Birth Plans: More General Thoughts

In my last post, I began a discussion of “what constitutes a good birth plan” in response to comments following a previous post. I addressed general issues and, after giving some more thought to the matter as I was falling asleep last night, I would like to continue the discussion of these before moving on to more specific concerns. It is important for the reader to understand that there are limitations and constraints on the part of both hospitals and providers that must be considered when a birth plan is developed.

All of us wish that pregnancy, labor, and delivery were always ‘safe' and uncomplicated processes, but they are not. From a health standpoint, they are among the riskiest events in a woman’s life. At the beginning of the 20th century, 1 out of every 100 women and babies died during pregnancy, delivery, and the immediate postpartum period. This is fact and not fiction. One cannot deny that pregnancy and delivery are ‘natural processes’ but, as I have stated in the past, they also were among the most powerful forces of ‘natural selection’ working to mold the human race. In many ways we have circumvented that process by aggressive management of medical conditions in pregnancy, the use of antibiotics and blood products, the use of drugs that can help the uterus contract (and therefore stop bleeding) following delivery, and by the use of cesarean delivery. Women, who never could have in the past, achieve, carry, and survive pregnancies successfully today when they never would have before. With that ‘success’ we are now routinely caring for pregnant women who would not even have crossed the threshold of our offices half a century ago. (It is interesting, but not appropriate to today’s discussion, to speculate on the long-term consequences of having ‘messed with nature’ in the way we have).

Expectations of patients have changed as well. In the past, it was readily accepted that some mothers and babies would not survive the process intact, or even alive. Poor outcomes were considered to be an “act of God” and now everyone expects a ‘perfect baby’ every time. Poor outcomes also are no longer attributed to divine intervention but to perceived (and sometimes actual) deficiencies in care, even when patients have not accepted responsibility themselves for factors that might optimize outcome (e.g., weight loss before pregnancy, cessation of smoking and other substance abuse, balanced dietary intake, compliance with care for medical complications during pregnancy, early reporting of ‘risk factors’ for preterm labor, etc…). And, even when everything is done ‘according to the book,’ sometimes bad outcomes occur anyway (sometimes very quickly) and cannot be anticipated before they do (e.g., congenital infections, cord accidents, placental abruptions, cervical incompetence leading to preterm birth, events that that lead to cerebral palsy prior to labor and delivery, etc…). As the result, providers and hospitals have had to take a more defensive posture (or close their doors to obstetrical patients) due to the high liability associated with the care of pregnant women rather than taking the approach that pregnancy and delivery are ‘natural processes’ in which the outcome will be accepted for what it is. This has led to the hospital routines, such as IV access and restricting oral intake, and the screening technologies, such as fetal heart rate monitoring, that are, admittedly, associated with high ‘false positive rates’ and low ‘positive predictive values,’ in the hope of improving outcomes in certain ‘preventable’ circumstances. The consequences of this are higher rates of intervention that are often perceived to be disruptive and unnecessary and, in many instances, are both of those things. Unfortunately, it’s the best we have to offer from the side of technology at the current time.

With all that said and done, you as the patient have several choices to consider. You can choose to work with the system and try to create the birth experience you want, respecting some of the constraints under which your provider and birth facility feel obligated to function; you can select a provider who, with a mutual understanding, is willing to go out on a limb with you within the ‘standard’ environment of a hospital or certified birth center; or, you can choose to take the risk entirely upon your own shoulders and contract with a certified or unlicensed provider outside of a certified facility. If honest assessment and careful triage are used, and attention is given to ‘standards of care,’ the risk to you and the baby can be minimized in each of these scenarios, but it never can be eliminated, even under the most controlled circumstances. It is also important to understand that not all options for management in both routine and emergency situations may be available to you, depending on your choice of provider and birth scenario. Personally, I would go with one of the first two options, depending on the status of my pregnancy, and if you would like to hear what I would do to optimize my birth experience as a young pregnant woman, then check in with my next post. I promise not to indulge in soliloquy again…!

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Developing a "Birth Plan"

A comment after my last post raised the question regarding “what constitutes a good birth plan” for a “first-time mother-to be.” I would love to answer this with a simple response that “a good birth plan” is whatever is right for YOU! And, even though that is true, anyone that would take the time to read through one of my longer posts deserves more of an answer than that alone.

I have mixed emotions about “birth plans.” Couples often develop birth plans based on what others tell them is best (that may or may not be based in reality) for themselves and their baby. The reason I know this is that most “birth plans” with which I am presented contain the same concerns, the same language (verbatim), and the same order of presentation. The script has been read and the blanks have been filled in. If you do not believe me, then go to any search engine, query “birth plans,” and see what comes up. If I am wrong, I will take it back! Indeed, there now are a number of websites that women can go to that will produce a written document based on a standard set of questions, concerns, strategies, and disclaimers. Unfortunately, this ‘quick fix’ and ease of preparation cannot replace frank inquiry and due diligence on the part of the individual. In fact, the ‘standard’ that results by such a process may produce a set of unrealistic expectations that can put a damper on the true importance of the whole ‘birth experience’ if it cannot be lived up to.

