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.

well aware

20131010-160825.jpg October is Spina Bifida Awareness Month, not that you’d know it, as apparently October is the most crowded of all awareness months, most famously shared with Breast Cancer Awareness, but also Down Syndrome, Autism, Domestic Violence, and Bullying Prevention. To make matters worse, the Spina Bifida Awareness symbol is apparently a yellow ribbon, which everyone else knows is the symbol for supporting the troops, spotted on many a bumper sticker. Basically, I think we need a new month and a new symbol, but they didn’t ask me.

Though “they didn’t ask me” could be the whole theme of my all-too-keen awareness of Spina Bifida in general. They didn’t ask me, and they didn’t ask Claire, if we wanted SB in our lives. They didn’t ask the other 1 in 800 babies born with myelomeningocele SB (the most severe form, what Claire has) either.

I feel like this week has been an effort on our doctors’ part to make sure we’re REALLY REALLY AWARE of this whole spina bifida thing. Last week we made 3 separate trips to the children’s hospital for various tests, and this week we made two trips to talk to the doctors about the results of those tests. And the news, while not surprising, is bumming me out a little bit. In addition to mobility limitations, bladder and bowel issues are very common for people with SB, and though we had been hoping to continue avoiding it, in order to prevent recurrent UTIs and prevent damage to Claire’s kidneys, our urologist says it’s time to start catheterizing to get her bladder fully emptied. This means cathing her every 4 hours. She’ll also now have to take a daily medicine to prevent bladder contractions. A side effect of this medicine will likely be constipation. I know (really, I do) that ultimately this is the best course of action and is what will help her maintain continence as she grows, but for some reason in my head, having to take this step, having to cath, seems like it makes her more disabled, more different, and I just don’t like it.

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WHAT? You’re gonna make me go to the doctor 5 times within 7 days?!

We also got x-rays and saw the orthopedist, and it looks like at some point, Claire is going to need a surgery on her left leg to fix the way her foot turns severely inward. For the short term, she’s getting a special brace to wear at night, but in 6 months to a year, we’re looking at another surgery. Again, I’m all for whatever we need to do to give her the most mobility and independence, but hearing that my baby needs another surgery is hard.

And yet, we have so much good news too. She got an MRI, and we saw our neurosurgeon, and her shunt is working beautifully. I really credit it with the way her mobility is exploding, and our surgeon agrees. We’ve officially been cleared by neurosurgery, and, barring any shunt malfunction or infection, won’t be seeing her for a YEAR! We’re definitely celebrating that milestone.

So, what would I like you to be aware of this month for Spina Bifida Awareness Month? Yes, I want you to know that sometimes it’s hard. Sometimes there’s more surgery and more hassle and more hard work than we’d like. Sometimes I get angry or sad. But mostly I don’t. Mostly, I focus on the fact that both of our girls are beautiful, funny, joyful, smart, silly, typical toddlers. Mostly, I think that’s what I want you to be aware of– Spina Bifida has challenges, but it’s just another way of being a human in the world.

If you’d like more info on Spina Bifida in general, I thought this post from a young woman with SB was excellent.

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Truckin’ along with her push cart. This is *without* her braces on. I know. I’m amazed too.

 

i have a seven year itch

Last week, my husband Jon and I celebrated 7 years of marriage, and in June, we marked 10 years of togetherness.

Everyone jokes about the 7 Year Itch. But I have it, y’all.

seven-year

Except…it’s on my finger.

First photo with my ring after we got engaged in 2006.
First photo with my ring after we got engaged in 2006.

You see, somewhere in the last year, I developed an allergy* to my white gold wedding rings. Sure, some skeevy dudes may say they’re “allergic” to their rings, when really they’re not wearing them so they can mac on chicks, but this is no lie. Wearing my rings has started to cause my finger to immediately break out into a red, bumpy, itchy rash. I figured out it was the gold because my silver stacking rings, a Valentine’s gift that I wear on my right hand, don’t cause the same problem.

