The Letter

We received “The Letter” from CCRM over the weekend. I’m referring to the annual invoice in which CCRM requires us to either pay $300 to store our three remaining embryos for another year, or to provide our signatures so that the embryos can be destroyed.

Note: Because I have a genetic condition, we cannot donate our embryos to another couple–I checked with CCRM’s genetic counselor, Danielle. Our options are to use, destroy, or donate for research.

When we received The Letter last year, we decided to pay the storage fee “just in case.”  Just in case what? I guess we were worried about making a rash decision and then having regrets. And now even though we have talked for months about signing away our rights to these three little embryos, it’s a sobering thought to actually have our signatures notarized. As much as I want a sense of finality, I have a tiny bit of fear that I’m making a decision I will later regret.

Before infertility, I had long thought I might like to have three children someday. Both of my parents have two siblings, and my grandparents’ lives always seemed so full with three kids and eight grandchildren. Two parents + three kids always felt to me like an ideal family.

But honestly, a rough pregnancy and our girls’ health complications have caused me to feel very negatively about going through any of it ever again. I’ve been robbed of any desire to repeat the process.

My pregnancy was filled with 8 full months of daily morning sickness (not complaining about this–I know how lucky I was to have experienced it at all!), dislocating both kneecaps, three broken bones after falling in the shower, invasive left knee surgery, a total of 11 days in the hospital, three months on almost complete bedrest, wheelchairs and walkers, sciatica and enormously swollen feet and legs (I was prescribed a water pill after giving birth and lost FIFTY-THREE POUNDS of fluid in two weeks–no joke!).

Then September 13th arrived, the birthday of our gorgeous twins and those perfect first three weeks of normalcy. The pain and discomfort of pregnancy subsided as my body healed quickly now that it was not allocating resources to my babies. Although we were sleep-deprived, we felt like we were in a wonderful dream. And then, the drama resumed:

  • Eight fractures in about a month from little to no trauma.
  • Complete and utter fear when thinking about handling our own babies (our absolute emotional low point was holding our newborn niece in our arms the night she was born, and then going home knowing that we could not hold our own newborn babies because we might cause a fracture).
  • Starting PAM infusions… seeing our kids hooked up to IVs.
  • Clubfoot treatment, first surgery (double tenotomy), months of casts and braces.
  • Craniosynostosis… seeing our child emerge hysterical from a 7-hour surgery during which her head was cut open from ear to ear and a piece of her skull was removed.

I must add, I know so many parents have had to watch their child go through so much worse. I follow bloggers who have kids with cancer, and I even have a friend whose baby just had open heart surgery last week and is still on a ventilator. My heart goes out to parents of sick kids, as I clearly do not have half the strength that they do. The past 18 months of seemingly nonstop medical intervention has been draining, to say the least.

So that’s why I was pretty surprised when we casually discussed “The Letter” last weekend and my husband said, “Maybe Violet and Layla will need a little brother or sister someday.” I felt my heart drop into my stomach because this was the first time he had ever expressed an interest in further expanding our family. In fact, he has always seemed pretty opposed to trying to have another child.

I stewed over it for a few days, and then broached the topic again. During those couple of days, I searched my soul to try to unlock my feelings about the idea of another FET. All I felt was dread thinking about it, honestly. Sure, it would be cute to have a little baby boy toddling around, looking up to his big sisters (what I pictured in my head was a little boy, but we don’t know the genders of our remaining embryos). But then I thought about the fact that he or she would have a 50/50 chance of having OI and that it’s a spectrum disorder, so his disease could be more or less severe than V&L’s. I thought about the possibility of more complications, like Violet’s clubfeet or craniosynostosis.

What I realized is that I am spent. I would not change a second of the past 18 months, because those experiences have shaped our family and we are stronger for having gone through it. But I can’t go through that with another baby, and I know it from the depths of my heart. I know 100% that the best choice is for us to sign away our embryos, to eliminate any future waffling and to move on with my life. I had my fallopian tubes ligated during my c-section, so the three embryos in question are our last chance at expanding our family.

So when I broached the topic again with my husband, I put it all out there. And thankfully, he agreed. He said his remark was just a fleeting thought and that when he thought about the ramifications of having another baby, he was no longer on board. We are comfortable with just being “Violet and Layla’s parents,” and we are planning on making it official next week by meeting with a notary. Although I am a tiny bit scared that I will hate myself someday, right now this feels right. And I have to trust that feeling.

