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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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.

Third Pamidronate Infusion

The babies’ third Pamidronate infusion went more smoothly this time. I think I know better what to expect now, and more importantly, I know THEM well enough to know when a cry is just a cry and when it is more serious. I went into Tuesday a little more relaxed than at the end of December.

For those who are just finding this blog, both of my babies inherited from me a brittle bone condition called Osteogenesis Imperfecta. They are Type I, the most mild type and will not have any skeletal deformities. My Twin A has broken each femur once, and my Twin B has also broken each femur once, plus her tibia, humerus, ulna, and skull. They are receiving a bisphosphonate via IV infusion called Pamidronate, which helps to strengthen their bones. They have these infusions every eight weeks for now, and as they grow up the frequency will gradually decrease. So far, it has been a miracle drug for us, although they still could fracture at any time.

Anyway, we traveled to Omaha for this appointment. Omaha, Nebraska has a children’s hospital with an actual dedicated OI clinic–the only one in the country, I believe. We found out about it when our babies were about a month old, thanks to FB, and they have been receiving care there since the end of October. They have an infusion center there, which is where Pamidronate infusions are administered. I walked into the infusion center and immediately saw four or five young children with no hair, clearly receiving chemo. That really puts our situation into perspective. Across the hall, a mother was questioning a nurse about what type of chemo her bald daughter was receiving that day. Later they had to switch rooms because a smell emanating somewhere near their original room was making her nauseated. Seeing all of this was heartbreaking. When my babies received Pamidronate the first time, they were inpatients. The second time, we were in the Kidney Department at our local children’s hospital, and we didn’t see any other kids there that day. Tuesday was a stark contrast.

Our babies were champs this time about having an IV placed. Violet was less than thrilled (can’t say I blame her!), but settled down quickly.

Layla did not even flinch when her IV was placed! Brave girl!

The infusion takes about three hours and 15 minutes to run, plus another 15 minutes to flush out the IV. During this time, the babies hung out on the bed together, sat in their bouncy seats, and napped.

After their infusion was complete, the nurse removed their IVs, and we were on our way home! They’ll return for their fourth infusion toward the end of April. We are lucky that this medication is available!

Second PAM Infusion

My girls had their second infusion of Pamidronate one week ago today. Pamidronate is a bisphosphonate intended to help strengthen bones that are weakened by OI. We went to our local children’s hospital this time around, but I think in the future we will head back to the hospital with the nation’s leading OI clinic (in Omaha). We will also likely go there for Violet’s clubbed feet treatment, since the nation’s leading OI orthopaedic surgeon can likely do this treatment for her (more on this later)! These infusions will be given once every eight weeks until the babies are a few years old, and then the frequency will lessen to once every 12 weeks. They’ll receive these infusions for approximately five years. Prior to their first infusion, they broke a total of eight bones in 5.5 weeks. Since their first infusion, they have each broken only one bone (a femur). We are really encouraged by these results and hope our lucky streak continues!

Layla is in the first picture below. She did very well with the infusion. We have them insert the IV into their scalp in order to avoid anyone having to hold them down to insert an IV into, say, their arm (because a bone could break from the pressure). She obviously was not thrilled about having a needle stuck in her head, but she calmed right down after it was over. The first IV fell out, so they actually had to stick her twice! Poor girl.

Violet was much less happy about the whole situation. She cried inconsolably for at least 30 minutes after having her IV placed, and she started to make me paranoid that she had a fracture. (We have the option of having a port placed in their chests to help this process be less traumatic, but that’s not something to be taken lightly, so we’re in “wait-and-see” mode.) My dad joined me for the appointment because my hubby had to work, and Violet eventually fell asleep on him. When she woke up, she was all smiles, thankfully!

It takes a little over three hours for the infusion to be administered. Side effects can include flu-like symptoms, but thankfully our babies have not suffered from any side effects at all so far. We are very fortunate that this medication is available to them! The next infusion will take place around the first week of March, and with any luck, we will be fracture-free this cycle!

3 months 2 day old

Since our babies were born (see my former blog, My CCRM IVF Journey), life has been a whirlwind. The first three weeks were absolutely perfect–we reveled in having two baby girls and all the joy and love that goes along with newborns. Then on October 7th, at 3 weeks and 3 days, our world turned upside down when the fractures started.

A summary of the past few months:

October 7th: Violet breaks her femur when we lifted her legs to change her diaper. An x-ray in the ER confirmed the fracture. Placed in a pavlik harness and given Oxy.codone. Clinically diagnosed with the bone condition (Osteogenesis Imperfecta Type I).

October 13th: Layla breaks her tibia after twisting her leg the slightest bit in my husband’s t-shirt. ER confirmed fracture. Placed her in an ace wrap because casts are too heavy for infants with bone condition. Clinically diagnosed with OI.