“I’m allergic to my wedding rings,” I announced to my doctor hubby one day, showing him the rash. He asked if I meant symbolically or literally, but thank goodness, the only itch I’ve got is the one on my finger. I’m not itching to get out of our relationship or marriage at all.

And my best friend and hubby was handy in diagnosing my problem too. It turns out it’s fairly common for people to develop allergies to the nickel used as an alloy with the gold to make it strong enough to stand up to the wear and tear it gets as jewelry. But he’s seen enough nickel allergies to know I don’t have it, since nickel is also commonly used in the hardware on things like jeans, and I don’t develop a similar rash to the rivet on the waistband of my blue jeans. Also, the gold posts on my pearl earrings have started irritating my ears, too. So, I am forced to conclude, I’m having an issue with gold, not nickel.

I’ve taken to wearing one of my silver stacking bands on my left hand as a placeholder, but I’d really like to get back to wearing my rings again. I hear a temporary solution is to coat the ring in clear nail polish, and a permanent one is to get it plated with rhodium.

As for seven years, I’m happy to celebrate how far we’ve come. Becoming parents has truly been the hardest thing our partnership has endured, far more stressful than moving cross country, grief, and trauma. Still, there’s no one else I’d rather be raising my family and living alongside.

wedding

We celebrated lucky number seven with a little road trip to Texas, spending a night in a bed and breakfast in Dallas, checking out the 6th Floor Museum at Dealey Plaza and the Dallas Museum of Art, and then continuing to Austin for LOTS of tacos, a visit to the LBJ library (thankfully for me, my husband indulges my political geekery), visiting with friends, seeing the bats, and eating some BBQ. It was a lovely getaway, and we’re super thankful that family took care of our kids and pets so we could get that time together.

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We stayed at the lovely Corinthian Bed and Breakfast in Dallas.
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Beautiful glass work by Dale Chihuly at the DMA.
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Visiting the LBJ library on the UT campus. He passed such an amazing amount of progressive policy!
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Pretty sure Jon wanted to keep the Jeep we rented for the trip.
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Stopped by Wendy Davis’ office, and even though she wasn’t in, some of her staff let me take a picture in her office. Big fan!

*Allergies can develop at any time, even after years of exposure without event to the allergen. This is why when people tell me they “aren’t allergic” to something like poison ivy, I always tell them, just wait! With enough exposures, you’ll eventually trigger a reaction!

surgery for Claire tomorrow

I’m back from an amazing weekend in NYC helping my sweet sister Jessica shop for a wedding dress. Now it’s back to reality in a big way because Claire the Bear is having surgery tomorrow. She’s having a shunt placed to help treat her hydrocephalus (the fluid that builds up in her head because of her spina bifida), which has gotten to the point of causing fluid to build up in her spine. This is called a syrinx, and because it could compromise her mobility, we have to finally do the surgery this time, almost exactly a year after we first thought we’d be getting it done.

I’m glad we could wait this long. Since she’s older, the procedure is much less risky than it would have been on a tiny baby, and that’s always good news. We trust our surgeon completely and know she will be in the best hands. All you lovely folks: please be praying for or sending positive thoughts her way, whichever you do. Thank you for always cheering Claire on. She will be in the hospital overnight, but it should be a fairly quick recovery, so let’s hope that’s true!

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Color Me…Bieber

Disclosure: The kind folks at Color Me Rad gave me a free spot in their colorful 5k, as well as a hat and a pair of socks. I was not required to post anything, and I haven’t even run the thing yet. Everything I’m about to say is 100% from me.

photo 3-1

I’ve come a long way in the last year. Just over 14 months ago, I had just birthed my two beautiful Bufflo Gals when I stopped breathing and wound up in the ICU on a ventilator. My official diagnosis was ultimately a congenital heart defect called left ventricle non-compaction syndrome, a defect in which the chamber of my heart that squeezes blood out into my body didn’t form correctly, so it doesn’t pump blood as efficiently as it should. This defect, which had never reared its head before, though I have had it since embryohood, apparently, was exacerbated by the stresses of twin pregnancy, pre-eclampsia, a c-section, and the fluids and blood I was given after surgery. Basically it was a perfect storm that landed me in congestive heart failure.