How Do You Know About New Posts On My Blog?

V&L are growing and doing so much new stuff that I need to write about! I am working on it, but in the meantime, I have a question…

When I publish a new blog post, how do you know about it? Do you use Google Reader?

If you haven’t already heard, Google is retiring Google Reader at the beginning of July. I’ve noticed that some of the bloggers I follow are recommending that their readers connect with them on Twitter to find out about new blog posts. I’m still in the Dark Ages and have never used Twitter. I also don’t have a smartphone yet (crazy, right?!). So with Google Reader going away, I’m wondering if the time is right to retire this blog. After all, if no one will be notified of new posts, no one will be reading anyway. And although I find writing therapeutic, I don’t have much time to sit down and really focus on it. When I do write, it’s usually because, like right now, I’m procrastinating about starting something on my never ending to-do list.

When I started this blog, I was a stay-at-home mom with two health-challenged babies. Life was busy, but they napped often enough that I had plenty of time to record those first 11 months. Then Fate intervened last August, and I was offered a consultant position and have been working full-time (mostly from home) for the past seven months. I’m lucky to have a very flexible schedule and only have to work on-site 1-2 days per week, but my days have honestly never been more full. Because I work remotely most of the time, I end up doing the majority of housework, and the girls are at home with me two days per week (they attend a day care center the other three days to give me time to attend meetings or simply to focus [or regain my sanity!]!). My contract is slated to end at the end of the year, and then I’m not sure what my next step will be. But right now, I struggle to find time to write in this blog even though I have plenty to write about! I hate that I’m not recording my girls’ lives like I had envisioned (although I do post hundreds of pictures and dozens of videos each month on FB). I’m certainly not doing this blog any justice by only writing once every several months. And with Google Reader closing up shop, maybe that is a sign.

I will still leave up my CCRM blog, though, so no worries about that! In the meantime, I would love to learn how you are notified of new blog posts so that I can make a more educated decision about this blog’s future. Please share with me!


It seems like I didn’t have much time to take many pictures in the month of January! Work and life got the best of me. I’ll share a few photos below…

We said goodbye to bottles in January! I had lingered on retiring bottles because part of me wants them to stay babies! But by mid-January, it was clear they were ready. Violet started drinking only an ounce or two and refusing the rest. Layla would drink more, but didn’t seem all that interested. They were eating more at dinnertime and getting fuller bellies, so we figured they were ready. One night we just decided to put them in bed and see what happened. I was afraid they’d wake up starving at midnight, but they slept all night and have ever since. They are seriously the BEST sleepers. They’ve been sleeping through the night for a year now, and I can only remember getting up in the middle of the night once (and that’s because Layla threw up in her crib). We are very, very lucky that they retired bottles so easily.

Here they are lounging on their animal pillows one night just before weaning, while their dad was not home:



A few weeks ago, I ordered some nap mats for them to use at daycare. When they arrived, their daddy decided that they needed to try them out to make sure they wouldn’t be swallowed up in the fabric. They LOVED them and stayed bundled up inside for several minutes.


I’m going to try to take more photos this month to share!

Also, I forgot to mention in yesterday’s post that they both say “Mama” and “Dada.” Talk about heart melting! Every morning that they’re home with me and my husband is at work, Violet looks around and asks, “Dada?” Layla is also saying “No” a lot. She hears it often enough, it does not surprise me!🙂


Busy Start to the Year!

These little girls are keeping me busy lately!!! Believe me, I’m not complaining!

Violet is WALKING!!! We began really encouraging her to walk by holding one of her hands and having her walk pretty much everywhere in our house that we would normally carry her–to her booster seat in the kitchen for meals and snacks, to her bedroom at naptime and nighttime, to her changing table for diaper changes, etc. We could tell that she was SO close, but she was just afraid to let go of our hand. She went to her daycare on January 25th while I worked onsite at my job, and when we picked her up that evening, the teachers were so excited to show us that she had started walking independently. In fact, they put her down and she walked across a hallway to reach us with a huge grin on her face! The fact that Violet has achieved this milestone feels to us like such an amazing accomplishment. This child who broke both femurs in her first two months of life, who went through so much with bilateral clubfeet, and who had her skull cut open less than six months ago, is walking!!!! We are so ecstatic for her, and she is very clearly thrilled with her newly discovered mobility. She is walking more and more every day.

