Musings of a Marfan Mom

December 14, 2010
by marfmom
1 Comment

Abbey’s Birth Story

Awhile back I invited readers to submit their birth stories, to be posted while I take a maternity leave. Since Baby J is here, it’s time to post them! Some of the women have Marfan syndrome like me, but not all. If you have questions for the authors, feel free to leave them in the comments section; I’ll also try to email them any questions I see (as soon as I have time).

This is Abbey’s story. You can find her at The Naptime Report.

When I was in 5th grade, I was diagnosed with scoliosis. I can remember complaining of back pain, but the first ‘red flag’ came during one of those routine the-school-nurse-will-check-your-back-in-the-locker-room things in elementary school. My parents immediately took me to our family doctor, who referred me to my orthopedic specialist.

He diagnosed me with a 32-degree s-curve. I clearly remember two things from that first appointment. One, I finally knew why I wasn’t flexible like the rest of my friends. I’d spent years being embarrassed that I couldn’t complete the ‘sit and reach’ portion of the Presidential Fitness Test, no matter how much I stretched at home. Two, I remember Dr. B telling me that if I ever chose to have a baby, I shouldn’t get an epidural. He said the curve of my spine versus the location of my spinal cord could lead to paralysis if it wasn’t done correctly. Even in the 5th grade, I knew I would be a mother someday.

I wasn’t given any real restrictions, and for the most part, lived my life just like any other child. I learned to water-ski and snowboard, and I enjoyed horseback riding. I even learned to do round-off back handsprings on a gym floor. Most of these are activities that many people with scoliosis don’t get the chance to enjoy. My doctor wanted to fit me for a daytime brace, but I opted for the nighttime brace instead. I wore that for about 5 years, and that was essentially the end of my regular treatment.

After I graduated from college, I took up running as a hobby. This caused me to have pretty regular lower-back pain, but it was manageable, and the enjoyment from running far outweighed the pain. In 2007, I found out I was pregnant. That pregnancy, and the one after, was an ectopic pregnancy. Though the doctor didn’t know why, and half of my reproductive system was removed, she was positive that we would be able to have a healthy pregnancy. Just a few months later, we found out I was pregnant again (three times in one year!), and this one was in the uterus.

Aside from harmless (yet heavy) bleeding from time-to-time, this pregnancy was low-risk and normal. The most fantastic part was that the counterbalance of my baby against my back completely erased my lower back pain from about 5 months on. We’d find out later that the pregnancy would cause my s-curve to completely correct itself (meaning, each curve is exactly 16 degrees….something my experienced doctor has never seen).

Per my doctor’s orders in 1995, I planned to have this baby au natural. I don’t think I would have planned for that type of birth otherwise, but I felt it was best for my body. My OB was completely supportive (she’s a fantastic, fantastic doctor) and gave me ideas and strategies. I got a doula and prepared via hypnobirthing. I also have severe endometriosis, and I’d been using a form of self-hypnosis to deal with the pain from that for several years.

At 38 weeks, my doctor encouraged me to be induced based on the pitted edema I’d had for 4 months. I was generally uninformed, sick of being pregnant, and completely trusted my doctor, so I agreed. While I don’t think it was a bad choice, I think being induced made my natural birth next to impossible.

I was given pitocin starting at 8:00am. I labored pretty easily until about noon, when my doctor broke my water. I had limited mobility, and was walking around as much as possible. A few hours after that, the pain was more intense than I’d imagined. My doula was fantastic, but my hopes were dashed when I was checked after 15 hours of active and painful contractions, expecting to be at an 8 or 9, and I was at a 3.

At some point, probably about 6 hours into it, the baby’s heart rate was erratic. My doctor toyed with the idea of a c-section, but I begged for another option. They inserted an internal fetal monitor (a screw in the baby’s head), which was less than ideal, but better than surgery. While I was avoiding the OR, I could no longer get out of bed. I was happy to know my baby was doing okay, but it was awful to be bed-ridden.

After I realized I hadn’t progressed past a 3, I asked my husband to go out to the car for the films of my back. I’d brought them along, just in case. I asked the nurse for some Nubain, and then almost immediately wanted an epidural. I (hopefully politely) demanded the chief anesthesiologist to be the one who reviewed my films. He wasn’t sure it would work, but agreed to try. He assured me the risk of paralysis was low, and I knew it was the only choice I had left. My mind wasn’t going to let my body go any further.

Thankfully, the epidural was administered well, and it worked like a charm. I was comfortable and relaxed. About 45 minutes later, I asked the nurse to check me again. She reluctantly agreed, but let me know that she wasn’t going to check me very often. She explained that it can be frustrating if I’m not progressing and it also puts me at a greater risk for infection.

As she was checking my dilation, she got a strange look on her face, and called for another nurse. The second nurse confirmed what seemed impossible: I was at a 10, and ready to push. The next few minutes were a flurry of activity as they prepped the room for delivery. Everyone, myself included, thought we’d be there all night. My doctor got there in just enough time to deliver my daughter, who was born just an hour after the epidural was administered.

When she was born, my OB guided her from the birth canal to my belly, where she crawled up and started nursing. I didn’t believe it until I saw it happening. I was nursing her within minutes of her birth. I think that has everything to do with why she was such a champ at nursing.

I made the nurses wipe her off and check her breathing and heart rate from my arms. I refused to let them weigh or bathe her until 2 hours later. I did not see an immediate need. Also, for one hour after birth, no one came in our room. We hung out and practiced nursing and Nic and I introduced ourselves to our baby girl before our parents knocked down the door. I highly recommend this.