So, what do I suggest? The first step in the process is to identify your goals, the ones that are right for you, for the pregnancy, labor, and delivery. This step seems to be often overlooked when folks jump to specifics of ‘the plan’ before doing their homework (and there is nothing more certain to turn a provider off). I am suggesting that you think in general terms at this point, not in the details. Go ahead, start with a list. Sit down with your partner and free associate and focus on the positives: “I want a healthy baby.” I want this to be the happiest moment of my life.” “I want to feel I did all that I could to give my baby the best possible start in life.” “I want my partner and me to share the moment and be brought closer by the experience.” “I want to challenge myself and find out what my limits are.” “I want to feel safe and secure even if I am frightened.” “I want to be alert, aware, and focused so that I can enjoy and remember the experience.” “I want to participate in nonemergent decisions regarding my care.” “I want to be kept informed, be told what is going on and what my options are.” “I want to feel free to ask questions without being dismissed.” “I want to understand when it is appropriate to exert some control, and when I should relinquish control.” “I want to feel confident that my provider and her/his staff have my interests at heart and will do everything in their power to make my experience safe and worth remembering in a favorable light.” “I want to come out of this feeling proud about what I have accomplished.” I could go on, but that is the general idea. By the way, consider substituting “we” and “our” for “I” and “my” and this approach takes on a whole new dimension.

Then, I think the birth plan should be developed in conjunction with your provider. Present the “wants” developed in your list and ask how they might best be accomplished. Most providers do not want to be told what to do, but if you remain positive, nonconfrontational or demanding, and focused on the general goals, you are more likely to develop a true bond and get more buy in to what you would like to accomplish. Maintaining open lines of communication and flexibility are the keys here. Becoming educated is essential to success. Attend birth classes. Explore the internet with an open and critical mind. In discussions with your provider and their staff, nurses at the birth facility, other professionals such as certified midwives and doulas, and friends who have had babies before, find out what options are possible, what options are actually available and practical at the birth facility, and what can be done within the constraints of your provider’s practice, experience, and comfort level. ASK QUESTIONS AND REQUEST EXPLANATIONS ABOUT THINGS YOU DO NOT UNDERSTAND.

In the end, develop your birth plan. Be realistic. If something doesn’t feel right, it may well NOT be right for you. Realize that a ‘plan’ is not something that happens all at once, it evolves over time, and it may have to be modified to suit the current status of the pregnancy and your own changing expectations. Prioritize your desires and concerns. KEEP IT SHORT (bulleted points are more likely to be acknowledged). Be clear and precise. Different folks come to the moment with different backgrounds, innate resources, and coping mechanisms. Different individuals may also have very different ideas about what a word or phrase means and your provider cannot read your mind. I have had some patients tell me that their birth plan was that they wanted a “natural childbirth.” To one woman that might simply mean being conscious at the time of delivery; to another it might mean withholding pain medication “even when I beg for it, my head turns completely around on my neck, and I vomit green fluid and profane expletives on everyone in the room.”

Once you have drafted a plan, review it with your provider and ask who else might need to review it (e.g., other providers in the group, L&D staff, hospital administration, The National Enquirer). Remember, the “plan” is not the be all and end all. And also remember, if the birth plan cannot be adhered to entirely or, even at all, in the end because of unanticipated complications for you or your baby, you are NOT a failure. The work you have put into the process reflects the extraordinary motivation and dedication to a new life that only begins with the delivery itself. In my next post, I would tell you what I would consider putting into a birth plan of my own…

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Poop is Part of Parturition

I was reading a post by another blogger today that addressed the delicate issue of feces in the delivery room. I sort of chuckled at the report of the writer’s (a neonatologist’s) repulsion with the situation (guess most babies in the NICU don’t poop very much, or maybe it just doesn’t smell so bad, and when they do, they are probably ready for discharge from the NICU anyway), but fully realize, and have commented to the effect on many occasions, that the only reason I have survived all of these years in OB is because my sense of smell is very blunted. That was one of the advantages of having been raised in New Jersey. More intriguing to me about the post was the collection of comments that followed bemoaning the generally abysmal state of the typical delivery experience in the U.S. today. I happen to agree (with some of them) and started to post a comment myself, but thought I had more to say than would be appropriate in that context, so I have linked interested readers back to here to get a whiff of my thoughts (and nothing else, I promise).

