A Claire Bear update: neurosurgery on Monday

We 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.

Claire Bear: an update

It’s been a while since I updated all of Claire Bear’s fans on how she’s doing these days. Most readers know that she has spina bifida, that she had surgery just days after birth, and that we’ve been sort of waiting to see how much her spinal defect will affect her.

The short answer is: she’s doing great. She’s a chilled out, happy girl who is a bit of a ham. She can charm any stranger with her bright eyes, big smile, and penchant for waving, giggling, and clapping. She is also a very observant little person, and seems to constantly be watching and figuring the world out. Even though she’s not into eating solid food yet, she loves to swipe pieces of it off her sister’s high chair tray, and before she got moved into the older room at daycare, would sit on the mat and swipe toys from smaller babies as they crawled by. She even figured out, on her own, from observing Etta, how to get from sitting unsupported down to her belly so she can roll around to wherever she wants to go. Where I used to be able to count on finding her wherever I left her, now she’s known to roll out of her room and down the hall. They even call her “the mechanic” at daycare, because she likes to roll under all the cribs and appears to be inspecting and fixing them.

Medically, she is doing really well also. She had a looooong day at the spina bifida clinic yesterday, and we saw rehab, urology, and orthopedics. Ortho continues to be impressed with how much function and sensation she appears to have in her legs considering the location and severity of her spinal defect, and the good news from urology is that we don’t have to start using catheters or anything at this point (bladder issues are very very commonly associated with spina bifida). Rehab, formerly a sore spot for us since one doc declared “she will never walk” after a very poor examination even after we said that she supports her weight on her legs for short periods, went OK too. We actually got to show the doctor how she can stand with support, and we got our first prescription for some AFOs, essentially her first pair of leg braces, which we hope will support her ankles and knees and help her learn to crawl, stand, and walk.

She’s been going to physical therapy for a couple of weeks now, and we are so happy to finally have that started. The therapist turns out to be the older sister of a friend from high school, and I have to say I just love her. Despite a very teary first session in which Claire *wailed* the entire time (her stranger anxiety has really ramped up lately– she also recently wailed at ZaZa’s, a local pizza joint, when the most grandmotherly, sweet-looking woman in the world dared to approach her), Claire has realized her PT is pretty cool and has neat toys, and now only cries when tired or frustrated with an activity.

Because Claire needs 3 PT sessions per week, 3 OT sessions per week, and now we’re talking about adding in speech therapy to help with her oral issues, we are working on getting her into a developmental preschool where she could receive all these therapies on site. With another one year old to wrangle, coordinate care for, and generally deal with, taking her to and attending that many sessions per week myself would really just be a logistical nightmare, and we’re so thankful this is even an option, that I could drop her off and know she was getting care from folks who don’t have a single issue accommodating her needs. Much as we LOVE LOVE LOVE our current daycare, the fact that she’s the only kid in her room who can’t feed herself or take a sippy cup is a bit of an issue. Even better, the preschool takes siblings, too, so if I get a job in the fall, Etta could join her. And the best news of all? Claire’s Medicaid TEFRA, a benefit she qualifies for because of her disability, for which we pay an income based premium, which covers basically all of her care not covered by the insurance we get through my husband’s work, would completely cover the cost of the preschool for Claire. AMAZING!

So, now I’m on the hunt for cute shoes that fit over AFOs (I’m thinking a sweet pair of mint green Vans might be my choice), and just generally excited that our sweet girl is finally getting the help she needs to make some progress in the mobility department. She’s starting to realize that Etta can do things she can’t, and it has her raring to go!

 

Ms. Bufflo goes to the capitol

Image I took on my way inside to testify before the committee, via my Instagram feed.

Today, I spoke before an Arkansas senate committee. Last night I was on the evening news.

Rep. Andy Mayberry is making national headlines by trying to ban abortion after 20 weeks of gestation (that’s the halfway mark, FYI). He claims this is because this is the point at which a fetus begins to feel and respond to pain, and he cites some studies, but this is hardly an established fact, and is controversial in the medical community. In the committee today, he testified that 98% of abortions happen before 20 weeks. Why would the minority get such a late abortion? It’s not because they just didn’t get around to it or had a sudden change of heart. Something big happens at 20 weeks. It’s the point in a pregnancy when the “big ultrasound” happens. The one that tells you if you’re carrying a boy or a girl (or two girls, in my case), but also the one that tells you for the first time that there could be something seriously wrong, even life-threatening, with your fetus. I know what it’s like to sit in that ultrasound room and get bad news. Like Andy Mayberry, who also has a daughter with spina bifida, I am fortunate that our news wasn’t as bad as it could have been. My daughter and the Mayberry’s daughter have a condition that is treatable and manageable and won’t stand in the way of a full life. Many parents are not so fortunate. For many parents, that moment in the ultrasound room is what turns a wanted pregnancy into a nightmare of heartbreaking news and difficult choices. Placing an abortion ban at that point in a pregnancy leaves these parents without options right when they need them most. It places a legislature between families and their doctors, right when those families most need compassionate care.