While the birth didn’t go as planned, I was able to have a natural-ish birth amidst the technology and ‘routine practices’ present today. Despite the drugs, IVs, and fetal monitors, I was able to have the birthing experience that I’d desired. About two hours after my daughter was born, the epidural wore off and I regained feeling in my legs. While I’d trusted the anesthesiologist, both he and I were beyond thrilled when I was able to walk to the bathroom.

I don’t know what my birth plan is yet for this baby (I’m due in June), but I do know that I won’t compromise on those first hours of bonding with my child. To me, that was the most important part of our story.

December 13, 2010
by marfmom
11 Comments

He’s Here!

I’m rather late on this, but the Menininho wanted to let you all know that his little brother Smudge, aka “Baby J” has arrived! J was born on Dec. 4th, weighed 6 lbs 15 ounces and was 20 1/4″ long.
Baby J’s birth story will be published at then end of the Women With Disabilities and Birth series.

December 9, 2010
by marfmom
2 Comments

Jennifer’s Birth Story #2

Awhile back I invited readers to submit their birth stories, to be posted while I take a maternity leave. Since Baby J is here, it’s time to post them! Some of the women have Marfan syndrome like me, but not all. If you have questions for the authors, feel free to leave them in the comments section; I’ll also try to email them any questions I see (as soon as I have time).

Jennifer Levesque, 38
Diagnosed with Marfan at age 12 -inherited from father
Mother of two
Methuen, MA

In May of 2005 I became pregnant with my 2nd child. Luckily, I wasn’t as sick as my first pregnancy. I went to the cardiologist, had echoes and everything was fine.

When it came time to give birth, again I had a good experience. Things went a lot quicker. This time my water broke on its own. My husband, daughter and I were at a Barnes and Noble bookstore when it happened. I thought I had peed my pants. I waddled into the bathroom and realized my water had broke. We rushed home, put our stuff in the car, called the grandparents to pick up our daughter at the hospital and then left for the hospital.

I was in labor for about 7 hours before James Thomas Levesque was born. I had another epidural, which went fine. I only had to push about 5 times before he came out.

When we left the hospital I knew I had post-partum depression. I did not feel happy to have another baby to take care of. I had forgotten how hard it was. My breasts were so sore from breast feeding and I was so tired. I felt guilty that I felt this way.

January 12, 2006 was a normal day. James was 10 days old. I went to the ob/gyn in the morning because I thought I had mastitis. I had it with Meghan. It turns out I didn’t have it. I was relieved. In the afternoon we all took a nap. I woke up before the kids. I felt so much better. I even put on my favorite jeans (they fit!) and some make-up. I told myself that I would be okay. When my husband came home from work we decided to go out to dinner. It was our first time out together as a family. After dinner we went to Kohl’s to look around. While my daughter was looking at toys, I had a really sharp pain in my abdomen. It hurt a lot and lasted for a few minutes. I just had a baby so I attributed it to that.

My husband was worried about my pain, so we went home. The pain subsided, but I was exhausted. I had never felt so tired before. I took a shower and put on my pjs. When I was walking down the stairs my husband asked me if I was okay. I said I was fine. I sat on the couch and fed my son. After he was done I was holding him and then I felt a very sharp pain in my back. It felt like someone was stabbing me. I put the baby in his bouncy seat. I asked my husband to get me Tylenol, but I knew it wouldn’t work. By the time Randy came back with the Tylenol, I was screaming. I didn’t know what was happening to me. Randy called 911. I was so scared. My 3 ½ year old daughter was crying. I felt a ripping feeling down my chest. I knew this was bad!

I was taken to a local hospital, the same hospital where my son was born. The nurses and doctor worked quickly. Everyone was aware that I had Marfan Syndrome. I had a CT scan within an hour of being in the hospital. The pain was excruciating. After the CT scan the doctor came in the room and told my husband and me that I had an aortic dissection. I had heard those words from my cardiologist before. I knew that was bad, but I didn’t totally understand what that meant. The ER doctor explained what had happened and that I would be med flighted to Mass. General. I was in shock. How could this happen to me?

At Mass. General it was confirmed that I had a type B dissection. I would not have to have surgery. I was relieved. I felt like if I had to be cut open, then I would definitely die. I spent 5 days in ICU. I was on morphine and I felt like I would have to be on it for the rest of my life. On the fifth day in the hospital, the pain started going away. I got to get out of bed and practice walking. I was starting to feel human again. I spent 3 more days on a step down unit and then my husband got to take me home.

I knew that my life had changed. I now had to take medication and I was so afraid that my aorta would suddenly rupture, but I was so happy to be back at home reunited with my family.

It has been over 4 years since my dissection. My descending aorta is growing and I am getting closer to surgery. Some days have been difficult, but ultimately I have realized how precious life is and how wonderful it is to see the smiles of my children’s faces everyday.

During those 4 years I also experienced organ prolapse. My bladder, vaginal wall and uterus literally fell down. I have had 2 surgeries, including a hysterectomy, to repair the problem. I had no idea this could happen. It makes sense that a person with Marfan could have this problem since everything is held up by connective tissue.

I don’t want other women with Marfan or other connective tissue disorders to be scared by my story. I want other women to be informed. I never knew that something could happen after the pregnancy. I also did not realize that a dissection could occur without dilation of the aorta. My entire aorta was within normal limits on the day of my dissection. I think it is important for women to know that every pregnancy is a risk and to think it through thoroughly. I wouldn’t change a thing about my decisions, but I do wish I had known more.