Roanoke Times
Copyright (c) 1995, Landmark Communications, Inc.
DATE: SUNDAY, May 27, 1990 TAG: 9005250390
SECTION: EXTRA PAGE: E1 EDITION: METRO
SOURCE: CHARLES HITE MEDICAL WRITER
DATELINE: LENGTH: Long
"I bet there were five or six people who came in. They began lining up around the room and then a doctor came and sat down real close to us," Craig recalls.
Cradled in his arms was 6-month-old Katie. She cooed and gurgled, unaware she was being tested for the genetic disease most responsible for killing children and young adults in America.
"I felt this rise of fear, a rush of adrenalin, everything just swelling up," Craig says. "I don't know how Wayne Craig thumps Katie's chest during physical therapy. to describe it, but I knew right then before he said a word."
Katie had cystic fibrosis.
Craig doesn't remember much about that visit to the University of Virginia five years ago. Driving back to Roanoke from Charlottesville, he took a wrong turn near Lynchburg. He got lost and drove an hour in the wrong direction. A couple of times he had to stop the car. He and Linda embraced and cried.
They talked about a book they had read a few weeks before Katie's birth. It was called, "Alex, The Life of a Child" and it detailed the struggle with cystic fibrosis by Alexandra Deford, the daughter of sports writer Frank Deford. Alex died when she was 8.
A passage in the book still sticks in Craig's memory. On the day she died, Alex had asked her father to go to the store and buy some root beer.
How odd that felt, to go to the store. There were all these other people in the store, going on about their lives, buying things, standing in line, living a Saturday. . . . I kept thinking there must be something wrong with everybody else in the store, because they weren't buying root beer for a child of theirs, dying back at the house, a few blocks away.
That night, after Alex had died, Frank Deford took a walk in his front yard and poured the contents of the root beer bottle on the ground. He looked up to the sky and saw his daughter, cut out in all the stars. He said good night to the stars and Alex, put the empty bottle in the trash, and walked back into the house.
On the drive back from Charlottesville, Craig remembered that passage and others. He felt lonely and helpless, a man who knew he, too, could one day end up wandering in his front yard looking up at the heavens.
Looking back, Craig and his wife, Linda, feel that reading the book was part of a divine plan to help prepare them for Katie. A child with cystic fibrosis is born once in every 2,000 live births. "It was as if God had chosen us to be the parents when it was time for that child to be born."
`Clinging and creepy'
About one in every 20 persons in the United States carries the gene for cystic fibrosis, meaning there are about 12 million potential parents with the gene.
It is primarily a disease of whites. It occurs in only 1 of every 17,000 black babies and is rarer still among Asians.
About 30,000 people in the United States suffer from cystic fibrosis. Because of advances in treatment, those affected with the disease have an average life span of 27 years. In the mid-1950s, a child with CF could not expect to reach kindergarten.
CF is a disease that undermines breathing, digestion and reproduction. It attacks the lungs, the pancreas and, in males, the testes.
The common route of attack is through glands that normally produce thin, slippery secretions such as sweat, mucus, tears, saliva and digestive juices.
Mucus creates the major problems for CF patients. The body manufactures too much and it is thick and sticky. The mucus obstructs airflow to the lungs and clogs the ducts of the pancreas and testes.
Respiratory complications include difficulty breathing, chronic coughing, infections and sometimes heart failure.
About 80 percent of CF patients have digestive problems because enzymes from the pancreas that help break down food cannot reach the intestines.
Katie Craig is lucky. The disease has affected her lungs very little. Most of her problems involve the digestive tract. She overcomes those problems with the help of enzyme pills that she takes with every meal.
Looking at Katie, it is difficult to see a child with a fatal disease. She is blonde with rosy cheeks and the healthy glow of a child who still hasn't lost all of her baby fat.
Yet Katie and her family live with daily reminders of cystic fibrosis.
At least once a day - more during the cold and flu season - there is the ritual of physical therapy that helps drain the thick mucus from her lungs. Wayne or Linda hold Katie in several different positions, some sitting up, some lying across their laps. Then they clap, thump or vibrate her chest. Each position helps a particular section of the lung. Wayne's sessions are usually marked by a few tickles to make Katie laugh and thereby dislodge some mucus.
