Today, I’m sporting red pants and red lips for heart health awareness. Most of the attention to heart health this month is on women and heart disease, which is a worthy cause. But this week is also Congenital Heart Defect Awareness Week, though it’s kind of going under the radar. I mean, I have a CHD and I didn’t even know it was my awareness week until a friend told me on Twitter.
Which is really just about perfect, because I didn’t know I have a congenital heart defect until I almost died. The cliff notes version is, my twin pregnancy was very hard on my body. By the end, I developed pre-eclampsia, which is not uncommon for twin pregnancies. I had a cesarean section (due to the pre-e and other factors, like my daughter’s spina bifida, and the fact that both of my twins were breech), but my blood pressure was slow to stabilize, and my counts were low. I received blood transfusions. And on day 3 post-delivery, I crashed, ended up intubated in the ICU, and was in congestive heart failure. What happened to me is known as peri-partum cardiomyopathy, but later, a cardiac MRI revealed the root cause of all of my problems: a congenital heart defect known as left ventricle noncompaction syndrome.
This means that the muscle fibers in the left side of my heart didn’t lay down properly when I was developing in the womb, so the part of my heart that is supposed to pump blood out into my body is weak, and doesn’t pump the blood very effectively. I’ve had it all my life, but my body used a fast heart rate and higher blood pressure to compensate, and so while I’ve never been very good at things requiring stamina and cardiovascular endurance (let’s be real, I have the constitution of a fainting Victorian lady), I never had enough symptoms to know something was wrong until the stress my twin pregnancy put on my body.
This makes me something of a unicorn. My cardiologists are always telling me that no one studies this condition, and it’s super rare, and they’re just sorta trying stuff on me. It’s kind of scary. I don’t really know how the course of this goes, or what to expect, or what my long-term prognosis is. I know that it’s a good thing that the medications I’m on have gotten me back up to a “normal” level of cardiac function. I take a beta blocker and an ace inhibitor. They make me very tired, as if having a weak heart didn’t already make me tired. Last week, my doctor let me try taking less of the beta blocker, though, and I already feel like I have more energy.
So, why am I telling you this? I’m not entirely sure. I guess I don’t like being a unicorn. It’s also a strange thing to effectively have an invisible, possibly life-threatening, disability. I look fine. I shouldn’t be tired all the time. I shouldn’t get so winded on the stairs. But I do.
Looking back, I probably should have discovered this sooner. I was always easily tired and intolerant of exercise. I had slow weight gain. I required (still do) a lot of sleep. I experienced strange feelings in my chest. So, in the name of awareness, here are some signs you should watch for in infants and children that might be worth a referral to a pediatric cardiologist:
Parents should be alert to the following symptoms in infancy:
Tires easily during feeding (i.e. falls asleep before feeding finishes
Sweating around the head, especially during feeding
Fast breathing when at rest or sleeping
Pale or bluish skin color
Poor weight gain
Sleeps a lot – not playful or curious for any length of time
Puffy face, hands, and/or feet
Often irritable, difficult to console
Some children with CHDs may not have any symptoms until later in childhood. Things to look for include:
Gets out of breath during play
Difficulty “keeping up” with playmates
Tires easily/sleeps a lot
Change in color during active play or sports (looks pale or has a bluish tint around mouth and nose)
Frequent colds and respiratory illnesses
Slow growth and weight gain/poor appetite
Complains of chest pain and/or heart pounding
So, this month, do think about things you can do to make your heart healthy, like eating healthy foods and exercising and not smoking. But also think about little signs that might point to underlying problems, and get yourself or your child checked out if you have concerns.
On this day in history, Jon and I went to my first prenatal appointment. 8 weeks pregnant, and so excited to hear our baby’s heartbeat and get the official confirmation that we were indeed having a baby.
Because the doctor was a friend, she gave us a quick ultrasound so we could peek at the baby. She jiggled the wand a bit and showed us a little blob inside a bigger blob on the screen. “There’s your baby,” she said. We heard the thump thump thump of the heartbeat. We watched the blob for a bit, tears in our eyes, and then she jiggled the wand again and said the words that I will never forget:
“And now we’re going to take a look over here at baby number two.”