I have practiced OB for more than 27 years. I too am dismayed by the atmosphere (and I am not talking about the smell) present in many delivery rooms around the U.S. today. A little bit of poop is not the problem, and has, indeed, been well-documented to be a 'natural' bodily function (and, besides, we could easily fix that by going back to the days of the enema, which probably isn’t such a bad idea for lots of reasons!). There often are too many people in the delivery room, family, friends, and nursing staff shouting exhortations at the top of their lungs (as if that will compensate for the fact that the patient cannot feel anything to push and hoping sound waves might accelerate or at least provide an immersion crash course regarding the process), the room is brightly lit (how can you chart in the dark?), the patient is laying on her back because she demanded an epidural at 2 cm and asks for a ‘top off’ whenever she senses any return of discomfort and is, therefore, ‘paralyzed’ from the diaphragm down, the incessant counting to 10 goes on to the point of distraction (even when the patient isn’t having a contraction, or isn’t making any effort to push because “I’m too tired, haven’t had anything to eat, and American Idol is on”), empty containers from various fast food chains are stacked in several locations around the room, the fetal monitor has the volume maxed out, the television is on (as already mentioned), and there is usually a collection of cell phones (with every imaginable ring tone), cameras, and video equipment that rivals major production companies. This is not delivery “medicalized,” it is delivery completely depersonalized. It’s show time, Papa John’s garnished with meconium, and remember to get your popcorn before you sit down to watch. Does this situation sound familiar to any of you out there who actually get up to your elbows in the blood, poop, and other bodily fluids that characterize a normal delivery? The whole scene needs to be treated with Ritalin.

I have also pondered long about how things have gotten to this point. Even during my training, in nonemergent situations, we tried to keep the room quiet, dimmed the lights, eliminated external distractions, and limited the peanut gallery. Maybe we had to do this because most deliveries were actually done in an operating room. But, there was some sense of intimacy, control, and quiet anticipation. (Incidentally, cesarean section rates back then were less than 10%). Nowadays, physicians rarely spend enough time in the room with the patient (they may not even be at the hospital) to have sole responsibility for the current state of affairs, but we are to blame for being tacitly complicit in allowing things to have evolved to this degree. We have become impatient bystanders, distracted by liability concerns, burgeoning paperwork, other patients at other hospitals, and in the process, have relinquished control, or acquiesced, rather than taking the time and effort to improve things. (Incidentally, today the cesarean section rate is 35%).

My greatest disappointment has been from the nursing side, and some of this may have gotten beyond their control. I remember when the routine was for a nurse to sit with her patient throughout labor (and sometimes into the next shift), taking her under wing, and maintaining control over the environment by offering support, camaraderie, explanation, and reassurance (and contributing immensely to my own education). I imagine the good ones still do, or would if they could. But, there are many times now I see nurses sitting in a central monitoring room, observing mother and baby from a distance (fetal heart rate monitor, contraction monitor, automated blood pressure cuff, pulse oximeter), only going into the room when the call light is illuminated, the fetal heart tracing deteriorates, a monitor falls off, or it’s time to start pushing (“1, 2, 3, 4…,10”). Admittedly, some may have too many other patients (due to staffing shortages, hospital cutbacks, and excessive requirements for documentation) to keep tabs on things in any other way. Unfortunately, I have been around long enough to watch more senior nursing staff successfully pass on these bad habits to junior staff (who don’t know anything different and think that this is the way things are supposed to be), so the whole process is now self-perpetuating. During the same time period, the relationship between physicians and nurses seems to have shifted from protagonists to antagonists. Then again, I may just be getting old and could just be viewing things in the past through the rose-colored lenses of the retrospectoscope.

Patients contribute their share to perpetuating this scenario as well. In the U.S., we still have high teen pregnancy rates, an extraordinarily high rate of pregnancies that are 'unplanned' (even among women in stable relationships), poorly prepared (“I didn’t have time to get to the classes”), but demanding, patients, insistence on completely”painless” deliveries, and a sense that everything must be accomplished on some predetermined schedule (“Mom, I will push if you can tape Grey’s Anatomy for me or you will have to wait to become a grandmother”). The apparent lack of concern or understanding of the magnitude of the moment seems lost and as unreal to me as ‘reality’ television! The focus is often not on the baby but on the comfort, convenience, and style of the central actor in this performance. Unfortunately, reality really will set in, but only after the baby is taken home.

Now, I know I will take some flak for these comments from many different sides; and, I readily admit that I am prone to hyperbole. I also know there are places around the country where nothing I have said is true, but there are many other places where things are even worse. The real tragedy is that, the scenario I have laid out seems to be more common at busy “teaching institutions” where the OB providers of tomorrow, both nurses and physicians, are being trained. I have worked well with many midwives over the years and, even though I fully believe that learners who will be providing OB care could benefit lots from the skills and experience of a good midwife, I also know that home deliveries are not the answer either. Besides, someday, in the not-to-distant future, when the cesarean section rate approaches 100%, this whole discussion will be moot and these issues will only be a problem for the patient who delivers in the ambulance (or taxi) on the way to the hospital and misses her opportunity for a planned, feces-free, operative delivery. Then she can sue the driver, rather than the physician, for her anal incontinence because he didn’t run the last six red lights on the way to the hospital….

