Girl in the war

Peter said to Paul you know all those words we wrote
Are just the rules of the game and the rules are the first to go
But now talking to God is Laurel begging Hardy for a gun
I got a girl in the war man I wonder what it is we done

Paul said to Peter you got to rock yourself a little harder
Pretend the dove from above is a dragon and your feet are on fire
But I got a girl in the war Paul the only thing I know to do
Is turn up the music and pray that she makes it through

Because the keys to the kingdom got locked inside the kingdom
And the angels fly around in there but we can’t see them
I got a girl in the war Paul I know that they can hear me yell
If they can’t find a way to help her they can go to hell
If they can’t find a way to help her they can go to hell

Paul said to Peter you got to rock yourself a little harder
Pretend the dove from above is a dragon and your feet are on fire
But I got a girl in the war Paul her eyes are like champagne
They sparkle bubble over and in the morning all you got is rain
They sparkle bubble over and in the morning all you got is rain
They sparkle bubble over and in the morning all you got is rain

—”Girl in the War,” Josh Ritter, The Animal Years

I originally planned to call this post “My Doctorate Is Bigger Than Your Doctorate.” But after recently listening to Josh Ritter’s ballad—one of the most-played songs on my Luke playlist—I realized that the song, though originally about the Iraq War, could also apply to my last pregnancy, when I apparently unwittingly stumbled into the middle of a latent war between researchers and obstetric practitioners.

My first exposure to this dueling doctor syndrome came in February when I was attempting to set up a preconception consultation with Dr. H., the maternal-fetal medicine (MFM) specialist affiliated with my OB. It was shortly after our trip to see Dr. Harvey Kliman, a placental pathologist at Yale, and I was armed to the teeth with information about my placental condition and potential treatment options, including having estimated placental volume (EPV) measurements taken throughout the pregnancy, a technique developed by Dr. Kliman to help detect signs of trouble.

I traded several messages with Valerie, Dr. H’s assistant (all through the receptionist, since Valerie doesn’t have voicemail), before finally managing to speak to her on the phone, a conversation in which she promised me that Dr. H. would be calling me sometime after 4 p.m. in the next two to three days. I waited dutifully by the phone for a week, but the call never came. At this point, I was so frustrated by all the futile attempts to reach Valerie that I just gave up.

Then I got pregnant, and it was time to schedule the first ultrasound at the MFM. As I was talking to Valerie—again, after several attempts to reach her—I asked her if the office would be able to do the EPV measurements on ultrasound. She then informed me that the doctors at the practice didn’t think they needed to do the EPVs but would talk to Dr. Kliman, and that “That’s not for you and me to worry about; that’s for the doctors to hash out together.”

I was stunned—the only thing more condescending would have been if she’ dethrone in the words your pretty little head—and I’m not sure anything coherent came out of my mouth, but the conversation in my head went something like, Really, Valerie? My last baby died at the end of his pregnancy when I was receiving very little monitoring, so frack yeah, it is for me to worry about. And I may not have a PhD, but I’ll bet I know more about my diagnosis than anyone in your stupid practice.

* * * * *

A few days later, I actually got a call from Dr. H. I was completely taken aback because I’d given up on actually hearing from him directly. I was in the middle of making dinner and totally not prepared for his call. He basically repeated what Valerie had said: that they weren’t going to do the EPVs because they just don’t do that in his practice. He then proceeded to brag about his “12 years” in practice and how he had never had a patient with recurrent term stillbirth. He also made a disparaging comment about Dr. Kliman, asking, “Does he sit in front of an ultrasound machine all day? No, I don’t think so.” To which I replied, but only in my head, OK, but do you look at diseased placentas all day?

Since I have more than 15 years of experience in my field, I wasn’t super impressed with his barely a decade in practice. After a while of listening to him blab, without getting much of a chance to speak, I finally interrupted to explain that the condition in Luke’s pregnancy has a high rate of recurrence, that I’m not in the category of stillbirth moms who don’t really have to worry about it happening again, and that the placental volume could be an important clue to the state of this pregnancy because Luke’s placenta was so incredibly small.

Dr. H. was obviously in the car, and after I gave my little speech, he suddenly seemed in a big hurry to get off the phone, so he quickly agreed, at my request, to at least talk to Dr. Kliman and also said he would talk with another perinatalogist at a nearby university to see if that doctor would do the EPVs. Then just like that, the conversation was over.

* * * * *

A few days later, Dr. H. left me a voicemail in which he was clearly chewing food and stated that he’d spoken to the other perinatologist, who also wasn’t willing to do the EPs. He ended the message with something to the effect of “No one in our community does EPV” and “if you want the EPV done, you will need to find another practice.”

I was then left to make the decision of whether to stick with this MFM and forgo the EPVs, or try to find a different MFM who would do the EPVs, without having advanced knowledge of high-risk pregnancy monitoring and which observations and techniques are truly critical. After talking to the helpful folks at the Star Legacy Foundation and my therapist, I came to the realization that regardless of the EPV question, this MFM had been fairly awful to deal with: difficult to get ahold of, condescending, and disrespectful to not just me but to a researcher at a prestigious university with many years of experience and a clear desire to help bereaved families.