I said: “SAY WHAT?”
And sure enough, there was another little blob inside a bigger blob, thump thump thumping away.
My sweet husband, who had suspected he saw another blob before the doctor showed me, well, his first words on the subject were, “You’re going to get soooooo big,” squeezing my hand.
I just kept saying “WHAT?!”
Twins had not even been on my radar. It never even occurred to me to think or fear the possibility. On the way out of the doc’s office, we started calling friends and family, and to a person, they all thought we were joking. I kinda felt like the universe was joking.
To anyone newly pregnant with twins out there, let me tell you, the shock is normal. I think we just kept saying “Holy shit,” to each other for a couple of months. And let me also say, it’s normal, I’ve learned, to have many complicated feelings about the whole twins thing. I really think I had to mourn the loss of a normal pregnancy, of the images I had in my mind of one newborn and a lot of snuggling and gazing into each other’s eyes. I only ever typed or thought of the word as “TWINS?!” for months. And some days I still can’t believe that I have two babies.
Mostly, though, I can’t imagine not having twins. There has never been one without the other, from the minute I saw them on that ultrasound screen. There is no Claire without Etta and no Etta without Claire. While I can honestly say that there is much of the exhausting early days I barely remember because of the mind-numbing sleepless HARD of it all, this twin gig keeps getting better. They ask for each other. They wake up talking to each other in the mornings. They tickle each other and hug each other and kiss each other, and yes, bop each other on the heads and steal each other’s toys and pull each other’s hair.
It’s been a crazy ride from blobs to baby buddies, but it’s also been a beautiful one. I wouldn’t have it any other way.
Some folks may be wondering, since my last post over a week and a half ago was OMG ONE DAY TIL BABIES LET’S FINISH THE NURSERY, whether or not I did in fact have those babies.
And what a crazy story it is.
Things started out as planned– we didn’t sleep at all the night before from excitement, and we showed up at the hospital at 7:00 am on Wednesday, March 28 (exactly one month before my 40 week due date) for a C-section that had been moved up 5 days because I suddenly had an elevated blood pressure and had started retaining fluid. I was really nervous about the surgery, but despite a little trouble getting the spinal/epidural combo in place (apparently folks with scoliosis and an extra vertebra are a bit challenging for anesthesiologists), the whole operation went very smoothly. It’s super surreal to be lying on a table, numb from the waist down, knowing you’re being cut open and having babies pulled out, but feeling nothing but pressure and tugs and then suddenly hearing cries. I’m pretty sure I was saying “I’m afraid I’m going to feel it!” to the anesthesiologist who was standing next to me as I was already being cut open.
It turns out my girls, who had been in “bunk beds” or in the transverse position for my entire pregnancy, had turned breech somewhere between my last appointment and the surgery– so I’d have definitely needed a C-section even without my blood pressure issue or Claire having spina bifida. Jon joked afterward that there are about 5 ways I could have died in childbirth this week if not for modern medicine– breech twins are one of them. Jokes aside, there is no one more supportive and awesome than my husband, who was right by my side for the whole surgery. The surgical team kept telling him when to pull out his camera for a good photo op, but he mostly focused on supporting me and keeping me calm. He did have the anesthesiologist give me some “goofy juice” at the end when I started to freak out a bit that I might be feeling more than just pressure (pretty sure that was mental), but overall, he kept me relaxed.
After both girls were pulled out, Etta weighing 6.2 lbs. and Claire weighing 6 lbs. (I am SO FREAKING PROUD of those weights for 35 weeker twins, I must say), Jon went with the babies to the resuscitation area, which sounds scary, but doesn’t necessarily mean the babies are getting like, CPR or anything. They were checked out by a team of neonatologists and pediatricians, and there was a transport team standing by ready to take Claire from the university hospital where I was delivering to the nearby Children’s Hospital (where my husband works), where she would be having surgery to repair the opening on her spine caused by her spina bifida.
