Siamese

I saw in the news a couple weeks ago that Siamese twins were surgically separated somewhere in the U.S.A., and that reminded me of my own experience with Siamese twins. My first brush with them was actually my roommate's experience in medical school. He was at a supposedly fairly routine cesarean section for twins when the attending obstetrician said matter-of-factly, "Hey, they're Siamese," which seemed like an anticlimactic way to describe an extremely unusual occurence.

I have personally seen Siamese twins only once. The babies were born at 26 to 27 weeks gestation - about 13 weeks early - and we knew from prenatal ultrasounds that many of their internal organs, such as their liver, were shared, although their hearts were separate. I was the attending neonatologist at their delivery and subsequent admission to the NICU. When they were born it turned out they were fused from their chins down through their chest. Their heads were thrown back by the fusion, their necks permanently extended. They were born alive, with beating hearts, but without much breathing effort or other signs of life. What to do? Their heads and neck were such that it would have been difficult to intubate them and put them on ventilators if we wanted to. We didn't know if their lungs were fused or anomalous in some other way, besides being premature. It was a number of years ago, and the survival rate for babies at that gestation, which is now about 80% to 90%, was less then, and much less for very premature babies with major birth defects. The parents were aware that they might be so severely affected they might not survive.

Should I give them a try? Should I attempt to put both on ventilators and call our pediatric surgeons to try to sort out the awkward mess of fused organs? Should I start treatment that, if they survived, would lead to months of hospitalizations, several surgeries with painful post-op periods, and a next to nothing chance of anything near a normal existence?

No. I let them die a quiet, peaceful death.

Trio

"Rita" was nineteen. He was about fifty, and the father of her baby born at 26 weeks gestation. She was petulant and immature, even for her age, and gave our nurses a hard time. Her baby had the problems typical of a baby born that prematurely and was in our NICU for about three months.

They were both Americans who were converts to Islam. Although not legally married in the eyes of the state, they were considered married in the eyes of their religious community. Rita was the man's second wife. The problem was that his first wife was still alive - and still married to him and living with him and Rita. Their religious community accepted the polygamy. Sometimes father, first wife, and second wife would visit baby together, a weird situation as you might imagine. Rita seemed very uncomfortable at such times and looked to be the lowest of the trio on the totem pole. Father - generally considered a scumbag by the NICU staff - sat with a grin like a Cheshire cat, looking very proud of himself.

I cannot imagine why a person, even a young insecure person, would let herself be exploited in this way. What sort of upbringing must she have had to allow herself to live in such a humiliating way? What sort of sad living situation must she have left to make this one seem acceptable? What sort of insults must she have sustained from her parents to end up with such low self esteem?

I will probably never know. I only hope her life gets better.

P.S. A new Grand Rounds is up at NHS Blog Doctor.

BUFA

"Kimberly" was supposed to have a BUFA baby. BUFA stands for Baby Up For Adoption and is a well known acronym in our hospital. This mother had planned to give the baby up for adoption for some time. She said she did not want to see the baby after birth, did not want the baby with her in her room, and did not want her family to see or be given information about the family. We are willing to comply with those wishes. I was in attendance at mother's cesarean section, and the baby did fine. Before leaving the C-section room I told mom that her baby was doing well and was normal - I think every birth mother deserves to know that, so they don't wonder or imagine things - and asked her if she wanted to see the baby. She did, so before we took the baby to our nursery mother saw the baby and touched her face.

The nurse who was with me and I commented to each other that we wouldn't be surprised if mother decided to keep the baby; mothers frequently change their mind in these situations.

I don't mind admitting that I feel a lot of ambivalence about adoption. I know it can be great for families unable to conceive children and I know that many kids through adoption are given families and lives that wouldn't be possible otherwise. But seeing a mother say good-bye to her baby for the last time, seeing the baby taken from mother, and seeing the expression on mother's face, makes you realize that has got to be one of the most heart wrenching experiences on earth. I suspect, too, that the birth mother thinks about the child she gave up every single day the rest of her life. Whenever we have a mother who is considering adoption and wavering about it, I find myself rooting for the birth mother, hoping that she'll keep the baby, hoping another person won't have to go through that awful separation experience.

