Sixth and Seventh PAM Infusions and ER Visit

Last week, I took V&L back to Omaha for Pamidronate infusion #7 (for Violet) and #8 (for Layla). Layla has now received one more infusion than Violet because our local hospital made a mistake. When Violet had her skull surgery for craniosynostosis in August locally, we decided to do the 6th Pamidronate infusion while Violet was in the hospital, healing from surgery. They were due for it the following week anyway, and I couldn’t imagine bringing Violet back to a hospital for more needles after just going through major surgery.

Layla received her infusion a couple of days earlier than Violet, and it went fine. After Violet’s infusion a few days later, the staff came in and admitted that they had overdosed her on Pamidronate. She received more than three times the calculated dose, and more than half of the amount she should receive in a year. Obviously, we were livid. Thank goodness it wasn’t a narcotic or a medication that could have damaged her organs!

The OI clinic doctors decided that she should therefore skip the October infusion to balance out the amount of Pamidronate in her body. That means she went 16 weeks between  infusions (August 13-November 30!). We were very afraid she would become fragile toward the end of the 16 weeks after going so long without the medicine. Luckily, she was fine.

It is getting VERY difficult (and exhausting!) to manage the girls during infusions. Oh, how I miss the days when they would hang out in their bouncy seats and allow me to entertain them! Oh, how I miss the days before they developed opinions and hospital anxiety! Don’t get me wrong–I love watching my babies grow and develop and learn, but each infusion has been more complicated and stressful. My hubby came with us for the October infusion, and we were both absolutely exhausted afterwards from chasing them around and keeping them from pulling out their IVs. There is no baby-proof area in the Infusion Center, so we had to watch them like hawks. I learned a few things from that experience and came better prepared this last time.

First of all, I brought their booster seats and lots of yummy snacks. Intermittently, they sat in their booster seats and played with toys and ate snacks. That was a godsend! Second, I finally spoke up to Child Life Specialists and requested at least one exersaucer, and they went to another floor and retrieved one. The only negative to this was that the seat swiveled all the way around, so they kept spinning in circles and getting wrapped up in the IV tubing. That was an easy fix though. Third, my dad brought the pack and play he keeps at his house, but we never needed it. My thinking was that I could place them in the pack and play with toys and let them play if I needed a rest. They will not nap at the Infusion Center (too much to look at, I guess!), so at least we had plenty of ways to contain them this time around. It helped my exhaustion level quite a bit.

We were in the Infusion Center from 10-5:30. When I was changing their diapers before heading home, I was horrified to find a bunch of blood in Layla’s diaper. A nurse quickly called the OI clinic, and they sent the diaper off to the lab to confirm that it was actually blood. Long story short, the lab confirmed that it was blood, so we ended up in the ER. After more than three hours (during which I had to entertain increasingly fussy and hungry 14-month-olds), they diagnosed it as a fissure. One of the possibilities was that it was a blood clot, so her actual diagnosis was a best-case scenario. We’ve been using cream and warm baths to help, and she’s had no more bad diapers. It was gross!

We were in the ER until 8:30, and I ended up driving home. We arrived home a few minutes before midnight, and fortunately they slept almost the whole way. It was a really miserable day for all three of us! My dad and stepmom were nice enough to come to the hospital and help me out with Violet, so that was lucky. I’m hoping our next infusion at the end of January is a smoother experience!

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First Birthday

My girls are growing like weeds, and we were ecstatic to celebrate their 1-year birthday on September 13th. They were kind of sick on their actual birthday, so we decided to lay low at home. We did dress them up in their “It’s My Birthday” tanks and cute tutus (a gift from a family member) for an impromptu photo session.

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Thankfully, they were feeling better on the day of their birthday party, September 15th. About 25 of our closest friends and family arrived at our house to celebrate. It was a beautiful day outside, so we enjoyed the outdoors in our backyard.

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We wanted to prepare lunch for everyone, so we scheduled the party to start at noon. That is V&L’s naptime, so the rest of us enjoyed lunch and visited for a few hours while the guests of honor snoozed in their cribs. My hubby smoked pork shoulder and I prepared a bunch of food myself. Not sure we’ll go to that much trouble for future birthdays, but it was fun this year!

The loose theme of the party was “You Are My Sunshine.” The cake baker modeled the cakes after their invitations:

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Otherwise, we decorated in bold colors–red, blue, green, orange, yellow. I didn’t want to be too theme-y; that’s not my style. We ran out of time to fully decorate as we had envisioned, but that’s okay.

Around 2, V&L were ready to join the party. We set up their booster seats in our backyard in an attempt to keep our kitchen free of becoming a smash cake disaster zone, and we set their cakes in front of them.

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Violet did not like it much. She is meticulous when she eats, with a very precise pincer grasp, and didn’t think much of digging her hands down into the cake.

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Layla, on the other hand, completely destroyed her cake. She was fun to watch!

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Everyone said they might have upset tummies afterwards or have some bad diapers, but they seemed completely fine afterwards. Lucky us!

They received plenty of new toys, and it was so much fun to celebrate with those who are closest to us. Violet and Layla are so lucky to have so many people who care about them!

