer programmer at a Chevron plant in Belle Chasse. He’s all about calculations, action plans, mission statements. Twenty dollars is $20. But this was different.

He wasn’t just seeking car insurance. Jim wanted a date with the blond woman he had first met months earlier when she briefly dated one of his co-workers. The calculation paid off. She said yes.

Missy thought Jim was funny. She liked the way he danced and his solid, middle-class background. They were similar, she thought. But not too similar. Jim believed in what he called the Lego Principle. Flat pieces, he told her, don’t work with other flat pieces. They don’t mesh. A flat piece needs a bumpy piece, he said, and vice versa. Then the pieces stick together.

He was outgoing and energetic. She was calm and measured. Jim and Missy stuck.

They were married in a rainstorm in May 1988. Streets were flooding, the electricity knocked out. Without power, the church organ wouldn’t work. Candlelight was all they had to read by. And Janet Pease, Jim’s sister, kept waiting for someone to scream. Many brides would have lost it over less. But as usual, Missy was calm. Everything, she kept saying, would be fine. And it was.

The couple wanted children and they had them: Adam first in January 1990, followed by Chelsea in April 1992. They were healthy. They were a family. They had a three-bedroom house in Metairie and plans. Jim was always planning. Then on Nov. 23, 1994, Amanda Ward, their third child, was born, and plans changed.

Amanda was discolored and bloated. She had spleen problems, blood irregularities, then liver failure. Missy cried -- at times Jim couldn’t get her to stop -- while Chelsea asked, "Where is our baby?" They didn’t know what to say or do.

Amanda needed a liver transplant to live and she got one three months after she was born. But that didn’t solve her problems. Doctors found that she also suffered from cystic fibrosis, a disease that causes the body to produce a thick, sticky mucus that makes it hard to breathe.

This, Missy decided, they could handle. It was the spring of 1995 and her daughter finally was leaving the hospital, going home. That’s what mattered. Children can live for decades with cystic fibrosis. But more tests that summer revealed yet another problem, one that would change everything.

In addition to cystic fibrosis, Amanda suffered from Niemann-Pick Type C, a rare genetic disorder that strikes the nervous system and tears it apart over time, robbing people, mostly children, of both mental and physical capabilities.

Jim and Missy, now both 43, gasped when they got this final, devastating diagnosis that October. There was no other known case of this disease in Louisiana at the time. No cure, either. But the Wards pushed on, distracted that fall by other problems in the house, mysterious clues that bewildered, then disturbed them.

Adam, their perfect son, was 5 years old and losing his mind piece by painful piece.

. . . . . . .

He was their first-born child, solid at 7 pounds, 11 ounces. He ate well. He almost slept through the night his first week home from the hospital. He crawled at 8 months, walked on his first birthday, and was hitting a baseball in the yard at age 3.

Jim was proud of Adam. Big plans filled his mind. His son would be an engineer, he thought. Or a baseball player. Or a writer. Or a genius who designed the cars that Adam loved so much.

From the moment he began talking, Adam identified cars, pointing, as the Wards drove down the road: Omni, Honda, Buick, Toyota. Adam knew them all by name. Talking, though, didn’t come as easily for him.

He suffered from what appeared to be a mild speech impediment. He slurred his words at times. His parents enrolled him in speech therapy. But they weren’t too concerned. Don’t many children have trouble saying certain words?

Missy worked with him. In the summer of 1995 -- as Amanda came home from the hospital and Adam prepared for kindergarten at St. Clement of Rome -- she used flash cards to help him learn his colors, numbers and letters. By the fall, the Wards had been told, Adam was expected to know these things as well as how to tie his shoelaces, count to 10, and spell his name.

These were guidelines -- not directives -- but Adam missed them anyway. As best Missy could tell, her son had marginally mastered only half of his assigned tasks by the time school started that year. And soon he was having other problems as well.

On the first day of school, he couldn’t find his desk, even though his name was on it. On the second day of school, he cried and failed to color within the lines during a drawing exercise, filling the page with wild, angry lines. The teacher was concerned by his performance and his attempt to hide his work from her. It was as if he knew he was doing something wrong. She sent a note home to the Wards.

Missy didn’t need this. Not now. She met with the teacher. Tests followed. It was believed that Adam had a learning disability, but extra attention didn’t seem to help. He continued to cry at school. He wet his pants. He lost friends. Missy drove by school one day and found her son standing alone at recess, playing with no one, a lost boy on the outside looking in.

The Wards were troubled and they became more troubled that winter when Adam began falling down, bumping into things. He appeared dazed in the months ahead, almost drunk at times. Simple everyday activities, such as playing on a slide or finding his shoes, now eluded him. He stared blankly at his parents from time to time. He lost control of his bowels.