Kimberly didn't change her mind, and in this case I was privileged to see what I usually don't observe, the happy part of adoption, where adoptive mom meets her new baby. One of the pharmacy workers in our hospital was looking for a baby to adopt, and she was given this baby. When she came to take her home she was thrilled. I've seen her several days since then and it is great to see the persistent smile on her face. I can't help but wonder, though, if the height of her joy might be matched by the depth of the birth mother's sorrow.

Youth

"Are you sure my baby can't go home today?"

I explained to the mother again why her baby couldn't go home, because he still had an IV in and wasn't eating enough to take the IV out, and assured her we would send her home as soon as possible. She was very nice. She was also only 16 years old, but was trying to do her best in the strange environment of the NICU. I asked her if she was going to school, hoping that she was enrolled in the special high school in our area for pregnant teens. She said she was home schooling for now but was planning on going back to school.

I cannot imagine having a baby when I was 16. It seems I barely knew where babies came from at that age. Sixteen year olds should be out at the mall with their friends, or playing a high school sport, or doing homework. They shouldn't have to be caring for a baby all the time. This mom is missing her teenage years.

Today we admitted another baby for antibiotics. That baby's mother was only 20 years old, but she looked like she was about 30. This was her third baby. She had a five year old and a two year old at home. Can you imagine having the responsibility of three children at age 20? It's almost mind boggling.

I feel sorry for these young moms. I know they love their kids and are appropriately concerned about them, but I think even they would admit their lives would be better if they could have delayed child rearing for a while. Even if we ignore the economic consequences - teenage mothers have a tough time getting a good education and job - the social consequences alone, the absence of a relatively carefree adolescence, is enough to make teenage motherhood an unfortunate thing.

P.S. So long to Geek Nurse, whose blog - for unclear reasons - was recently shut down. I think I'll keep blogging anonymously.

Management

I think there is problem with the way nursing management is structured in the U.S. The problem is that nurse managers do not actually work in the units they manage, so they often don’t experience first hand the problems they need to help solve. For example, in our hospital, and in almost all U.S. hospitals, the chief of neonatology takes his turn working in the NICU just like the other neonatologists, rounding and taking night calls. The same is true for the chiefs of other clinical departments. But the nurse manager of our NICU never does a shift alongside the NICU nurses. She never does the same work the other nurses do. Instead, she spends most of her time in the office dealing with scheduling, policy, and other matters.

As a result, I and the other neonatologists know more about her nursing staff than she does. We know which nurses are good and which are not. We have to inform her when they are doing a good job or making mistakes. I’m not sure why nursing management has evolved this way, while physician management has kept the chief doing the same work as the others, but it should change. I think every nurse manager should do at least one shift every week or two simply doing the regular work of a unit.

Don’t get me wrong. Our nurse manager is an excellent person, but the structure of her job is flawed. Also, this is not a diatribe against nursing. Most of our nurses are good, and working with a good nurse is very gratifying, knowing you can trust them to do what is best for the patient, and knowing they’ll probably catch something if you miss it. But this is one instance where I think the medical model is better.

Care

I don't get it.

A mother delivered a 34 week gestation baby this morning. The baby is doing fine. Mother's history is remarkable for chronic high blood pressure and 4 strokes, although it has been hard to find evidence of those strokes on imaging. Also, in a tragic note, the mother lost two children eight years ago in a house fire (an all too common occurence in the poor urban area in which I work.)

Wouldn't you think that a pregnant woman with chronic hypertension would seek prenatal care? Wouldn't you think that a pregnant woman with 4 strokes would get prenatal care? Wouldn't you think that maybe a mother who had two children die in a house fire would consider each child precious and get prenatal care? Wouldn't you think that such a mother might be able to forego her marijuana smoking during pregnancy?

This mother did none of those things. She said she had no health insurance, but in our state any woman who is poor can get Medicaid, and since she was hospitalized for 6 days at about 27 weeks gestation - the extent of her prenatal care - I know a social worker discussed getting Medicaid with her. This mother simply did not follow through, did not do the responsible thing for herself and her baby.