I know some will say that the bill has been amended to include exceptions for the health of the mother, for fetal anomalies, and for rape and incest. But as one of my own doctors testified before another committee, when we’re talking criminality for doctors who provide abortions, how much of threat does there have to be before it’s “enough” to justify an abortion? I have a congenital heart defect and a previous severe cardiac pregnancy complication, but no one can say exactly how risky another pregnancy would be for me. My doctors agree that I should not have more children for the sake of my health, but my condition is very very rare, and there isn’t much data on it, let alone actual odds of my survival. Do you think my doctors are willing to risk jail time and the loss of their career and livelihood on my chances of survival? I don’t. And yet I am not willing to risk leaving my girls motherless, and should my IUD fail (as it could, I personally know people who became pregnant with an IUD), I would not think twice before terminating to protect my own life and stay here to care for the girls who need me.

The bill passed the committee despite my testimony. It will probably pass the Senate. The governor will probably sign it. I fear for the state my girls will grow up in, and I fear for their rights and mine.

For every mother who testified that she’s glad she carried her anencephalic baby to term (that’s a baby with no brain and a damaged skull, with no chance of survival outside the womb), there are mothers thankful they had the opportunity for a post-20-week abortion (essentially an induction of labor), to prevent needless suffering for her and her doomed child. For everyone like Andy Mayberry and me, whose kids will have challenges but lead full and happy lives, there are people who got literally fatal news. For everyone like me who survived pre-eclampsia and peri-partum cardiomyopathy, there are people whose fatal complications developed too early to save themselves and their babies, and were forced to deliver to save their own lives, meanwhile their babies could not be saved. For everyone on the other side who calls themselves a compassionate conservative fighting for life, there is someone like me, literally fighting for her own, asking for compassionate choices when we need them most.

If you’re in Arkansas, please start writing to your senators and the governor and urge them not to let this bill pass. Post 20-week abortions are rare because they only happen in the most dire of circumstances. These people deserve compassion.

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.

A quick update on Etta and Claire

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!

thankful that no news is good news

It’s possible I’ve gotten a little spoiled in that I’ve yet to go to an OB appointment alone. Jon’s got a flexible schedule, and he’s been willing and able to go with me every time. Until today, when some very important disaster training coincided with my appointment, and he couldn’t make it. Because I’m spoiled, my stepmom came with me instead. It was an uneventful appointment, and for that I am very thankful. Just a quick check in, a chat with my doctor, and off on my way, with a pat on the back because I’m still gaining the weight I need to for the babies. No bad news in the ultrasound room. No difficulty finding two little heartbeats on the Doppler. No real problems to report except that I’m still insanely insanely tired, and the constant heartburn is a little annoying, but overall I’m pretty happy for 6 months pregnant, which still feels insane to say.

I am so thankful for this uneventful appointment. I’m thankful to have a great team of doctors and nurses and genetic counselors and care coordinators. I’m thankful that, even though Claire will have to be transferred to another hospital right after birth for surgery and care, it’s the hospital where my husband works, right by our house, and one of the best in the country. I’m thankful to have family nearby to take care of all of us.

In short, I guess I’m just feeling really thankful. Possibly because I’m reading One Thousand Gifts, which is a book all about the practice of gratitude as the central practice of the Christian faith, which is a really great read so far, even though I don’t agree with all of it. The author seems to suggest that God causes all things that happen to us, be they joyful or painful, and I just can’t get on board with the idea that God causes things that are bad. That isn’t to say that I don’t believe that God works all things for the good, or that we can’t be drawn nearer to God in all things, or that we can’t learn and grow from every experience that happens to us. But I just can’t agree, though it has been suggested to me by well-meaning people, that God caused, for example, my Claire’s spina bifida. I am sure it will be an opportunity for learning and growth and drawing nearer to God, but I don’t believe God gives anything but good gifts to his children. I can’t believe God would cause disease or suffering and pain and still maintain faith in God. Spina bifida just happens sometimes. Even if you take your vitamins. Even if you say your prayers. Because that’s the way this world works. And God’s heart is broken along with mamas’ and daddies’ when they find out something’s wrong with their baby. And God rejoices when the defect isn’t as bad as it could be. That’s what I believe.

And so, I’m thankful for uneventful appointments, and for hope for the future, and for days when we don’t get any bad news. And I’m thankful for arms that hold me when the bad news comes too.

six month pregnancy update

Jon and I last week.

I’m now 24 weeks pregnant. That means 6 months. Before I was pregnant, I thought 6 months was a whole lot pregnant. Now that I am actually 6 months pregnant with twins, I’ve discovered that while it is indeed a whole lot pregnant, I still have a long ways to go. Apparently I don’t look as big as folks think I should, because when they ask me how far along I am and I tell them “6 months, with twins,” they always remark how tiny I am. Well, the babies are just now breaking a pound in the weight department, and I have to get to 6 pounds each, so I have a feeling the growing is going to start speeding up in a hurry.