Hateful Comment

Recently I received my first-ever hateful comment. I’ve been lucky that almost everyone who has reached out to me by commenting or emailing me has been supportive of our decision to move forward with IVF despite the fact that my Osteogenesis Imperfecta could be inherited by my offspring. This is what the commenter wrote:

 

So you have OI and knowingly used your eggs to create embryos that would have a 50/50 chance of a potentially devastating disease? And CCRM went along for the ride. I’m speechless.

 

I wrote this person back and haven’t received a reply, so not sure if she was a troll or is simply choosing not to respond. I sent the comment to my trash instead of approving it, but I want to address it in a post in case others out there are thinking the same thing.

I have Osteogenesis Imperfecta. I have had about 12 fractures, mostly occurring my first few years of life. In my family, our bones are most fragile when we are newborns. I have knee problems and have had four knee surgeries. I wore a lift on my left shoe as a kid because femur fractures affected my growth plate and made one of my legs slightly longer than the other, rendering me very clumsy and prone to falling. My joints pop and crack when I walk sometimes. I became fragile again while pregnant with my twins and hurt myself quite badly, so my days of adding to our family are over (I had my tubes “tied” during my c-section). I need future surgeries on my right shoulder and both legs to improve my quality of life. I spent some time in doctor’s offices over the years, but much more of my time outside in the fresh air, doing what normal kids do. All of these things are true, and they suck, and OI at times has been a bit of a burden.

But you know what? I also grew up pretty normally, playing softball and basketball. I ran in P.E. and played with my friends after school. I rode my bike, swam constantly during the summers, and played tennis. I graduated high school, earned my college degree, fell in love and got married, and turned my attention toward having kids. We met with no less than three geneticists to discuss the impact of OI on our future children. We understood the risks and took the leap. I knew that because my quality of life growing up was so good, my kids would have a similar experience. My mom may not have let me play soccer or roller skate (pretty much her two big no-no’s), but I would say my childhood was pretty great. There has been nothing “devastating” about my own experience with OI.

Furthermore, at this point I have met a LARGE number of families who have MUCH more severe OI kids than what I personally have experienced with V&L or myself, and guess what? Their kids’ quality of life is pretty great too. They may spend more time in a hospital like I did, but they feel joy and feel love from their parents. I have witnessed it personally. And I am 110% sure that not a single parent I have met has thought twice about bringing life into this world, despite the risk of passing on this disease. These kids are a gift, no matter how severe their condition. And unlike when I was a kid, there are minimally invasive treatments available to improve quality of life. When I was little, bone rodding was a very invasive surgery with a long, painful recovery time. Nowadays, it’s the complete opposite. When I was little, there were no Pamidronate infusions like my girls receive to improve bone strength. These two therapies have turned even severe OI kids into walkers and runners, and research continues.

OI is part of who I am, but not all of who I am. I am also a wife, daughter, sister, and friend. I am also infertile, and wanted a baby more than anything just a few years ago. I’m guessing if you are reading this blog, you are likely going through infertility or are close to someone who is. So you are probably familiar with that deep yearning in your heart to expand your family. That yearning consumed me once we decided the timing was right to have a child. I really didn’t feel at the time that mild OI was any reason to quell that yearning and remain childless. And I’m so glad that I made that decision, because my children, OI or not, are incredible.

I think this commenter is also trying to say that CCRM should have stopped me from using my own eggs because of this mild condition. Are they expected to know about and test for every possible gene defect? I volunteered the knowledge that I have OI, but I could have just as easily decided not to share that information. What about parents who carry a gene for cancer, for heart disease, for diabetes, for high blood pressure? Technically, these could be potentially devastating diseases as well. Does this mean that no one should procreate, that the human race should just end because we are all genetically flawed? Of course not. A genetic counselor at CCRM sat down with us, explained the 50/50 risk, and left it up to us to decide if we were prepared to welcome a baby with OI. Of course, we had no idea what we were getting ourselves into, but in hindsight I wouldn’t change it for the world. We could have screened out OI by sending our embryos for testing in Chicago, but I chose not to. What if my parents had had that option? Would they have passed on me? I believe this is a VERY personal choice, and I don’t judge anyone who decides to screen out a serious medical condition, but I just couldn’t do it. And I am glad.

What do you think? I will not be offended by your responses, but am curious to hear your thoughts. The commenter left an email address, and it is hjgreene66@yahoo.com (she listed her name as Hanna).

Rant over. My next handful of posts will focus on getting caught up!

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Ahhh… there are not enough hours in the day! I have been meaning to write ever since the surgery… thank you to Meg for reminding me during a time when I have a few minutes!

Violet did GREAT during her surgery! Here is an update in pictures:

She was the happiest girl in the world just before getting wheeled off to the operating room:

Her surgery lasted SEVEN hours! The surgeons were oh-so-careful with her because of the bone disease and the fact that kids with OI tend to bleed more, so they certainly took their time! Here she was just after surgery, in the PICU:

She had IVs and tubes everywhere… IVs on every limb, a Broviak catheter (also known as a central line) in her groin, a catheter, blood pressure cuff, monitors for her vitals, etc. She was hysterical because she was disoriented from anesthesia and because her pain meds hadn’t caught up to her pain level yet. The next few hours were among the worst of my life.