Up until now, they had chosen not to worry much about Amanda’s strange disease, Niemann-Pick Type C. Jim and Missy figured their infant daughter wouldn’t suffer from its crippling symptoms for years. And maybe by then, they told themselves, there would be a cure to save her. They had time.

But now they were forced to recognize the obvious. Adam didn’t have a learning disability or a mild mental handicap, as some experts believed. He had the disease, too. He was dying, too. And much faster than his sister was.

Doctors delivered this news to the Wards in October 1996, one year after they had received Amanda’s diagnosis. A swift decline followed. Like an old man suffering from Alzheimer’s disease, the boy became increasingly confused, unable to find the chest of drawers in his bedroom or the bathroom down the hall. He could no longer name the cars he loved or, in the end, identify his own father. But dementia was only part of his problem. His body also was slipping away.

Within a year, Adam stopped talking in complete sentences. He soon couldn’t walk or feed himself, either. Seizures floored him. Brittle bones snapped. His body became a prison collapsing upon itself. Adam was dying from the inside out, fluid filling his lungs. It became harder and harder to breathe.

So when Adam finally died of respiratory failure in June 2000, leaving the Wards with two children -- one healthy, one sick -- Missy didn’t know what to feel.

She was numb.

. . . . . . .

Dr. Michael Kiernan has worked with thousands of patients since 1980, hundreds of kids suffering from cystic fibrosis and dozens with neurological disorders. But until he met the Wards in the mid-1990s, Kiernan, a pediatric pulmonologist at Tulane Hospital, had never seen one patient with Niemann-Pick, much less two.

The odds are stacked against it. It is estimated that only one in 200 people carry the NPC1 gene that causes the disease. Both parents must carry this gene, found on chromosome 18, in order for their child to have a chance of getting the disease. And even then, researchers estimate there’s only a 25 percent chance that the child will inherit the gene from both parents and become infected.

Adam and Amanda were among a mere 500 cases estimated to have been diagnosed worldwide. And Kiernan would have to turn to textbooks and to the Wards themselves to understand what was happening to the kids: how they couldn’t metabolize cholesterol in their cells like other people, how this would lead to cholesterol buildups in the spleen, liver and brain, and then ultimately to neurological problems and death. Brain cells -- for reasons still unknown to researchers -- begin to die.

The disease, Kiernan learned, was like a steamroller. Nothing could stop or even slow it. And he eyed the Wards, knowing that parents of far healthier children have been torn apart by lesser diseases. When dreams shatter, so can the relationships that built them. There is guilt. There is blame. People argue. People snap. Even close couples grow apart. Some get divorced.

But the Wards refused to give in to the disease that had begun stealing Adam from them. Like an outmatched boxer on the ropes, they jabbed back, determined to stay on their feet and answer the bell at the start of the next round.

That was the victory. And while their optimism, faith and upbeat attitude stunned Kiernan and others -- what was there to be optimistic about, they wondered? -- the Wards knew this was the only way to survive.

. . . . . . .

They fell into a routine. Church on Saturday evenings. A visit to Adam’s grave on weekends. Blessings from the priest once a month and vacations in the summer. It wasn’t, however, a typical life.

Jim and Missy vacationed apart at times. It was the only way they could get a break. Someone always had to be with Amanda. She had never advanced as far as her brother. She never ate solid food like he once did, outgrew diapers, or attended preschool with other healthy children. She attended special education classes instead, wore diapers that needed to be changed by others, and ate through a "button" surgically implanted in her abdomen.

These feedings structured the day. Jim, wired on chicory coffee, took care of them at night. He fed Amanda, pumping her full of medication, protein shakes from a can, and three large syringes of water. Then he cleaned the syringes and laid them out on a tin platter in the kitchen for Missy, who would shoulder the feeding in the morning after Jim went to work and before the girls went to school.

There were breathing treatments, too -- attempts to keep Amanda’s lungs clear of mucus and infection. This was partly a complication of the cystic fibrosis. But Niemann-Pick made it worse. Many children who suffer from the disease, including Adam, die of respiratory problems. Soon, the Wards knew, breathing treatments alone wouldn’t be enough to help Amanda. They would have to suction mucus from her with a machine while she grimaced and they soothed her.

Chelsea was growing up. She was taking French horn lessons, and going to sleepovers, and dancing with a boy for the first time. Amanda was just growing.

"Tomorrow you’re going to be 7," Missy told Amanda in November 2001 on the eve of her birthday. Amanda smiled, but said nothing.

She had stopped speaking suddenly in the months before her brother died. The few words she had mastered -- doll, mom, dad, "Chel" for Chelsea, and "Bud" for Adam -- were gone. By that fall, she couldn’t walk, either -- something she had been able to do at her brother’s funeral some 17 months earlier. Amanda rolled instead on a toddler’s walker or teetered on her tiny feet while her parents held her hands above her head.

