Tag Archives: pregnancy

My Monkey Baby

For today’s Mama Kat’s Writers’ Workshop, I’m writing on my strangest dream ever (prompt #2).

I am so glad that I thought to type out my first pregnancy dream the day after I had it. I had forgotten how crazy it was until I re-read it last night. I was about 2 months pregnant at the time and preparing for my 5 year high school reunion. So here goes:

It was late at night and I was standing in a parking lot, outside of a small car. I had just given birth in that car, at the same time as another Latina woman. She’d had quadruplet boys and I’d had a girl. I entered the car to bring my daughter home, but the other woman tried to tell me the baby was hers. She had even named the girl Attila.

So, this baby girl was huge…like at least 10 lbs huge. The weird thing? I’d given birth at 4 months. All I could think was “darn, I never even got to show!”

Somehow I get my daughter from this other woman and her quadruplets, and my baby and I are just walking around. She is the PERFECT baby. She was very smiley and quiet. However, she was totally naked, just having been birthed and all, and we had no clothes for her. Mark bought her some bright yellow diapers.

Then, I decided to take Attila to my high school reunion. As I was showing her to various classmates, I noticed she was growing more and more hair.

She turned into a Pygmy Marmoset!!! An evil one! One who jumped out of my arms and bit my classmates on the head!

from http://www.aqua.org/animals_ pygmymarmoset.html

And I was like “CRAP! What am I going to do with a Marmoset baby?!?! Is her tail going to shrink? Will her fangs go away?”

My mother told me “that’s what happens to premature babies,” and that I couldn’t breastfeed anymore or she’d bite me.

What was YOUR strangest dream?

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Snapshot

Today I’m writing on prompt #2 for Mama Kat’s Writer’s Workshop: If you could have given yourself a snapshot 5 years ago of what your life is like now, what would the picture be of and how do you think you would have felt about it?

I thought about this for a while and finally chose this picture, which I took a couple of nights ago. Menininho is going to town on some beef and beans, totally grossing out my mom and Mark, and cracking me up.

Five years ago I wasn’t sure I’d ever have a biological baby. I desperately wanted to experience pregnancy (you know, some day after I was married and all that; not as a sophomore at college) and it was just too early to tell if that would be able to happen for me. I worried about it a lot, increasingly so as the years went by…right up until I was able to hold Menininho for the first time, when he was REAL.

So, it would have alleviated a lot of anxiety to be able to see this snapshot and know that things would turn out ok, that I’d have a spunky, independent little boy with a stubborn streak as long as I am tall.

Menininho’s learning so much now it blows my mind. His new thing is “reading” books. He’ll sit in the book corner we’ve constructed for him (Mark installed a small bookshelf next to the kitchen table, full of board books) and just open and close books forever. Today he came when my mother called him, and tonight he tried to crawl out the door after Mark and I when we left to see a movie. Then he cried when he realized he wasn’t coming with us. He also knows what will get him into trouble. For instance, he’ll sneak over to grab the TV remote and then try to crawl away fast as soon as he’s caught. I can’t believe how quickly my little baby is growing up, and I can’t wait to see what happens five years from now!

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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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The Body is BACK (mostly)!

Feb. 2nd, 2009

Jan. 5th, 2010

It took almost a year, but yesterday morning I stepped on the bathroom scale and low and behold! I’m at my pre-pregnancy weight! My body’s not *quite* the same…I’m a little curvier than before I got pregnant so I don’t fill into all my old pants (and alright…I *might* be sucking in the c-section belly a bit in the first shot!) but it feels great to be back where I was weight-wise. My first #mamafit goal is complete! 🙂

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Genetic Testing for Marfan Syndrome

Today’s post comes from a question from Tom:
How did you ensure you didn’t pass Marfan on to your child? Any good links/resources on that process?

This is a great question! First, I’ll briefly explain how Marfan works.

Marfan is what’s called an autosomal dominant disorder. This means that it is a single mutation that causes Marfan and if you have the mutation, you have Marfan, at least to some degree (there are mild cases to severe cases). You can’t be a carrier, like you could with something like cystic fibrosis. This also means that children have a 50/50 chance of inheriting Marfan from the affected parent. Marfan happens equally among men and women and across all races.

If you decide to have a baby the old fashioned way, there is no way to guarantee that your child won’t have Marfan. This is the option my husband and I decided on. It was just luck that Menininho doesn’t have Marfan. If we have another biological baby, s/he has the same 50% chance of having Marfan that he did.

If you want to try to be sure that your biological child doesn’t inherit Marfan, genetic testing may be an option. First, the affected parent must be tested. 90-95% of people who meet the clinical criteria for Marfan test are able to have their mutation identified. A negative test result doesn’t necessarily mean that you don’t have Marfan (although it could mean you have a related disorder instead, like Loeys-Dietz syndrome, MASS Phenotype, or a form of Ehlers-Danlos syndrome). Dr. Dietz, one of the world’s pre-eminent Marfan researchers, explained it to the teens at conference like this. We know that Marfan is caused by a change to FBN-1 (fibrillin). Right now, we know how to test FBN-1 itself. However, there are all kinds of proteins that may create changes to FBN-1 that we haven’t yet identified, and the 5-10% who clearly meet clinical criteria probably have mutations that just haven’t been discovered yet. Confusing, I know.

[Moral of the story: rely more on clinical criteria for a diagnosis and save genetic testing for 1) pregnancy issues and 2) when it’s hard to get a clear diagnosis .]