The day after was the worst day of swelling:

Her left eye swelled shut and she started to bruise. However, her spirits improved dramatically when her catheter was removed and we could pick her up. Within a couple of hours, she was clapping and smiling again. It was incredible.

Two days after surgery, her bandages came off. Check out this major incision:

We estimate that she has 60+ stitches in her head. They are dissolvable, thankfully! It sure wouldn’t be fun to hold her as all those stitches were removed!

Later that day, Violet was in a great mood and really showed us that she is an incredibly tough little girl!

Her surgery was on Thursday morning (Aug. 9), and she was released on Monday, August 13th–their 11-month birthday! We were so glad to come home, and she has been doing so great with recovery ever since. It is amazing to see how quickly she is healing.

She was fitted for a helmet while in the hospital and received one a few days later. Her surgeon felt that because the bone disease causes bone to be lesser quality than normal, she should wear it when out of our care for protective reasons. So if she goes to daycare, she wears it, or if she is being particularly rambunctious. We don’t want her to hit her head and break one of those healing bones or damage a plate or screw that is holding everything together.

The weekend after Violet came home, we dressed them up to attend a family function. We usually don’t dress them alike, but these dresses were a gift from my mom and were too cute!

Layla’s on the left, and Violet’s on the right.

Both babies are starting to enjoy some solid food, finally! They are very picky about flavors and textures, and more often than not refuse what we feed them. I started out feeding them only made-at-home purees, but found that all they were eating consistently were bananas and sweet potatoes. We branched out and started in with the Gerber products–Puffs, Cheeto-like snacks, yogurt melts. They do like those, of course, because they are pretty much junk. They liked Cheerios briefly but now spit them out–must be too bland. They’ve refused all of our table food for a few months, but are finally starting to turn around. They like pizza and macaroni and cheese, so far!

Certainly not nutritious, but we are just happy that they are eating anything off our plates! We’ve offered them healthier options–blueberries, pieces of grape, corn, etc.–but they have refused it all. Work in progress, I guess.

They are crawling like crazy. Layla still only army-crawls, but she’s still getting wherever she needs to go, so we guess that’s okay. Violet crawls, and is quick! They are both also standing. Layla pulls up effortlessly on everything and has “cruised” a step or two. Violet can stand, but only if you stand her up. She’s a little behind, which I have read is normal with clubfoot treatment. She is also really scared of falling, and that is something we’re going to work on.

Now we are planning for their first birthday! They turn ONE on September 13th–only 15 days away! I can’t believe it. This has been an incredible year on so many levels. I just look so forward to watching their personalities emerge even more and for them to accomplish milestones and all of their goals. We are so lucky!

Surgery Plan

Violet’s surgery is scheduled for Thursday, August 9th, first thing in the morning. Upon closer analysis of her CT scan, she was diagnosed with both unilateral coronal craniosynostosis AND sagittal craniosynostosis. This means that the suture on the left side of her head and the suture at the back of her head have fused prematurely. In addition, her right orbital rim (the big bony ridge just behind the eyebrow) sits back 4mm further than the left, so that will be reconstructed during surgery as well. I took her to see the neurosurgeon on the 13th, so that he could look at her head and take some notes before surgery.

Here’s how the operation will work: we’ll arrive at the hospital at 5:30 in the morning, and she’ll be prepped and then go to sleep under anesthesia at 7:30. Then, she’ll receive a central line. This PICC line will be threaded through her groin up to her superior vena cava. Having the PICC allows the staff to give her blood more easily, since she will need blood transfusions during surgery. Next, the craniofacial surgeon will expose the skull and mark the bones that need to be taken off. The neurosurgeon steps in and removes those bones, with all precautions to avoid touching the brain in any way. Then the craniofacial surgeon molds the bone as necessary to reinstate a proper skull shape. The craniofacial surgeon will also be the doctor who reconstructs her orbital rim.

The surgery will last approximately four hours. She’ll be in the PICU for most of her stay, where she can be closely monitored. The scar will be big, but it’s done in a zigzag fashion so as to be less noticeable. Doctors used to cut horizontally from ear to ear, and that was obviously much more noticeable. Her head is going to swell tremendously afterwards–her eyes will swell completely shut, and some babies even get blisters all over their forehead because of the swelling. She’ll wear a little cap on her head afterwards for a few days to protect the skull, but after that she won’t need a helmet or anything. Some doctors choose to employ the use of a helmet for further reshaping, but Violet’s surgeon aims to have her skull shape corrected before she leaves the OR. She’ll be feeling much better within a few weeks, though–it’s just the few days immediately following surgery that are going to be rough for her (and for us).

I am pretty much numb when I think about her surgery, which is pretty much all the time. I believe that’s my body’s way of protecting myself from the panic and anger and sadness that are boiling in my heart. I have no doubt that those four hours that she’s in the operating room are going to be the worst of my life. We’ve already met the nurse liaison who has the duty of relaying information from the surgeons to us, and she was very nice.