After our last ultrasound ended in scary news and tears, I was a little nervous about this morning’s appointment, but I have to say it went so much better. It’s always fun to get to peek at our girls, and we had a great ultrasound tech and a MUCH warmer Maternal Fetal Medicine specialist this time around. The great news is the girls are growing very well, with Etta (baby A) weighing in a little ahead of schedule at 2 lbs. and Claire (baby B) just slightly behind schedule at 1.2 lbs. There was a little fluid in Claire’s skull, but it looked pretty good, and there appears to be no sac on her spine, so again, the best possible scenario for spina bifida.We met with a genetic counselor and a nurse for the Arkansas Fetal Diagnosis and Management Program which will help coordinate all the various care Claire will need both before and after birth. We feel great to have such a good team of healthcare providers and are very hopeful for the best for Claire.

Both girls were still as wiggly as ever, and the tech managed to get one really awesome shot where it looks like Claire is giving Etta a kiss on the cheek. I realize it’s entirely possible that I’m only looking at this pic with a mama’s eyes, but I’m sharing it anyway. It’s sort of like a magic eye– don’t look too hard, and you’ll see two little faces, I hope. Can you see it?

Overall, I feel pretty good. I’m really exhausted practically all of the time, have been experiencing more and more rib and back pain, and am completely annoyed by all the heartburn. Still, I’m thankful I don’t feel too bad, and also thankful that I have plenty of time to rest when I need to. The craziest development is that we can now SEE the babies moving around in there from the outside. It’s a completely trippy experience.

good news and the best possible bad news

Friday was my birthday. I think 27 is going to be a good year. It was also the day of our “big ultrasound” or “anatomy scan” for the babies, the day we would finally find out if Baby B was a boy or a girl.

The tech was very sweet and showed us right away that we have two very wiggly baby GIRLS! Baby B, the one on the top if you remember this early ultrasound picture (they’re now too big to both be seen at once) is named Claire Elaine, and Baby A, the one on the bottom, is named Etta, with an as-yet-to-be-determined middle name. I’ll tell you all about the names and their significance later, I promise.

We got to see our girls kicking and punching and flipping around, and at one point, I swear they high-fived across the membrane that separates them. It’s so cool to think they’re already interacting, and that they will ALWAYS have each other. We couldn’t be more excited about them.

The atmosphere in the ultrasound room got a little weird right at the end of the scan. Instead of wiping me off and sending us on our way, the tech draped me with a towel and said she had to go talk to one of the doctors, who may come back in in a few minutes. We were soon joined by a maternal fetal medicine specialist with an all-business demeanor. She took the helm at the ultrasound machine and told us that Claire has a slightly lemon-shaped head, and this was a red flag that made them want a better look at her spine.

She then got to work looking at Claire’s spine, and I got to work freaking out. Hormones and fear took over, and though I was trying very hard not to cry so as not to have a heaving belly making the scan more difficult, the tears just came. I was confused and scared and all I had heard was that there was something wrong with my baby’s head and spine and no one was speaking to me in a way that I understood. Jon held my hand and tried to comfort me, but he was also trying to get all the information so he could explain it to me later, which I am very grateful for. I have never felt more like a mama bear than I did in that ultrasound room, wanting to protect my tiny baby girl.

Ultimately, Claire has what we hope is a best-case scenario of spina bifida. This is known as a neural tube defect, and means that her neural tube didn’t close all the way when her brain and spine were forming very early in my pregnancy. This means there is an opening at the bottom of her spine, in the section known as her sacral region. There is a little sac there, but there appears to be no neural tissue in the sac, which is a good sign. It’s hard to know how this will affect her until she is born, but she might have disability in her legs and some other issues. However, her legs looked good anatomically, and though the MFM specialist said that movement doesn’t necessarily mean anything in utero, she was kicking and wiggling her legs all around. She will most likely not have neurological or cognitive issues. The abnormality in her head shape is known as a Arnold-Chiari malformation. She might have problems with excess fluid building up in her head, which may require surgery and a shunt to drain the fluid, but we don’t know this yet either.

We do know that I will definitely be having a scheduled c-section, and that Claire will most likely be having surgery within a few days of her birth to close the opening on her spine. That’s all we know for sure.

We are thankful that the opening on her spine is down so low, and we are thankful that there appears to be no neural tissue in the sac. We want it to stay that way. We are thankful to be at UAMS, where we have access to some of the best specialists and surgeons around, and to know that our OB will definitely be there at the girls’ birth. I am personally thankful that I’m married to a pediatrician who can listen to all these doctors speak and translate it into a way that I can understand and that calms me.

We are so excited and so in love with Etta and Claire already. They are moving like crazy inside me, and every night at bedtime, they seem to have a little dance party. Jon has been able to feel them moving too, and it’s just the craziest experience in the world. I love that they’re still keeping a little secret to themselves, and that we won’t know until after they’re born if they’re identical or fraternal twins.

Friday was a hard day with a lot to take in, but after a couple of days of reading and settling in, we are feeling much better. We, all of us, are going to be OK.