Women With Cystic Fibrosis Defy Odds to Get Pregnant : Health: More mothers are giving birth as new treatments help extend their lives. They say their ‘miracle’ babies give them new life and hope.
Having struggled with cystic fibrosis all her life, Sharon Dingman knew pregnancy would be risky. Even if she succeeded in having a baby, she might not live to see her child through grade school.
After all, when she was diagnosed at birth with the incurable, smothering disease, she wasn’t expected even to live through childhood. When she met her husband, John, medical advances had stretched the life expectancy for people with cystic fibrosis to 21 years. She was 21 already.
“All the accounts I had read about pregnancy and CF were very negative,” said Sharon Dingman, now 26. “The women often died within the first year of giving birth, or got very sick during pregnancy.”
As she spoke, her fingers trailed over the silky hair and chubby toes of her infant son, Ian. He was born June 11 at Albany Medical Center Hospital.
The birth was celebrated as one of the first for a woman with CF in the hospital’s 146-year history. Within several weeks, two more mothers with CF gave birth to healthy babies.
Dr. Anthony Malanga, head of pulmonary medicine at the hospital, said the successful pregnancies signify optimism that CF patients can live full lives if their symptoms are carefully controlled through new treatments.
Cystic fibrosis is the most common inherited disease among Caucasians. One in 20 people carry the gene responsible for the disorder, and one in 2,000 babies have CF, said Dr. David M. Orenstein, director of the Cystic Fibrosis Center at the Children’s Hospital of Pittsburgh.
A few decades ago, most children with CF died before their second birthday, Orenstein said. Now the life expectancy is near age 30, with many people surviving into their 40s.
The disease causes the lungs to become clogged with thick, sticky mucus that provides a breeding ground for bacteria, leading to frequent infections such as bronchitis and pneumonia.
Patients require continuous antibiotics to stave off infection, and daily physical therapy to expel mucus. They frequently are hospitalized when an infection sets in.
The pancreas also is affected, causing problems absorbing nutrients from food. Thus, people with CF are usually thin and often malnourished.
Therapeutic advancements include new antibiotics, mucus thinners and anti-inflammatory drugs, improved methods of loosening and expelling mucus, and enzyme tablets to improve nutrition.
Joanne Washburn, 24, who lives 30 miles east of the Dingmans in Hoosick Falls, had to contend with the threat of miscarriage caused by a weak cervix as well as the debilitating symptoms of CF.
“I grew up wanting a family,” she said. “The doctor said it would be hard to get pregnant” because the thick mucus of CF hinders conception. A year after she married her husband Tim, she discovered she was pregnant.
“My mom cried,” Joanne Washburn said. “She didn’t want that. Everyone had tried to discourage me, even my husband. They didn’t want to lose me.”
Terribly sick with CF symptoms and unable to gain weight, she lost her first baby. She had another miscarriage a year later.
During her third pregnancy, she developed asthma and was hospitalized three times for lung infections.
“Toward the end of the pregnancy, I got so big it restricted my breathing,” she said. “They put me in an oxygen tent at 29 weeks.”
Her son Andrew was born by Caesarean section six weeks prematurely June 20. While Washburn was under anesthesia, a pulmonary team poured a liquid mucus thinner into her lungs.
“He’s given me new life, new hope,” said Joanne Washburn, cradling her son at the kitchen table of her parents’ farmhouse. “Andrew’s my miracle baby.”
Caring for a newborn as well as herself is difficult, said Washburn, who doesn’t work outside the home. She spends four to six hours a day giving herself treatments with bronchodilators, antibiotics, steroids, mucus thinners and chest-pounding exercises that help her cough up secretions.
Dingman, who works full time as a respiratory therapist, and her husband, who manages a shoe store, said they rely on help from their extended families within the community.
Both infants tested negative for CF. The parents were offered genetic testing to determine the odds of passing the disorder on.
If both parents had CF, the child also would have it. If the father was a carrier of one of the 32 most common defects in the CF gene, there would be a 50% chance that the child would inherit the disease. If the father tested negative, there would be a one-in-500 chance that the child would have CF.
If the mother’s lung function is good, pregnancy doesn’t make cystic fibrosis progress more rapidly, said Dr. Renee Samuelson, a high-risk obstetrician at the medical center.
“But if pulmonary function is low,” Samuelson said, “the stress of pregnancy can cause respiratory problems for both the mother and baby.”
Mothers with CF may have trouble meeting the fetus’s nutritional needs as well as her own, Samuelson said, and there is an increased risk of diabetes.
Dingman, who eats three times the calories a normal person would just to maintain her weight, said it was hard to do that after she developed diabetes during pregnancy.
The joy of new life mingled with sadness at the death of a friend a month after Ian was born, Dingman said.
“We went to the funeral of a girl [with CF] who was just 29. I’ve lost nine friends in the past few years.”
“I hate the thought of not being around for him,” she said, rocking her sleeping son. “I hope gene therapy comes through. I try to keep a positive attitude, even as I’m watching my friends die one by one.”
Medical researchers hope that gene therapy will cure CF by replacing the defective gene with a normal one, Malanga said. Scientists already have constructed the corrected gene, he said, but have not found the right “biological syringe,” such as a virus, to carry the gene into the patient.
John Dingman said he and his wife did a lot of soul-searching before they decided to have a child. “I was concerned that pregnancy would shorten Sharon’s life,” he said. “I told her I’d rather have 10 years with her than one year with her and a child.”