I've always considered myself sort of a bleeding heart liberal, one who tries to see the problems and difficult life of a woman like this and feel pity for her, and try not to blame her for her lack of prenatal care. But I get a little tired of it when I see case after case similar to this. Maybe it isn't as easy for her to get prenatal care as it is for a suburban woman with good health insurance. Maybe it would require her to take a trip to the Medicaid office, or to fill out some forms and mail them in. But that's not a valid excuse; doing the right thing in life takes a little effort sometimes, and this woman simply didn't put out the effort. I'm sick of it.

The Institute for Healthcare Improvement recently surveyed patients and families about what they want and expect from healthcare providers. The first item they wanted was to be listened to, taken seriously, and respected as a care partner. I would love to do all those things for my patients and families. But first they have to respect themselves.

Attention

She was 19 years old, sitting at the bedside of her 10 hour old baby born at 24 weeks gestation. It was my first time meeting her, so I was trying to give her "the talk", where I go over the baby's problems and try to tell her what to expect. We had discussed the survival rates; I had told her about the immature lungs, their honeymoon period, and how they would likely worsen towards the end of the first week. I was now into the sometimes trickiest part of the talk, where I tell them about the chances of bleeding into the brain when -"What's that thing on her arm," she interrupted, pointing to a mild bruise. She didn't seem to be hearing a thing I said.

Later that morning I talked to an expectant mother of about 34 weeks gestation. I say "about" because there had been no prenatal care and mom wasn't sure of her dates. My partner had already talked to her a couple of hours previously, but her nurse asked me to see her again, saying she hadn't comprehended what my partner had said. I went to see her, but she kept falling asleep while I talked. After a few minutes I left, thinking she probably didn't comprehend much of what I said either.

Doctors are sometimes criticized for not talking to their patients enough. I know that criticism is often justified - I've experienced it myself as a patient - but I also know how hard it can be to talk to some patients, and how frustrating it can be when they don't pay attention or don't seem to care what you say. It seems to me that when you're discussing possible bleeding into your baby's brain and possible brain damage, you would most likely be listening closely. But the mother of the 24 weeker wasn't even close to paying attention. Maybe I'm more boring than I realize, but still - it's a pretty important topic. Also, I don't really blame the mother of the 34 weeker for falling asleep - it was morning , and she probably hadn't slept much that night - but that, too, was still a little frustrating. Am I being egotistical for expecting them to listen? I don't need to see rapt enthrallment on their face, but is polite attention too much to ask?

These situations actually occur pretty commonly, and I bet that other doctors would say the same. I'll keep trying to talk to patients and families, but do me a favor: if you're a patient, at least look like you care.

Happy

A regular reader of this blog whom I know personally asked me "Doesn't anything good happen in the NICU?"

"Sure'" I said, "babies get better and go home."

When we think of neonatal units, we tend to think of "micropremies", the extremely small babies who have high rates of complications, or of babies with birth defects. People have commented to me that neonatology must be an interesting but sad specialty, and when I review the subject matter of many of my postings - holoprosencephaly, drug addicted moms, extremely premature babies - I can see why they might think that. The truth, though, is that most of our patients do very well, and that neonatology is actually mostly a happy specialty. The kids are cute and the parents are pleased and (usually) grateful. For every 23 or 24 weeker with residual deficits, there are two or three 31 or 32 weekers who need just a little help and a few weeks of hospitalization and then go home healthy as can be. For every baby with severe birth defects, there are dozens of babies who have transient breathing problems and go home fine after 3 or 4 days.

And many of the babies who do well now would not have done well in the not so distant past. President Kennedy had a premature baby at 34 weeks gestation who died at the age of two days 43 years ago. Today, babies of that size and gestation, even with respiratory distress syndrome, have a survival rate approaching 100%.

The problem - and this is not really a problem - is that these babies who do well usually don't make for interesting stories. They are born, get better, and go home. So those of us who write about neonatology, either on blogs or elsewhere, tend to write about the more complex or ethically vague cases - cases that often have a sad ending.

This post, I guess, is my way of justifying all the stories here about less than happy things. So when you read them, don't get discouraged; just think about all the pleasantly boring babies whose parents are beaming but who didn't make the news.

Honesty

I try to be honest in my dealings with patients. It's good ethically both medically and generally. When I discuss a patient's condition, I neither sugar coat it nor try to scare the family but am just straight forward.