The last step before surgery is to attend a craniofacial clinic on August 3rd. It sounds similar to the OI clinic that we attended in April, during which specialists performed some evaluations. Because there is some risk of additional surgeries needed (she may develop soft spots the size of pennies that would require bone to be placed over them), she’ll attend this clinic annually for evaluation until she’s five. At that time, she’ll “graduate.”

Her head has become noticeably more misshapen during the last few weeks. Her brain is growing, and because it doesn’t have enough room, it’s pushing her bones out to compensate. So the left side of her forehead looks very misshapen, and her forehead in general looks asymmetrical.

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See the bulge protruding from the left side?

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The top of her skull looks like a bunny slope. There is a “valley” in the middle of her head that is becoming increasingly more prominent.

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We’ve noticed over the last few weeks that her neck appears to be very stiff, so that is a new concern. I actually took her to the ER last Tuesday night for neck and spine x-rays, because I was worried about a compression fracture. The x-rays were negative, but something is going on. Sometimes craniosynostosis causes vision problems, and babies can develop torticollis because they are trying to overcompensate for their lack of vision. However, I had thought this usually happened with very young babies, so I’m not sure. She doesn’t seem to be able to turn her head completely to the right, and she gets VERY angry when I try to stretch her neck in that direction. She also can’t seem to look down at me when I lift her up in the air (she looks straight ahead instead), and I feel that she arches her back when I lift her and that she hunches her shoulders when she tries to turn her head to the right. I addressed all of these concerns with the ER doctor, who didn’t seem terribly concerned. But I think this is one of those circumstances where it takes her mama to know that something is wrong. I have a call in to the craniofacial surgeon’s nurse to inquire about whether this is common for a 10-month-old with craniosynostosis, so hopefully I’ll hear from her tomorrow. The babies are scheduled for physical therapy on Thursday, but I’m going to see if I can move it up so that the PT can evaluate Violet. We certainly want to be proactive so the problem doesn’t worsen.

My next post will contain some milestone updates (they’re crawling!) and pictures of something other than Violet’s head. : )

Diagnosis

Violet was diagnosed yesterday with unilateral coronal craniosynostosis. A CT scan showed that the bones on the left side of her skull have fused prematurely. Left untreated, her brain won’t have room to grow, and developmental issues will occur. Surgery is the next step and will occur in August. A cranio-facial surgeon and neurosurgeon will separate the fused bones and remove a chunk of skull to give her brain room to continue growing. She’ll spend 3-5 days in the hospital. Obviously we are very upset and reeling from this news. It’s unbearable to think about sending my child off to an operating room to undergo a complex surgery. We have read that her head will swell tremendously and that the 48 hours after surgery can be difficult. I am just devastated.

The Latest–9 Months

I have been thinking about blogging, but it seemed overwhelming to sit down and try to cover everything that has been going on with us. And honestly, I wasn’t sure anyone was even reading this, so I wasn’t sure I wanted to bother. Thanks to Mini for commenting so I know at least someone is out there. Here goes…

Health

The biggest thing going on health-wise is that Violet has a new diagnosis: craniosynostosis. This is a birth defect in which some bones in her skull have fused too early. Her pediatrician diagnosed her after I broached the subject of her funny-shaped skull, and she referred us to a cranio-facial specialist. We saw the specialist last week, and we have a CT scan scheduled for this Thursday and should receive the results the same day. There are different types of this condition, and depending on what’s going on with her bones and brain, she may either need surgery or a helmet. The surgery sounds terrible (they take out a piece of her skull so that her brain has room to grow), and we’ve read that the first few days afterwards are rough, but babies are resilient and recover well. The helmet doesn’t sound like much fun either, but left untreated, she could have seizures or go blind. Clearly, we have to treat it. She will be sedated for the CT because she has to be completely still in the machine.

We are still fracture-free (knock on wood) since November 5th and 6th! There have been many, many situations lately that could have easily caused a fracture for an OI baby–a leg has become stuck between a crib slat (this happened yesterday, and the breathable bumper immediately went back on the cribs), or we’ve struggled to push the babies’ legs through a jumperoo or exersaucer. And our babies’ bones have stayed strong and unbroken through these incidents, which is excellent.

They received their 5th Pamidronate infusion last Tuesday at the children’s hospital in Omaha. Both babies have opinions now and were not happy about getting an IV. My husband had to work, so I drove there solo and dealt with the IV placements and immediate aftermath by myself. A few hours later, my dad stopped by to visit and ended up just staying the rest of the time. The College World Series was in full-swing in Omaha, and both teams that competed in the finals–University of Arizona and University of South Carolina–stopped by our room to visit. My dad loves baseball and was so thrilled to meet them, and they gave us signed baseballs that my husband was excited about. We didn’t get any pictures with the South Carolina team, but the Arizona starting shortstop and star pitcher each held a baby, and we snapped a few pictures. It made a long day a little more exciting, and I think the babies enjoyed the distraction.