Aaaanway. So, you want the genetic test to locate your mutation. There are a few different labs that do the test, but I hear that all labs are not created equal. Tulane University’s Matrix DNA Diagnostics lab was the first in the country to do the FBN-1 test and is spoken highly of. I had my test done there 4 years ago. Click here to download their FBN-1 testing information. Many insurance companies are now covering this test, but if not, the initial test will cost $1400 (from Tulane). You can have your blood drawn anywhere and shipped overnight to Tulane. If you live in the US, it will take 5-6 weeks for you to get your results.

Once your mutation has been identified, you and your partner will need to find a center that does pre-implantation diagnosis in-vitro fertilization. Basically, the doctors will create embryos in the Petri dish and test them for the mutation that you have. [Each family shares the same mutation but manifestations of that mutation vary, meaning some family members might be mildly affected and others more severely affected.] If you’ve produced an embryo that doesn’t have Marfan (which is not a guarantee), one will be implanted in the uterus and if the implementation works, your child shouldn’t have Marfan.

If you’ve gone the old-fashioned route, you can still find out right away if your child has Marfan (providing you had the genetic test). You can test the baby in utero using amniocentesis, or you can wait till he’s born. We used Menininho’s cord blood and had the sample shipped to Tulane the same day he was born (needed to be the same lab where my test was done). When doing testing on other family members like this, the test is only $350 and results can be had in 2-3 weeks.

If you do not do genetic testing, it may take much longer to find out if your children are affected. It is my understanding that the aorta is not stable until 3 years of age so it may not be useful to do an echo prior to this point. A slip lamp eye exam should be done around 18 months of age so that any potential vision problems can be addressed so as not to interfere with physical development, like walking. Because Marfan is progressive, it may take years to know if children are affected. Routine echoes and eye exams are recommended to catch any problems early, since preventative medicine is best.

OK, I think that covers the bulk of it. I’m sure there are things I’m forgetting. Always feel free to post questions on this or other questions and I’ll help you find the answers! Further information on genetic testing and labs that will do the FBN-1 test can be found via the National Marfan Foundation. Amy Kaplan is their nurse and you can contact her at akaplan@marfan.org.

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.

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Pregnancy 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. This week is Pregnancy in Marfan and next Monday come back for Delivery in Marfan.

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.

Contrary to the information doctors had a generation ago, pregnancy is ok for many women with Marfan. For women with an aortic root under 4.0 cm, they are at the least amount of risk of aortic dissection (just slightly above the risk for what it would be if they weren’t pregnant). For women between 4.0 cm – 4.49 cm pregnancy is still ok, just with an increased risk. Pregnancy is not recommended for women with an aortic root of 4.5 cm or above unless valve sparing surgery is done first (Meijboom, et. all, 2005). I can imagine that pregnancy would also not be recommended right away for someone with a rapidly growing aorta. I have not found measurements for other parts of the aorta. There is also the risk of descending aortic dissection without a pre-existing dilation of the aorta. I found out about this after I delivered Menininho and plan to ask my doctor for more information when I see him next month.

Medication should be continued during pregnancy to help prevent growth of the aorta (Elkayam, Ostrzega, Shotan, & Mehra, 1995). Not all medications are safe during pregnancy. For instance, Losartan/Cozaar is not recommended during pregnancy, particularly the first trimester, so you should talk with your doctor about moving to another medication before trying to conceive. Talk with your doctor about your particular medication. My OB monitored my baby’s heart rate at every prenatal visit because of a couple of case studies that mentioned bradycardia (slow heart rate) as a possible side effect for babies whose mothers were taking beta blockers (which I was taking at the time). However, my team also told me that since there were only two case studies it’s possible the bradycardia was not related to the medications at all.

Aortic monitoring throughout pregnancy is vital. It should be done at least every trimester. This way potential problems can be caught early and your safest delivery can be planned. An echocardiogram is the least invasive measure, although MRIs are also considered safe. CT scans are not typically used during pregnancy because the risks posed to the baby from the radiation.

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:
Elkayam, U., Ostrzega, E., Shotan, A., & Mehra, A. (1995). Cardiovascular problems in pregnant women with the Marfan syndrome. Annals of Internal Medicine, 2, 117-122.

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.

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Tips ‘N’ Tricks: RG Maternity

Starting when I began to wear maternity clothes, I’ve been keeping a list of items and tricks that have been really helpful to me as a mother with a chronic health condition, although some of these are certainly applicable to all moms. From time to time I’ll write reviews on the items (tips) or offer up suggestions of ways I’ve adjusted doing things (tricks) to make being a mom easier on my body.

All of the products are ones I purchased myself, unless otherwise specified.

Having a 37” inseam and trying to find reasonably priced pants is not an easy task, but it’s particularly hard when you’re pregnant. I don’t know why, but most of the stores I usually shop only carry 34” maternity inseams, as if tall women don’t ever get pregnant. Go figure. So, I was very excited when I came across RG Maternity.

Being a family-owned store, the selection is small but changes every few months and the deals can’t be beat. I purchased a pair of dark bootcut jeans with a versatile panel, gray dress pants with a full panel, and a red rouched top. Having a disproportionately small upper torso, I welcomed the long shirt because it gave me the illusion of being more equally proportioned. I also personally prefer the full belly panel. I carried high and the versatile panel kept slipping later in my pregnancy, but the full panel fit smoothly and kept my pants up well.

RG’s customer service is excellent! When I emailed questions I got a response right away, and was emailed when new items became available. Shipping only takes 1-2 days. The only thing that complicates returns is that you must email the company for authorization for a return before mailing back the item, and you have two weeks to do it. I cut it reeeaaalllly close when trying to exchange my dress pants for a different size. I’m a wee bit of a procrastinator.

Overall, great small business and if I ever have another baby I’d use them again!

Below is a picture of me wearing the gray dress pants.
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