"What do you want?" Missy would ask her when she became agitated. "Come here. Walk with Mama. Walk with Mama. Come on now. Use that foot. You know how. You can do it."

Amanda struggled against her body while her parents held her up. They believed she still was in there, understanding them even if she couldn’t speak anymore.

Others did, too. And in July 2002, months after Amanda’s birthday, many of the faithful gathered in New Orleans for the 10th annual Niemann-Pick family conference, a four-day event that included medical discussions, research updates, fellowship and finally a candlelight vigil.

"I light this candle in honor of Amanda. I hope and pray for a cure, not just for her but for all the children, so that no more children have to needlessly suffer."

As Missy spoke these words, a hush fell over the darkened hotel ballroom. It wasn’t a huge crowd. There weren’t enough people to fill the room. There were only about three dozen there. But these people understood the Wards. Each family had at least one child suffering from Niemann-Pick and a reason for being there. Jim spoke next.

"I light this candle in memory of Adam."

He bowed his head and fought back his tears. The candlelight flickered, then spread around the circle, each parent speaking for someone or something. They lit their candles for Michelle and Andrew and the diagnosis they had prayed for and received. They lit their candles for the brothers and sisters of Niemann-Pick children, and for the parents who raised them, and for the inspiration they found in each other.

"I light this candle in honor of my son Roy who drives me nuts but keeps me fighting this disease."

"I light this candle for my baby."

"I light this candle in honor of every one of you."

Outside the doors of the darkened ballroom, their children filled the halls. Strangers stared or looked away. The disease’s external toll -- crooked bodies, gnarled hands, and blank eyes -- was everywhere, a parent’s worst nightmare. And the Wards had lived with it long enough to know that many couldn’t deal with it.

These people asked awkward questions, fumbled over themselves in the presence of Amanda, or simply didn’t get it. In one such conversation years ago, Jim listened as a relative questioned whether or not Amanda was even "in there" anymore, if she could understand anything at all.

"Boy," the man said to Jim, "it’s awfully hard to tell."

It was a legitimate observation, an issue that had once troubled Jim as well. The first time Adam couldn’t identify him, Jim broke down and cried. "Where are you?" he remembers asking of his son. "You were in there, buddy. Where are you?"

Now he refused to think that way. "Our kids are just a little more difficult to read," he told those who asked. You do your best, he added. "Then, you just kind of guess."

. . . . . . .

On a Wednesday morning four weeks later, Amanda suffered her first seizure, the beginning of the end, and her mother lingered over her limp body on the living room floor.

"What’s the matter, baby?"

"Why are you like this this morning?"

"It’s all right. Mommy’s with you."

Amanda was exhausted and Missy wasn’t surprised. Seizures, once they started, had always left Adam lethargic. What troubled Missy was the pattern she had noted lately. Amanda had appeared tired for months, drooling more, laughing less. Now Missy knew it was about to get worse.

By her 8th birthday that November, Amanda did not sit on her mother’s lap as everyone else sang "Happy Birthday." She was strapped in a wheelchair, groggy and staring at the ceiling while her father prodded her, saying, "Smile. Smile. Wake up, little Amanda."

Brief spells of violent coughing awakened her. But Amanda’s smile was elusive. She frowned now as her parents suctioned mucus out of her throat and called for towels to mop up her face. The suctioning machine was loud. The birthday party somewhat deflated. "Where’s your smile?" Jim asked the girl.

The seizures continued -- not that night, but throughout the winter and into the spring. The Wards called it "dancing," a euphemism to describe Amanda’s bobbing extremities. But they knew it was serious.

"Relax," Missy told Amanda during a seizure at the kitchen table in February 2003. "Relax. . . . No. No dancing."

Missy locked the wheelchair to keep Amanda from rolling away and moved closer to her, speaking to her in a mother’s whisper. "You think you’re Ginger Rogers? Trying to dance?"

Amanda looked at her mother, helpless. "Relax," Missy said again.

The girl finally did.

"It’s OK," the mother told her, soothing her.

The stress was beginning to take a toll on Missy. A year earlier, Amanda could laugh, smile and embrace her mother. Now, if Missy wanted a hug from her daughter, she had to take Amanda’s arms and drape them over her own shoulders.

"Let it out," she told her coughing daughter. "Get this stuff moving."

Missy daydreamed about the future. She picked up a cruise brochure. Jim had always promised that one day he would take her on a cruise. But it wasn’t long before she threw the brochure away. They probably couldn’t afford it anyway, she told herself. Also, she felt guilty.

"There you go," she told her wheezing daughter. "It’s all right. It’s all right . . ."