An orthotist measured Violet today for a new clubfoot brace because she has pretty much outgrown the one she received in February. Her current one was custom-made for her and is especially lightweight, but her orthopedist thinks she is strong enough now to go with a standard brace. So we are going with the Ponsetti shoes and bar. We should be getting it in the next few weeks. She only has to wear it at night at this point, so hopefully it won’t be too bothersome for her. The bar is metal and is quite a bit heavier than her current carbonfiber one, but the advantage is that her current brace is experimental while her new one is proven to aid in clubfoot correction. She still has a tendency to pull her feet inward, and I have some fear of relapse. Her orthopedist always tries to reassure me by saying that worst-case scenario, if he thinks her feet are relapsing, he will cast her for two weeks or perform a tendon transfer surgery. He says this in the most nonchalant way possible. Obviously he has never tried to entertain a child in double casts or comfort a baby who has just become awake after surgery. I’m hoping the Ponsetti shoes and bar will continue to straighten out her feet. Currently the little Houdini slips out of her shoes every single morning when she wakes up, so it’s getting more and more difficult to keep them on her feet.

Layla is teething and recently had a miserable cold, but she is doing really well otherwise. I’ll post more about her below.

In my last post, I mentioned that we sent off Violet’s blood for DNA testing to receive an official OI diagnosis. There are many different types of OI, and while our symptoms are characterized as Type I, we wanted confirmation. Plus, if anyone ever becomes suspicious that our babies are suffering abuse or something like that, we can present the results as evidence. Anyway, the results are in, and we have a gene mutation that has never been described before. Similar mutations do cause OI, so the conclusive results are that she does have OI, but they cannot match her to a type because she doesn’t fit any of the existing categories. Very interesting. This doesn’t really mean anything, except that any geneticist would probably be fascinated by our DNA. We have a long family history of this condition (dating back to at least the Civil War era), and Violet was the first to have her DNA tested, so this is significant for our family.

Are you exhausted yet? Sometimes I am amazed at all of the medical appointments on our calendar, but we remind ourselves that at least none of these issues hinges on their survival. Everything is cosmetic or maintenance, and not life-or-death. In that sense, we are very lucky.

Milestones

V&L turned 9 months old on June 13, and developmentally they are doing great! A physical therapist works with them bi-weekly to improve their strength and meet milestones.

Violet is sitting up (as well as sitting herself up) with ease. She rocks on her hands and knees, but hasn’t connected the dots yet. She rolls and gets around pretty easily. She is discovering her voice and lately has been screeching at the top of her lungs randomly. It’s an assault to our ears, but we love it. She went through several weeks of repeatedly saying “ma-ma-ma-ma,” but lately we can’t get her to say it. We must have worn out our welcome by constantly requesting that she say it. She has some stranger anxiety and separation anxiety, which is new over the past week or so. She calms down instantly when I pick her up, which makes me feel like such a mom. And reminds me of how lucky I am! She weighs 18 lbs 5 oz and has plenty of rolls, and she’s 26.5″ long. She still has her same two bottom teeth, with no signs yet of the top two front teeth. She loves jumping in the Jumperoo, loves sitting in front of a basket of toys and exploring every angle of every toy. She loves tags on things. She loves splashing in the bathtub. She makes the funniest “mean” face where she scrunches her face up like an old lady and rapidly inhales/exhales. My husband’s family says he used to do the same thing. And speaking of my husband, she looks exactly like him. Everyone remarks on it when they see her.

Layla looks exactly like me, except she has my husband’s long eyelashes. There is no question that CCRM grabbed the correct tube of embryos! Layla army crawls across the floor and gets better at it daily. She also gets on her hands and knees and rocks, and maybe seems a little closer to figuring this out than Violet. She is suddenly sitting up with ease, finally! She says “da-da-da” (music to my husband’s ears). She is clapping, which is new as of last Friday. She also likes clicking her tongue to mimic us and also shaking her head back and forth. She is not as loud as Violet, but she cries a lot more forcefully. She doesn’t really have the stranger/separation anxiety yet and is pretty gung-ho about everyone who pays attention to her. She also loves splashing in the bathtub and breaks into a huge smile while we set her in the water. She has her two bottom front teeth, and now the top left front tooth is very nearly breaking through. She has been impossible the last two days because of it. I stay patient by reminding myself that it’s just a phase. : ) Layla is more dramatic and more demanding than Violet. She definitely lets us know when she needs something and when she’s not happy with something. She weighs 18 lbs 8.9 oz, so she is now 3 ounces heavier than Violet! She was almost two pounds smaller at birth, so she has really caught up. She’s a quarter inch shorter–26.25″ long.

Both babies are eating lots of fruits and veggies now, along with puffs and Cheerios. I think they’ve tried 18 foods now. Their favorite food seems to be bananas. They still sleep all night in their own crib–I don’t remember the last time either of them woke up in the middle of the night. We did the cry it out method, and it worked so quickly. It was the right decision for us.