Both Violet and Layla are so, so fun right now. They’ll be one and a half next month! They are interested in everything I do, from preparing the coffee pot to cooking and cleaning. I can almost see their brains soaking everything up!

Violet has had a language and personality explosion over the past few months. Words that they are both saying are:

  • Hi!
  • Bye-bye (and they wave while they are saying it)
  • Night-night
  • Love you

Layla is more the singer and Violet is more the dancer. When we sing Twinkle Twinkle Little Star, Layla says “Up A-Ba” (then we finish with “the world so high”). Then she says “Di Di Di Di Di Di” and I believe she’s trying to see “Like a Diamond in the Sky” because there are so many long “I” sounds in that phrase. She sings by herself too, just gibberish but it is very cute. Violet sways back and forth in her booster seat and makes some singing sounds as well. She loves to dance to Yo Gabba Gabba, and she crouches down and shakes her booty or swings her arms left and right across her body. Her dancing is especially cute because she has a bit of a bubble butt! : )

It seems like they’ve had a cold for months now. It goes away for a week and comes back. Today we are in runny nose mode. A few days ago we were in sneezing-fountains-of-snot mode. I caught acute bronchitis at the very beginning of the year and was so sick. A doctor at urgent care prescribed me an inhaler, a z-pak, a steroid, and cough syrup. I was afraid I might get admitted to the hospital, and I was terrified of Violet or Layla getting it. Luckily, no one else in my house got it. It made for a very miserable couple of weeks! I’m ready for spring and hopefully healthier little girls.

Both girls are in a terrible hitting phase currently. We are consistent with our correction–we grip their hand and tell them “No No” and remind them that hitting is bad. We show them soft touches and encourage them to be nice. So far our correction has done little to no good. Most of the time when I correct them (especially Layla), they turn around and hit me. Ha! Time-out is just no good at this age, at least for our kids. We tried and they thought it was funny. We’ve been told by parents who have been there that this is normal. However, Violet also had a biting incident at daycare last Friday. I take one for the team and let her show interest in biting my index finger, then correct her when she does it. But it’s not clear to me how much it’s sinking in. I don’t want to have “that kid” at daycare who bites the other little kids, so I hope this phase passes quickly!

I must go, but next time I will post some recent pictures!

2012 … Another Year of Ups and Downs

2012 … Another eventful, unpredictable, crazy year for us. Reflecting on all that has transpired since last January, I feel a little bit exhausted! Still, I remind myself to count my blessings. First and foremost, I am blessed with little ones who we so desperately wanted. Because of my unique fertility challenges (a huge benign tumor that required my ovaries to be deconstructed and then reconstructed, plus stage IV endometriosis), there were no statistics we could rely on to hope that we would ever have children. We were told in 2008 that we should bypass a second round of IVF and try donor eggs. A few years ago, I would have never dreamed that I would have TWO little girls underfoot, and yet here we are. Very lucky!

I so wish, however, that Violet and Layla would have been blessed with a smoother 2012. It was no picnic for me, either, but they were the ones being poked, prodded, and operated on. They have developed medical professional anxiety and have spent way too much time in hospitals. I am counting my blessings to have access to great medical care in our area, but just wish it wouldn’t have been needed!

In the spirit of not being too negative and counting my blessings, I present our highs and lows for this year. I will not be sad to bid this year adieu and am hoping that 2013 leads to much less medical intervention, zero surgeries, and plenty more milestones!

January Highlight: Layla rolled over for the first time!
January Lowlight: Violet had to endure casts for clubfoot treatment and a double tenotomy procedure, her first time under anesthesia.

February Highlights: Both V&L finally started pushing up a little during tummy time! Also, Violet finished the casting process for clubfeet and started wearing a much more comfortable brace.
February Lowlight: Tummy time made them hysterical and made us very nervous worrying that their femurs would fracture underneath their weight.

March Highlight: Six-month birthday!
March Lowlight: Layla was hospitalized for RSV.

April Highlight: Six months fracture-free! First teeth! (They have 12 as of the end of 2012.) First OI clinic. Started showing interest in books!
April Lowlight: Fourth PAM infusion… they are really starting to not like IVs!

May Highlight: Started rolling across the room! Started eating solids and babbling. Violet started wearing her clubfoot brace at nighttime only.
May Lowlight: ??? (The fact that I can’t remember is a good thing!) : )

June Highlight: Lots of walking outside with the stroller in nice weather! Oh-so-close to crawling, and starting to notice more about the world. Violet started sitting up by herself!
June Lowlight: Craniosynostosis diagnosis, Roseola, another round of sickness earlier in the month, and another PAM infusion.

