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Delivery in Marfan

There’s seemingly a dearth of information about pregnancy and delivery in Marfan patients and it’s a subject I get a lot of questions on. I was really lucky to happen to live in a city with an OB who specialized in Marfan and have access to one of the country’s top cardiologists during the latter half of my care (he’s now my regular cardiologist, since we moved to California). So, I’d like to share what I’ve learned from my experience, my doctors, and reading up online in a two part series. You can read the first entry, Pregnancy in Marfan, here.

Please keep in mind that I am not a doctor myself, and (like everything else in Marfan), what works for MOST patients may not be what’s right for YOU. Do your own research and talk with your doctors and experts! The National Marfan Foundation has resources and my OB has expressed a willingness to talk with any pregnant/soon-to-be-pregnant Marf woman. Email me at marfmom@gmail.com for his information or for copies of the papers I cite.

There are different points of view on what the best way for a woman with Marfan to deliver. I’m going to present the benefits and dangers of each, so that you’ll be able to work with your obstetrics team (OB, cardiologist, anesthesiologist) to plan the safest delivery for you and your baby.

Natural (unmedicated, vaginal) delivery: No one recommends this for Marfan patients. When your body is in pain, your blood pressure rises. A rise in blood pressure, particularly one that would be as prolonged as with labor, puts you at risk for dissection. You can dissect even if your aorta is not dilated prior to labor. Your descending aorta seems to be the most at risk for dissecting without prior dilation.

Medicated vaginal delivery (epidural or spinal catheter): My OB, Ohio cardiologist, and California cardiologist all agreed that this is the optimal form of delivery for many women with Marfan (those who do not have an aortic root close to 5 cm and those who have not experienced rapid aortic growth during the pregnancy). Prompt pain medication eliminates pain and therefore decreases the risk for aortic dissection. Recovery time for the mother and child is also greatly reduced.

Traditional c-section: This can post a similar risk to the natural delivery. Any time your body undergoes surgery, especially such a major surgery as a c-section, your blood pressure rises. In addition, Marfan skin does not always heal properly and there could be scarring complications. However, some OBs feel that a c-section is safest because it is in a more “controlled” environment than a vaginal delivery and is much shorter than labor would be. My c-section took 45 minutes; the average first labor is 12-18 hours (of active labor, usually defined as at least 4 cm dilated). And as I stated above, for women with significant aortic dilation or a rapidly growing aorta, c-section is the safest route. It is important to note, however, the several papers have documented the risk for uterine rupture after a c-section. In one paper, Dr. Reed Pyeritz (a member of the NMF’s Professional Advisory Board) wrote that he saw this in 4 of his 11 patients (Pyeritz, 1981). That said, that was also in 1981, long before Loeys-Dietz syndrome (LDS) was discovered. LDS carries with it a risk of uterine rupture and it is possible that some of these patients had LDS, not Marfan, and didn’t know it (my own posturing here).

C-section under general anesthesia: Dural ectasia, to a varying degree of severity, affects 60-70% of people with Marfan syndrome. Dural ectasia is like the equivalent of an aneurysm of the dura sac, the sac of fluid that protects the spinal cord. While it can occur anywhere along the spine, most often it is at the base of the spine, right where the epidural or spinal catheter would be inserted. In patients whose dural ectasia is severe enough, a c-section under general anesthesia might be performed. The reasoning for this is that the needle from the epidural or spinal cath would cause a tear in the dura sac, which would lead to a leak of the spinal fluid. This causes a TERRIBLE headache, which can last up to a month. “Marfriends” of mine who have had a spinal leak say the pain and nausea is only lessened by remaining flat on the back. You can imagine the blood pressure issues that could arise from such a headache, not to mention the postpartum problems, like trying to breastfeed.

Difference between an epidural and a spinal catheter: My understanding is that a spinal catheter has a slightly thinner needle and provides a stronger dose of medication. While a woman using an epidural might feel it begin to wear off towards the end stages of labor, my anesthesiologist told me that a spinal cath would leave a woman numb for quite some time after delivery. I am not sure of the additional risks of using a spinal catheter, although I would think it would make it slightly more difficult to push. I have heard some OBs do not allow their Marfan patients to push at the very end of labor and instead using their hands or a vacuum to assist the delivery.

Postpartum care: There is still the risk of aortic dissection after delivery. Women with Marfan must be closely monitored postpartum. An echo no later than a week after delivery is recommended, and again at one month postpartum. Many beta-blockers are safe for breastfeeding, but not all, so discuss your medication and dosage ahead of time with your OB and cardiologist if you intend to breastfeed. You should know that a prolonged high blood pressure postpartum could signal an aortic dissection, even if you have no pain.