I began to research other MFMs in the area and found one, also named Dr. H.—this one a female—who specializes in treating pregnant women with clotting disorders. While I do not have a clotting disorder per se, I figured her experience and knowledge would be applicable to my situation, since massive placental clots killed Luke. Luckily, at my first ultrasound with my OB, I found out that the new Dr. H. is one of the MFMs my OB works with.

I called the new Dr. H’s office and it was immediately a different experience, with a pleasant receptionist who quickly conferred with the new Dr. H. and the sonographer and reported back that they would do the EPVs (though not until after 20 weeks). At our first appointment, the new Dr. H. even said they have worked with Dr. Kliman’s patients before.

* * * * *

I’ve decided I’ll stick with the new Dr. H. in a future pregnancy, unless I can get enrolled in a study at a research hospital (something I’ll be investigating and may write about in a future post). So that part is settled, but the whole experience left me bewildered by and despondent about the state of the obstetric profession here in the U.S.

If researchers looking into a pregnancy condition—armed with reams of data from cases they’ve studied and research they’ve conducted in the lab—are developing methods to diagnose and treat that condition, why would practitioners be so resistant to implementing those methods in their everyday medical care, even if just on a trial basis? And even more so, I wonder, why would they resist when that condition is stillbirth? And when the method being proposed is 1) simple and quick to implement; 2) poses no harm other than to the practitioner’s pride; and 3) at the very least, will provide additional information that could be useful to managing the pregnancy and preventing another stillbirth?

If researchers work in a lab all day and doctors sit in front of an ultrasound machine all day, wouldn’t individuals from both environments have valuable perspectives to bring to patient care? And wouldn’t it benefit patients, and bring about happier outcomes, for those individuals to get together and talk about what they have seen, and to together come up with new treatments to test? And most of all, don’t doctors care more about saving lives than having their egos wounded by someone making a simple suggestion that there may be a better way to do things? Don’t doctors want to advance their professions? Or would they rather cling dogmatically to what they learned in academic settings long ago?

In speaking with Lindsey Wimmer at the Star Legacy Foundation, I learned that this kind of internecine battle is common in obstetrics. OBs don’t always think outside the box, and they prefer what’s been tried and true—even if it’s not all that true, or at least useful to preventing stillbirth. And there is some justification for this; admittedly, it’s not always clear what practitioners should do with any extra information they collect about placentas or umbilical cords. If a problem is detected at 28 weeks, for example, is it better to deliver right away, when survival is not guaranteed, or to take a gamble and wait a few more weeks, when the risks are fewer? There’s simply not enough clinical, real-life data for doctors to make these decisions, Lindsey says.

In the case of EPVs, the data Kliman has collected so far has been retrospective, looking at placentas from previous pregnancies and matching those up with outcomes. He is now trying to gather prospective data, in order to predict which placentas will be problematic. It’s not yet clear how critical EPVs could be in pregnancy monitoring, but we can’t answer this question without the cooperation of OBs.

* * * * *

And all of that is a fracking shame. In a recent Washington Post article tellingly headlined “Stillbirth is more common than you think and we’re doing little about it” (subtitle: “In the United States, there are more than 20,000 stillbirths each year, a rate worse than that of many other countries”), author and stillbirth mom Sarah Muthler writes that the U.S. ranks 25th in the world for stillbirths and “has made some of the slowest progress of any country in reducing stillbirths. Between 2000 and 2015, the U.S. rate declined by 0.4 percent per year, putting us at 155th out of 159 in the world. We were joined at the bottom by Chad and Niger.”

Unlike in countries like the Netherlands, where autopsies and placental exams are provided for free and a medical team reviews each stillbirth to pinpoint weaknesses in care, “the United States has no national system to report and evaluate stillbirths,” Muthler writes. And many parents opt not to shell out thousands of dollars for autopsies and genetic tests not covered by U.S. insurance companies, meaning crucial data is left uncollected.

Moreover, Muthler writes, “Stillbirth has not received the same interest [as Sudden Infant Death Syndrome] because the public underestimates its devastating toll and also tends to view it as inevitable.” People equate it with miscarriage—I myself have seen that firsthand.

In contrast, rates of SIDS declined 50 percent, Muthler says, after researchers figured out why it was happening and a government campaign galvanized doctors to educate parents about how to prevent it.

And yet, stillbirth is 10 times more common than SIDS, and no one seems to care. We have a fracked-up system here, people. In memory of Luke, Lydie, Matthew, Isobel, Larkin, Baby 2, Josie, Savanna, Meredith, Maeve, Eloise, Leo, Lily, Quinn, and all the countless other babies stolen by stillbirth, and in honor of the families left here on earth to grieve forever, we need to do better. And it starts with doctors setting aside their stupid silos and scaling back their enormous egos to get together in the same fracking room and figure out how to help their patients. It involves them working together to collect data on when things go wrong in pregnancy, so they can figure out what the hell they are going to do about it. It involves them being willing to listen to parents about new research and work with the experts they have found. It involves them acknowledging our losses and vowing that they never want to see it happen again.

