“For as long as he is here, we are his parents.”
Mt two latex-gloved fingers extended as far into Greta's vagina as they could through the tight rubbery of of her cervix, grazing the firm, smooth, slippery surface of her baby’s lowest part, but I could not feel the narrow gaps between the bones in the baby’s head. The “sutures” of the infant skull are what we use to confirm that the hard structure meeting the examiner’s fingertips is the baby’s head. I couldn’t be sure.
“We’ll need to do an ultrasound to make sure the baby’s head is down,” I told Greta as I extracted my fingers and snapped off the glove. She nodded, knowing from my previous explanations the importance of making sure that her baby was presenting “vertex” (head down) and not “breech” (butt or legs first). About 3 percent of babies are breech at the end of pregnancy; these babies have unacceptably high complication rates during vaginal delivery and therefore must enter the world via cesarean section. Earlier in the year, three consecutive first-time-pregnant patients of mine had breech babies. Many times we can turn these around via a procedure we call “version,” but the first we could not turn and the other two went into labor early, so all three had C-sections.
Two evenings later, on Wednesday, my afternoon off, my pager interrupted me during dinner with its rude vibration against my belt. I can’t seem to help getting annoyed when I’m paged on my days off. Often, these pages come to me by mistake. The answering service that connects patients to their physicians on call has trouble finding employees capable of reading a schedule and paging the right doctor.
This one wasn’t a mistake; it was Greta. “The doctor said the ultrasound showed a serious problem with the baby’s heart.” Her Swiss-German accent didn’t hide her concern.
Few things upset me more than patients calling me with questions about abnormal test results before I know about them. It should never happen. In this instance, the radiologist interpreting the ultrasound should have called me the moment he finished. The facility where the test was done was now closed. So here was Greta, full of anxiety and questions, calling her doctor, who could not help her. I apologized and promised I would figure things out first thing in the morning and call her back.
“She [the radiologist] said there was something in the heart,” Greta explained.
“Ultrasounds are not always that great for seeing things as they really are,” I offered in an attempt at reassurance. “I need to speak to the radiologist before I can give you advice that has any meaning. I’m really sorry I don’t know more right now.”
Worried and seething, I called my office, knowing that someone would still be there doing evening office hours. “I passed that message on to Sherri,” Bart, one of our better medical assistants, assured me, “and she told me to go ahead and arrange for Greta to see a pediatric cardiologist in two days.” Sherri is a great nurse practitioner, but sometimes she goes overboard to protect me. I’m the only provider in our practice who does obstetrics, so I have to know every time something important happens to a pregnant patient, and I have to decide who goes where and when.
I called the radiologist first thing the next day to get the news: the baby had a three-centimeter mass in the left ventricle. I don’t know what the average diameter of a newborn’s left ventricle is, but it’s not much more than three centimeters, so this mass was occupying most of the ventricle, which makes it difficult for the ventricle to serve its purpose: pumping oxygen-containing blood through the aorta into the entire body. This was, therefore, a disaster. It was also a surprise. Her ultrasound 20 weeks earlier had been normal. My wife Terry believes that all things happen for a purpose, and she may have been right to claim that the reason I had the had luck with three consecutive breech babies was to make sure I would order the ultrasound for this one so we could discover the heart problem before the baby’s birth. Masses in the heart are not common. They usually are rhabdomyomas, or lumps of smooth muscle, which grow because the person has an inherited disease. Greta’s baby appeared to have a single large mass in the heart, but none anywhere else, which gave the baby about a 50 percent chance of having tuberous sclerosis. TS causes these masses to form in many different organs, including the skin and the brain. Most people with TS have central nervous system involvement, which often causes seizures and leads to mental retardation in about a quarter of its victims. TS is passed on in families, but most people who have it acquire it through a spontaneous mutation and do not have any family history of the disorder. Of the other conditions associated with rhabdomyomas, most carry a similar or worse prognosis than TS.
So when I called Greta and Ken, her African-American software-engineer husband, whom I had met once during one of her prenatal visits, I told them to come right in to the office because I knew this would not be appropriate to handle over the phone. I didn’t have any appointments free that morning, but when something like this happens, other people have to wait. I had to tell them their son had a huge cardiac mass that would pose a formidable threat to his life within hours after birth. At birth, babies have a connection between the pulmonary artery and the aorta called the ductus arteriosus. The ductus allows blood coming from the right ventricle into the pulmonary artery to go straight into the aorta, avoiding the lungs and left side of the heart. Since this blood does not go to the lungs and exchange carbon dioxide for oxygen, the body cannot allow this to continue. The ductus almost always closes in the first 48 hours after birth, at which time the left ventricle must pump all the blood into the aorta. It seemed obvious that Greta’s baby’s left ventricle would not be able to do this unless a cardiac surgeon either redirected the circulation or removed some of the mass soon after birth.