My experience: Because of my dural ectasia, my obstetrics team, my orthopedist at Johns Hopkins and I decided at 37 weeks that it was safest for me to have a c-section under general anesthesia. My blood pressure spiked while I was under, to 170/107 (for comparison, I try to keep it at around 100/60 – 110/70). Even on a host of medications it took 5 days to get me to a systolic of 140 and 8-10 weeks to get down to a systolic of 120. No one knows why this happened. Luckily, I didn’t suffer any permanent damage (and no aortic growth). I wrote more about it here.

Moral of the story? Each method of delivery brings its own set of risks and benefits. Keep an open dialogue with your team (and if your OB isn’t meeting regularly with your cardiologist and the anesthesiologists, insist on it) during your pregnancy to determine what is safest for you.

And again: The opinions offered at Musings of a Marfan Mom are for informational purposes only and are not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of a qualified healthcare provider with any questions you may have regarding Marfan syndrome and any medical condition. Never disregard professional medical advice or delay in seeking care because of something you have read here.

Citations:
Meijboom, L. J., Vos, F. E., Timermans, J., Boers, G. H., Zwinderman, A. H., Mulder, B. J. M. (2005). Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. European Heart Journal, 9, 914-920.

Pyeritz, R. E. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med. 1981;71:784-90.

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Menininho’s Birth Story

Warning: This is going to be a rather lengthy post, because I want to record all the details. It’s a balance how much information to give, but I want other future “Marf Moms” to have an idea of what they might expect when it’s their turn to deliver.

Mark and I reported to the hospital at noon on Feb. 2nd for my scheduled c-section. They were pretty good about getting me back to the prep room right away, so I thought my surgery might actually be on time (hahahaha). It ended up being a lot of hurry up and wait however, with having to justify my c-section to every nurse that came to see me thrown in. The IVs weren’t so bad but oh my goodness no one told me I was going to have to get a catheter while I was awake (sorry if that’s TMI but it’s true and it was awful and it made me briefly wonder if this was all going to be worth it)! Every once in awhile a doctor would pop his or her head in to check on me, but no one could tell me when I’d actually be going into the OR.

I really appreciated my anesthesiologist. Throughout the time we worked together I found him to be very honest and cautious. He came in and explained to me that I would be needing an arterial line (a monitor through one of the arteries in my arm) in order to best monitor my blood pressure during the surgery. I really, really did not want this done and I started to cry a little. I think at this point it was about 3:30 PM and I was supposed to have had the c-section at 2:00. I was hungry and tired and had already been stuck all over from various IVs/the catheter. I wasn’t afraid of the surgery itself, but I think at that point I was just tired of the pain and not looking forward to the pain that was to come.  The anesthesiologist was very kind though, and as he wheeled me away to the OR he gave me a mild sedative through my IV to make getting the arterial line a little easier. One of the nurses also held my hand and talked to me throughout the process to try to distract me (I say process b/c it was a med student doing the line and after 3 tries he’d butchered me so badly that Dr. Small had to put the line in himself on my other arm). Below is a picture of my arms a few days post-op. The bruises are mostly black now and not hurting quite so much.

My memories immediately post-op are a little fuzzy. The way Mark tells it, he was waiting for me in the recovery room when he heard me from down the hall repeating “Ow. Ow. Ow. It huuuuurts. Ow. Ow. Ow. It huuuuurts” in a loud but monotone voice. I kept this up for a little while, then suddenly stopped, looked at him, and asked “What is it?” “It’s a baby boy,” Mark replied. Mark said I got a smile on my face and proudly announced:

“I KNEW it! I WIN!”

And then I promptly returned to my monotone chanting.

Mark and I had to spend the first night apart from Menininho: Mark and me on the telemetry unit and Menininho on the maternity ward. I didn’t find out till the next day, but this was because Menininho and I had both had complications from delivery. Menininho had a low body temperature that they couldn’t regulate and then developed low blood sugar as a result. We weren’t allowed to see him until 10:30 at night and then only for a 15 min. visit so I could try to nurse. With some pleading, I convinced the nurse to bring Menininho back every 3 hours so I could feed him. However, since social visits to other floors are not allowed for babies, the nurse would give Menininho to me, he’d feed, and I’d have to give him right back. Mark didn’t get to hold his son till Tues. afternoon, when we were reunited downstairs on the maternity ward. By then everything was fine with Menininho and he’s still doing really well.  However, I was extremely frustrated by the lack of communication to me or Mark about my condition and the telemetry nurses didn’t allow Menininho on the floor the next morning, so we were separated from about 6 am to 1 pm and I was yelling and panicked.  I wanted to be nursing my baby!