I don’t want to be a girl in the war anymore. I just want my baby back.

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Foam on the grass

Sometime in the moments after my OB, staring at the ultrasound screen, softly uttered the words that are frozen in my brain—I’m afraid it means the baby has died, in answer to my question of what it meant that there was no heartbeat—she turned to me and said reassuringly, assuredly, that most women who experience stillbirths go on to have living babies; having more than one stillbirth is incredibly rare, even more rare than having just one. (Oddly, she never actually used that word, stillbirth; I had to put it together myself after I began to grasp that I would still have to go through labor.)

The MFM doctor we were whisked to immediately after, to confirm that Luke had died, said much the same thing: To have multiple stillbirths is extremely rare. Many women go on to have normal pregnancies? I asked him. Most do, he replied.

Sometime in the weeks after Luke’s death, I read another baby loss mom’s blog about being treated as “low risk high risk” by the staff at her MFM; her second child had died of an umbilical cord knot. She was pissed about being treated this way, but I remember thinking, OK: Low risk high risk—I can live with that; I like the sound of “low risk”; that lessens my anxiety.

And I saw the statistics. About 1 in 160 pregnancies (0.5 percent) end in stillbirth, defined in the U.S. as a loss after 20 weeks of gestation. Of those stillbirth moms, fewer than 1 in 100 have more than one stillbirth. You probably won’t get hit by lightning twice. And I have a healthy daughter, who was born at almost exactly 40 weeks after an uneventful pregnancy. Plus, on the way to the hospital, we saw rainbows by the side of the road, twice, and days later I learned the term rainbow baby means a child born after a loss. And so, from somewhere deep in the dark grief, a little kernel of hope sprouted.

All of that was robbed from me the day I received the autopsy results.

In the medical literature, massive perivillous fibrin deposition and its associated conditions are described with bluntly dire language such as “high recurrence,” “severe consequences for the fetus,” and “associated with stillbirth” and a host of other issues including miscarriage and intrauterine growth restriction. In the face of such odds I questioned whether I should even try to have another child. On top of so many other secondary losses (friendships, my job, my innocence), it was devastating to think another pregnancy might not be in the cards.

Then in early January, we went to see Dr. Harvey Kliman, a placental pathologist at Yale, to talk to him about his review of our placental sides, and a teeny, tiny seed of hope was reborn.

How a placenta normally functions

As Dr. Kliman explained it, one of the placenta’s many roles is “to immunologically appear to be invisible to the mother”: to protect the fetus, via cells known as trophoblasts, from attack by the mother’s immune system. Otherwise, the maternal immune system would reject the baby’s foreign tissue in the way that it would attack tissue transplants from an incompatible donor.

Dr. Kliman noted that the ability to distinguish self from non-self is an evolutionary advantage predating life on land by hundreds of millions of years, and that even single-celled sponges, if mixed together, will separate back out into themselves—”they know what is me and what is not me.”

Why placentas occasionally fail in this role is poorly understood because scientists don’t yet understand how a normal placenta is able to function in this immunologically invisible way. In the 1950s, Medawar described this as “the paradox of pregnancy”: the puzzle of why most animals, and humans in particular, don’t reject their pregnancies. As Dr. Kliman said, “We have this pregnancy inside of a woman when really everything that’s made about her is designed to not let that happen.”

From four weeks on, the developing embryo and its support organs take on a structure that can be likened to a plant: the embyro is the plant, the maternal blood is the soil, and the placenta is the root system, with the “soil” circulating between the root fingers, known as villi.

The maternal blood is in constant contact with the villi, much like water circulating around your fingers if you put your hand in a bucket of water. Crucially, one of the trophoblast cells’ many functions—in addition to manufacturing steroids, absorbing nutrients, and expelling wastes—is to prevent this blood from clotting, like deicing a wing; “there’s constantly deicing going on,” Dr. Kliman said.

The grass analogy

Some trophoblasts also leave the placenta, enter the maternal blood circulation, and destroy the walls of the maternal blood vessels in order to open them up and allow for more blood flow. In some cases, for genetic reasons the trophoblasts don’t perform this function, resulting in such low flow to the placenta that the tissue actually dies.

Dr. Kliman compared this to grass dying after a sprinkler system is shut off. “You have brown grass, but you have nothing in between the grass.” In Luke’s case, he said, there was “foam on the grass”: the fibrin, or clotting material, that built up in the placenta. Not only was the placenta infinitesimally small, at the 0.01 percentile (meaning smaller than 99.9 percent of the placentas in his age group), it was only 5 percent functional.

“This is not turning the sprinkler off,” Dr. Kliman. “This is, someone has covered the whole grass with a foam that doesn’t let any light in, no water, and the little leaves are just stuck in this gluelike stuff. … The intervillous space was just obliterated in this placenta. There were very few places for the mother’s blood to interact with the fingers.”