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The Smell of Rain

A dear friend of mine sent this to me. I do not know its origin, but thought it was especially appropriate during the Holiday season. It relates an experience many of my patients live through every day. To them and the friend who thought enough of me to send it my way, I dedicate this post.....

THE SMELL OF RAIN

A cold March wind danced around the dead of night in Dallas as the doctor walked into the small hospital room of Diana Blessing. She was still groggy from surgery. Her husband, David, held her hand as they braced themselves for the latest news. That afternoon of March 10, 1991, complications had forced Diana, only 24-weeks pregnant, to undergo an emergency Cesarean to deliver the couple's new daughter, Dana Lu Blessing.

At 12 inches long and weighing only one pound nine ounces, they already knew she was perilously premature. Still, the doctor's soft words dropped like bombs. "I don't think she's going to make it," he said, as kindly as he could. "There's only a 10-percent chance she will live through the night, and even then, if by some slim chance she does make it, her future could be a very cruel one"

Numb with disbelief, David and Diana listened as the doctor described the devastating problems Dana would likely face if she survived. She would never walk, she would never talk, she would probably be blind, and she would certainly be prone to other catastrophic conditions from cerebral palsy to complete mental retardation, and on and on.

"No! No!" was all Diana could say. She and David, with their 5-year-old son Dustin, had long dreamed of the day they would have a daughter to become a family of four. Now, within a matter of hours, that dream was slipping away.

But as those first days passed, a new agony set in for David and Diana. Because Dana's underdeveloped nervous system was essentially 'raw', the lightest kiss or caress only intensified her discomfort, so they couldn't even cradle their tiny baby girl against their chests to offer the strength of their love.

All they could do, as Dana struggled alone beneath the ultraviolet light in the tangle of tubes and wires, was to pray that God would stay close to their precious little girl.

There was never a moment when Dana suddenly grew stronger. But as the weeks went by, she did slowly gain an ounce of weight here and an ounce of strength there. At last, when Dana turned two months old, her parents were able to hold her in their arms for the very first time. And two months later, though doctors continued to gently but grimly warn that her chances of surviving, much less living any kind of normal life, were next to zero, Dana went home from the hospital, just as her mother had predicted.

Five years later, when Dana was a petite but feisty young girl with glittering gray eyes and an unquenchable zest for life, she showed no signs whatsoever of any mental or physical impairment. Simply, she was everything a little girl can be and more. But that happy ending is far from the end of her story.

One blistering afternoon in the summer of 1996 near her home in Irving , Texas , Dana was sitting in her mother's lap in the bleachers of a local ball park where her brother Dustin's baseball team was practicing. As always, Dana was chattering nonstop with her mother and several other adults sitting nearby when she suddenly fell silent hugging her arms across her chest, little Dana asked, "Do you smell that?"

Smelling the air and detecting the approach of a thunderstorm, Diana replied, "Yes, it smells like rain." Dana closed her eyes and again asked, "Do you smell that?" Once again, her mother replied, "Yes, I think we're about to get wet. It smells like rain."

Still caught in the moment, Dana shook her head, patted her thin shoulders with her small hands and loudly announced,"No, it smells like Him. It smells like God when you lay your head on His chest."

Tears blurred Diana's eyes as Dana happily hopped down to play with the other children. Before the rains came, her daughter's words confirmed what Diana and all the members of the extended Blessing family had known, at least in their hearts, all along. During those long days and nights of her first two months of her life, when her nerves were too sensitive for them to touch her, God was holding Dana on His chest and it is His loving scent that she remembers so well.

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First Trimester Screening for Aneuploidy

In my November 29, 2006 post, I promised that my “next post” would focus on a discussion of first trimester screening for aneuploidy. It has taken awhile to get back to this, having had a few distractions along the way, but blogging does offer some degree of flexibility and allowance for distractions!

First trimester screening for aneuploidy, or as it is commonly referred to as ‘combined first trimester risk assessment,’ has gained widespread acceptance in other developed nations over the past decade, and is slowly coming into its own here in the U.S over the past couple of years as well. This screening test uses a combination of fetal measurements, maternal blood pregnancy ‘markers,’ maternal characteristics (age, weight, race, family/pregnancy history, and certain medical problems, such as diabetes) and a large, and continuously expanding, data repository to generate risk numbers for trisomy 21 (Down syndrome) and trisomies 13 and 18. The primary measurements of the baby include the ‘crown-rump length (CRL)’ (top of head to bottom of butt) and the ‘nuchal translucency (NT)’ (clear space between the skin and underlying soft tissues of the back of the neck, head, or upper trunk). The wider the nuchal translucency, the greater is the likelihood that the baby has aneuploidy. A special certification process has been developed for performing NT measurements and completion of this in an acceptable fashion is required before access to the database for combined risk assessment can be obtained. The presence or absence of a fetal nasal bone can also be factored into the risk assessment, since the absence of the same (or the presence of one that is smaller than usual) has also been associated with aneuploidy, especially trisomy 21. Separate certification is required for this to be used in the risk assessment process.