My complications were a little longer lasting. Tuesday morning the anesthesiologist came to meet with me. He told me that during the c-section, my blood pressure had shot up to 177/107 (that’s super high, esp. for me). Unfortunately, the doctors were still not able to get it down, and that’s why I’d had to spend the night on the telemetry unit being monitored. I actually ended up having to spend an extra day and a half in the hospital so the doctors could try to get my blood pressure down to a more manageable level.  The doctors aren’t sure what caused it, but they also refused to investigate.  I was corresponding over the computer with my cardiologist in California, who was pressing for an echo of my aorta and an ultrasound of my kidneys, but the cardiologist on the floor refused (and, I later learned, refused to even call my local cardiologist.  My very nasty message on his answering machine was the first he’d heard I was having problems).

By my 2nd night in the hospital I was on quadruple my normal dose of beta blockers.  I realized that this might not be safe for my son, and I asked my nurse about it.  She called the pharmacist, who responded with (direct quote here): “Why the hell is anyone letting her breastfeed?”  None of my doctors had realized my dosage was toxic through breastmilk and I had to stop breastfeeding immediately.   I was devastated.  However, I am sooo grateful to the nurse I had that night.  I had brought my pump to the hospital and she showed me how to use it.  Every 3 hours she had me pump around the clock to keep up my supply, in case I would be able to breastfeed down the road.  She encouraged me and told me I was not a failure for this momentary setback.  The next morning the cardiologist mocked me in front of his interns for persisting in my desire to breastfeed, so I threw him out of my room (yes, you CAN do that).  I found a replacement medication on my own, had it OKd by my local cardiologist, and was breastfeeding 36 hours later.  Later on I hit one more hurdle when the hospital pediatrician tried to get me to supplement with formula because Menininho had lost 6% of his birth weight, but I knew that it was normal for a baby to lose up to 10% and once I informed her of that, she left me alone.

I’m feeling a lot of emotions right now.  I’m glad to finally be a mother.  I’m tired, too, of course.  But I’m also really upset about how crappily I was treated post-delivery.  I have more medical knowledge than a lot of patients.  Some of those doctors have known me for years.  And still, still I was helpless.  I still have hypertension with no idea why.  I’m hoping to get more information in 2 weeks when I’m in California.

But, we’re VERY glad to be home together now! We’re thankful for those nurses who helped ease the stress of me being sick, and for all of our friends who have provided support/meals/visits/other help this far. 🙂

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A Change in Plans

One lesson I’ve learned from having Marfan syndrome is to expect the unexpected. I also think having Marfan is why I haven’t really been nervous at all about delivering the baby. I’ve spent a lot of time in hospitals and though I’d never call the experiences “fun,” I am comfortable there. I know what to expect: the smells, the sounds, the general flow of the environment.

So, I wasn’t totally taken aback when I found out on Thur. that there was a hitch in our well-thought-out delivery plans (though I was mad). Because I’m a high-risk pregnancy, I have an obstetrics team comprised of my OB, my cardiologist, his nurse practitioner, and 3 anesthesiologists (so whoever is on call when I come in to deliver will be familiar with me). They meet once a month to discuss my case. Well, Thur. was my first time meeting one of the anesthesiologists face to face. I was surprised and angry to learn that he had NO idea I have a rare spinal condition called dural ectasia. It’s not found outside of people with Marfan and Loeys-Dietz syndromes. Really, I have no clue what my team has been doing in those meetings if that didn’t come up, b/c it’s been my #1 concern with the pregnancy as far as complications for me go. In any case, the anesthesiologist told me he and his resident would stay up late researching my condition and talk to my specialist at Hopkins, and that as of now he didn’t feel comfortable doing an epidural or spinal catheter on me because of the potential I’d have for permanent disability due to my dural ectasia.

[Future doctors, take note: to me, this is the mark of a great doctor. Not all doctors can admit when they don’t know something/don’t feel comfortable doing a procedure, but if you can, a patient will trust you so much more.]

After I talked with my Hopkins doctor and my OB, prayed a lot, and my OB consulted with many other doctors, we decided it is best to deviate from my birth plan. I am going to be having a c-section under general anesthesia to deliver Baby Z. The date for that will be set tomorrow at my OB appointment.

I feel really at peace about the decision. In some ways, it’s easier for me to wrap my head around this than going through labor because I know what general anesthesia is like and how I react to it, as well as what the recovery process from surgery tends to be like. I would be lying if I said I wasn’t a little sad though. I think it will be kind of weird to not be “there” when my baby is born (and I assume Mark won’t be allowed in the OR, so he won’t be there either). It will be an hour or two before I can meet him/her. And just so everyone knows, Mark isn’t allowed to call/text/email anyone to announce the birth till I see the baby because I don’t want to be the last one to find out the gender. 🙂 However, in the grand scheme of things an hour or two away from Baby Z is not a big deal and it’s more important that we’re all safe so we can start our new life together as a family of 3!

