Call Professor X, I’m a Mutant

Many of you are probably aware of the fact that I almost died three days after I had my babies. I experienced an often-fatal complication called peripartum cardiomyopathy, something I had never heard of until it happened to me, but according to The Daily Beast is actually on the rise. The first sentence of that piece, where the woman describes feeling like her head was being held underwater, just felt so true to what I experienced as well– I often describe it to people as feeling like I was drowning inside my own body. As my heart went into failure, my lungs literally began to fill with fluid, and I could actually hear it crackling as I struggled to breathe, before I was sedated and intubated and moved to the ICU. Thank GOD I was still in the hospital 3 days postpartum, because there was a code team ready to save me. If I had not asked for one more day inpatient to work with the lactation consultant on breastfeeding, I could have very easily been at home, where I might have died. (After my near-death, I was put on some serious medicines which meant my breastfeeding days were over, but you could say that my desire to breastfeed saved my life!)  Continue reading “Call Professor X, I’m a Mutant”

i have a broken heart, but i’m ok

Sporting red for heart health awareness. Photo by Whitney Loibner.
Sporting red for heart health awareness. Photo by Whitney Loibner.

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.

Claire update: no surgery this week

After seeming to talk us into the shunt surgery in the ER, our neurosurgeon seemed to spend our clinic visit talking us out of it. While she seems to agree that the surgery is basically inevitable, there is still a slim slim chance the hydrocephalus will arrest, and since she’s not having severe symptoms at this time, we have a little time to wait. In the meantime, we’re probably going to start her on Zantac and see if that helps the vomiting.

I’m glad this means no surgery this week, though I’m pretty sure she will need the shunt at some point. Guess we’ll be staying in style when that happens, because the new infant and toddler unit at our children’s hospital will be open by then.

I also realize I haven’t updated about MY health issues in a while. Based on my slow recovery and echo-cardiograms, my cardiologist had me do a contrasted cardiac MRI. It was kind of crazy to actually feel the contrast get warm in my chest every time the MRI machine was on, and I now totally see why claustrophobic people totally wig out in those things. Personally, I pretended I was an astronaut about to be blasted into space. Anyway, the upshot is, it looks increasingly like I have a congenital heart defect called left ventricle non-compaction syndrome, which basically means the tissue in my left ventricle didn’t form correctly, which is why it’s weak and has such a “poor squeeze.” The strain and stress of the pregnancy on my body is what finally made my condition severe enough to notice. I now feel vindicated and want to go back to every PE teacher I ever had and explain that my poor performance in everything endurance related is actually the fault of my heart.

The MRI also showed that my ejection fraction (a measure of how well the heart pumps blood into the body) has improved from 15% to about 30% (normal is still 50% or better). I’m still doing well on my ever-increasing cocktail of meds, and I think my doctor will be adding a third medicine (I’m already on a beta blocker and an ace inhibitor) this week, and I hope the transition goes well.

Anyway, that’s what’s going on with Claire and me health-wise. Thank you again for all of your support, thoughts, and prayers.