On the slides, Dr. Kliman noted that the villi had branched in an effort to obtain more surface area and more contact with my blood, in the way that a plant growing in low light will become long and spindly. He also found evidence that Luke, incredibly, had made his own blood cells in response to lower oxygen delivery. “I think that’s just a testament, if you will, to how healthy he was, in the sense that even though he was in such an adverse environment, he really managed to survive so well for so long.”

Luke was also abnormally large compared to the size of his placenta; a normal fetus-to-placenta ratio is 6:3, and his was 7.8:1. Despite his tiny placenta, Luke himself was still in the 10th percentile. “I think it’s an amazing thing that he made it that far,” Dr. Kliman said.

Villitis versus intervillositis versus MFI

Dr. Kliman also clarified the diagnosis as MPFD/CHI. Whereas the pathology report from the hospital had referred interchangeably to MPFD and maternal floor infarction, the latter signifies fibrin deposition confined to the maternal floor, or the area where the placenta attaches to the uterus, whereas in Luke’s case fibrin was found throughout the placenta.

He clarified the difference between conditions associated with MPFD. In villitis of unknown etiology, the villi themselves are attacked by the mother’s T-cells, the tissue-recognizing cells that reject foreign tissue. While there was a little of that happening in Luke’s case, Dr. Kliman said he believes it to be closer to intervillositis, an attack on the cells between the villi. All these conditions are part of a spectrum, and while it wouldn’t seem to matter which specifically I had, it’s important to note that villitis can be treated with immunosuppressive therapy, whereas intervillositis cannot.

Because I have a healthy daughter who delivered normally at 40 weeks, Dr. Kliman suggested that the etiology in Luke’s case may have been an immune response to his particular genetic makeup, citing a case of twins where one baby was immunologically attacked by the mother and the other was not. “The genetics of that particular fetus was expressing something that caused [the attack] to happen and the other one was not,” he said.

Although autoimmune disorders can be implicated in MPFD cases, Dr. Kliman said it’s unlikely I have one. When I asked about antiphospholipid antibody syndrome, which can be asymptomatic, manifesting only in pregnancy, he said it’s overdiagnosed.

Dr. Kliman was careful to express repeatedly that Luke’s death was not my fault. When I noted that Luke appeared to be of normal weight at his 32-week growth scan—suggesting to me that the condition had kicked in sometime after 32 weeks—he pointed to a recent study on how common it is for ultrasound technicians to overestimate fetal weight (resulting in pressure on mothers to deliver by C-section). “This was a process that I’m sure was happening from the very beginning of pregnancy,” he said.

Treatment for a subsequent pregnancy

We spoke at length about a treatment plan for a subsequent pregnancy. Because immunosuppressive therapy doesn’t work in cases of intervillositis, Dr. Kliman suggested starting with one baby aspirin per day from the beginning of pregnancy, to prevent clotting. I would start low molecular weight heparin, which can also prevent clotting, only if the same problem started to present itself, as there may actually be no issues in the next pregnancy, if in fact the immune attack in Luke’s case was a response to his particular genetic makeup. “If the placenta is normal size, there’s no indication in my opinion to do something that has some risk to you, and heparin is low risk but not zero risk,” Dr. Kliman said.

I’ll also be receiving more frequent ultrasounds in a subsequent pregnancy, for which Dr. Kliman wants to use the estimated placental volume technique he developed with his father. This simple set of measurements of the placenta’s width, height, and thickness can be compared to normative curves for placentas of the same gestational age. (Unfortunately, ultrasound technicians normally don’t measure the placenta or the umbilical cord; the standard of the care in the U.S. is to “follow the fetus” and the fetus only.)

“You did extra nothing in the second pregnancy and you made it to 37 weeks,” Dr. Kliman said. “That pregnancy would have survived if we would have known beforehand and we delivered you. So we can at least do that for you. But I think we can do a little better. We can at least have better growth and be aware of it. If we see nothing and there are no problems, then we just continue to follow you. If there’s another very small placenta, I would not go to 37 weeks.”

Dr. Kliman noted than in cases of renal failure, the kidneys can go all the way to a 90 percent loss of their units because the remaining 10 percent are operating at 500 percent efficiency. “This is how all organs that have many little units fail,” he said, likening the scenario to pregnancy. “The problem is this crash,” he said, as he drew a graph with a straight line that plummeted at the very end. “We want to see that we’re going down this pathway. If you’re only looking at this axis, which is the fetus, everything looks OK right until the very end. So we need to understand what’s happening here”with the placenta, pointing to the line before the crash.

Ray of hope

I’ll write a more detailed post when I have some time, but our visit to Yale on Tuesday yielded unexpected hope and cleared away much confusion. Dr. Kliman clarified the diagnosis as massive perivillous fibrin deposition, not maternal floor infarction, related to inflammation (intervillositis) of the placenta’s intervillous space, caused by invading white blood cells from my immune system. In maternal floor infarction, the fibrin, or clotting material, is limited to the area of the placenta closest to the mother’s side. In my case, fibrin built up throughout the intervillous space, obliterating it.