The maternal blood samples assess levels of free β-hCG (the ‘pregnancy hormone’ secreted by placental tissues, the trophoblasts) and pregnancy-associated plasma protein A (PAPP-A) (associated with differentiation of the placental trophoblasts). Free β-hCG levels usually decrease after 10 weeks’ gestation in normal pregnancies, whereas they often remain elevated or increase in cases of trisomy 21. Indeed, the difference between these and normal pregnancies increases with gestational age. In contrast, babies with trisomy 21 tend to have lower levels of PAPP-A than normals, but the difference between these and normal babies tends to decrease with gestational age.

For combined risk assessment, the fetal crown-rump length must be between 45 and 84 mm, roughly corresponding to 11-14 weeks’ gestation. The maternal blood screening can be done at the same time the fetal measurements are obtained, or as early as 8-9 weeks,’ and then combined with the fetal measurements when these are performed. The advantage of this latter approach is that a ‘final result’ can be obtained the same day, whereas about a week is required for the former because that is the time required for processing of the maternal blood samples. The test is most reliable (has the greatest combined sensitivity of the NT and blood screens) at the gestational ages between 11 and 12 weeks and this is also the time when it is easiest to obtain the most accurate NT and CRL measurements.

The ‘sensitivity’ of the test relates to the probability if the disease (in this case aneuploidy) is present, the test will be positive. Neither the NT measurement nor the blood tests by themselves have sufficient sensitivity to stand alone as a good ‘screening test’ that would be widely acceptable. At the risk of great over-simplification, combined risk assessment has been shown to have the capability of detecting trisomies 21, 13, and 18 in 85-95% of cases with false positive rates (probability that the test is abnormal, but the baby is not aneuploid) in the range of 5% or less. In our own experience over the past two years, we have not missed a case of one of these trisomies in ‘at risk’ women (women expected to be 35 years or older at delivery), younger women at increased risk for aneuploidy, or who simply wanted reassurance and had this screening performed.

There are distinct advantages to combined first trimester screening. In most instances, it provides early reassurance to ‘at risk’ and even low risk women who undergo the screening process. It also offers the opportunity for early detection of babies with the chromosomal abnormalities noted above, and for detection of other chromosomal abnormalities (and congenital heart defects as a side benefit in babies that may or may not have a chromosomal abnormality) that might also be accompanied by abnormalities of the NT. This allows patients more time to consider their diagnostic and therapeutic options at a time in the pregnancy where privacy and confidentiality are much easier to preserve. At the same time, the cost, sensitivity, and false positive rates are comparable to, if not better than, that of midtrimester screening.

There are also some limitations of first trimester screening that must be conveyed to all women considering this procedure. The test is still just a ‘screening’ test and therefore cannot replace an invasive diagnostic test such as chorionic villus sampling, amniocentesis, or umbilical cord blood sampling for the definitive diagnosis (or not) of aneuploidy. Although ‘false positive rates’ are low, a “positive result” does not mean that a baby definitely has a genetic abnormality. Indeed, many will not. By the same token, a “negative result” does not guarantee the absence of a chromosomal abnormality, other heritable or syndromic problems, or fetal birth defects that are not clearly related to a specific genetic cause. For that reason, even to women who have had ‘reassuring first trimester screening, we offer both maternal serum alpha-fetoprotein (MSAFP) screening at 16 weeks’ and a ‘targeted’ genetic sonogram at 18-20 weeks’ gestation.

In the next (or some time in the near future) post, we will discuss what it means to have an “abnormal” first trimester screen as well as the options for follow-up of the same…

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Thanks to Jon and the AADT Crew for Grand Rounds Vol. 3 No.12

Thank you to Anxiety, Addiction and Depression Treatments for including my personal recollections regarding the first HIV patient, who just happened to be pregnant, I ever took care of (too many years ago), even before we had a name and a cause for 'acquired immunodeficiency syndrome.'

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World AIDS Day: Remembrance of My First Patient with HIV

December 1, 2006 was World AIDS Day. It reminded me of another very special patient in my life and I wanted to tell her story…

About a week before I was to finish my residency in OB/GYN and start a fellowship in Maternal-Fetal Medicine, the Dean of Admissions at Duke University School of Medicine, Dr. Art Christakos, who also happened to be a senior member of our department, took me aside to ask a “personal favor.” Since I was going to be staying on as a faculty member, he was wondering if I would consider taking on a “special case.” The daughter of a fairly well-to-do family in town, with whom he was close personal friends, had left home and taken off to see the world for several years without leaving a trace. Apparently, she had relied on her feminine wiles to support herself with regard to a basic place to sleep, eat, and supply the heroin to which she eventually became addicted as she traveled around the country. She had finally come home, begun counseling, gotten started on methadone, and then become pregnant. Although she was living with her parents again, ongoing supervision on the methadone program required her care to be received at the local health department, and Dr. Christakos thought she would benefit from having a physician she could call her own. He had actually been asked to see her himself, but thought her social situation, and the relationship he had with the family would interfere with her care. Besides, he hadn’t practiced obstetrics in years.