July Highlight: Violet was finally considered strong enough for a regular clubfoot brace. Started pulling up. First swim! Crawling everywhere.
July Lowlight: Started preparing for surgery

August Highlight: Layla pulled up to a standing position on her own for the first time! Watching our baby girl bounce back from major surgery!
August Lowlight: Surgery and everything associated with it.

September Highlight: First birthday!!!!!
September Lowlight: ENT evaluation and plans for Violet’s ear tubes.

October Highlight: First “real” Halloween! (Last year they were six weeks old and had broken bones, so there were clearly no costumes or fun to be had at our house.)
October Lowlight: Violet’s third procedure, ear tubes

November Highlight: Layla started walking!
November Lowlight: A very stressful and exhausting PAM infusion

December Highlight: Violet started standing on her own for a few seconds at a time. Layla started singing to Twinkle Twinkle Little Star, and Violet started doing hand gestures to Itsy Bitsy Spider. Started climbing up our stairs by themselves (with us hovering closely behind).
December Lowlight: Lots of yucky sickness, but nothing major.

New Pictures!

What we’ve been up to in December…



Violet practicing standing just before heading out to our favorite local Thai food restaurant!





Layla showing off her new walking skills


Layla giggling while I tickled her.


Violet giving me some giggles before heading out to see Santa Claus.


Layla’s pretty green eyes.


Violet practicing standing in her new chair.



The biggest milestone-related news is that Layla is walking everywhere now! The first time she walked from one end of the living room to the other was November 28th, and she stumbled every couple of feet. She has improved every single day after that, and is getting fast! No more crawling for her!

She is still the feistier of the two. We call her our firecracker, because she can explode! She’s starting to throw tantrums that involve throwing herself on the ground and banging her head against the floor. Scary, but at least it’s not the twin who is four months removed from a $250K skull surgery.🙂

She also appears (right now, anyway) to be more of the schemer. When she knows something is forbidden (like lifting the heavy lid of our ottoman that has caused some sore baby fingers in the past), she peers at me out of the corner of her eye while she attempts to lift it. I tell her No-No and she bursts into tears. She 100% knows she is not supposed to do it, but she tests me.

She does most of the talking at this point. She says “Hiya!” and loves to go “Mmmmmmmm-mamaaaaaa,” really dragging out the M sound. She voices her approval over foods she likes. She loves to press buttons, including endlessly turning our DVR on and off. This has resulted in her Daddy placing duct tape over the buttons, but that hasn’t stopped her from peeling off the strips of tape. She is tenacious and already can be relentless when she wants something.

She is VERY sociable, grinning broadly whenever she gets extra attention. A family member, someone she had only met once briefly before in the waiting room during Violet’s surgery, visited our house last week and stretched her arms out for Layla to come to her. Layla flashed her a huge smile and beelined over to receive the hug with no hesitation. She loves people and also laughs hysterically at our dogs when they play. Recently one of our dogs had a little bit of a cough because she had apparently eaten some leaves outside, and Layla laughed at every cough. I guess she thought it was a funny noise!

Layla plays peek-a-boo, and it sounds like she tries to mimic us when we sing Twinkle-Twinkle-Little-Star. When I sing to her, she claps loudly afterwards and yells Yaaaaayyyyy. She has been practicing turning in a circle while she stands up, which is funny to watch.

Violet is, in a lot of ways, the polar opposite of Layla. Not in every way–they are quite a bit alike, but in some ways they couldn’t be more different. Violet is very reserved and cautious. Whereas Layla ran straight for the family member who visited, Violet wouldn’t even look her way.

My husband thinks Violet is going to be Type A or have a little bit of OCD. She is very methodical in so many ways–she has the most precise pincer grasp I’ve ever seen. Whereas Layla drags out toy after toy, Violet picks them up and puts them away. She seems to prefer order and routine and structure.

Violet is also very tidy when she eats. She makes an effort not to dirty her face when she eats. I think she will be a very well-groomed adult!