There have been times when a family has asked me to be dishonest, like when they ask me to write a work excuse for the father and ask me to make it for longer or a different time than it should be. I decline those requests. I figure if I lie for them then they won't trust me when I'm giving them information about their baby.

Sometimes, though, I wonder if I have to tell them all the truth. I think specifically of obtaining "informed" consent. The most common procedure for me to obtain consent for is a spinal tap, also known as a lumber puncture. The purpose of this is to find out if the baby has meningitis. If you miss meningitis in a baby, it can ruin the baby's whole life. In general it is a very safe and useful procedure, and we don't do it unless its benefits outweigh the risks. In one instance I am aware of, though, in a case of a pediatrician acquaintance of mine, a baby was paralyzed by the spinal tap. It was a two month old baby who had hemophilia, although the hemophilia had not yet been diagnosed. The baby had increased bleeding from the spinal tap because of the hemophilia, and the blood compressed and damaged the spinal cord, leaving the baby a paraplegic.

That is a tragic case. It is also extremely rare. My question is, do I have to tell parents about this when I tell them the risks of a spinal tap? Do I have to mention paralysis as a risk? If I were being totally honest, I guess I would, but there is a very real concern that some parents, already skittish about a spinal tap, might decline it if I tell them that, and that would not be good, because it is extremely important we don't miss menigitis. Like I said, I wouldn't do the spinal tap unless the benefits outweighed the risks. A further question might be, should we even have to obtain informed consent for spinal taps? Why do we? I know that patients' rights advocates won't like this, but think about it. As a doctor, I am trained to evaluate the risks versus benefits; the parents are not. Why do they get to choose an option that might not be in the baby's best interests?

I'll stop here. I've probably already said enough that I'll receive some flak. In the meantime, though, I'll try to be honest with parents. I hope I'm honest enough.

Modesty

I was heading to a delivery and before I even entered the room I could see the mother from the hallway, her legs up in stirrups and her business end on display for anyone passing to see. This is not uncommon; we take sort of a cavalier attitude towards most mothers' privacy in our hospital. Our delivery rooms are divided in half by a curtain, with the newborn's warmer in the half closest to the door, then a curtain that can be drawn, then the mother's bed. The OB staff try to keep the curtain drawn, but with people coming and going it's often not doing its job of protecting mother from being seen in this awkward pose. There is a door to the room also, but it's even more rare for that to be closed.

I'm not sure why we're this lax about privacy. The mothers usually don't seem to mind. Maybe it's because they have much more important things on their mind, such as contractions and pushing and trying to get a creature the size of a small bowling ball out of their uterus. Let me assure people that it is in no way a planned effort by the men so they can voyeuristically view womens' private parts. Trust me, there is nothing sexy about the bottom of a woman in labor. When I used to work at a suburban hospital, we seemed a little more conscientious about keeping mother from public view. Maybe modesty is just not that big a deal in the inner city.

The exceptions, not surprisingly, are some of our Moslem mothers from Central and Western Asia. I went to a delivery of one of them recently only to be greeted by an older woman related to the delivering mother, perhaps her mother or aunt, waving her hands at me and saying "No man, no man!" I managed to hide behind the curtain until the baby was delivered and we were sure he was fine. Sometimes not even the father is present at their deliveries.

I suppose we should pay more attention to this and do better, but until the mothers start complaining themselves, that's not likely to happen.

Dollars

I hadn't intended to write about 23 weekers again so soon after my previous posting, but the comments to that post raised a couple of issues I can't resist addressing. The first issue is about the "cost effectiveness" of care for extremely premature babies. Cost effectiveness studies are inherently a little distasteful, trying to put a dollar value on a human life. Roughly speaking, they are usually done by looking at the cost of certain interventions - such as NICU care or angioplasty - and comparing the ratios of the cost of the treatment to the amount of money generated by the survivors of that treatment in the productive years of life they live following the treatment. The good news, at least for us neonatologists, and a surprise to most people is that in such studies NICU care is usually more cost effective than many other common treatments, such as coronary artery bypass graft surgery and dialysis for kidney failure. Although sometimes you might hear someone complain about the high cost of NICU's, when's the last time you heard anyone say we shouldn't do heart bypasses because they're not cost effective? You won't hear anyone say it, because people the age of bypass patients have a pretty good political voice - unlike premature neonates.

