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.

Vroom, Vroom

I think I’ve figured out why my blood pressure is still not totally back to normal. I’ve decided at this point it has little to do with my recovering from childbirth and everything to do with driving in California. Today I had to drive Menininho to the pediatrician (about a 25 min. drive) to get him checked for reflux (which he unfortunately has). Over the course of my trip there and back:

# of times I was cut off: 2
# of times I was stuck behind a car going at least 10 mph BELOW the speed limit: 3
# of times I got lost on the way to the doctor: 1
# of times I got lost on the way home from the doctor: 2

*Sigh*

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. 🙂

A Perfect Valentine’s Day

Mark and I have been blessed to have many friends and family members stop by since Menininho was born. Since we have a full day of Cleveland friends tomorrow, we decided to try to celebrate Valentine’s Day today. I think this was the best Valentine’s Day I have ever had 🙂

Mark woke me up at 7:30 with breakfast in bed. Now, this is HUGE. I have always wanted breakfast in bed (I guess b/c when my siblings and I were little we used to do that for our mom for special occasions and so in my mind it became an expression of love). My husband is a much tidier person than I, however, and thinks food in bed is unsanitary. I was really impressed that he was willing to break his rule for me today. However, not only did Mark get me breakfast in bed, but he snuck out of the apartment while I was asleep, drove to Bob Evans, and bought a half dozen of their biscuits (which I love). Awwww! 🙂 As soon as we started to eat though, the baby began to fuss and Mark realized Oliver had somehow climbed into bed with us (no idea how the cat got in through a closed door). In that moment of slight chaos I realized that 1) my life is now forever changed and 2) this is exactly the life I want to be living.

Later that morning, we got the good news at the pediatrician that Menininho has gained enough weight that I can continue to breastfeed. He’s now back up to his birth weight of 7 lbs even. Hooray!

Mark spent most of the afternoon napping. I’m on a host of new medications just between the c-section and my delivery-induced hypertension and they’ve really knocked me on my rear end. I’m so exhausted from them that it’s hard to function, so my dear husband has been up with Menininho a lot at night so I have the energy to care for him during the day while Mark is working. I was grateful Mark could take today off work to sleep! While he slept, my Uncle Alex, Aunt Chris, brother Matt, and his girlfriend Deanna all came to visit Menininho and me.

Menininho took a nap right around dinner time, which meant Mark and I were able to enjoy a romantic meal of Wendy’s and watch 2 episodes of M.A.S.H. Perfect! And, both of us were well-rested enough (as well-rested as 2 new parents can be) to be able to really sit down and enjoy playing with Menininho afterwards.

Today was certainly different from Valentine’s Days in the past, where Mark and I would dress up and go out to Little Italy for dinner. However, I wouldn’t trade today for anything. Sitting on the couch next to my husband, watching him bounce our son in his arms, I was overcome with gratitude. Ever since I was young I’ve wanted to be married and have a family. And, while I am sleep deprived and a little stressed, I am so very, very grateful to be part of an eternal family: married to a man who is good, kind, generous, and always striving to be a better person and now with a beautiful baby boy for us to raise and love.

🙂