Still, over a year later, regularly taking my old man meds (seriously, I’m on a beta blocker and an ace inhibitor, the type of thing usually marketed to old men via commercials that feature things like fly fishing), and I’m doing great. My heart is functioning at a range that puts me into the “normal” zone. And as I keep up with two babies rapidly heading into toddlerhood, I’ve been thinking that I’m ready to be more active.

Enter my friend Kyran with a fun opportunity for some of us Little Rock bloggers to participate in a fun little thing called Color Me Rad. It’s a color run. It’s not a marathon, it’s not for a cause, it’s just for the joy of it. And for me, it’s a celebration marking an end to a year in which I considered myself sick, and a step into a life of health. I know I don’t have the stamina to run the whole thing at this point, but this is my kickoff. I plan to effing frolic, man, and I’m pleased to be doing this thing with the rest of team Rad News Bears: Kyran, Kerri, Amy, Sarabeth, Alison, Whitney, and Jacklyn.

If you’d like to join this little celebration, you can still sign up online for the Little Rock race through tonight, and after online registration closes tonight, you can sign up in person on Friday 10-7 at: Cardinal Health, 5426 Landers Road, Sherwood, AR 72117 (Next to the Tractor Supply Company).

Special bonus? I tried on my swag, and well, let’s just say I found my doppelganger:

bufflo and the biebs

A Claire Bear update: neurosurgery on Monday

IMG_3548We just got back from a visit with our (much loved) neurosurgeon, and based on new information in last week’s MRI, we have decided in consultation with her and another neurosurgeon she asked to give a second opinion, that it’s time for Claire to get a shunt placed to treat her hydrocephalus. The lay explanation is, due to her spinal defect and chiari malformation, Claire’s cerebral spinal fluid doesn’t drain from her head and spine properly, causing fluid to build up in her head (aka hydrocephalus). While she has had the hydrocephalus from the start, we and our surgeon chose to take a conservative approach instead of operating when she was a wee baby, because surgery is always risky, because the risk of infection and complications is greater in younger babies, and because clinically, she was having no symptoms or problems from the hydrocephalus other than a big head as a result of the fluid buildup.

However, the MRI we got last week showed that not only are the ventricles in her head very large, indicating a lot of fluid has built up there, but also she has developed a syrinx, or pocket of fluid within her spinal cord. This has the potential to cause her to have problems with her arms and legs, and given the great strides and progress she has made mobility wise, and the hope we have for continued progress, we do not want to risk this syrinx compromising her physical abilities in any way.

It is for these reasons that we’ve agreed to have the shunt placed on Monday morning. We are of course a little disappointed that she has to have neurosurgery, but we are ultimately confident that this is the right time, and hopeful that taking this step now will give her the greatest chance to achieve all she can developmentally. She’ll stay one night in the hospital, and then should be back to her normal activities within a week or two. Thanks for always cheering Claire on– her internet fan club means more to us than you can know.

Awareness

I found out today that October is Spina Bifida Awareness Month. My first thought was: what a crap choice in awareness months. I mean, everyone knows that October is Breast Cancer Awareness Month, as pink has pretty much blanketed everything we see.

But then I thought, well, it’s just as well, since I’m generally skeptical of “awareness” in general. I mean, I’m not usually sure of what it accomplishes. Half of the pink I see this time of the year seems to have no real point, as most of us are aware that breast cancer exists, and the pinkwashing is often unaccompanied by anything about breast exams or early detection or risk factors or anything.

