When Beverly Ray Garza went in for her 19-week prenatal ultrasound, she thought she'd leave knowing whether she had a baby girl or boy on the way. While the ultrasound answered that question — the child, her fifth with her husband Gabriel, was a girl — it raised some new, and frightening, questions. The image showed that the fetus' stomach was in the area where her lungs should be. Her doctor had never seen anything like it. That same day, he sent Beverly to a high-risk pregnancy specialist in Fresno, 35 miles away from their small town in California's central valley.
The specialist couldn't make a definite diagnosis, either. But as an instructor in the UCSF Fresno Medical Education Program, an outpost of the UCSF School of Medicine, he knew who could.
"He told me they couldn't help me there, and I would have to go to UCSF," says Beverly. "He called the Fetal Treatment Center and I spoke with someone right then and there." Two days later, Beverly made the four-hour drive to San Francisco for the first time. It was the start of a long, demanding journey to bring her daughter into the world safely.
At UCSF, a comprehensive two-hour ultrasound gave Beverly and her husband a diagnosis: Their baby had congenital diaphragmatic hernia, or CDH. In CDH, the diaphragm — the flat muscle separating the lung area from the abdomen — doesn't form completely as the fetus develops, leaving a hole. The fetus's stomach, intestine, liver and other abdominal organs may bulge through the hole and up into the chest area, crowding the lungs and preventing them from developing.
This doesn't cause immediate trouble in the womb, where the baby gets oxygen from the mother through the placenta. After birth, however, when the baby must rely on her own lung power, severe cases of CDH are often fatal.
For these babies, Beverly learned, UCSF offers an experimental procedure called tracheal occlusion, performed on the fetus during the pregnancy. UCSF was the first institution to perform a tracheal occlusion. The official birthplace of fetal surgery, it was also the first in the world to perform surgery on a fetus still in the womb.
In a tracheal occlusion, a fetal surgeon uses tiny instruments, inserted through a keyhole incision in the mother's abdomen and uterus, to place a small balloon into the fetus' trachea (windpipe). The incisions are then closed up. As the pregnancy continues, the balloon prevents fluid produced by the fetal lungs from escaping through the trachea out into the amniotic fluid, as it normally does. The accumulating fluid expands the lungs, spurring them to develop and pushing abdominal organs down toward their proper place.
Dr. Hanmin Lee, director of the Fetal Treatment Center, told Beverly that unfortunately, her case was bad enough to warrant considering the surgery.
Lee explained her options: She could continue the pregnancy and wait to see how the baby fared after birth, terminate the pregnancy or receive the fetal surgery. He made it clear that the surgery was far from a guarantee. UCSF offers it as part of a research study, approved by the FDA, to help find solutions for what would otherwise probably be fatal cases of CDH. He told her that at that point, they'd performed six such surgeries and two of the babies had survived.
"Dr. Lee made sure I knew all my options, he was very honest, he answered all my questions very openly and never rushed me," says Beverly. "He said he didn't know if the surgery would help or not, but it was an option I had. I decided right then that a small chance was better than no chance at all."
Once Garza made her decision, things moved quickly. That same day Beverly had an amniocentesis test to check her baby for other defects or genetic disorders. The Fetal Treatment Center's nurse coordinator, Jody Farrell, arranged Beverly's appointments, free lodging at UCSF's Koret Family House for Beverly, her husband and their youngest daughter, sent her directions and instructions, and made sure every question she had got answered.
When the day of her surgery arrived, on Dec. 19, 2008, Beverly had a crowd of relatives keeping vigil in the hospital, waiting for the good news that the surgery had been successful.
The good news came. Because the surgeons had used small, minimally invasive instruments and one tiny incision, Beverly didn't receive general anesthesia and was awake off and on throughout the procedure. She came out of the operating room with low blood pressure and mild contractions, but within a few hours, her blood pressure normalized and the contractions stopped.
Two days later the team told her to take it easy, watch for signs of early labor — a complication associated with fetal surgery — and sent her back to the Koret Family House, where she could stay close by and be monitored with weekly ultrasounds.
Four weeks later, Beverly returned for a second procedure to remove the balloon. Although doctors aren't yet sure, they believe removing the balloon from the trachea a few weeks before delivery may help the lungs mature. Ideally, the mother can then return home to wait out the rest of the pregnancy.
As it turned out, Beverly's case wasn't ideal. The next morning, an ultrasound revealed that her amniotic sac had separated from the placenta and there was a high risk that her water would break. Beverly would need to stay in the hospital where the doctors and nurses could keep close watch over her.
"They said that if it did break and it was a trickle, they were going to try to keep me pregnant as long as possible," says Beverly. "My water broke the next day. And it did not trickle — it broke like a water balloon. I had her that day." Beverly and her husband named the girl Samara, the one name both she and Gabriel liked. Once Beverly's condition stabilized her nurse wheeled her, bed and all, over to the Intensive Care Nursery to see her daughter.
Soon after birth, Samara had her first surgery outside the womb, to repair the hole in her diaphragm. "In this surgery we take the contents that are up in the chest that should be in the belly, and we push them gently back into the belly," explains Lee. "Then, because the hole in the diaphragm is too big to close with sutures, we put in a patch — kind of like a pair of jeans that has a large hole that's too big to sew up, so you put on a patch." Tiny Samara needed a relatively large patch; she had almost no diaphragm at all.
The first few months of Samara's life were, says Beverly, "a bumpy road." Her digestive system wasn't working well, she couldn't hold food down and she hadn't gained nearly enough weight. In March, Samara had another surgery to insert a g-tube, a feeding tube placed directly into the stomach. Three weeks later, the baby girl finally started to grow and thrive. Sixty-eight days after she was born, she went home.
The next major bump came over Memorial Day weekend. Samara began throwing up frequently and suffering from diarrhea. Initially diagnosed with pneumonia, Samara wasn't getting better, so her local doctor called the Fetal Treatment Center. The doctor on call — Dr. Shinjiro Hirose, who had assisted with several of Samara's surgeries — asked that she be airlifted back to UCSF.
CT scans and a series of ultrasounds revealed that Samara's patch had broken and her intestines had moved back into her chest, something Beverly and her husband had known was always a possibility after CDH repair. The surgeons performed another procedure to fix the patch, but this time they were able to use minimally invasive techniques instead of open surgery, leaving Samara with just two, dime-sized scars on her chest and back.
Six months and three surgeries after her birth, Samara's personality is beginning to emerge. She knows what she likes (playing, being held and getting attention) and what she doesn't (cold stethoscopes, wind and car seats).
As one of the brave pioneers of fetal treatment, Beverly has given more than anybody to earn the pleasure of watching Samara's personality unfold, but she's quick to share the credit. "If it hadn't been for UCSF and Dr. Lee, my daughter wouldn't be here," she says.
Story written in August 2009.
Sierra Senyak is a freelance writer in San Francisco.
Fetal Treatment Center
400 Parnassus Ave., A123
San Francisco, CA 94143
Phone: (415) 476-0445
Fax: (415) 502-0660