Violet is not walking yet, and is only just now starting to try to stand independently. She is very obviously afraid of falling and only attempts to stand when she deems it safe. Sometimes her choices are questionable–for example, she received a little armchair for Christmas, and for whatever reason, she has deemed it safe enough to attempt standing upon. Of course, she runs the risk of toppling off the armchair and onto the floor, so I’m not sure why! On the flip side, if you hold both of her hands and gently coax her to stand by herself, she has deemed that an unsafe situation. She becomes afraid that you’re going to let go, and she immediately sits down. We’ve learned that what seems to work is placing a pillow on the floor. We say, “Violet, will you stand?” and she pushes off on the pillow and stands for a couple of seconds. She also pushes off my husband’s chest if he’s stretched out on the floor. She’s getting better and better at this and sometimes stands for 5-6 seconds at a time. She still lacks balance, though.

I believe I may have babied her too much because of her various medical issues and allowed her to become a little less independent. If you direct the girl to a push toy, she can stand up and show that thing who’s boss. But when it comes to independent standing/walking, she is just not there yet. She lacks a lot of confidence. Her clubfeet might play a role, but I’m not sure. I’m not going to worry a lot about it until she’s closer to 2 years old, because others have reassured me that some kids don’t walk until closer to that age. I’m hoping that one day it will just click.

To compensate for not walking yet, she walks on her knees and is really good at it!

The biggest milestone for her lately is that she has starting doing hand gestures to the song “Itsy Bitsy Spider.” She just started doing this a few days ago, and it is pretty neat!

Since putting tubes in Violet’s ears, she is vocalizing a lot more. It’s mostly babble, but “s” and “z” sounds primarily. However, this morning it sounded like she tried to say “Bye bye, Da Da.” She mixes up D and B sounds. For instance, when playing peek-a-boo, she says “Da” instead of “Boo.” But it’s an attempt, at least! She says dada all the time.

Violet hides in corners or behind objects and waits for you to find her. At Christmas, she kept wandering back into my in-laws’ hallway and waiting for me to peek at her. Then she would laugh. She does the same thing with her sister in our living room–she hides on one side of the couch and waits for Layla to find her, and then squeals with delight. She clearly understands cause-and-effect.

Violet loves stuffed animals, and also loves to feed us food. She holds out a Cheerio or Goldfish and laughs excitedly if we eat it out of her hand. She has even tried to offer me a sip of milk from her sippy cup. She seems like a born nurturer. Layla, on the other hand, will place a Cheerio on my palm but then snatch it back and eat it! : )

They fight terribly! Probably the most surprising thing to me about having twins is that they don’t seem to have this unbreakable bond yet that I hear so much about. We really never allowed them to sleep close to one another because of SIDS fears, and we had to separate them when they were so fragile because they may have somehow caused the other one a fracture, so I wonder if that’s why they’re not as close. At this point, they have slept in their own cribs since January and prefer having their own space.

Layla is assertive and rips toys right out of Violet’s hands. Violet vocalizes her displeasure but doesn’t attempt to fight back. (I am working on this behavior, btw.) They head-butt each other and stick their hands in the other one’s face to block them. They have each bitten each other at least a few times. I plan to be very consistent about correcting this behavior, but first they have to fully understand that “no-no” means they are being corrected. Layla knows better at this point, but Violet is just now grasping the understanding. I told her No-No yesterday about something else and her face crumpled, so I think it is sinking in.

They are still picky eaters. They love bananas the best over anything else. Violet or Layla yells at me while I shovel a piece of banana into the other one’s mouth, until I return back to the first one and shovel in another bite. Goldfish and cheese round up second and third for favorite foods. They love apples and peaches, yogurt and animal crackers. They’re turning into little carnivores and love turkey sausage and bacon, hot dogs, pork chops, pepperoni, etc. Layla loves eggs and chocolate (obviously not served together), and Violet hates both. They are still very texture aversive, and it’s difficult to find things they will eat. They hate veggies and a lot of fruits. We started out striving to feed them only healthful foods and at this point are just happy that they eat! We try hiding veggies in their food, and they pick them out or just refuse to eat. Violet loves whole milk and could seriously go through a half-gallon every day if we let her. We limit her to a few cups a day and offer her water the rest of the time. They still have a nighttime bottle, but we’ll have to wean them off of that soon. Our goal is to be completely done with formula by 18 months. Their dentist suggested that we start offering them a bottle filled with water instead of formula. We haven’t started this yet, but we’ll see if it works. Honestly, I am savoring their last few months of nighttime bottles, as that is really one of the few baby things left in our lives!