Life in General

We have been tested so much this year, and as rewarding as it has been, it has also been exhausting and hard. I think it has taken a huge toll on me, but I just keep going and put one foot in front of the other. That’s all I can do. It has probably taken a toll on our marriage, but we are still going strong. Because I don’t work (although I am looking for a job now that the babies appear strong enough for daycare!), I shoulder the brunt of these medical appointments. I take them for shots, take them for clubfoot stuff, take them for their infusions, take them to the cranio-facial specialist, help with physical therapy, etc. Not to mention just the day-to-day care that goes along with 9-month-old twins. It can be a very lonely job. I am doing the best I can, but know that I could do better. When I return to work, my husband and I will share the medical stuff so that neither of us is taking too much time off from work, so I think that will help us to be more on the same page. I feel that I am my own very last priority, and it really shows in my appearance and my energy level. I need to work on that. My husband is wonderful and encourages me to do things for myself, but I just don’t have the energy most of the time. I am going to do better.

Pictures

#1–Playing on the floor together.
#2–Studying a Puff the first time we gave them one.
#3–Layla hanging out on the deck on a nice, breezy evening.
#4–Violet wearing her daddy’s baseball cap.
#5–Layla meeting University of Arizona’s starting shortstop Alex Mejia at Omaha Children’s during a Pamidronate infusion.
#6–Violet meeting Arizona’s star pitcher Konner Wade at Omaha Children’s the same day.

Bullet Point Parent

It has been over a month since my last post. V&L are now 7.5 months old and growing and changing so much every week! This is a really fun age. I am now officially a bullet point parent, as I try to summarize the most recent developments in bullet points below.

  • They are both rolling! And when I say rolling, I mean as in all the way across a room, just for the fun of it. My daytime routine now revolves around picking them up in order to get them out of the jams they’ve rolled themselves into–smashed up against the pack and play, partially underneath a sofa, pressed up against the entertainment center. Layla started first, and Violet followed a week later. They were a little late to achieve this milestone (about 6.5 months), but considering their challenging start to life, I think that’s not too bad!
  • They are eating solids! We started with green beans on March 16, a few days after they turned six months old. At first they were confused and pretty grossed out, but they have really caught on, . At this point we’ve tried rice cereal, oatmeal, green beans, avocado, white potato, sweet potato, bananas, and most recently apples. Layla has been relatively accepting of everything except white potato. Violet has hated everything except the sweet stuff–sweet potato, bananas, and apples. She LOVED apples yesterday, and my hubby couldn’t shovel it into her mouth quickly enough. Looks like she has my sweet tooth! : )
  • They finally conquered tummy time. This was a huge battle at my house. Those first several months of having to be in the pavlik harness and basically living in their car seats left their core muscles very weak. They would scream and get hysterical when we would place them on their tummies. Very traumatizing for two parents who had dealt with the broken bone scream five times in one month. I remember thinking they would never be able to lift their heads up. A therapist began working with them, and we became more committed to enduring tummy time even though we hated their screams. And eventually, they began lifting their heads up. Now they primarily play on the floor on their tummies with their heads held high and proud!
  • They have teeth! Layla cut her bottom right front tooth on April 15, and Violet cut the same tooth on April 17. Violet’s left bottom front tooth came through a few days later. We can see Layla’s left bottom front tooth, but we can’t yet feel it above her gums. Layla has been a little fussy and has a stuffy nose, but you wouldn’t really even know anything was bothering Violet. It is so cute to see their tiny teeth when they grin at us now!
  • Violet is less than a month away now (26 days and counting!) from transitioning to only wearing her clubfeet braces at night! I have been so amazed at how well she has tolerated the braces, but of course it will be nice to reduce the amount of time spent in them. Especially as the weather heats up, she’ll be so much more comfortable not having to wear socks and shoes during the day. Her feet look amazing!
  • We attended OI Clinic at the beginning of April. The babies had x-rays of their spine and hips, bone density scans, and then we met with several specialists: a social worker, nutritionist, physical therapist, occupational therapist, dentist, geneticist, and the orthopedist himself. Dr. E is also in charge of Violet’s clubfoot treatment, so he examined her feet and was very pleased with her progress. The bone density scan showed that their bone density is low (Layla’s is the lowest), but that is to be expected for a brittle bone disease. It is something we will monitor for many years. We found out that Dr. E and the geneticist are planning to write a case study on Violet, which is pretty cool. She is Dr. E’s first OI patient with clubfeet, and with his guidance last fall we made the decision to hold off on clubfoot treatment until she received a few Pamidronate infusions to strengthen her bones. The case study will show that although it is a good idea to start clubfoot treatment as early as possible after the baby’s birth, nothing is lost by postponing treatment in special circumstances. She will be anonymous in the case study, but we still think it’s neat! Layla will be mentioned in some degree also. The doctors promised to send us copies for their baby books!
  • Speaking of Pamidronate infusions, we just had the fourth infusion this past week. This time the nurse was able to access a vein in their hand instead of having to resort to their head. For the past two infusions, Layla has not even flinched when they have stuck her with the needle. She is one tough cookie. Violet cried a little, but not too bad.
  • After the infusion, the nurse drew 7 cc’s of Violet’s blood to be sent off for an official OI diagnosis. They have been clinically diagnosed based on fracturing due to little trauma, but we want the actual piece of paper identifying the type. There are eight types of OI, and our family history strongly represents that we have the mild form, Type I. But we just want to know for sure and figure it may help others in my family who have kids with the condition. None of us have ever been officially typed because even in my generation, this wasn’t really done much. Only three labs in the U.S. do this kind of testing, and Violet’s DNA will have to be mapped out in order for the gene mutation to be identified. It may be six months before we have a result, but it could be less. Some parents face child abuse allegations or even have their children take away by the system due to OI, so it’s a good idea for us to protect ourselves. We already had the babies’ first orthopedist write a letter that we could present at an emergency room explaining the diagnosis, but actual test results would be a lot better evidence.
  • And speaking of broken bones, if we make it through the next eight days, May 6th will mark six months without fractures! I’m majorly knocking on wood as I type this! Last October, we would never have thought this would be possible. They were breaking so often and despite our best efforts to be as careful as possible, basically handling them like glass and not letting anyone else breathe on them. Pamidronate has truly been a miracle drug for us, and hopefully it will continue working its magic!
  • One toy we wondered if they would ever get to enjoy: their Jumperoo. They love it, and so far they have tolerated the bouncing very well. We get nervous about these types of things, but have decided to treat them as normally as possible until we have a reason not to. So that is another new pastime at our house, to go along with the rolling.
  • Layla is babbling! She woke up six days ago saying sounds like “Dadada” and “Bababa.” Violet is not quite there yet, but I’m sure she’s not far away from achieving this milestone.
  • Nothing makes me happier than when they see me first thing in the morning. They sleep on their bellies now by choice, and they bang their little fists on their mattress because they are SO happy to see me. That daily greeting alone makes the process of cycling at CCRM a million percent worth the time and effort. It’s the best way to start out my day!
  • Speaking of waking up in the morning, they are great sleepers! We are finally on a really predictable schedule. Because they don’t go to daycare, they sleep until about 8:30, then nap from about noon-3. They eat five bottles a day and eat their solids right after they wake up from their nap. They go to bed at about 8:30 p.m. I cannot complain about this schedule at all!
  • I haven’t done a great job of prioritizing myself, unfortunately. As long as things continue to go so well with their health, I’m going to look for a job again in August. I want to enjoy the summer with them and then go back to work. I have been extremely lucky to have this time with them, but I think I have discovered that I need a job as part of who I am. I’m doing an important job, obviously, but I think I will value my time at home more when I have a career again. Nothing is stopping me from prioritizing myself, except I seem to lack the ability to flip the switch. Tomorrow marks one year since my first knee dislocation, the one that happened at work. From April 29 until the end of the year, I wasn’t in good enough condition to get out often. So I am committing to spending at least 45 minutes outside each day starting tomorrow through the end of the summer to make up for not being able to last year.
  • We decided to keep our three frozen embryos for another year, so we paid the $300 a few weeks ago to keep them stored. We won’t have any more kids, but we just didn’t feel quite right yet about discarding them. I checked with Danielle, the genetics counselor at CCRM, and she let me know that CCRM won’t allow embryo adoption to another couple who have a history of a genetic condition, even if the adopting couple consents. So our options were to discard or store.

