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.

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15 thoughts on “0.01 percentile

  1. This is really interesting Angela. I will ask my consultant next week if she knows the information about placental volume. She’s certainly never mentioned it to me. Will have a look at our post mortem report but I think my placenta was average in size. Awesome you are getting meeting with the expert!!! I know how hard it is to read all the medical information and try to process it when you can barely function for day to day tasks, you’re doing great x

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    • Thanks, Alanna — I’m trying to just take it one step at a time. There’s a link to more about EPV at the bottom of the post if you are interested. I can’t remember if autoimmune disorders are associated with MPFD or just MFI – brain is a foggy soup right now – but did you ever hear anything about that in connection with your case?

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      • Yes but only from people in the FB group, my doctor doesn’t really know anything about MPFD specifically and won’t prescribe the hydroxychloroquine that others are on. It does make me nervous but I do trust her too. I think monitoring is the most important thing and delivering as early as possible. So far my little one is growing perfectly on track, long may it continue!

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      • Yay, that is wonderful! Little one needs to just keep growing. I agree with that – monitor and monitor and just pray you get to the viable point. So did you ever find out if you have an autoimmune disorder? Argh, the whole thing is so confusing and overwhelming.

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  2. Thank you so much for sharing this information! I am so glad you were able to find resources through Star Legacy Foundation and now Dr. Kliman, and I’m happy Dr. Kliman seems to be giving you some answers (not that it makes any of this easier). I am sending you well wishes for your appointment at Yale and hope you’ll update on it.

    I admire your ability to look into all this. I tend to be a researcher too, and it isn’t always easy when we’re already going through so much. I hope our efforts, and then speaking out about said efforts, will not only help us in potential subsequent pregnancies, but will also help others, and will possibly even help spark a movement where patients ask more questions of their doctors, forcing them to pay attention. Did you end up confronting your doc about why she never alerted you to the report? I’ll be interested to read that post too. xoxo

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    • Thank you – that’s a really good point. When I’m feeling discouraged and like I just want to bury my head in the sand, I try to remember there are likely others out there who are just as confused and could use the information too. I haven’t talked to my doctor yet because I just haven’t had the emotional energy for it. I’m honestly not sure what I’ll say when I see her again, but it’s why your post about lone wolves resonated so much with me because it seems like she just doesn’t have any desire to learn more about this and I don’t get how a medical professional – especially someone devoted to bringing healthy, live babies into the world – could be that way!

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  3. I hope your meeting @ Yale goes well tomorrow and you get some helpful information. I was told after our baby died that my placenta was “aged” but nothing they wouldn’t expect at 42wks nor should it have caused her death. The preliminary autopsy said it was more like 10% that wasn’t functioning anymore, but still didn’t list that as a cause of death. I am still angry, why allow me to go to 42 weeks, go through all the NST’s and biophysical profiles and tell me everything looked good, if this was even a cause for worry? They tried to scare me about the necessity of doing cervical checks at 36 weeks but never batted an eye about going post term or mentioned any risks involved with that. I know your experience with Luke was different, but I am still interested to see what you find out tomorrow. Thanks for sharing.

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    • Yeah, that seems incomprehensible – I’d be pissed too. I don’t understand why they are so concerned with making us pee in a cup or putting a cuff on our arm but seem to lose all focus on something important like the placenta. I’m trying to keep expectations low for tomorrow as I don’t have much faith in doctors at the moment, but I’ll definitely post an update here.

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  8. Hello,
    I am so glad to have found your post. I have just experienced this with 2 pregnancies (lost at 15 and 17 weeks). I am overwhelmed with the complexity of the information. I really just want to talk to someone who knows something about this and has treated it before. The information on treatment is so little, and I don’t know if I can hope for a live baby or not. I will look into Dr Kliman, thank you for that information. I will continue to follow your posts. Thank you for sharing your experience.

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    • Hi Sadie, I definitely recommend working with Dr. kliman. He was so kind and the first person to give us hope. Have you found the Facebook groups? If you email me (lukesmom0821@gmail.com) I can also send you some info I got from another loss mom. I am so sorry for your devastating losses. It’s so unfair. Thinking of you and your sweet children ❤

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