It turned out that Claire’s spinal defect was both higher and larger than we initially hoped. I got to see my baby girl in a plastic transport box for about 5 minutes in recovery before she was whisked away by the transport team. I’m pretty sure the “goofy juice” is what helped me not completely freak out over not getting to hold one of my babies, but we had also prepared ourselves for the fact that this is what would happen on delivery day, knowing it would be happening since week 20 of the pregnancy. I was also very thankful to have met and consulted with the team of doctors who would be taking care of her, so I knew she was in the very best of hands.
After recovery, Etta and I were moved to the peri-partum ward, and she was allowed to share a room with me. My blood pressure and heart rate were slow to go down, and labs revealed my blood counts were very low, so on Thursday I received a blood transfusion. Everyone who came in the room and saw me afterward remarked on how much better I looked, and the color in my face, even though I was about the same shade as the beige hospital blanket that covered me. I guess I was white as the sheets before?
On Friday, Claire had her surgery, a five hour process involving neuro- and plastic surgeons to cover the 4 cm. area where you could literally see her spine. I wished so desperately that I could be there for her, but was still not in shape to be discharged from the hospital, so a social worker set up a video-conference and I got to video chat with her before the operation. She opened her eyes when she heard my voice, which made me feel so much better that she still knew her mama loved her. She came through the surgery great, and continues to recover in the NICU at Children’s. She seems to move her legs of her own accord and also to respond to stimuli, which are great signs that she may not have disability in her legs. As with everything, though, we will just have to wait and see. She may also have disability in her bladder and bowel functions, but since no babies have bladder and bowel control at this point, we won’t know until she’s older if this is the case. We certainly hope it isn’t.
On Saturday, there was talk of discharging me from the hospital as I was doing much better, but I was still feeling very very weak and not very able to get out of bed, and when my doctor suggested keeping me another day, I said I thought that was a good idea. I needed the time to continue to recuperate.
By early Sunday morning, I was feeling well enough to get out of bed and walk unassisted the 5 steps to the bathroom, as Jon was holding Etta. I remember feeling so proud as I stood up and walked unassisted, thinking this was finally the milestone I needed to get to go home. As I walked back to the bed, I felt myself getting shorter and shorter of breath. I told Jon I was having trouble breathing, and he called for the nurse. It just got worse and worse, as I struggled for breath in short gasps, and a crowd of nurses gathered around. People were telling me to take a deep breath, but short gasps were all I could manage. I began to hear crackling in my lungs (Jon later explained that this was flash pulmonary edema), and I became more and more panicked. I knew from the way I felt and the look on Jon’s face that I was crashing.
The Rapid Response team arrived and soon I was being prodded and various breathing masks were being shoved onto my face, which only increased my feeling of panic. I know enough from being raised by medical professionals and married to an emergency physician that I needed to be intubated. I was looking at Jon begging him with my eyes to get someone to sedate and intubate me so I wouldn’t have to panic and struggle to breathe any more. From the look on his face, I could tell if he’d had a cart nearby, he would have done it himself. I truly believed I was dying, lying in that bed, surrounded by people bustling around to save me, with my husband watching and my baby girl lying next to me in a bassinet.
And then I don’t remember anything.
My next memory is vague, like a dream, where you know there is something you MUST do, and in this dream, what I had to do was pull something out of my mouth. So I just kept pulling.
It was Monday morning, and I had extubated myself. I was in the ICU, where I had spent about 24 hours on a ventilator.
After consults with cardiologists and pulmonologists and internal medicine specialists and more doctors than I can count, it seems I have developed peri-partum cardiomyopathy, which was causing congestive heart failure. My terribly lay understanding is that possibly due to the stress of the pregnancy on my body, I either developed or finally exhibited a pre-existing weakness in the muscle of my heart, specifically in the last chamber (left ventricle) that sends blood out into the body. The ability of this chamber to pump out blood effectively is measured in an “ejection fraction.” Most people’s is 50% or better, and mine was 15%.