It's only fair that I insert a disclaimer here. The cost effectiveness studies I've seen have not usually gone down to 23 weekers but have included slightly more mature babies. If anyone knows of cost effectiveness studies encompassing 23 weekers, I'd love to see them.

The second issue raised in the comments was the idea that, instead of spending money on NICU costs, we should spend more money on prenatal care so we can prevent premature births and NICU costs. This is a very appealing idea and a darling of the public health community. Unfortunately, though, it's probably not true. The data supporting the notion that prenatal care saves subsequent postnatal dollars is pretty scant. It makes sense, too, that it is not true. We don't know how to prevent preterm birth. If we did, the rate of preterm birth in the U.S. would be falling, but in fact it is rising. More than 10% of births are preterm now. Don't get me wrong: I think it's good to provide prenatal care, but let's not subscribe to the too-good-to-be-true idea that every dollar spent on prenatal care will save five dollars in NICU costs. (For reasons not clear, that concept always reminds me of Wimpy in the Popeye cartoon: "I'd gladly give you a nickel on Tuesday for a hamburger today.")

If you want to think about whether NICU dollars are justified, here's some food for thought. What if we took money spent on NICU care - about $2,ooo per day per baby - and spent it on food, clean water, immunizations, and medicine for kids in the developing world? There, the money probably could save more lives. But that's another big subject in itself, so I'll stop here.

P.S. If we're going to send NICU dollars to developing countries, we'd better send dialysis and coronary bypass dollars there too.

Twenty-three

I help keep track of the statistics for our NICU and in reviewing our 2005 data noticed that we had an unusually large number of babies born at 23 weeks gestation that year. We also had an unusually good survival rate for them. (I don't say that to brag - these things have a way of balancing out over the years.)

Mostly that (the good survival rate) is good news. I say "mostly" because babies born at 23 weeks gestation cause ambivalent feelings in those of us who care for premies. Twenty-three weeks is pretty much the lowest gestation at which a baby has a real chance of surviving. Nationwide, the survival rate for them is about 30%. Approximately 50% to 60% of the survivors will have moderate to severe impairments, and some of the remainder will have a mild impairment. Putting those numbers together means that a baby born at 23 weeks has about a 15% chance of surviving with no impairment or a mild impairment, and thus the ambivalence. Should we be resuscitating these babies? Should we do everything we possibly can to make them survive?

We spend lots of time and energy discussing this question. If we have time, we try to discuss this with the parents prior to delivery, but even with the best discussion, it's hard to make the parents really comprehend all the issues involved in the short time we tend to have before delivery. In our hospital almost all of the parents opt for full resuscitation and support.

Personally, I'm pretty comfortable with full resuscitation of these babies, although I know others in the neonatal field disagree. I think it's fair to resuscitate them because at almost any other time in life, except at the very beginning or end of life, we would usually opt to give full support to someone with a 30% chance of survival. I am also influenced by a study done several years ago in Canada. In that study, the investigators compared the feelings of self worth of impaired adolescent survivors of premature birth with those of a control group of normal adolescents who had been born at full term. Interestingly enough, the impaired survivors had higher feelings of self worth than the control group. Granted, there were some survivors so impaired they could not be tested, but still the study sure has to make us wonder: who are we to judge the worth of a child with handicaps?

This post only scratches the surface of this huge neonatal issue; I suspect we'll return to it in some future posts.

P.S. The latest edition of Grand Rounds is up at Emergiblog.

Fertility

She fit the profile. She was in her late thirties, it was her ninth pregnancy, she hadn't had much prenatal care, and sure enough, her urine drug screen showed she had cocaine in her system. Her newborn baby had it too.

Most of our mothers who use cocaine tend to be older, at least older as far as mothers of newborns go. It's not surprising, too, that they often don't get much prenatal care, given their chaotic lives. But the part that puzzled me was why they so often had so many pregnancies. I know that some of these women will earn money for drugs by prostitution, either overt prostitution or by attaching themselves to a man who provides for them even though they don't really care for him. You would think, though, that it would be to their advantage to use birth control or even get their tubes tied, that they would do better in prostitution in a non-pregnant state. Then one day an obstetrician who has worked for years in our urban area explained to me that a woman's value on the street was higher if she was fertile, was lessened if she could not get pregnant.