For some reason not clearly understood by science, in cases like mine the placenta fails its job of keeping the baby “invisible” so that the mother’s immune system doesn’t attack it, as any mother’s would otherwise do. Dr. Kliman felt the condition was present from the beginning of the pregnancy and not one that developed toward the end, as I had previously written. He also doesn’t believe an autoimmune disorder was involved and thinks it was a response to Luke’s particular genetic makeup—which helps explain my prior uncomplicated, full-term pregnancy.

Dr. Kliman had hope for a subsequent pregnancy as Luke fared well and strong under the circumstances despite a small and failing placenta, as evidenced by his weight, his survival to 37 weeks, and the fact that he even tried to make some of his own red blood cells. We discussed treatment and monitoring methods and his research, and for the first time in a long time I felt I had met a medical professional who truly cares and is trying to make a difference.

0.01 percentile

In October, shortly after I found out the diagnosis of maternal floor infarction/massive perivillous fibrin deposition (basically, huge clots formed around the villi, the little fingerlike placental protusions that delivered nutrients to Luke, making them unable to function), I connected with the folks over at the Star Legacy Foundation, the only national U.S. organization dedicated to stillbirth awareness. They responded quickly and kindly and put me in touch with experts who answered a few questions; they also let me know about Dr. Harvey Kliman, a Yale University placental pathologist who consults on stillbirth cases.

We soon began working with the hospital to have slides from my placenta sent to Dr. Kliman’s office. On Dec. 22, I received Dr. Kliman’s report. (This is probably a post for another time, one in which I recount all the ways people suck, but the report was dated Dec. 2 and sent directly to my doctor, who never bothered to contact me; I only got the report after following up with Kliman’s office. My doctor has always been really nice and kind to me and that’s why I’m still with her, but I’m incredibly pissed about this. I had even taken the time to give her a heads up that the report would be coming.)

The gist of the report, and the finding that was new and shocking to us, is that my placenta was EXTREMELY small; Dr. Kliman even used all caps in his report, as shown below. It actually weighed less than the 0.01 percentile for his gestational age of 37 weeks. For those of you who have all but forgotten about your SAT scores, this means that 99.99 percent of placentas of the same gestational age, statistically speaking, are larger than mine was. Given this fact, it seems astonishing that Luke survived to 37 weeks, and indeed Dr. Kliman notes in his report that “the survival of this fetus to 37 weeks [was] remarkable.”

To me this seems to suggest that the placental clots must have formed rapidly, because there was no indication of this problem at Luke’s 32-week growth scan, and in fact at that time he measured large for his age. So some time between 32 weeks and 37 weeks something went very wrong. Unless the ultrasound technician royally screwed up—and there was a substitute working that day, a technician from a different office who wasn’t used to the machine—but I’m inclined to think that wasn’t the case, because at birth Luke weighed 5 pounds 6 ounces, which while small, to me doesn’t indicate that he’d had a super tiny, infintesimal placenta for very long. He was also quite the kicker and squirmer until the end.

Zack and I will be traveling to Yale on Tuesday to meet with Dr. Kliman, ask him all of our questions, and hear about his recommendations for a future pregnancy. Except that I don’t really have any concrete questions at this point, just a swirling hot mass of confusion and despair. I have a stack of printouts about MFI/MPFD that I made right after we received the diagnosis, but I haven’t been able to read through them because they are so technical and dire. It’s something I’ll have to bring myself to do in the next few days, but I’m not looking forward to it, as what little research I have done shows there’s not a lot of global consensus on how to treat these conditions—and everything depends on what the insurance companies will pay for anyway.

Speaking of which, Dr. Kliman in his report recommends for a future pregnancy a technique called estimated placental volume. It seems like a simple process in which the health care provider regularly measures the width, height, and thickness of the placenta. Because I’m now extremely cynical about everything, I’m not holding out much hope that I’d be able to persuade the doctors to do this, even the high-risk doctor I’d be seeing for regular ultrasounds. I’m sure it’s not approved by the American College of Obstetricians and Gynecologists, and I’m sure that without ACOG sanctioning, insurance companies won’t pay for it, even though it seems like it would be simple enough to grab those measurements while they’re already in there poking around.

I’m not even sure that ACOG has published standards for what size the placenta should be; I know they haven’t for the umbilical cord. Similar to Dr. Kliman’s EPV technique (and I’m just assuming at this point that it’s not ACOG-approved), a dedicated and courageous umbilical cord researcher named Dr. Jason Collins came up with a technique and equipment for monitoring babies in utero so that moms could be alerted right away when their babies are in distress, but he never got the doctors and insurance companies on board, and he could never find the funding to mass-produce the equipment, so it’s not even available anymore.