I could go on and on about all the things they are doing, but I lack time or memory. It is amazing to see all that they are learning and understanding from one day to the next. They are so incredibly precious to me, and my bond with them continues to grow. Words cannot express how thankful I am to have the privilege of seeing them achieve all of these milestones! A year ago, they were three months old and basically still little blobs. To be able to interact with them and help them learn and grow is the greatest reward of my life. I am very lucky!

15 Month Well Child Visit

Stats from V&L’s 15-month appointment this morning:


Weight: 22 lbs 5.1 oz (40th %)
Length: 29.5 inches (21st %)
Head Circumference: 19 inches (98th %)


Weight: 22 lbs 3.2 oz (38th %)
Length: 30 inches (35th %)
Head Circumference: 18.5 inches (87th %)

Apparently they have some big heads! Must mean they are really smart. : )

They received two vaccines today, and they were understandably not happy about that. They have upper respiratory infections, so they were already not thrilled in general. I had to wake them up early to be on time for the appointment, so they started the day a little grouchy. I crept into their room and watched them sleep for several minutes before waking them up… sleeping babies are so angelic! When I have to wake them up early, they have drastically different reactions… Violet stands right up and starts jumping up and down in her crib with a big smile and some giggles (much like my husband, who is a morning person), whereas Layla usually wakes up and starts crying (much like me, a grumbling grump for the first few minutes of the day).

Because we can never go to the pediatrician without some type of new diagnosis, the doctor today diagnosed Layla with KP (Keratosis pilaris). It’s basically a minor skin condition that causes little red bumps to appear on the backs of her arms. She already has eczema, so we had been applying tons of lotion to the backs of her arms, thinking that it was just a particularly dry spot. But when I actually took time to examine the backs of her arms last night, I noticed that it was actually a rashy-looking patch of skin. It doesn’t seem to bother her, but they recommended that we put cream on it twice a day.

This is gross, but maybe good information for a parent in the same situation… I also asked today about a rather large skin tag that Layla now has in the crack of her bottom. I never noticed it until her ER visit for her anal fissure, and I didn’t know if having a skin tag in that vicinity of her body would have any negative implications. The doctor said it’s common after a fissure or after a bad bout of constipation, and that it’s nothing to worry about. Good to know!

I’m surprised at how similar their weight is to each other. They have different body types… Layla’s legs are longer, Violet has adorable thunder thighs, Layla has a narrow bottom and Violet’s is a little rounder. Violet was nearly 2 lbs heavier than Layla at birth, so it’s amazing to see how Layla has thrived and caught up.

Thankful that they are doing so well and that we can mark yet another medical appointment off the list. The next well child visit is at 18 months in March. They are getting some serious medical environment anxiety (Layla started crying the minute we entered the exam room, before anyone had even paid any attention to her), but we are dealing with it. There isn’t much I can do about it, but I feel terrible seeing them cry like they did today. In one year, they have had the following medical interventions:

  • Eight broken bones between the two of them (plus a few false alarms that resulted in an ER visit)
  • Eight Pamidronate IV infusions
  • 10 or so pediatrician visits
  • Casts for clubfoot treatment
  • Double-tenotomy surgery for clubfoot treatment
  • Fittings for two braces for clubfoot treatment
  • OI clinic
  • Exams and CT scan for craniosynostosis
  • Complete skull and frontal orbital advancement reconstructive surgery for craniosynostosis
  • Two craniofacial clinic days and evaluations
  • Exams for hearing concerns and plans for tube placement
  • Tubes placed in ears
  • ER visit for anal fissure
  • Tons of PT and speech therapy since February

I’m sure I’m leaving some stuff out, but that’s a lot of medical appointments all by itself. Even when the medical issue applied only to one child, I had to take both of them most of the time because my husband obviously had to work. The unaffected child sitting in the stroller still felt the anxiety and stress of her twin, I believe, and learned to associate medical environments with the crying that ensued. I’m sad for them… I wish they hadn’t had to deal with any of this, but their OI had to be addressed, Violet’s clubfeet and craniosynostosis had to be addressed. Broken bones had to be x-rayed, vaccines had to be given. I tell myself that they are lucky, that many other babies deal with so much more, but I don’t want to invalidate their feelings. They don’t understand why they have to hurt, why they have to get poked and prodded, and their feelings are justified. It’s just a shame that they are now so fearful of these appointments. And I’m sure as they grow older and more aware, it’s going to get worse!  Blah! : )

Next up: Milestones update!