Remembering why we got the ultrasound in the first place, I told them the baby’s head was down, as it should be.
Greta and Ken didn’t hide their anxiety as we discussed this information. I made sure they understood that we did not yet have enough information to make any real plans; those would have to wait until after the next morning’s consultation with Stuart, the pediatric cardiologist.
He called me during my lunch hour the next day, Friday, with the confirmation. “This is the biggest cardiac mass I’ve ever seen,” he told me. It was so big he did not see any blood getting around it. This would mean that the aorta could not have received the usual blood volume as the baby developed, which leads to poor development of the outflow tract, and the ultrasound seemed to show this as well.
Stuart had offered Greta and Ken two potential surgical options. The first would assume that the mass had prevented the left ventricle from full development, a condition called hypoplastic left heart syndrome. A hypoplastic left ventricle never becomes strong enough to pump the necessary volume of blood to sustain life. Babies with this syndrome undergo three surgeries; one just after birth, one at about age three months, and a third between age three and six years. These convert the heart to a single-ventricle system that pumps blood simultaneously to the heart and the lungs. Under the best circumstances, a person undergoing these surgeries can expect to live into his or her 20s and cannot do any vigorous activities.
Option two was to take out as much of the mass as the surgeon could, working through the aortic valve, which is about four millimeters, or a sixth of an inch, in diameter. If the ventricle were strong, this would allow it to fill with enough blood to sustain the baby for the first few months while the mass shrank in size. Rhabdomyomas shrink and usually vanish somewhere between six months and a year of age. Stuart thought this baby would have about a 50 percent chance of having a strong enough heart to survive after this one procedure.
Stuart and I figured Greta and Ken would choose the first option, given that it offered the best statistical chance for survival Most people when confronted with information such as this about their unborn baby near the end of the pregnancy don’t have to think much about their decision; they do whatever they can for the baby. My wife and I would not have had to think five minutes before deciding to send the baby to surgery and hope for the best.
Greta and Ken were more inclined to think about the baby’s suffering and our inability to give precise information, given that this was a one-in-a-million case.
“Our baby is not having three surgeries.” Ken’s soft voice on the speaker phone held a defiant note. “He is our son. He is not a guinea pig.”
So that left the tumor-reduction procedure, but as we continued our conversations, the prospective parents made it clear they weren’t in favor of that either.
Later that day Greta’s father Karl, a physicist at Stanford University, emphasized the mental retardation risks. “Having a baby with so many needs would put too great a strain on our family.”
Ken concurred, from a different angle. “My mother is 80 years old and has a very weak heart. Putting her grandchild through heart surgery would kill her.”
Greta spoke the least, but during our Thursday and Friday telephone conversations her tones became more measured and uneven, as she fought off the fatigue of late pregnancy to focus her thoughts on her child who would die so soon after birth.“It’s hard enough just to have a baby,” she observed, her voice wavering through tears. “This is killing me.”
I knew that. This was my first experience in private practice with a mother about to deliver near her due date a baby that wouldn’t survive outside, but when babies die in the womb, mothers want them out as soon as possible. Greta would want to get this done to end the suspense, although there would be guilt, and she knew it “As long as I keep him inside, he lives. When he’s born, he dies.”
They had made their decision: no surgeries. Stuart wanted them to see Elaine, a medical geneticist, to get the proper advice about what the odds were that their baby would have tuberous sclerosis or other inherited disorders, and they made an appointment to see her Monday, but they assured me this would not make any difference. Therefore, there wasn’t any reason to delay her delivery. Inducing Greta’s labor would increase her chances of a cesarean section, but she did not mind that. Labor would not present any unusual problems for her baby because he would not redirect his circulation to rely on the left ventricle until the ductus arteriosus closed sometime after birth. I set her up for an induction the following Wednesday. I notified the neonatalogists, the hospital-based specialists who take care of seriously ill newborns and who would be administering“comfort care only” to Ken and Greta’s baby. Vishu, the neonatologist on duty that day, agreed to the plan but gave me a friendly warning: “These little fellows don’t always do what you think they will [die]. They have their own agenda.” I knew that; adults sometimes do the same thing.
