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.

Overdue Update

Wow, I can’t believe it’s been so long since I posted! Time is speeding up, it seems. Here’s what we’ve up to since my last post:

Violet and I traveled to see her orthopaedist for her two-weeks-post-tenotomy check-up February 15th. The physician’s assistant used the very loud saw to cut off her casts, which terrified my poor baby. She screamed and her eyes were as big as saucers. But on the plus side, her feet looked great!

For comparison’s sake, here is what her feet looked like shortly after birth:

And here is what they looked like in January just before starting round two of clubfeet correction:

So as you can see, there has been much improvement! After her casts were removed, she and I settled into a bottle, and the orthotist arrived to take measurements for her braces. He created special, lightweight braces for her so that she wouldn’t have to withstand the weight of the normal braces.

Once the orthotist had completed his measurements, Violet had her last clubfoot-related casts applied!

She adapted to the casts quickly this time, and they never seemed to bother her. We continued with the next two weeks focusing on tummy time, introducing the exersaucers and jumperoo, visiting with family and friends, taking walks outside, etc. And before I knew it, the time had come for the next trip to the children’s hospital.

On February 28th, the very loud saw scared Violet again, despite the headphones we put on her head to try to help with the noise.

After the casts were gone, the braces went on! We are referring to them as her “shoes.”

It took her just a minute or two to figure out how to lift her legs in the air at the same time.

So far the shoes haven’t even seemed to faze her. For the next three months, we can only take them off briefly to bathe her or for general hygiene. Her feet are a bit big for the shoes right now, so we are doubling up on socks to try to keep her aligned in them correctly. After the three-month mark, as long as there is no sign of relapse, we can reduce the number of hours she spends in her shoes. She’ll definitely still wear them for naptimes and at nighttime, but her orthopaedist will determine how much she must wear them beyond that. I must admit that I’m a little concerned that her right foot is still slightly turned in. I’m making a conscious choice to trust her orthopaedist and not bring her back in there for my concern. She will be seen again in eight weeks for her next infusion, and I’ll bring it up then if I’m still concerned.

This post is getting rather lengthy, so I will cover their third Pamidronate infusion in my next post!

The Long and Winding Road

My heart exploded for my little family today.

I watched my 4-month-old baby get wheeled off to an operating room to have her Achilles tendons snipped on both feet. Before anyone thinks I’m too melodramatic, I know that so many families deal with so much worse. But I’m sorry… there is nothing normal about your infant having a tube shoved down her throat and being put under. We were at a children’s hospital with staff taking very gentle care of her, but still… it felt unnatural. It was also the very first time I’ve ever let her out of my or my husband’s care without a loving grandparent there to watch her.

Before she was wheeled off to surgery, she reached for my husband for the first time ever. I watched his eyes flood with tears, and my heart just about burst. I am so very happy that we got to share that moment during such a tense time. He loves these little girls with every fiber of his being, and I adore him even more for this fact.

Everything went okay. She did wake up absolutely hysterical, convincing us and her nurse that something was wrong. The nurse phoned her surgeon, who came in personally to see her. The doctor gave us the option of admitting her. But after a dose of Lor.tab, she seemed better. We ended up driving home, and she’s sleeping comfortably in her crib right now. I think she is exhausted.

She was totally parched when she woke up and drank 8 oz of glucose solution before they brought her to us. I could tell that she was very, very hungry, so I insisted on giving her a bottle (a brave move on my part considering she was perched on my lap) regardless of the fact that she could throw up. That child has always had an ironclad stomach (completely opposite of her sister). She only ate about an ounce before falling asleep. She kept waking periodically and crying hard, so that’s when the staff became worried. Her reaction basically never occurs during this procedure, but yet she’s been through a lot already, and usually clubfoot treatment occurs earlier and doesn’t involve brittle bones.

Anyway, the surgery went well. The doctor had to make two incisions in her right foot because it was more severe than the other, and he wasn’t happy with the correction that occurred from one incision. He said both feet practically corrected themselves from the snipping. She’ll wear the casts for two weeks, then have them changed out, then wear them for 1-2 more weeks. Then we put braces on her feet pretty much 24/7 for three months before proceeding with wearing them only at naptime or nighttime. That will continue for a longtime–until approximately age three or four. Ugh.

After we arrived at our car in the afternoon, we picked up Layla. She had such a huge smile on her face. She stayed with my dad all day and charmed the heck out of him. My heart warmed watching her smile and grab her feet and just generally act adorable. She has the art of cuteness down to a fine science.

I love my family!

Clubfeet – Update #2

Whew! I meant to write sooner, but it has been a very busy few weeks! We were supposed to return for Violet’s next set of casts on Monday the 23rd, but Layla was sick on Sunday the 22nd. She woke up throwing up and continued getting sick all day long. I knew we would probably need to take her to the pediatrician on Monday, and my hubby’s work day was jam-packed, so I postponed Violet’s appointment until Wednesday the 25th.