Ear Tubes and Muenke Syndrome

I failed to post at the time that Violet had tubes placed in her ears October 9th, on her two month “cranioversary” (in other words, two months since her skull surgery). We became increasingly concerned about her hearing capabilities because she was barely vocalizing at all, and the very little vocalization she did sounded strange, almost as if she couldn’t hear herself. I would describe it as very nasally and sometimes it sounded more like groaning or moaning. Many times she would not turn to sound, and we had been told for months that she had fluid in both ears. She has only suffered maybe one or two ear infections, but the fluid was a constant presence.

Additionally, at her post-op cranio clinic appointment in September, a geneticist told us that he thinks she has something called Muenke Syndrome. Our research leads us to believe that she doesn’t exhibit very many of the symptoms, but apparently Muenke Syndrome is often demonstrated by head and feet abnormalities. For Violet, the connection is her craniosynostosis (specifically relating to the coronal suture that was prematurely fused on the left side of her skull) and her bilateral clubfeet. Many patients with Muenke Syndrome also suffer from hearing loss, so that combined with her speech delay led us straight to an ENT for evaluation.

The ENT observed the fluid in Violet’s ears and wanted to start a six-week steroid regimen to attempt to clear up the fluid. He said her hearing was equivalent to a person just stepping off of an airplane. He admitted that because of her craniosynostosis and recent surgery, this treatment option was unlikely to work, and tubes were probably inevitable. He figured the steroid was worth a shot. I left his office with the medicine, but immediately had “buyer’s remorse” and started thinking that we should bypass the steroid and move forward with the tubes. Six weeks is a long time to wait for the inevitable, and I didn’t want to worsen her speech delay. Plus, he pretty much told me that the steroid would not work. After talking with my husband, I called the ENT’s office a couple of days later and asked to bypass the steroid. Thankfully, her ENT agreed immediately. I was all prepared to advocate on her behalf, but he made it easy on me!

Here she was in pre-op just before the procedure…


The procedure was no big deal at all, just as we had been told by friends. I believe she was the first procedure of the day, so we were there bright and early (5:30, I think). The ENT was ready and waiting, so she was actually taken back about 40 minutes ahead of schedule. I had my usual paranoid discussion with the medical staff about how to handle her because of her OI and then walked with her to the door of the O.R. This time was much less traumatic than last time, since I knew this surgery was very minor. I went back to the waiting room, and literally seven minutes later, the ENT walked in to let me know that she was done and waking up. It was so quick!

I went back to the recovery bay, and she was TICKED OFF. She must be getting so tired of all this medical intervention, and she let me know it! She screamed and cried for about a half hour as I struggled to feed and dress her by myself (Daddy was at home with her sister). Finally she calmed down, and we were out of there. By the time she arrived home, she was totally fine. We’ve had no issues with the tubes whatsoever.

Everyone has seen a huge improvement in Violet’s hearing and speech since the tubes were placed. She is seeing a speech therapist for monthly evaluations just in case, but there is no question that she is hearing and starting to vocalize more. At her two weeks post-op ENT appointment, she passed a hearing test in a booth, so the discussions about sedating her for an ABR (auditory brainstem response test) ceased. She is now saying “Da-da” and often sounds like she is babbling consonant sounds. She mimics a LOT of sounds now, even when she hasn’t turned to look at us. She plays peek-a-boo and says “Da!” (instead of Boo), and she fake-sneezes by bobbing her head and saying “Ahhhh-cha.” We feel much better about her hearing now, and the speech therapist is impressed with her progress.

We have an order from the geneticist to have her blood tested for Muenke Syndrome, but we haven’t gotten around to it yet. I want to wait for a week during which we have no other medical appointments, and sadly that is a rare occasion. I hate for her to be messed with too often during a given week. We want to have her tested for a few reasons:

  • If she tests positive for Muenke Syndrome, she is still at risk of hearing loss as she grows. Knowing for sure can enable us to receive proactive assistance from her school someday.
  • She has a 50/50 chance of passing it on to her children if she has the condition.

Her hearing will continue to be monitored very closely. She goes back to her craniofacial surgeon in February and will have a hearing test there, we have OI clinic in May when she will likely have another hearing test, and then we’ll move forward from there. Several doctors have suggested we have her tested at least twice a year, so she has many years of hearing tests in her future. Hopefully we can catch any problems as they arise, but for now the tubes are a success!