I went home that night and discussed the case again with Terry. I knew it troubled her. “I’m sorry, but if they think it’s inconvenient for their family to have this child, then they should put him up for adoption.” Was the cardiac mass a “fortuitous” circumstance allowing Ken and Greta to perform what would amount to a late abortion so they would not risk having a mentally retarded child?
My philosophy is not to take sides in these situations. My first responsibility, in my mind, is to be my patients’ advocates and to try to help them, within reason, to achieve the outcomes they desire. Ken and Greta cared about and grieved for their baby. “He is our son” was Ken’s tearful reply when I had raised the adoption question.“As long as he is here, we will be his parents. We can’t give him away.”
That weekend, two of my other expectant mothers went into labor, and I assisted their babies’ safe landings into the world, welcome respites of normalcy to break the craziness of the previous two days. Stuart got things rolling again on Monday with a phone call.
“My issue is this,” he began.“If they have decided not to do anything, that puts me in a difficult position. I have made a judgment that their baby is not viable based on the results of an ultrasound done through the mother. I’m not God. I do not feel comfortable giving this baby a death sentence because of an intrauterine echocardiogram [ultrasound of the heart].” He needed me to talk the family into allowing us to do whatever was necessary to keep the baby alive long enough to do another echocardiogram after birth, which would give us better images so we could be more certain about the size of the mass and the prognosis. The open ductus arteriosus would probably give us enough time to do the study without giving the baby any medication, but I would need to get permission in advance for the neonatologists to give supplemental oxygen and put an intravenous catheter (IV) into the umbilical vein to administer any necessary medications.
Ken and Greta put me on their speaker phone, and we discussed what all of this would mean. We would take the baby away right after birth. Most likely, he would turn as pink as a normal newborn and the pediatric cardiologist would do the echocardiogram. If necessary, we would give him extra oxygen from a bag and mask and put the umbilical catheter in. Placing an IV always involves puncturing the skin except in newborns, because cutting the umbilical cord leaves three blood vessels facing the outside world in cross-section: two umbilical arteries and one umbilical vein. We clamp the cord before we cut it, but if you take the clamp off, you can thread a catheter into the open vein without the need for any needles to puncture skin. So these measures would not be painful for the baby. If the baby required more intensive interventions, such as CPR, a breathing tube, and a mechanical ventilator, we would not do them because we knew he would not live anyway. Doing the test would cause Greta and Ken to lose some time with the baby, and since we could not predict how long the baby would live, this was not a trivial concern. We agreed to meet for an appointment to finish all the arrangements the next day, Tuesday, at 11:45 a.m.
About a half hour before their appointment, as I was leaving an exam room after finishing with patients, I noted Beth, a gynecologist upstairs from us, in our hallway telling us to evacuate the building. “There’s smoke in our office, and we don’t know why.” An electrical fire in the elevator shaft was the cause, and one of their patients was trapped in the elevator. When I found her in the parking lot, crying but physically fine, she told me she had picked up the red emergency phone and told the person on the line that she was trapped in a smoking elevator. Unimpressed, he responded: “Can you hold, please?” From now on, I’m taking the stairs.
When Greta, Ken, and Karl arrived, we had to meet standing under our overhang behind the office in the back parking lot. They had seen the geneticist, and I had spoken to Ann, the genetics counselor, twice over the phone. Ken’s features, including his cherub cheeks, one of which overlay a cyst in the wall of his mouth, raised the possibility of something called Gorlin syndrome, an inherited disorder that causes, among other things, rhabdomyomas. If Ken had Gorlin syndrome, so did his baby, but it would take three weeks to get the genetics testing done. Most people with Gorlin syndrome have normal intelligence. But this possibility did not alter their plans. Karl summarized their thinking: “If there is a chance that the baby will be retarded, we cannot put him nor us through the surgeries.” They did agree with Stuart’s position and consented to an echocardiogram after birth.
That was that. Greta would come in that evening so I could place Cervidil, a prostaglandin that promotes cervical “ripening” (softening and opening), in her vagina behind the cervix to improve the odds that when I gave her pitocin the following morning, she would go into labor. We would deliver the baby, do the echocardiogram, and let nature proceed if the test verified Stuart’s original conclusions. I drove ten miles to our Alameda office to see my Tuesday-afternoon patients and make the final call to all the specialists involved. Lani, the excellent physician assistant student whom I was teaching, took care of most of my patients, giving me the room I needed to spend 90 minutes on the phone. Watching and listening to me sucked my colleagues and consultants into my whirlpool of uncertainty. Shouldn’t the baby be put up for adoption? Should an ethics committee review this? But Stuart and Vishu had the most experience in similar matters, and they reached the same conclusion that I had: this was a highly unusual case, which made our predictions uncertain, and the family was acting with their best intentions. Therefore, we would do as they wished.