Feeding tiny Claire in the NICU.

I guess people are less aware of the realities of spina bifida. SB is a congenital defect of the “neural tube” which is the part of a fetus that eventually becomes the baby’s head and spine. Claire’s neural tube didn’t close properly, and when she was born, she had 4 centimeters of her spine visible from the outside. As a result of this defect, things like nerves weren’t hooked up properly, so she has/will have certain amounts of disability in her legs, bladder, and bowels, in addition to hydrocephalus, or fluid building up in her head (which for many people with SB requires surgery to place a shunt and drain the fluid, though we haven’t had that yet). While her spinal defect was one of the more severe types, she seems to have good enervation and musculature in her legs, and her doctors and physical therapists believe she will walk and will only need braces to support her ankles, though some people with SB require more extensive bracing or even use wheelchairs.

I certainly didn’t know all of this or really much about SB at all, and it really wasn’t even on my radar until my birthday last year, when we went in for a 20 week ultrasound, excited to finally learn our babies’ sexes, and instead learned that the baby we’d later name Claire had SB. It was a really scary, sad day.

But the thing I needed awareness of that day wasn’t just “spina bifida” as some vague concept. I needed to be aware of the beautiful reality that would be my daughter’s life. Yes, we both had a rocky start. She had surgery at two days old. She was separated from me for 9 days. She was in the NICU for two weeks. She had to stay on her belly for 6 weeks while her back healed. But despite all of that, she’s really just a baby. They’re all very needy. They’re all very fragile. They’re all very tiny. They’re all amazing little creatures. If you looked at my two girls today, you might not be able to guess which one has SB.

If I could go back to last December 16 and make myself aware of anything it would be this: Claire is beautiful. She is funny. She is sweet. She has a radiant smile. She loves to eat. She loves her mama and daddy. She is exploring and learning and growing every single day.

I was so worried about all the ways she’d be different from her able-bodied twin sister, but the reality is, they’re both just babies. They are completely different and yet so very much the same. And almost all of my worrying was completely unnecessary. That is what I needed to be aware of: that there was nothing to be afraid of.

So, no, you likely won’t see NFL teams raising awareness for SB this month, or yellow covering all your favorite products in the name of raising funds. And while you may not personally know anyone affected by SB, now you know a little more about our story, and a little more about my baby Claire, who is special, just like everyone else.

Claire the Bear today.

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.

surgery for baby claire

Last Thursday we spent most of the day in the ER with Claire, because her already more-than-average spit up had become projectile vomiting in the last couple of weeks, and had escalated to every other feed or so in the last few days. Dr. Dad thought she might have pyloric stenosis, and additionally, since increased vomiting is a warning sign that her hydrocephalus (extra fluid in her head because of the way her spinal defect pulled down her spinal cord and blocked the exits for the cerebral-spinal fluid) is getting more severe, so we wanted her to get worked up.

She got an ultrasound of her pyloris, which ruled out pyloric stenosis, so she got an ultrasound of her head, which revealed more fluid in the ventricles of her head than the last time we looked. She also got a head CT to get an even clearer picture. Her neurosurgeon, whom we love, insisted on coming down from clinic to see “her girl” personally, and we discussed whether or not it’s time to move forward with surgery to place a shunt that would drain the excess fluid from her head into her belly where her body could then absorb and deal with it.

The bottom line is, it looks like it’s time to get the shunt surgery. We had been hoping to avoid it, but we think that since the fluid continues to increase, we’d rather go ahead and do something about it instead of waiting for things to get worse, which would involve symptoms like trouble breathing and swallowing.

We hate that our wee girl has to have surgery again, but we know she’s a trooper and will come through great. We see the neurosurgeon again on Monday morning, and it looks like the surgery will be Tuesday or Wednesday. Not sure how many days inpatient would be involved, yet. Please keep our Claire Bear in your thoughts and prayers, and us too.