I don't know if that's true or not. It has the ring of urban myth. Maybe it's just their chaotic lifestyles that also explains their frequent pregnancies. But truth can be stranger than fiction, so it could very well be true or at least partly be based in fact.

I've written before in a post that some people get angry at mothers who use drugs during pregnancy, but that doesn't do any good, except for letting the miffed health care worker blow off some steam. Instead, we should recognize that the birth of a baby can be a time when a mother might have incentive to change her life, to make things better for her baby, and we should capitalize on that by trying to get her into rehab. It seems like a small step compared to the problem of women who remain fertile to increase their value, but hey, it's at least something.

Cuts

In a comment to my last posting entitled “Jane”, paedsrn – author of the blog geeknurse – expressed his surprise and dismay at the fact that they cut off part of her ear. I agree that it is cringe inducing, and we were not at all happy that it happened. But it is not the only time that I have seen parts of a body cut from a baby. I once saw a piece of cheek cut from a neonate, and when I was a resident a nurse cut off a tip of a finger of a toddler. In each case a nurse was cutting some tape. For the cheek and ear removal, they were cutting tape that held an endotracheal tube in place, and for the finger they trying to remove tape and gauze that held an IV in place.

Are these mistakes excusable? No, not really. We who work in the NICU always have to be mindful that little things can make big differences in little babies. If you’re cutting tape that is on a baby you have to always know where the tip – and every other part - of your scissors are. Better yet, cut the tape the right length before putting it on the baby’s face, instead of afterwards. Unfortunately, though, sometimes one has to make a mistake like this to learn how picky you have to be around neonates. Fortunately the majority of us learn it without having to cut off some flesh.

What to do with the person who cut off the body piece? In my experience the perpetrator is so horrified that no disciplinary action can punish them further than they are punishing themselves. When I was a fellow in neonatology, I cut my forearm in a cooking accident badly enough that it needed stitches. I went to the emergency room of the same hospital where the tip of the toddler’s finger had been cut off, and who should be my nurse but the very one who had cut off the toddler’s fingertip. Frankly, I didn’t even remember her - it had been a couple of years since the incident. But she clearly remembered it (of course) and told me her gratitude that I, the toddler’s physician, had recognized her mortification and hadn’t raked her over the coals for it. It was weird: an incident I barely remembered had had a huge impact on her. I still kept a pretty close eye on her, though, when she pulled out her scissors.

What to do with people who twice cut off a piece of a body? They have to go. Fortunately, I’ve never seen that happen.

Jane

I recently got some follow-up on a graduate of our NICU - we'll call her Jane - a former 23 weeks gestation patient. I remember this specific patient because one of our nurses, while cutting tape on the baby's face, had accidentally cut off part of her ear, and one of my partners sewed it back on. She is now three years old and a pediatrician friend of mine told me she saw Jane for a check-up.

Respiratorily, Jane has recovered nicely. She had gone home on oxygen but now was having no trouble breathing. Developmentally, she was behiind - saying only a few words at age three - but not so far behind that there was no hope for improvement. Socially, things were not looking so good. The pediatrician heard, from outside the room, the mother yelling at her. It had been seven months since her last pediatric visit, instead of the recommended one month, and her teeth were rotted to the nub by milk bottle cavities. She had been referred to the dental clinic but mom hadn't taken her, so she had some dental abscesses and needed some teeth extracted. Her mother didn't know what medications she was on.

There's an article in the February issue of Pediatrics that confirms what we have seen before: as a group, very premature babies are at risk for lower IQ's and developmental delay. It also showed another discouraging thing. Babies and children who grow up in a home environment where there is lots of stimulation, where they are read to and spoken to often, played with and paid attention to, tend to develop better than children whose home environment lacks those qualities. This difference seems to be exaggerated in premature babies. Premies brought up in "good" homes can overcome, at least partly, the inherent developmental disadvantage of extremely premature birth, but that disadvantage is amplified for premies raised in "bad" homes.

What's going to happen to Jane? I don't know. She's probably not neglected enough that Protective Services would do much. We cannot completely re-engineer society to place every baby in homes to our liking. I guess we can only try to make their lives a little better in the short time we have with them.