Distraught over the baby’s death sentence, Terry confessed to me that evening that she had considered calling the Department of Social Services to see if there was anything it could do to arrange an adoption or some other rescue plan for the baby. Showing her characteristic calm wisdom, she had not called because she knew it was not her place to get involved. For that, she got a thank you and a hug from her relieved but sympathetic husband. About an hour earlier, I had put the Cervidil in Greta’s vagina, starting the baby on the road to enter our world with a heart ill-suited to its purpose of allowing him to live here.
So much of how well situations such as this play out depends on random factors, including who happens to be working what shifts at the hospital. Maureen, an exceptional nurse who had herself lost a child, had Greta Wednesday morning. When her even, intense gaze met me at the nurse’s station, I got that secure feeling you get when your smooth-riding sedan with steel-frame doors eases onto the highway. It gave way to a choke and a wheeze when she spoke. “The night shift didn’t start the pitocin this morning because they were not comfortable with the plan. It isn’t clear enough from the chart what the plan is.”
Rereading to verify my sanity, I pointed to my two-page admission note from the previous evening, in which I explained the clinical issues, complete with detailed test results, and the plan for delivery. We would not do a vacuum delivery, cesarean section, nor any other procedure to protect the baby because we expected the baby to die; we would only do them if necessary to protect Greta’s health. After birth, I would hand the baby to the neonatologists, who would stabilize him, do the echocardiogram, and keep him comfortable. Maureen looked it over.
“Seems clear to me,” she admitted. “I’ll start the pitocin.” Nine hours and about 400 contractions later, Greta’s baby remained in her womb behind a cervix that was now three centimeters dilated and much thinner than when the day began. Rather than continue pushing on late into the night, we all decided to turn off the pitocin, let Greta rest overnight, and have the baby the next day.
Or the next night. I broke her water bag first thing in the morning to stimulate stronger labor and commit us to taking her labor to its completion. Once the bag of waters breaks, vaginal bacteria have open access to the uterine contents and will cause infection that can cause serious problems for the baby and mother, most often between 18 and 48 hours later, so we cannot delay delivery. It took until the early afternoon for active labor, with its regular strong contractions and consequent gradual cervical dilation, to set in. We recommended and she accepted an epidural anesthetic to take her pain away; she would get enough pain after her baby arrived. By 6:00 p.m., her cervix had completely dilated, and she was ready to push. Maureen had finished her second consecutive day shift with us, and I had finished seeing all of my patients in the office. Maureen hugged and kissed the expectant parents good-bye, and I called Terry to tell her where I would be for the next few hours.
Slim but large-boned and well-muscled, Greta pushed with power, but two grueling hours later, her drooping facial muscles betrayed her fatigue. Her boy’s head jammed into her pubic bone with each of her pushes because she had a shallow pelvis, meaning that she did not have much room to squeeze that head out between her pubic bone and rectal muscles. Many women in her situation cannot push hard enough to have a vaginal delivery, and the in-house obstetrician that night, who would be interested now that she had been pushing more than two hours, was Benjamin, who was not a patient or pleasant fellow. He was so abrasive during one vacuum delivery he did for a patient of mine that she said afterward, “I will never let him touch me again.”
A half-hour of position changes, exhortations, and pushing with each contraction every three minutes yielded little progress; the baby’s head was still high in the vagina, and therefore if she didn’t bring him down in the next 30 minutes, I knew that Benjamin would call for a C-section.
“Greta,” I told her, “you have 30 minutes left to push. That’s it. You can only have a vaginal delivery if you give it everything you have.”
She did. She pushed harder. The impacted head began moving down again. Twenty-five minutes later, about a quarter-size area of black baby hair showed through Greta’s parting vaginal lips with each push, but she had little energy left and would need help. Pleased that we would be doing a vacuum delivery instead of surgery, I nonetheless dreaded bringing Benjamin into this situation. I prepared Ken and Greta. “He’s brusque, but he’s an excellent technician, and he’s who we need right now.”
I need not have worried. Benjamin said little, and under the bright overhead lights, he applied the Betadyne-soaked plastic suction cup to the baby’s head and performed a gentle vacuum extraction to bring Eric slowly out to safety. We clamped and cut the cord, and Eric made a successful early transition to his new environment, relying on his patent ductus arteriosus to circulate his blood, which turned him pink. He spent a few short moments with his parents before Hank, the neonatologist, took him upstairs to see Schmuel, the cardiologist who was standing by with a technician to do the echocardiogram.
