Several holidays in 2003 will never be forgotten by Melyca and Scott Waterman of South Paris, ME. A routine ultrasound showed their first child was likely to have a rare and serious birth defect.
The ultrasound revealed large, echogenic lungs — lungs overinflated with fluids that showed "bright" on the image. The couple was quickly referred to the Center for Fetal Diagnosis and Treatment at Children's Hospital and traveled to Philadelphia for further testing.
The Watermans got the news on Halloween — their daughter had chronic high airway obstruction syndrome, or CHAOS.
CHAOS results when there is an airway obstruction blocking the fetus's trachea (windpipe) or larynx. CHAOS is associated with a variety of conditions, ranging from large, overinflated lungs to a flattened or inverted diaphragm; dilated, stretched out airways; excess abdominal fluid (ascites); and fetal heart failure (hydrops).
"We were in shock when we found out," says Melyca. "Shocked and nervous. But being at Children's was so reassuring."
As soon as they arrived, a social worker met them and explained the further testing they would need. She also explained how the Hospital could help with medical expenses, and arranged for them to stay at the Ronald McDonald House in Camden, NJ.
"The doctors explained CHAOS to me," Melyca recalls. "They said it could entail fetal surgery, and that I would need weekly stress tests and ultrasounds to check the baby's condition throughout my pregnancy. They explained the baby's chances of survival." They also pointed out that long-term hospitalization for the baby could be necessary until she was able to breathe on her own. Further surgery after birth would be necessary to remove the blockage and repair the airway and perhaps allow her to speak.
The couple now knew that fetal surgery was their only chance, says Melyca. They would continue the pregnancy, knowing there was hope. "We were in good hands," says Melyca.
Melyca was cleared to return home for the holidays, and she continued her weekly ultrasounds and stress tests. There was some good news — ascites was not developing.
A cesarean section was scheduled for February in Philadelphia and the couple returned to Children's Hospital and the Hospital of the University of Pennsylvania. On Friday the 13th, 2004, Faith was born weighing 5 lb., 6 oz. The Fetal team used the EXIT (ex utero intrapartum treatment) procedure to deliver her head and neck while keeping her attached to the placenta.
"The EXIT went extremely well," says Melyca. "They pulled out her head and right shoulder and made a secure artificial airway. They then cut the umbilical cord and took her right to the Newborn/Infant Intensive Care Unit (N/IICU).
"She had large lungs and no airway of her own. A web in her skin was fused to her vocal cords. But she was perfect — she was beautiful," said Melyca.
Faith stayed in the N/IICU for three months. And despite the worry and stress, Melyca says her experience was awesome.
"The nurses were so supportive and taught us so much. I wasn't able to hold my baby for the first 17 days, but we still learned how to take care of her and feel like real parents," Melyca says. "They taught us how to take care of the tracheotomy — how to clean and suction it, and how to change it. We learned how to put the feeding tube down her nose. In fact, when we were allowed to go to a hospital closer to home in Portland, we had to teach them how to take care of her."
Faith remained in the Portland hospital for another month and a half before going home. Now age 3, she soon will have surgery to remove the blockage and then another to reconstruct her airway. The surgery once again will be at Children's Hospital.
It's not known whether the surgery on her vocal cords will permit her to speak or whether her vocal cords are too damaged, Melyca says. But not being able to speak hasn't slowed Faith down. The other kids in her daycare group have learned sign language to communicate with her, and her mom says they all love Faith and are protective of her.
"She is very expressive with her body and communicates that way, as well as with sign language," says Melyca. "She is extremely outgoing, and also loves to dance." And although Faith knows sign language, she is also learning how to form words with her mouth in preparation for vocal cord surgery. "We are preparing her to communicate either way," says Melyca.
Melyca recalls how they first were told the baby wouldn't make it or that pregnancy termination was appropriate. "Children's was the only place of hope for us," she says. "They said those options are not the only answers for the baby."
"I know it sounds strange, but throughout this ordeal we were blessed. We were in the safest place we could be for us — and for Faith."
Originally posted: June 2009