I met this young woman on a hot summer day in July. The air-conditioning in the health department did not work very well and the place smelled like...like a health department. When I walked in the room to meet her, I was taken aback. Sitting in front of me was a stunningly beautiful and healthy-appearing young woman. Not what I expected, but very easy to understand how she had supported a very expensive heroin habit over the years. We reviewed her medical history and the events of the past several years about which she was quite open. Her only complaints were about “feeling tired all the time” (preceding the pregnancy), a chronic cough (“I smoke 2-3 packs of cigarettes per day”) that frequently had required antibiotics over the past year for “bronchitis,” intermittent "night sweats," and a “swelling in her neck.” Physical exam was remarkable for not just a “swelling in her neck,” but a diffuse prominent lymphadenopathy, an enlarged spleen, and oral and vaginal yeast infections. She had been told that she kept getting the latter because of the frequent need for antibiotics to treat her “bronchitis.”

In addition to the routine pregnancy laboratory work, I screened her for TB, sent off serologic studies for cytomegalovirus and Epstein-Barr virus, and did an antinuclear antibody test as a preliminary screen for an autoimmune disease. Her CBC came back with a low total white blood count and very low lymphocyte count. She did not have acute mononucleosis and she had prior immunity to cytomegalovirus. The ANA was also negative as was her test for TB. Somewhere along the line we got a hematological consultation and a lymph node biopsy, neither of which led clarity to the diagnosis, but did rule out malignancy. Now, I know all of you reading this today wonder what the hell I was doing because you knew her diagnosis the minute I started this post, but things were not so clear back then, so stick with me on this just a little longer!

I headed off to the library to dig out what I could about her combination of physical findings, laboratory results, and somatic complaints. What I came upon were a couple of recent reports from the CDC describing similar findings in homosexual males in San Francisco and New York who ended up dying with Pneumocystis carinii, a condition that had only been seen with any regularity before in patients with severe inherited or malignancy-associated immunodeficiency syndromes, or who required immunosuppressive therapy for a variety of conditions. Indeed, these young males were labeled by the CDC as having some sort of “acquired immunodeficiency” although at the time the etiology for this was completely unknown. Could this be an autoimmune consequence of their sexual promiscuity, a toxic effect of the IV drugs (or contaminants) they had used, or perhaps an infection with some previously unknown organism?

Regardless, I went back to our young pregnant woman and told her that I thought I knew what she had, but I still had no idea what it was! Nor did I know what effect her condition might potentially have on her baby. She let me confirm my suspicion by sending off lymphocyte subtype analysis that confirmed the presence of very low T4 cells, a finding that had also been described in the men with “acquired immunodeficiency.”

As things turned out, she had a relatively uncomplicated pregnancy, except for chronic yeast infections and bronchitis. She delivered at term, a healthy, and large, baby boy, who had no apparent medical problems at the time (and never developed any afterwards). Sometime near the end of her pregnancy, a possible connection between infection with a virus that could grow in T lymphocytes and the cases of acquired immunodeficiency was raised in scientific circles. I told her of this association, but at the time, there was no readily available test that could be done to establish the diagnosis and investigators were still not certain that this was the actual cause of the immunodeficiency syndrome. However, my graduate training in viral immunology made me suspect that they were right on the money.

About a year after her delivery, she returned, complaining of weight loss, increasing fatigue, and persistent yeast infections. By that time, a very clear association between what is now known as human immunodeficiency virus (HIV) and the “acquired immunodeficiency syndrome” had been established. I told her again that I really thought that this was her problem, even if she wasn’t a homosexual male, and that we should confirm the diagnosis so that she could take advantage of any therapy that might someday come along to treat the condition. I also told her that at that time, we did not have a lot of information about potential transmissibility to babies in the uterus and cautioned her about getting pregnant again before we had a diagnosis. To my surprise, she REFUSED to be tested. She also refused to tell her partner what I was thinking. And, two months later, she showed up on my door step again, now early pregnant with her second child! She admitted that she did this quite intentionally and I could have strangled her…(not).

By now, she was really feeling awful, between the pregnancy and her medical condition, and I recommended that she be seen by a close friend of mine in infectious disease. He finally convinced her to be tested for the virus and it did not take long to confirm the diagnosis of HIV. She blew the ELISA off the wall. AZT was in clinical trials by that time (bless you Burroughs-Wellcome), but had not yet been approved by the FDA, so we really had no treatment to offer her. Despite that, with aggressive management of her respiratory and yeast infections, she did surprisingly well as the pregnancy progressed, again delivering a healthy baby boy at term who also, fortunately, did not have any evidence of the virus infection.