Violet is demanding a bottle, so I must close this out. My next post will contain pictures!

Seeking Balance

My babies are over six months old, and they are becoming more delightful every day. I’m not sure if all moms feel the way I have, but bonding with my girls has been gradual. I loved them from the moment I first laid eyes on them, of course, but the bonding process was not immediate. At this age, I know what makes them laugh and what makes them mad. I recognize when they are tired and when they need to be held. I know which toys they like, and I recognize their hunger cues. I comfort them and help them to feel more secure, and their eyes search for me when someone besides my husband holds them. Suffice it to say, I am enjoying them more and more as they grow more interactive.

Life seems like it is getting slightly more manageable. We still have clubfoot treatment in our future, but we are past the serial casting process and the tenotomy, so the frequency of those appointments has lessened. We still have Pamidronate infusions, but those occur only every eight weeks. We still have bi-weekly visits from a physical therapist, but those appointments are no big deal. Their next well visit with the pediatrician isn’t until 9 months. They are recovering nicely from their RSV. We will have OI clinic annually and will hopefully have no more broken bones in our future. We have babies sleeping 10-12 hours per night in their cribs and napping during the day. Life has gotten easier, and I am grateful.

Now I feel like it is time to re-focus on myself. Of all the members of this household over the past six months, I have been my own absolute last priority, and it shows. During my pregnancy, I gained 35 lbs. I can’t believe I didn’t gain more, as I was absolutely starving toward the end and ate tons of (junk) food, both to satisfy my hunger and to help with the morning sickness that lasted my entire pregnancy. I was so incredibly swollen afterwards from pregnancy and my other injuries and lack of activity, I had to take a presciption medicine to help flush me out. So in the two weeks after V&L were born, I lost 53 lbs. My body maintained that weight for about a month, despite me eating whatever I wanted, as it was working hard to recover from childbirth and my injuries. And then the weight started coming back on. Over the past five months, I’ve gained back about 20-25 lbs. I blame it on depression from all of the medical intervention needed for my girls their first three months of life, all of the pain they were in and the helplessness I felt, having to quit the job that gave me social interaction, and my own pain and inability to be active because of my injuries. In addition, I was feeling overwhelmed by life with twin infants. I comfort ate, and I don’t blame myself for it because I was at my breaking point. But it’s time to change.