Anyway, I digress. The only small upside to Dr. Kliman’s report is that he doesn’t seem to think it was villitis of unknown etiology, as another expert suggested, which is somewhat more dire because it can’t be treated (it seems not even with anti-clotting agents, though I’m not entirely sure) and can worsen with subsequent pregnancies. However, the MFI/MPFD diagnosis suggests I may have an autoimmune disease, which is odd since I’ve never had any other kind of symptoms—and Zoe was born on time, with no growth restrictions. So one of the next steps, I’m sure, is to figure out what the fuck is up with all of that.

(From the report:)

This placenta was EXTREMELY small, weighing less than the 0.01 %ile for gestational age. There are three major causes for such a small placenta: decreased maternal perfusion of placenta, chronic maternal immunologic rejection or an intrinsic genetic abnormality. In this case there was a combination of decreased maternal perfusion, immunologic rejection, and massive intervillous fibrin deposition (as a consequence of the first two issues). Not only was this placenta extremely small in weight, only about 10-20% of the villi were functional, making the survival of this fetus to 37 weeks remarkable. The pathologic progression of this process can be seen in the images, with the top image showing evidence of intervillositis with monocytes, macrophages and lymphocytes. This progressed to total trapping of the villi in the middle image, followed by death of the villi in the bottom image. There was no evidence of genetic abnormality seen, nor evidence of an intraamniotic fluid infection. This condition can recur.

It would be useful in subsequent pregnancies to follow the placental volume using the Estimated Placental Volume (EPV) technique (see http://klimanlabs.yale.edu/placenta/epv/index.aspx) and if this recurs to deliver by section as early as is prudent for the fetus.

Maternal floor infarction

On Thursday, Oct. 15, my doctor called with the results of my clotting disorder tests and Luke’s autopsy (clotting disorders such as a Factor V or Factor II mutation are one cause of stillbirths).

First, she said, all my labs came back normal except one. I have the xx mutation of the MTHFR gene, involved in metabolizing folic acid. It’s a common mutation, she said: “80 percent of the female population has it.” I would need to take baby aspirin throughout a subsequent pregnancy.

Next, the autopsy. “The baby was fine,” she said. “There was nothing wrong with him. It was the placenta.”

The cause, she continued, was determined to be an extremely rare placental disorder known as maternal floor infarction (MFI). (The condition may also be known as massive perivillous fibrin deposition, or MPFD, although at least one journal abstract I found seems to suggest that they are different conditions on a spectrum.) In any case, what happened was that a clot formed, resulting in placenta insufficiency: a placenta unable to sustain Luke. In the scientific language, fibrin (a clotting protein) built up in the spaces between the placental villi, the small fingerlike projections that we all learned about in health class. The placenta was actually 75 percent “infarcted.”

From the autopsy report: Massive perivillous fibrin deposition is a condition characterized by enmeshment of chorionic villi in a fibrinoid material and in placental insufficiency of various degrees. The perivillous fibrin deposition essentially strangles the chorionic villi. This in turn leads to a marked reduction of fetal blood flow in the affected villi and secondary stromal fibrosis. Thus, functional villi are reduced in number, and if the process is sufficiently large, placental function is compromised. The consequences include a significant risk of intrauterine growth restriction, intrauterine death, and preterm delivery.

“We do not know what causes it,” my doctor said, “and the downside is that it has a high risk of recurrence.” Around 30-32 weeks of a subsequent pregnancy, she said, I would be monitored more closely, with twice-weekly ultrasounds, and would be induced at 37 weeks (which was the plan anyway, before we knew the cause). “The ultrasound can show some things, but not everything,” she said; I would also have to be hyper-vigilant about kick counts, but in the end, we would have to leave things up to a certain amount of chance.

I reeled from shock. I had always figured the autopsy would come back inconclusive, or with some kind of freak cord accident. I knew the chance of having two stillbirths was incredibly rare, much more rare than the risk of having one (I think it’s like 2 percent of all stillbirth moms go on to have a second stillbirth), and it never occurred to me that a subsequent pregnancy would really, truly be high risk and that my rainbow baby could be just a fleeting dream. Suddenly I was launched into the grieving process all over again, and now trying to figure out how we could decide whether to try to have another child.

After digesting this information for a few days, I contacted the Star Legacy Foundation for Stillbirth Awareness. In online searches for both maternal floor infarction and massive perivillous fibrin deposition, I had found mostly technical journal abstracts, nothing much written from the lay perspective or by other mothers in the same situation. I asked the foundation if they had any information on the condition(s) written for a lay audience. To my surprise and eternal gratitude, they responded within hours and forwarded my inquiry to two members of their board.

The first, placental pathologist Dr. Mana Parast of the University of California, responded with a recommendation that I be screened for autoimmune conditions such as lupus and antiphospholipid antibody syndrome (a clotting disorder). She said if an autoimmune condition is indicated, she has known women treated with low molecular weight heparin or other treatments during subsequent pregnancies who delivered healthy babies (and normal-looking placentas).