After delivery, she continued to be seen by the folks in infectious disease and was eventually begun on AZT. I would only see her periodically as our paths crossed in the hospital when she returned for follow-up visits. She always reported that the boys were doing well and that she felt better now than she had in years. She took my advice regarding contraception, motivated by the published reports of the high morbidity associated with intrauterine fetal infection with HIV, and her awareness of having rolled the dice twice and come away with two beautiful babies.

By that time, my experience with her and the pregnancies, and my own interest in viral infections, had motivated me to include the topic of HIV infection in pregnancy as a regular offering when I was invited to speak. I still have the 100’s of 35mm slides I put together more than 20 years ago sitting in a carousel in my office. Many have faded beyond recognition of their content. A portion of the talk, however, even back then, included a plea that all pregnant women should be encouraged to undergo HIV screening so that treatment could be initiated for both their benefit and that of their unborn babies.

When I finally left Duke in 1987, to move on to the University of Tennessee, I hadn’t seen our patient in several years, but I was updated periodically as to her ongoing condition by my friends in infectious disease. After arriving in Knoxville, I got so wrapped up in my work that I lost touch even with them. I did not, however, forget our patient and made a point of including her story with every opportunity I had to speak about HIV in pregnancy.

Then, around 1994, I was invited to present a symposium on sexually transmitted diseases to the high school students at the preeminent private school in Knoxville. It was to be a “no holds barred” sort of presentation, and I again decided to include the details of my experience with this patient. The day I was to give the talk, I thought it would have an even greater impact if I could give them recent information about her status. The folks I contacted in Durham told me that she had finally died from AIDS and left her two children to be raised by her family. When I related this information to the high school students, teachers, and parents at the end of my talk, I couldn’t stop my voice from cracking. The audience was deathly silent after my display of emotion but many of the parents afterward told me that it had really helped to punctuate the message of the day.

I have watched over the years as the primary mode of HIV transmission to women has shifted from IV drug use to the consequence of heterosexual activity with infected males. I have seen the dramatic reduction in fetal infection as the result of access to treatment in countries like the U. S., and the continuing tragedy of the disease in women and babies in underdeveloped countries where such treatment is not readily available. At least 20,000,000 women are infected with HIV worldwide, and I would bet that if we had truly accurate numbers from Africa, China, and Southeast Asia, the numbers would be even higher. In our own country, it has only been within the past year that standard of care to offer screening to all pregnant women has been changed to a standard that expects such testing to be performed as part of routine screening in pregnancy. I can only ask now after all these years, why has this taken so long?

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Go Get Your Darn Flu Shots!!!!

Sorry, but I haven’t been online in awhile. My last full post seems to have taken an awful lot out of me for some reason. Maybe it was because I had been on call for most of the 3 weeks preceding the events detailed in that story. Then again, I am probably just turning into a real wussy in my old age.

On top of that, I have spent most of the last 3 days worrying about my 9 year old daughter who has had a persistent fever as high as 104.5 degrees F. Her pediatrician tells us that Keegan has the first documented case of the flu seen in their office this year. She has all the typical aches and pains, an aggravating dry cough, and has not been able to hold anything down long enough for the medications she has been given to work. The vomiting has been more a function of the congestion and the low “vomit threshold” she has had since the day she was born. I have been worried sick about her because she has asthma, the residual of having been born at 34 weeks’ herself, and is at increased risk for superimposed bacterial pneumonia. Sometimes a little knowledge about medicine can create a lot of anxiety.

Anyway, please pardon the paucity of my posts so far this week. I can promise you that I have been putting together some good things for the very near future. And, if any of you have ideas for topics you would like to see addressed, please leave a comment to that effect.

By the way, the main reason I wanted to mention my daughter today is to remind ALL you pregnant women out there that it’s not too late to get your flu shot. I got my shot 3 weeks ago (hope they got the strains right this year) and am very glad now that I did. You guys (sorry folks, I was brought up in NJ) are at the same (if not greater) risk for complications as my daughter. And, I don’t need to be worrying about all of you too! Besides, there are no extra beds on my ‘High Risk’ floor this week.

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Thanks to Emily DeVoto at "The Antidote..."

Emily,
Great job on this week's Grand Rounds and thanks for including a link to my recent post on "The Human Value of Knowledge." I have found that sharing special moments in patient care, happy and sad, is definitely an antidote to trying to keep it all inside. Thanks for giving me another outlet and glad you enjoyed the post.
Regards,
Ken

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The Human Value of Knowledge

Since we have been on the subject of aneuploidy screening, let me tell you about a situation that we had on Friday that epitomizes the importance of knowing as much as you can about a baby, including its karyotype (chromosomal status), before delivery….