Every day Katie also takes antibiotics to help ward off respiratory infections.
Later, if Katie's respiratory problems worsen, she may face treatments with aerosol mists to help clear secretions from the lungs.
Eventually, most patients get caught up in a cycle of respiratory infections that impair lung function. Death results from pulmonary failure.
The Craigs live with the knowledge that the disease is progressive.
"It's always going to get worse. It's never going to be better," Wayne Craig says. "Next year is going to be more than this year. And you feel if you can't handle what's going on now, you've got a whole lot more to go in the future. That's the pattern that dominates everything."
Frank Deford describes living with cystic fibrosis this way:
Some diseases, even fatal ones, don't put too many demands on the family of the patient. But cystic fibrosis is a tyrant that rules everyone in the family. There is no avoiding it; it is at once clinging and creepy, smothering and penetrating; maddening for sure; often it can destroy the wh family, even as it takes the one child's life.
Robbed of time
Patricia Neece couldn't convince anyone something was seriously wrong with her 3-month-old son.
Antonio gulped down his formula but still lost weight. People told Patricia that she wasn't feeding him enough. They pointed out that because she and her husband are thin, it was only natural that their son would be.
A couple of months later, Antonio still looked emaciated and was having trouble breathing. A doctor suggested that Antonio be hospitalized and put in an oxygen tent a couple of days to help his breathing. Antonio stayed two weeks but he never did seem to get any better. He became listless and looked emaciated.
Several weeks later, Patricia, who lived in Christiansburg at the time, took Antonio to a Roanoke hospital. Doctors hospitalized Antonio again. They suspected cystic fibrosis. They ran a simple test.
Because of an abnormality in the body's exocrine glands, the salt content of sweat in CF victims is two to five times the normal amount. Parents and grandparents are often the first to notice this sign, tasting salt when they kiss a child's skin.
When the sweat test came back positive, Tony and Patricia Neece discovered their child had a chronic, fatal disease. The distraught couple asked if there was someone they could talk to, someone who had a child with CF. Wayne Craig volunteered.
The Craigs had gone through much the same experience in getting a diagnosis. Katie didn't gain weight, either. She vomited her milk, had lots of diarrhea, came down with a kidney infection and was hospitalized three times before she went to UVa for the sweat test.
Most people with cystic fibrosis are diagnosed in the first five years of life. But some cases aren't identified until much later because the disease is often confused with asthma, allergies, bronchitis, pneumonia and even a catch-all category of "failure to thrive."
The talk with Craig helped the Neeces. But some things have been tough to face, even knowing that others have faced them, too.
Perhaps most difficult for Tony Neece is knowing he probably will live to see his child die. Neece and his father are very close, and he assumed he and Antonio would have a long, close relationship. He feels cystic fibrosis has robbed him of years that he would have spent with his son.
Patricia Neece has similar feelings. They come especially before it's time for Antonio to get his periodic checkups at a special cystic fibrosis clinic every three months.
She thinks about the things she may never have that other people take for granted: watching her son graduate from college, seeing him participate in sports, being a grandmother to his children. "We don't know if he'll live that long," she says.
Antonio's illness has made Patricia savor her time with him. She tries to make sure she can hold on to memories.
Antonio is only 18 months old now. When he's ready to give up his crib, Patricia wants to buy him a race-car bed. She knows it's an expense they can't really afford, but she'll probably buy it anyway. "I look at it as a one-time chance," she says. "We don't know if we will have children again."
The subject of having children is one the Neeces have thought about a lot. Tony is more inclined to adopt. He's less willing to take the one in four chance of having another child with cystic fibrosis.
Patricia isn't sure. A child with CF, she reasons, still has a chance for a happy life. But she realizes that part of her reasoning in wanting another child is selfish. As Antonio gets older and his disease gets worse, she says her attitude could change.
They would have liked to know whether they were carriers, but abortion would not have been an option, the couples say.
Wayne Craig says he's advised his stepsons, one of whom is now in college, that they should consider being tested as carriers for the gene. "It might not change anything they would do, but at least they would know what they were in for," he says.
Then he adds, "We're still hoping for a cure. That's the biggest thing this genetics business may mean for us. At least maybe there will be some improved treatment, something that might allow Katie to lead a normal life."
by CNB