GRATEFUL TO BE ALIVE, MORE GRATEFUL, STILL, FOR MOTHERHOOD

Bracha Witonsky, 29, spends time with Shina, one of her daughters, during a hospital stay. Witonsky has cystic fibrosis, a disease that claimed her sister at age 16. Neither of her daughters has the affliction.

BY NEIL SAMSON KATZ
SPECIAL TO THE STAR LEDGER
PUBLISHED MAY 14, 2006
Star Ledger Link

NEW YORK — For Bracha Witonsky it wasn’t easy growing up with a sick sister who was fighting cystic fibrosis.

Shulamis got all the attention. Every day the family gave her physical therapy, they injected the intravenous antibiotics she needed to clear the thick mucus from her lungs and they watched helplessly as she lost pound after pound. When Shalumis died at 16, perhaps the hardest thing for 8-year-old Bracha was knowing she was sick, too.

"When my sister died, I felt when I would reach 16, I would die too," Witonsky said.

Today, Witonsky is 29 years old and living in Marine Park, Brooklyn. She even has been healthy enough to fulfill her lifelong dream “to have a baby girl and to be able to name her after my sister."

"That was my dream for life," Witonsky said. "Anything more than that was extra for me.”

This Mother’s Day Witonsky has plenty to be thankful for, notably her two baby girls — 6-year-old Shulamis Zahava (Hebrew for complete gold) and 5-year-old Shina. Neither has the deadly genetic disorder that afflicts Bacha.

Cystic fibrosis, a disease that attacks the lungs, pancreas and digestive system, has for ages been known as a childhood affliction because few patients lived past their teens. But over the past three decades, new medicines and better care have helped children with cystic fibrosis become adults. And increasingly women like Bracha are seeking out a new and unexpected challenge: motherhood.

"Now we have more and more patients living into their adult years," said Dr. Robert Zanni, a cystic fibrosis specialist at Monmouth Medical Center in Long Branch. "They don’t spend as much time in the hospital. They are going to school, getting jobs, having a family life. It’s a major difference from 30 years ago."

Indeed, half of all people with cystic fibrosis now live past 37, according to the Cystic Fibrosis Foundation, the primary advocacy group for the disease. Many patients live far longer, some into their 60s.

Still, motherhood remains a fairly exclusive club among those with the disease. There were only 107 births in 2004, according to the Cystic Fibrosis Foundation’s most recent numbers. Eight were in New York and New Jersey.

Though 10 million Americans are carriers for the disease, only an estimated 30,000 people have full blown cystic fibrosis. Patients have two defective genes that cause thick bacteria-trapping mucus to build inside the lungs and create constant infections. Over time, those infections degrade the lungs, making breathing difficult and eventually impossible.

Mucus build up also prevents the pancreas from producing enzymes vital for absorbing food. Many patients become frighteningly thin. And while there still is no cure, with powerful antibiotics, careful nutrition, and aggressive physical therapy, doctors are keeping patients healthy far longer.

"These women, when they were born, they were told they would die by the time they were 18 years old. Now they are told that the average age is 37. They are very hopeful that the number will continue to rise and they will beat the odds," said Dr. Moira Aitken, director of the adult cystic fibrosis program at the Washington University Medical Center in Seattle. "My job is to make these women live as long as they possibly can and have all the joys in life that they possibly can. And so far so good. These children bring them incredible joy."

'A TOTAL MIRACLE'

Motherhood puts new stresses on any woman. For someone with cystic fibrosis, it also means lung infections can get out control and they may have trouble gaining weight. The antibiotics used to treat their infections might also harm their fetuses. But many doctors have been able to keep mother and babies healthy, and genetic testing makes it nearly impossible for mothers to pass the disease to their children.

"Ten years ago we were concerned that it would be a real drain on them and they would lose lung function and couldn’t recover," Zanni said. "But I think we are in a new era now. They are being successful as mothers."

That doesn’t mean pregnancies are always easy.