Our patient is an 18 year old, having her first baby, who was a late entry to prenatal care at an outlying clinic. She was seen by one of my associates for a routine ultrasound to confirm gestational age and assess fetal anatomy. At that visit, she was estimated to be about 27 weeks’ pregnant but, unfortunately, the baby was found to have a congenital diaphragmatic hernia. She had come alone to that visit and, when told of the findings, she apparently became too upset to benefit from any attempt at counseling at that time. Arrangements were made for her to see me for a ‘second opinion’ and also to see our genetic counselor a few days later.

My findings confirmed the initial diagnosis. The baby had a large diaphragmatic hernia, with stomach and bowel filling the entire left chest, pushing the mediastinum and heart to the right chest wall. Very little lung tissue could be seen above the heart. The baby had a single (rather than the normal two) umbilical artery, an absent right kidney, generalized soft tissue edema (fluid accumulation), and polyhydramnios (increased amniotic fluid). The baby was also growth restricted. I expressed my concerns that the baby’s prognosis was poor because of the size of the defect and the paucity of normal lung tissue and also because the baby had a greater likelihood of having a chromosomal problem in view of the multiplicity of the abnormalities seen. She was encouraged to undergo an amniocentesis to determine the fetal karyotype and told that this would help to guide our counseling about recommendations for delivery, and the decisions that would have to be made with her regarding resuscitative efforts following the birth of the baby. She was not at all enthusiastic about having a long needle placed in her abdomen, but when she asked me if I had ever done one (an amniocentesis) before, I told her “No, but I did stay at a Holiday Inn Express last night” and, when she laughed, despite the gravity of the situation, I knew we had made some real progress in her care.

Ten days later, the baby’s karyotype returned abnormal with “mosaicism for an isochromosome of 12p.” To translate that into understandable terms, this means that in certain cells (not all) in the baby’s body, there are 4 copies of the short arm of chromosome 12 rather than the normal two. The abnormalities seen by ultrasound, coupled with the fetal karyotype, made it highly likely the baby had a condition called Pallister-Killian Mosaic Syndrome. This is a very rare chromosomal disorder that occurs sporadically, for no apparent reason, and is not likely to recur.

The patient returned for another ultrasound after the karyotype results were obtained. The ultrasound revealed worsening polyhydramnios (a further increase in amniotic fluid), progressive fetal growth restriction, and abnormal Doppler flow studies suggesting fetal blood flow redistribution, or ‘cranial sparing,’ a physiologic event that often accompanies placental insufficiency that helps to preserve the baby’s brain at the expense of temporarily underperfusing less essential organs. She was told in view of these findings that there was now an increased likelihood of delivery sooner than later, either because of preterm labor, spontaneous rupture of membranes, fetal decompensation, or preeclampsia that might develop as a consequence of the fetal compromise. She spent some more time with the genetic counselor that day and then told us that she thought she would not want heroic measures taken for the baby at delivery. We set up consultations for her with Pediatric Surgery and the Neonatalogists.

Not three days after this visit, and at about 31 weeks,’ she presented to our labor and delivery unit in active labor with spontaneous rupture of membranes. We reviewed the baby’s findings with her again, the prognosis, and her options for care of the baby after delivery. She was told that if she wanted to do everything that was medically possible, we still had time to send her to an institution that had ECMO (extracorporeal membrane oxygenation); although we could not guarantee that the baby would even be a candidate for it. She actually smiled and thanked us but said, “No, we just want to be able to spend as much time with the baby as we can after she’s born.” She and her whole family were at peace with the decision. At that point, we disconnected the fetal heart rate monitor, arranged for placement of epidural anesthesia, and contacted the NICU staff.

Early that evening, she delivered, vaginally. The baby was barely alive at birth but was pink, had her blue eyes open and a slow heart beat. Thankfully, the baby made no effort to breathe or cry and, with respect for the expressed wishes, no effort was made to ‘resuscitate’ her. We dried the baby off, swaddled her in a warm blanket, and handed her to the young mother to hold. At first, she could not look at the baby, but when her mother-in-law offered to take the baby from her, she wiped off the tears streaming down her face, pulled the blanket down to expose the baby’s face, smiled, and stated that “She looks just like her Daddy,” and then gently sought out and touched the baby’s tiny hands and feet. After a few minutes, she finally shared the baby with the grandparents, her sister, and other family members who had been there to support her throughout the day. As the baby’s color darkened and the heart beat slowed further, her eyes closed, and one of the grandparents held her in the air, looked up, and said, “Tell all the grandmas and grandpas up there that we love them, too,” (and let me tell you, when she did that, there wasn’t a dry eye in the room) and handed the baby back to her mother, on whose chest she finally succumbed.

I probably don’t need to tell you (and that’s the real reason for this post) that this touching scenario could easily have been much different if we had not had the knowledge of this baby’s physical and chromosomal abnormalities, and the opportunity to discuss the findings and prognosis with the patient before the delivery. In the end, this young mother was able to make the right decision for herself and she got to spend a few precious moments with her baby while she was still alive. She thanked me as we left the room for “everything we had done for her,” and I could only nod my head and think to myself that I should be thanking her for everything she did for us….

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