Putting me on the ventilator helped them pump fluid out of my body to take the load off my heart and lungs. They removed 7 liters of fluid from my body in my 24 hours in the ICU, and continued to take more off through diuretics and fluid restrictions after they got me out of the unit and back in the peri-partum ward.
Tuesday through Thursday were spent trying to fine-tune the cocktail of medicines I am now on to control my heart rate and blood pressure and allow my heart to strengthen and heal. Every day they tweaked the meds a bit, and every day I worked harder to get out of bed and walk and move. By Wednesday night, I finally was able to make half a lap around the ward, pushing Etta in a bassinet, Jon beside me to steady me, with nurses cheering– even those who hadn’t cared for me had heard about the girl who was seemingly ready to go home and had suddenly crashed and wound up in the ICU on a vent, so they were proud to see me looking better.
At first, the medicines made me feel worse, which my cardiologist told me would happen. I’d get my meds, and then for 4 hours afterward, feel hot flashes and jitters and general exhaustion, only able to sweat and sleep. But every day that got a little better. By Thursday, Jon and me, and Etta, if I may speak for her, were just DONE being in the hospital. Convinced that I would be compliant with my meds, diligent in home blood pressure checks, and sure to come back for follow-up treatment, I was finally discharged. I have never been so glad to breathe fresh air as when baby Etta and I were wheeled out to the curb in front of the hospital on that beautiful spring day when we finally got to go home. I’m feeling better and better since then. My heart condition may never be fully cured, but it can get a LOT better, and I have high hopes of being one of the successful cases. We will not be able to have any future pregnancies, however, because it would literally mean risking my life.
Another loss that I feel very deeply is the loss of the ability to breastfeed the babies. I had really hoped to be able to nurse them, and had been working on nursing Etta and pumping and building up my supply while in the hospital, however, after the whole almost-dying thing, my doctors convinced me that breastfeeding is a major physical strain on my body, and I needed to be conserving my physical resources to give my heart the best chance to heal, so that I could become strong enough to care for my girls. I have cried many tears over this loss, but I know that we made the right choice for our family. They need my beating heart a lot more than they need breast milk.
We’re still missing 1/4 of our family, with Claire still in the NICU, and we are anxious to get her home. I’m still pretty weak, though stronger every day, and my one outing a day is to go visit her. Blessedly, Children’s has a special “twin rule” that lets us bring Etta with us to visit her sister, and it’s super special just to have the four of us together for a little bit each day– giving us a taste of how great it will be to finally have all of us home. We don’t have a specific date that she’ll be discharged, but we hope it could be soon if she keeps meeting the goals her doctors set for her. Every day that I’m wheeled out of that NICU with only one baby feels incomplete.
Overall, though, I am so very thankful. I’m glad I was in the hospital where I could quickly get the care I needed when I crashed. I’m glad Claire has such awesome people taking care of her, and glad we have awesome families who would come and take care of her and Jon and Etta and me when we needed them. I’m thankful for two beautiful baby girls. I’m thankful to be alive.
Just a quick update on Etta and Claire: they’re not even born yet, but they have a birthday!
I saw my OB this morning for my now weekly monitoring, and the babies and I look great. She had spoken to the Maternal Fetal Medicine team about our last ultrasound, and they decided that to be on the safe side, we should plan to have the babies at 36 weeks.
So…..April 2 is BABY DAY! It’s completely surreal to think we’ll just go to the hospital, have a c-section, and have babies in our arms by lunchtime.
I feel it is an auspicious date because April 2, 2006 is the day Jon asked me to marry him. Now that happy day gets exponentially happier!
At the beginning of my pregnancy, blessedly free from the all-day pukes called “morning sickness” that so many women suffer, my one persistent symptom (aside from having to pee constantly from the moment we got that little plus sign) has been INSANE TIREDNESS. Like, mono tired. Ran a marathon tired. Completely senseless tired. I spent a lot of time napping with Tinycat (who, it must be said, is a champion napper):
Now that I’m in the home stretch, it’s become frustrating because I’m still tired, and I’m increasingly incapacitated by my ballooning body, but I can’t. freakin’. sleep.