Then she mentioned that she might question whether the cause was MFI, and not instead a condition that can mimic MFI called villitis of unknown etiology (VUE), in which the mother’s immune system attacks paternal antigens on placental cells. It can also be associated with fibrin deposition in the placenta. The recurrence rate is high, just as for MFI (up to 50 percent in subsequent pregnancies), but there is no treatment.

Since I had a normal pregnancy with Zoe, she questions the MFI diagnosis, unless Zoe had undetected fetal growth restriction or I developed an autoimmune disease after that pregnancy. On the other hand, VUE, she noted, gets worse with subsequent pregnancies (i.e., “mom’s immune system is now alerted to the paternal antigens, so she’ll fight them back faster”).

We are in the process of sending samples of my placenta to a Yale pathologist, Dr. Harvey Kliman, also recommended by the Star Legacy Foundation. Hopefully Dr. Kliman will be able to give us a definitive answer as to MFI/MPFD or VUE, and we can go from there.

Star Legacy also connected me with Dr. Alexander Heazell of the UK’s University of Manchester, who has created “rainbow clinics” to work closely with parents to plan and deliver subsequent pregnancies. Dr. Heazell was in a radiology residency program when his child was stillborn, and he changed his focus to OB/GYN, dedicating his career to finding answers. He didn’t have much to add to what I already knew, but it gave me hope to hear that his clinics have yet to lose a rainbow pregnancy.

In the meantime, I have a number of questions swirling around my head—ones I can’t seem to find many answers to because a) I don’t want to shell out hundreds of bucks for a journal subscription, and b) the disorder is so rare and so poorly understood that there aren’t many journal articles on it to begin with. Among my questions:

1. MFI is associated with central nervous system (CNS) injuries and “adverse neurodevelopment outcomes” such as cerebral palsy. What impact does treatment (aspirin, heparin) have on the risk of such outcomes?
2. Is VUE also associated with such outcomes?
3. Are MFI and MPFD truly on a spectrum? What does that mean for my situation? Where am I on the spectrum?
4. In the case of MFI, does treatment eliminate or merely reduce the risk of another stillbirth?
5. What exactly is the risk of recurrence with MFI?
6. Does my MTHFR mutation have anything to do with MFI? I’ve seen other women with MFI say they were prescribed extra folic acid (not sure if they meant folate) in subsequent pregnancies; is this because the MTHFR mutation is somehow related?
7. Is MFI/MPFD related to a clotting disorder, an autoimmune disorder, or both?
8. I’ve heard that some women develop clotting disorders only in pregnancy—so even if I came back negative for Factor V now, maybe I would have it during pregnancy?
9. Exactly which autoimmune diseases should I be screened for?
10. Why the fuck is this happening to me?

Thanks to the kind folks at Star Legacy, I have some of these questions out to the placental pathologists and hope to hear more answers soon—and hopefully Dr. Kliman will also be able to clear a lot of this up for us. All I can do now is focus on the next step.

Guinea pigs (AKA Effin’ Doctors)

In 2009, I contracted Lyme disease. I don’t even know how, as we were living in urban Frederick at the time. I had a fever and some muscle aches and then noticed a bull’s-eye rash on my back.

I found a specialist, and thus was my rude introduction to the controversial, little understood world of Lyme disease. It often goes undiagnosed because titers can yield false negatives, and some doctors don’t believe it remains in the body long-term. The medical community is split down the middle on how to diagnose and treat it.

I recovered quickly; I was one of the fortunate ones. Some people suffer debilitating symptoms for the rest of their lives (if you believe the Lyme activists).

In 2013, Zoe was born. Right away, I had problems with breastfeeding. It was excruciating, even though it’s not supposed to be, and it soon became clear that Zoe wasn’t getting enough to eat. The lactation consultants at my hospital weren’t much help. The pediatrician was no help; she just told me to supplement with formula. The pain eventually got better but for months I struggled with low supply. I fell into a mild depression. All I wanted to do was feed my baby like so many other moms could seemingly do effortlessly.

After about six months I was doing some research online and stumbled upon my tribe: a whole host of other mothers, also struggling with low supply. And for all kinds of reasons: thyroid disease, PCOS, hormonal imbalances, something called insufficient glandular tissue. These sisters had taken it upon themselves to educate and help each other because here again, the medical community is not much help. Many lactation consultants dismiss low supply; they just tell new moms that as long as you’re feeding baby often enough, you won’t have a supply problem. (Yeah, tell that to the moms who are taking fenugreek until they smell like maple sugar, feeding baby around the clock, pumping after every feed, drinking lactation tea and a gallon of water every day, eating oatmeal at every breakfast, and still not producing enough.) As far as doctors go, it’s hard to find ones who will do the tests to help you figure out which underlying medical issue you might have. And pediatricians are the worst; most of them don’t lift a finger to help mom figure out what might be the problem and instead just push formula.