So I have joined Weight Watchers Online, partly because my friend LisainSK has had a good experience with it. Hopefully I can achieve similar success. When all 53 lbs of that weight was gone right after childbirth, I felt fantastic. So that is my goal for now. I’m tracking the things I eat and trying to plan our meals around more fruits and vegetables. I can say it certainly hasn’t helped to spend all day at home, with my kitchen just a few feet away. And it hasn’t helped to enjoy a rum&diet or two with my hubby in the evenings out on our deck after our babies are asleep (2 oz of rum is 5 points!). But this change is needed for my mental and physical health. Moderation and exercise should help me to shed some pounds before the really sweltering summer weather gets here. I’ve been walking outside with the babies more often and increasing my mileage, and I will give myself an expectation of doing that every nice day this spring/summer.

A friend of mine, who is 31 years old (only one year older than me), suffered a severe stroke near her brainstem on March 11th followed by a series of smaller strokes. She is basically comatose, with a trach and a permanent feeding tube, lying in a hospital bed 20 miles away from me as I type this. She was thin and in good health, and basically the stroke was bad luck, but it is a definitely a wake-up call to me that I need to focus on my health. I want to be here as my daughters take their first steps, walk into their kindergarten classroom, move on to middle school and all educational institutions beyond, meet their first romantic love interests, and accomplish their goals. So I will be keeping that in mind as I start making myself a little more of a priority around here!

A Surreal Problem to Have

We are joining the bandwagon of trying to figure out what to do with our three frozen embryos. What an amazing problem to have–when we were awaiting our Day 5 report after retrieval, I thought it would be amazing if we only had one or two blasts. I never dreamed that 18 months later, we would have two babies and three frozen blasts in the freezer at CCRM. How fortunate we are! This week we received the notice from CCRM that we need to decide whether to pay for another year of storage, or somehow discard our embryos.

Even if I would want to try for a third baby someday, it is a really, really bad idea. After my accident last May, it became apparent that my body cannot make enough calcium to nurture a baby and keep my own bones strong. My joints also loosened and caused my kneecaps to dislocate. I also became very iron-deficient, which seemed like the very least of my problems at the time but could pose a danger to any future embryos in my body. We’ve determined that personally for us, we are not comfortable with the idea of someone else carrying our embryo for us. It’s just not for us. There is no way to tell whether my body would behave differently with a singleton pregnancy, but no doctor can tell me any differently, so I don’t really want to find out.

Then there is the OI issue. My babies had a 50/50 chance at inheriting the gene, and they both have it, so that tells me we either have really bad luck or it’s a really strong gene. A third baby would have the same 50/50 shot. In the thick of the fractures last October, I felt briefly like I was at my absolute breaking point. I remember a day when my husband and I drove to our pediatrician’s office to pick up several cases of formula that their Enfamil rep had kindly hooked us up with, and we decided to grab some lunch nearby while a family member watched our babies at home. We were both so exhausted and emotionally spent, we barely even spoke. I think he would agree that we were both at the lowest point of our lives, which was so opposite of how we were supposed to feel as new parents. In retrospect, I know that we were “only” dealing with broken bones and not so many worse afflictions involving survival, but it seemed like the end of the world in that moment. I do not ever want to repeat that experience, so that has turned me off even more from wanting another child. We can test the frozen embryos for OI, but then that’s a moral issue for me. I have OI, so I might not be here if technology had been more advanced when my mom decided to conceive me. We may choose not to transfer our frozen embryos, but it won’t be because of OI.

So now we are wondering about embryo adoption. I need to talk to CCRM to find out whether my OI even makes us eligible for this. Would an infertile couple want to deal with such an affliction? OI varies SO much from person to person. Even in my family, those of us who are affected have had vastly different experiences. Some of us have only fractured a few times, some of us have had more like 25-30 fractures (which in the grand scheme of things is still relatively mild). I’ve had 12, so I’m about middle-of-the-road for my family. Some of us have lingering side effects, like my loose joints that cause kneecap dislocations, but some of us have no side effects. All I’m trying to say is that there is no way to predict how mildly or severely our frozen embryos may be affected. They will all have Type I if they do have it, which means there will be no skeletal deformities, but they could have a few fractures or dozens. Would dealing with this be better than continuing with life childless?

If we can’t or decide not to pursue donating our embryos, I would want to know whether our embryos could be used to benefit OI research. That would probably be the next best thing to actually using the embryos for the purposes of conception. But I want to research the other paths first.

Or of course, we could simply pay the money to keep our embryos frozen for another year. My fear about this is that as the memories of my accident and recovery fade, as our daughters grow strong and seem less like the fragile little creatures that they were last fall, my resolve about not conceiving again might weaken. I find myself wanting a resolution to avoid mixed feelings later.

It’s a lucky but difficult position in which to find ourselves. We have some thinking to do, but getting this all down has at least put all of our options in a place other than our heads.