Daydreams of cruise vacations meant that Amanda wasn’t around, which meant that she had died, which meant that Missy’s family of five had become a family of three in a matter of years. She wanted Chelsea to have siblings. She wanted what they had before and feared what was to come.

But as the months passed and Amanda went on oxygen full time -- from a half-liter per minute in the beginning to 2 liters per minute near the end -- Missy also couldn’t ignore what was happening.

Amanda was dying.

"Look at her," she said on Christmas Day. "Look at what she’s been going through."

Amanda had been fine at church the night before. Her oxygen saturation numbers were good -- or at least as good as they had been. But sometime in the night, she took a turn. They had to take her to the hospital in the morning, much to her sister’s disappointment. And there, as it began to snow outside, Jim and Missy made a decision they had been unable to make six months earlier.

Instead of putting Amanda on a ventilator, as they had done to save her in June 2004, the Wards asked doctors to give Amanda antibiotics for the infection in her lungs, to make her comfortable, and let the disease take her.

Three days later on Dec. 28, she was dead, 19 years to the day of Jim and Missy’s first date.

. . . . . . .

Dr. Kiernan had told them to move on without second-guessing themselves. The Wards did that. They were at peace with their decision. It was the silence that bothered them on New Year’s Eve, hours after Amanda was entombed next to her brother.

For the first time in years, Jim and Missy had nothing to do. There was no one to suction. No one to feed. The oxygen sensors were still. The house quiet. They stared at the television, watching people ring in the new year. Chelsea, now 13, had asked to stay up until midnight.

But when the moment came, there was little celebrating in the Wards’ house. They clicked off the TV and went to bed, walking down the narrow hallway past the empty room with children’s stickers on the door.

"Adam," one sticker said.

"Amanda," said another.

The room was empty. Jim and Missy awoke in the night, listening for Amanda’s coughing. What they heard instead was Chelsea grinding her teeth, something Missy had not heard before. She visited the empty room and cried. Jim came home from work and wandered around outside. Missy wished he would get a hobby. She had her writing, and her love of movies, and her Mary Kay makeup demonstrations. What did he have?

Jim wasn’t sure. He began sleeping more and working overtime at the plant. His trips to Home Depot on weekends felt odd. Amanda had always gone with him. His runs around the neighborhood were different, too. She had been, in a sense, his running partner, there in her wheelchair, pushed along by her father as he jogged. Amanda was his balance. And without her now, without her wheelchair to hold him up, Jim began to trip and fall, bloodying himself in the street. He kept running, though, and things got better.

Amanda hadn’t beaten Niemann-Pick, Missy had told the crowd that gathered for her funeral on New Year’s Eve. But she had survived an organ transplant, surgeries, and desperate nights without air, managing to live with two diseases for a decade. And while that wasn’t the miracle they had wanted, it was a miracle nonetheless, Missy said. It awakened them from what Missy called "our little suburban life" and taught them lessons that may have eluded others along the way.

Patience and compassion. That babies are beautiful even with tubes attached to their faces. And that family life can include dance lessons and tube feedings at the same time and still be worth living, still be worth fighting for.

"Some people feel we have been cheated as parents, having two children taken from us at the age of 10," Missy told those gathered at the funeral. "True, it was never our desire to outlive even one of our children. But we have realized the blessing in having been chosen to be parents at all, and particularly of the three wonderful children we were given.

"Amanda will be missed," she continued, "as her brother, Adam, has been missed for these past 4_ years. But our faith has taught us that she will live with us in spirit, and in our hearts, and through each other. We will see something of her in other children at times. And all we need to do is close our eyes, and her smiling face will be there for us."

Slowly, the Wards found ways to move on. They began sleeping through the night. They prepared to give away Amanda’s hospital bed and wheelchair. They made plans to attend Chelsea’s band camp together in San Antonio as chaperones, something they had never been able to do. They went to dinner alone one night while Chelsea went to a dance with her friends. And then, at the end of February, Jim and Missy went away for the weekend, just the two of them, in a hotel on Canal Street in New Orleans.

It was a gift from their pastor. They never would have arranged something so extravagant on their own, staying in a suite, overlooking the Mississippi River. Twenty dollars is still $20. But the Wards were happy to have this moment, to drink wine and eat cheese compliments of the hotel, to hold hands and walk the city streets, tourists in their own town. They watched the cruise ships roll down the river and, for a while, appeared like any other couple.

It was a bittersweet moment. The only reason they were there was because Adam and Amanda were gone. The empty room was there to remind them of that when they got home that weekend. Like the disease itself, there is no escaping it. But they still have each other, memories of the past, and plans for the future. There is another child to raise.

Chelsea is blond like her mother. She is a budding musician and actress, filled with life and laughter. She will start eighth grade in the fall.

And she has big dreams that make her parents proud. When she grows up, she wants to be a pediatrician.