With the help of my newfound community, I located a new lactation consultant. She came  to our house, heard my story, watched Zoe nurse, and sent us to a pediatric dentist to have her tongue and lip ties revised. Yes, it turns out that a tongue tie is actually a thing. And tongue and lip ties can interfere with breastfeeding, make it painful, and cause milk supply to never be fully established because the baby cannot feed properly.

Guess what? Tongue and lip ties are not fully understood or recognized by the medical community, and there’s disagreement and confusion about diagnosis and treatment. Many doctors and lactation consultants will look in the mouth of a clearly compromised baby and declare there to be no ties. I belong to tongue tie support groups and have seen some of the pictures and just shake my head at how the diagnosis could be missed. Then these poor moms have to drive around to all these different dentists and ENTs before they find one who will actually listen to them.

How to treat tongue ties is a whole other question. Some do it with laser and some surgically. Some recommend chiropractic and cranial sac therapy as aftercare while others just send moms home with instructions for some simple stretches.

We had Zoe’s ties revised and nursing got better but I think they grew back, and by then it was way too late to do anything about my supply. Zoe had started solids at that point anyway and it mattered less and less as time went by. I ended up nursing her for 20 months, which I was really proud of because it was so, so hard for a long time.

Now, we come to Luke’s stillbirth. It turns out that stillbirths are—wait for it—poorly understood by the medical community. Apparently your full-term baby can just up and die inside of you after an otherwise normal pregnancy, and the doctors have absolutely no clue as to why. And kick counts, one of the few things that could actually possibly prevent a stillbirth, are—you guessed it!—controversial. Are you sensing a theme here? In the U.S., the Count the Kicks campaign says that third trimester babies should kick or move at least 10 times every two hours. Over in the U.K., the Count the Kicks campaign says frequency and pattern of movement is unique to each baby and as long as your baby is doing what is “normal” for her, you should be fine. Meanwhile, my eight-month-pregnant friend’s doctor told her, “We don’t really tell people to do kick counts anymore.”

By this point I’ve pretty much given up on the medical community. You have to work so hard to seek out professionals you can really trust, who a) you’re confident they know what they’re doing; b) if they don’t, will go to the ends of the earth to look for an answer; and c) will listen to you and not roll their eyes when you show them something you found online or in a book.

My grief counselor told me that Luke’s life is not meaningless because he taught us things, brought us gifts. The gift of empathy, of living in the moment. And for me, the gift of learning to speak up for myself. Through all of these experiences I was never really a self-advocate. I just listened to what the professionals had to say and took it at face value, often without really understanding. This tragedy has changed all that. I will speak and speak until they listen and if they don’t listen, I will go somewhere else and find someone who does. That’s Luke’s gift to me.

Autopsy report, part 2

I talked to the state’s social worker on Monday about finding out the status of Luke’s autopsy report and whether we could get a written copy instead of just a phone call. I hadn’t heard back, so I emailed her for an update. She called back and said that an autopsy had been declined because my daughter’s death at 38 weeks had been considered natural.

Zack and I lost it.

We requested an autopsy, I told her. I signed paperwork authorizing it. I had a son, not a daughter. And I was 37 weeks, not 38. Are you sure you even pulled the right case, I asked her? I’m freaking out right now, I said.

She apologized and said I should speak directly to her organization’s executive director. She gave me the phone number. I called right away and left a message explaining the situation and telling her that I was highly distraught. Within a few minutes, the woman called back. She asked me a few questions and then explained that it was the state medical examiner’s office that didn’t do an autopsy, that they defer to local hospitals in such cases, and that the hospital would have conducted the autopsy. She said the hospital is probably still waiting for lab tests to come back and that is the likely reason for the delay.

Well, Jesus. I wish the social worker had been better informed of how these things work. Isn’t that her job? She could have bypassed the medical examiner’s office and gone straight to the hospital to begin with—preventing us all this heartache in the meantime.

The executive director then offered to make some calls on Monday and try to find out more about the status. She kept getting the name of the hospital wrong, but we finally squared it away. (Franklin Square Hospital? Frederick Square Hospital? Oh, Frederick Memorial Hospital? Got it).

So, that’s where we are now.

Autopsy report

We’ve gotten a couple voicemails from a social worker who works for the state of Maryland. I hadn’t called her back because in one message she referred to Luke as “her” and in another she said she’d called on Aug. 17. Since we didn’t find out Luke was dead until Aug. 20, I thought that was interesting.

Anyway, the hospital didn’t send us home with anything about the autopsy—when to expect results, what to expect, who to call with questions. It’s been 4.5 weeks and we haven’t heard a peep. So I decided to call the social worker this morning and ask her what she knows.

She said she’d call the University of Maryland Center for Infant and Child Loss and find out what they know about the status of the autopsy. She said it usually takes 8-12 weeks. Will something be mailed to us? I asked. Usually it’s just a phone call, she replied. I told her we’d want a copy of any written documentation. I can’t imagine there wouldn’t be anything written. It seems crazy that your full-term baby can die inside of you, and all you get is a phone call.

So that’s where it stands now. She’s going to see if she can find out the status and let them know we want a copy of anything written.