The break with the natural order of things became obvious when Greta started the mournful wails typical of a mother whose child has just died. Benjamin had the awkward task of stitching up the small episiotomy he had cut with admirable care as he was delivering Eric, and he set to his task with his customary emo-
rteriosus to circulate his d, which turned him . He spent a few short nents with his parents re Hank, the neona-»ist, took him upstairs e Schmuel, the cardi-st who was standing by a technician to do the cardiogram.
The break with the nat-jrder of things became )us when Greta started loumful wails typical mother whose child ist died. Benjamin had wkward task of stitch-p the small episiotomy id cut with admirable as he was delivering and he set to his task his customary emotionless demeanor, speaking only to ask if Greta was feeling any physical pain. She wasn’t, and I’m not sure she could have. Laurel, another terrific and kind obstetrical nurse, stayed at her side with Ken and me. After Benjamin finished, we all thanked him, and soon thereafter I went upstairs to get the verdict on Eric. Ken waited outside the nursery in the hallway.
“He has two masses,” Schmuel advised me upon my arrival. “Take a look.” I recalled Ken’s account of staring at the ultrasound images for an hour and a half in Schmuel’s office the previous week as I appreciated the white, billowing, parachute-like shape that almost obscured what should have been a black blood-filled space in the left ventricle, with its smaller white partner adjacent to it. I would later read the official echocardiogram interpretations, which, in every instance, referred to the larger tumor as “the giant mass,” and this seemed entirely appropriate. Its three-centimeter size appeared enormous in that tiny heart.
Hank’s face sported a glum smile. “He’s a cute baby, wouldn’t you know it?” Eric was cute in a way most newborns are not. He had lovely, smooth dark brown skin and a round face with his father’s chubby cheeks and his mother’s wide eyes. Hank and I confirmed that we would give him only morphine when he began to turn blue and have trouble breathing. The nurses snapped some Polaroids and digital photos for the parents and then allowed me to take Eric back downstairs in his bassinet on wheels to his mother, with his father at his side.
Back in the birthing room. Jack, the hospital chaplain, met us. Seated on the left side of Greta’s bed and touching Eric’s forehead as the baby rested in his mother’s cradled arms, he began his spontaneous attempt to say something appropriate and meaningful. “I’ve been brought here to say a blessing,” he began with his silver hair and a pleasant, humble expression directed toward the doomed infant “But, really, you are the blessing.” Laurel and I became spectators, and Jack delivered a message as short and sweet as little Eric. Not wanting to bond too much with the irresistible new force in the room, Ken and Greta took turns holding him, but after about 25 minutes, they sent him to the nursery. They did not want to watch Eric suffer. Knowing that the terminal event, the ductus closure, could occur in two hours or two days, we again could not tell Greta and Ken how to handle this, so we did not.
Exhausted, I turned my attention to Greta, who I remembered had much more right to be tired than I did. I pointed out to her what a remarkable effort she had just put forth to get a vaginal delivery, and she thanked me. I had nothing to do now but go home, so I said good night. Laurel intercepted me on the way out and gave me the warmest professional-to-professional hug I’ve ever had.
When I arrived at the neonatal nursery the next morning, Eric was in his mother’s arms busy drinking formula. Greta couldn’t bring herself to breastfeed, but with every moment his life continued, Eric was drawing his parents in. “For as long as he is here, we are his parents,” Ken reaffirmed. He held and admired his son just as any proud father would. When I signed out that night to Don, the physician covering me for the weekend, Eric remained pink and happy.
All weekend I waited for the page from Don that would tell me that Eric’s short stay had ended. When that didn’t happen, Schmuel came in Monday to repeat the echocardiogram, and his news changed everything: the ductus was closed. We don’t know when that happened and it doesn’t matter. Eric’s left ventricle had done what none of us experts could: figure out a way to get its job accomplished with the masses still in there. Greta started to breastfeed Eric, and everyone went home two days later.
Two days after that, the three of them rushed in to my office. “His feet and hands turned blue,” Greta explained. And indeed they did have a bit of violet hue to them, but after a careful examination, it was clear that this was nothing unusual. Babies’ feet and hands often turn a little blue when they get cold. A few weeks later, another echocardiogram showed that the masses were shrinking. The three of them haven’t gone back to the geneticist yet. “We want some time for ourselves first,” Ken told me, and they’re right, of course. After a most unusual beginning, they are enjoying their first child. At the time of this writing, Eric is eight weeks old and thriving.