Bracha spent weeks in the hospital throughout her first pregnancy and delivered Shulamis eight weeks early, a common problem among cystic fibrosis moms. Shulamis was kept in the intensive care unit and went into crisis days later, unable to breath.

"I ran under my blanket and started crying," Bracha said. "I thought she had cystic fibrosis or something was really wrong with her."

Shulamis did not have cystic fibrosis, but there was a hole in each of her tiny lungs. The doctors said Shulamis had seven months to live if she made it at all, according to Bracha. But instead she went home in two and a half weeks and has been healthy since.

"It was a total miracle," Bracha said. "All of my family prayed a whole book of psalms every day."

Many mothers with cystic fibrosis have an easier time of it.

Melissa Shiffman, a 32-year-old with a blue-eyed, baby girl, lives on Manhattan’s Lower East Side. She also had a sibling who died of the disease when she was a child. Though Shiffman has had 11 surgeries to remove blockages from her nose and, like many other cystic fibrosis patients, requires daily physical therapy and antibiotics, she always felt healthy enough to tackle pregnancy.

"It never occurred to me that I wouldn’t give birth," Shiffman said. "My doctors freaked out at first when I told them I wanted to have a child. We had a very long talk about it. They were very clear on the pros and cons, well not the pros just the cons."

Luckily, her doctors’ concerns proved largely unfounded. Though she delivered baby Chloe two and a half weeks early, both mother and daughter’s health held firm.

"I loved it," Shiffman said while laughing. "There were a couple of times I was nervous because that is my nature, but for the most part it was just amazing. Just to feel the progression of it. Going to the doctor at seven weeks and hearing a heartbeat is an overwhelming feeling. You don’t realize it until that moment, watching the baby grow, feeling it kick, having her hiccup, talking, singing, and dancing. I was really excited and nervous, but mostly excited."

A DIFFICULT CHOICE


Melissa Shiffman, a 32-year-old mother with cystic fibrosis plays with her infant daughter, Chloe.

On a recent sunny afternoon, Shiffman rolled around the carpet with her 1-year-old daughter. Chloe put on a fashion show with mommy’s hat, a big pink floppy number, and wild sunglasses to match. Chloe accessorized the ensemble with three different wristwatches while mom beamed a thousand watt smile.

Shiffman is now considering her second child but she also has doubts. "I’m worried that I’m being selfish and playing Russian roulette with (husband) Steve and Chloe’s future because I want two kids," she wrote in an e-mail. "There is no guarantee that my health would be jeopardized at all, but there are no guarantees that it wouldn’t."

Counseling young women about the risks of motherhood is tough, according to doctors who treat the disorder.

"CF is still a life shortening disease," said Dr. Mark Tonelli, a medical ethicist and cystic fibrosis specialist at the Washington University Medical Center. "We have to talk about the possibility that they may not be alive in 20 years. They need to acknowledge there is a significant risk that they may not be there to raise their children."

But many cystic fibrosis patients, like Erin McNalty, a 31-year-old social worker from Yonkers, N.Y., who is thirty weeks pregnant with twins, face that question head on.

"When I was born, the average age to live was 13. In my teenage years I thought I would never go to college. Then in college I though I would never get married. So I am always waiting for another shoe to drop," she said, adding that "if my health starts to decline I will be able to teach my kids who I was. And they will be strong enough to go on."

Despite the risks, many doctors remain optimistic about their patients becoming mothers.

"These are people that don’t take their children for granted, that’s for sure," Tonelli said. "And I think they understand better than the rest of us that each day is precious."

Linda Clark, a 57-year-old, crisis counselor, from Chehalis, W.A. knows better than most. Despite battling cystic fibrosis she has two grown children and six young grandchildren.

“They are healthy beautiful children and very much a delight in my life,” Clark said. “They are such an encouragement and make me focus so much on being alive.”

“I am here for them and they are here for me and that is an awesome force.”

Neil Samson Katz is a freelance writer and photographer based in New York City. He may be reached at neil@neilkatzphoto.com.