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Labor and Delivery

So, last night I got a personal tour of Labor and Delivery, only it wasn’t really the kind of tour any mother-to-be wants to have. Instead of having my baby, I was there to make sure I still *had* a baby. (So, my fellow preggo friends, you may not want to read this depending on your hormones today, but I assure you this story has a happy ending).

I don’t have a super active baby. I was lucky and started feeling kicks at 16 weeks, but Lil Z is just not overall a mover and a shaker. However, at this point s/he does have a routine. I feel some kicks first thing in the morning, when I get up and check my emails from the night before. There’s some more kicking just before lunch, and then again when I have my afternoon snack during my 3:30-5:15 class. Then, more at night just before I go to bed, especially if I put the cell phone up to my tummy so Mark can talk to him/her.

Any way, lunch came and went with no movement from Baby. I was a little nervous but my mom told me I was being silly, so I headed off to run errands and to class. However, by 5:00 I still hadn’t felt anything. I had been leaning forwards and backwards, pushing on my belly, even did what I’m not supposed to do and sped-walked to my Wed. night class because that usually gets the baby kicking pretty hard. Nada. At this point I’m really worried. I also can’t get ahold of Mark.

By 5:30, my friends Danielle and Justin convince me that I should page my OB and ask him what to do. I’m also downing M&Ms (our prof. is awesome and brings candy to every class!) in the hopes that a sugar rush will get me just one little kick. My OB calls back and tells me that although no fetal movement for an entire day may not necessarily mean something bad, I need to get myself over to Labor & Delivery right away. Danielle sees me packing up my bag and offers to drive me back across campus so I don’t need to do this alone.

It was kind of weird walking in L&D and not being in labor. The registration process took forever (I hope it’s not this long when I’m actually having the baby) and then I had to wait for a bed. I was really hoping they’d just have me pull my shirt up, squirt on the gel, do the doppler really quick, and send me home.

Actually, they make you get totally undressed. Um? Why is this necessary? In any case, I get undressed and go to put on the robe and surprise! It has no arms. To my preggo friends: They apparently unsnap the arms of all gowns AHEAD of time to make the IVs easier for delivering women. However, it’s impossible to put on a gown with no back and no arms. So, FYI. I’m still trying to figure out how to create sleeves on this thing so I can get dressed when the nurse just walks in. Eeek! But, there’s no modesty in L&D and she’s just like “enh, I’ll dress you.” (I figure I’ve got to take the humor in situations where I can find it, haha).

I get all strapped up to the fetal heart monitor, but the nurse can’t find a heart beat. I don’t really have the right vocabulary to articulate how scary this was (I blame my lack of big words on pregnancy brain). She’s trying to play it cool and says she’ll just get another piece of equipment to try that out. Finally, she finds a strong heartbeat. WHEW!!! However, the baby isn’t kicking, not even with the buzzer thingy. Nurse A leaves, tells me the doctor will be in to talk with me. While she’s gone, I FINALLY feel 2 quick kicks. RELIEF!

Nurse B walks in. I kid you not, she looks like she could be the twin of my least favorite professor ever. I seriously thought it WAS this prof when the nurse walked in. Nurse B wants me to count kicks. I don’t feel anymore till she flips me on my side and starts pressing in diff. places. Maybe it was that, or maybe those M&Ms were finally kicking in, because Lil Z moved 7 times in about 10 minutes. Another sigh of relief. Creepy nurse ignores me and looks very serious as she click clacks on the computer. Doctor comes in, nurse leaves. I really just want to go home at this point.

The doctor begins to tell me that I really shouldn’t feel silly about coming in and having them check, but I should know that when a baby is kicking this much its just fine. What I wanted to say: No duh doc. Do you think I came in for giggles? I didn’t feel stupid until you started acting like I should. What I did say: My baby did not kick all day long. I know my baby’s usual movements. I came in because my doctor told me that’s what I needed to do. After the doc left, I had to sit on the monitor for a while longer because they wanted to make sure the baby’s heart rate was still stable.

Meanwhile, Danielle has been out in the waiting room this entire time. Apparently she called her mom to let her know what was going on, and her mom helpfully told her all these stories about women she knew whose babies stopped kicking and it turned out something was terribly wrong with the baby. So, Danielle was panicking because I was taking so long and she therefore assumed something terrible had happened. 😦

It was great to finally get home, curl up in the rocking chair with some pizza and ice cream with strawberries (hey, I think Baby and me deserved a night of junk food after all that!) and watch Heroes Season 1 with Erin. I passed out early too.

Today things are back to normal. Baby is back on his/her normal schedule. And I won’t feel bad about yesterday. At this point, nobody knows my baby better than me and I’d rather be safe than sorry.

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