Last appointment, Dr. E surprised us by saying she would only need a total of two casts before her tenotomy. He thinks cutting her Achilles tendon and letting it regrow properly will release the tight side of her foot and cause most of the correction. Previously he had said that it would take 4-5 casts before the tenotomy, so we were expecting a month or more of weekly casting. We were thrilled!

Shortly after canceling the appointment, Violet woke up, and I noticed that the cast on her right leg had slipped down. Based on past experience, I knew that we would have to soak it off because her foot was no longer properly positioned inside the cast. I called Dr. E and was told to soak her casts off Monday night and wait for our rescheduled appointment on Wednesday. My hubby and I were afraid that her feet would revert back to their pre-cast position during the 48 hours she would be without a cast, but we followed the ortho’s instructions. We considered wrapping her left leg in a plastic bag while she was in the tub to try to preserve the dryness of that cast so that it could remain on her leg, but then we would have worried that somehow that cast would bang up against her right leg and cause a fracture. So we soaked off both casts. I used scissors to carefully snip at her casts while they were submerged in her bathtub, and was able to rip the casts apart after only 30 minutes. The previous time, this took two hours, so I was happy that she didn’t have to spend that long in the bathtub this time.

I took these pictures on Monday night just after her casts were removed (progress after one week in casts):

Left foot

Right foot–more severely affected:

At her appointment Wednesday, I was surprised when Dr. E decided not to recast her feet. His thoughts were that she would just continue to kick the casts off, so they weren’t beneficial and actually posed more of a danger because of the risk of fractures. We’ll proceed with her tenotomy on Tuesday, but now it has turned into an OR procedure during which they will put her under briefly to try to mold her casts better so that she can’t wiggle out of them. Most kids are given a simple local for this procedure, so it’s now a bigger deal than originally envisioned. Scary, but I’m just trying to remain calm and reassure myself that she’s in great hands.

Clubfeet Treatment

Some of you may remember that during a routine ultrasound at 28 weeks, we discovered that our Baby A (Violet) had bilateral clubfeet. At the time, I didn’t know much about clubfeet. We breathed a sigh of relief when we found out that nowadays, correction of this condition has an almost 100% success rate. The correction is not a quick fix though, and I plan to document the process for parents out there who encounter the same issue.

Most doctors in the U.S. use the Ponseti method (also called “serial casting”) to correct clubfeet. This process consists of the doctor gradually manipulating the foot into the proper position using plaster casts. These casts are removed weekly so that the foot can be repositioned, and then a new cast is applied. The cast extends to the upper part of the thigh, so it is a full leg cast.

The earlier you can start this serial casting, the easier it is to correct the feet. Ideally, you want to start treatment during the first two weeks of the baby’s life. Our local orthopaedist wanted to wait until Violet was at a certain weight before she would begin treatment, so we had to wait until she was a little over two weeks old. At the time, Violet showed no signs of the bone condition, so we had no qualms about putting her in heavy plaster casts. This is what her feet looked like when she was a few weeks old, just before beginning the treatment:

And here she is after her first casts were applied:

Here was the amazing progress after one week:

The above picture was taken on October 5th. On October 7th, her femur broke against the weight of the cast when we were trying to change her diaper. Her orthopaedist cut off her cast, and we decided to postpone treatment for a while.

Recently, we decided to switch her care to the OI guru in Omaha. I drove her to the children’s hospital there for her first casts on Wednesday. Here is her “before” picture:

And here she is this morning with her casts on:

As you can see, heavy plaster casts are not holding her down much!

Her doctor said it may take 4-5 weekly casts to obtain the proper correction. After that, she must have a tenotomy during which the Achilles tendon is snipped, and a cast is applied for three weeks. Since her feet did not develop correctly, her Achilles tendon also did not grow correctly. In the three weeks after the procedure, her Achilles tendon will regenerate in its proper position. Once she has the tenotomy casts removed, she will have to wear an orthotic device consisting of tennis shoes with a bar between them, spanning the width of her shoulders. At first, she will wear them full-time, but eventually she will only wear them at night to maintain proper correction. When she is three or four years old, her feet will be considered successfully corrected, and she will no longer have to wear the brace.

Today we noticed that her cast is slipping off because it was applied too loosely. I suspect that the P.A. was afraid of applying much pressure to her legs, so she wrapped the casting material too loosely. Tonight we have to soak the casts off in water and vinegar (during which time Violet has to sit in a tub for three hours because they take forever to soften!), and we’ll return on Monday instead of Wednesday as scheduled to have new casts applied.

This process is no picnic! It’s no surprise that Violet does not like having heavy plaster casts on her legs. She tolerates them remarkably well, but she has her fussy moments. I’m sure the tenotomy is going to be worse for me than for her, and the shoes sometimes cause blisters and result in very fussy babies. I know other babies go through much worse, so I won’t complain too much. And obviously, I am very thankful that such a successful treatment is available!