I know! Right this second, you’re thinking something along the lines of: oh poor naive dear, if she thinks this is bad, just wait til she has actual newborn twin humans on her hands demanding to be fed and changed and held all the time.
And yet, it would be nice if I could sleep now. People say helpful things like, “Sleep while you can!” And boy do I wish I could. Between reflux (which is a nice way of saying “constantly throwing up in one’s mouth”), restless legs, difficulty rolling over which results in weird hip pains from being paralyzed in one spot, hot flashes, and having to pee every single hour on the hour of the night…. and I’m not sleeping much.
I’m thinking I might start “sleeping” on the futon in the nursery, not because Jon is somehow keeping me up, but because lying sleepless next to someone happily, deep-breathingly, sleeping away makes me irrationally, jealously angry. He’s off in dreamland, and I’m sitting there thinking NO FAIR NO FAIR NO FAIR.
In the meantime, not sleeping at night means lots of napping during the day, which perpetuates the vicious cycle. Perhaps I’m becoming nocturnal.
I’ve realized, while pregnant, that people really have no frame of reference for what pregnant bodies are supposed to look like. Every woman carries her pregnancy differently, every woman gains weight in different amounts and in different places, and as a result, even those who have been pregnant or have experienced a pregnancy up close have no idea what we’re “supposed” to look like at various stages along the way. Perhaps because there’s no “supposed” and we’re all special pregnant snowflakes. Anyway, I say all this because to everyone I meet I am either ginormous or way too tiny to be carrying twins.
Here’s the real facts: I’m 7 months pregnant, I have gained about 30 pounds (which my doctor is very pleased with), and I’m officially measuring about as big as I would be at full term with a singleton. And I still have two months to go.
Though lots of folks still tell me how tiny I am and are amazed that I really “have two in there,” I’m starting to feel the effects of carrying around all this weight all the time. Basically, as I have tearfully told Jon more than a couple of times lately, I hurt somewhere all the time. If it’s not my ribs from the weight of two baby heads, it’s now muscular pain in my back so bad I can barely walk from my living room, where I spend a lot of time snuggled with a heating pad, to my kitchen. I get short of breath easily, and any sort of movement is enough to trigger Braxton Hicks contractions, which, while not exactly painful, are definitely uncomfortable. Oh, and the only thing I’m allowed to take for all this pain is a couple of Tylenol. Fun times.
I’m glad I’m not experiencing pre-term labor. I’m happy to let the babies cook as long as they need to. But I can’t lie and say I’m enjoying this part. It’s harder than I thought it would be and I’m kind of over it.
Which is why I was particularly happy that my marvelously talented friend Christen Byrd took some gorgeous maternity photos for us. I got them back this weekend, and they made me feel pretty at a time when I pretty much feel awful. You can see more of the photos on her site, and if you’re local, I HIGHLY recommend you let her “shoot” you sometime– wedding, maternity, engagement, family, newborn, whatever. She will make you feel great, she will make you smile real smiles, and you will not be disappointed.
Just got home after another ultrasound with our maternal fetal medicine team. We got to look at the babies, which is always super fun–just looking at them reminds me that all this near-constant discomfort and pain are worth it. I also know now why my right ribs hurt all the time: both babies have their big ole noggins on that side.
Baby Etta was called an “Amazon baby” by the doctor because she’s measuring a couple weeks ahead of where we should be at 28weeks gestation at 3 lbs. 6 oz., and Claire is just slightly petite at 2 lbs. 9 oz. The discordance is nothing the doctors are concerned about.
The best news was that neither the tech nor the doctors saw a defect on Claire’s spine. Basically, based on her head anatomy, the presence of a “lemon sign” and the fact that her cerebellum has been pulled down trapping some fluid in her ventricles, mean that there must be a spinal defect somewhere. But, the fact that they can’t find this defect means it must be very small and is therefore unlikely to have a very large negative impact on her. It seems more and more like the neurologists will have to find it after the birth rather than seeing it in utero.
We are so thankful and hopeful. Both girls look great and are growing well and we can’t wait to meet them!