October 14th: Layla breaks the femur on her other leg after I just lifted her up to burp her like a non-affected baby. She let out a bloodcurdling scream, and I instantly knew a femur had broken. ER confirmed fracture. Ace wrap for tibia removed, and pavlik harness applied.

October 20th: Layla fell out of car seat onto hard ground because of our mistake. Fractured humerus, radius, and slight skull fracture on right side. Spent one night at children’s hospital for observation, but CT scan showed no permanent damage (thank God!). She had five broken bones at this point.

November 5th: Violet’s femur somehow broke either in her swing or when I lifted her out of it. Woke up at 10:45 p.m. hysterical, but ate 3 oz and slept for an hour on my shoulder. Then woke up hysterical again and continued to be inconsolable for an hour before we determined that there was probably a fracture. ER confirmed fracture. Placed back in pavlik harness.

November 6th: Layla’s femur fractured when I very carefully picked her up. I was supporting her femurs as we had been taught, but I guess sometimes you just can’t protect them enough. Decided not to take her to ER because we knew there was a fracture, and we had all necessary supplies/medicine at home. Gave Oxy.codone and placed back in pavlik harness. Orthopedist confirmed fracture from x-rays on November 8th.

So as you can see, it’s been quite a wild ride. Amazingly enough, the one dedicated OI clinic in the nation is only three hours from our house. I had no idea until I started researching groups on FB. We met an amazing OI mom who helped us to secure an ASAP appointment, and we headed there on October 27th. There is a medication called Pamidronate that has shown amazing results in OI kids. It is given by IV infusion starting as early as possible in the child’s life. Basically the earlier, the better. Our babies spent one night in the hospital, receiving half the dosage on Thursday, and the other half on Friday. We also met an OI mom who taught us better ways to hold the babies and offered advice about things like bathing, swaddling, setting limits on who can touch them, etc. They will receive Pamidronate locally every eight weeks for the immediate future. At some point, the medicine starts building up in their system, and they can receive the medicine less often. But for now, it is every eight weeks. While at the OI clinic, we met one of the country’s foremost experts on OI treatment, Dr. Paul Esposito. We are very fortunate to have this clinic so near to home.

Because of their fragility, I had to quit my job and begin staying home with them. No childcare facility wanted to take on the liability of caring for babies who may fracture from simple, everyday tasks. My husband has been on FMLA leave all this time and is finishing up his last 11 days of part-time leave. Starting the week after Christmas, he will be back at work full-time. It has been so wonderful to have him at home with me, and I am dreading being the only verbal person in the home. Oh well, it is a blessing to stay at home with them, and I won’t forget it. Financially, it is not ideal for us, but we will make it work because that’s all we can do. Hopefully when they’re around a year old, I can return to work. For now I will just do freelance projects intermittently. If you need any kind of editing work performed, contact me! : )

The babies are now three months old and really starting to interact with us. They smile at us constantly and love it when we talk or sing to them and when we play pat-a-cake, Violet especially. Just today, Violet grabbed onto the toy bar that accompanies her bouncy seat for the first time ever. They absolutely love to sit on our laps and hold their heads up. Violet is better at this than Layla. Violet is literally a replica of my husband, and Layla is a complete replica of myself. Yet, everyone asks if they are identical. Weird, because to us they look totally different. They hate tummy time with a passion and so far have lasted less than five minutes on their bellies because they get too hysterical. An early interventionist came to our home today, and the babies will receive physical and occupational therapy in our home until they are at least three years old. They are still not sleeping on their backs and currently sleep in their swing (Violet) and bouncy seat (Layla), while I sleep on the couch. Sleeping downstairs was my idea, so that my husband could be properly rested for work. They wake up generally between 3-4am, and again around 7am. Really, they are pretty decent sleepers, but I am looking forward to when they start sleeping through the night.

A couple of weeks ago, Violet weighed 10 lbs 11.3 oz, and Layla weighed 9 lbs 9.3 oz. Violet is in the 28th percentile for weight, and Layla is in the 8th percentile. We won’t allow our pediatricians to pull on their legs to accurately measure their length, because we don’t want any bones to break. So they are still in the 0 percentile for length for right now. Their heads are growing on target.

I have so much more to write, but I am tired so it will have to wait. I will do some picture posts to get you up to speed. Originally I was planning to keep our experiences private, but with the chaos of the past few months, I decided that blogging was once a good outlet (with IF) and probably will be again. Bear with me, since my life has gotten quite a bit busier and therefore may not leave much time for blogging, but I definitely plan to update once in a while. And knowing myself, I imagine that I will blog more frequently than I imagine right now. My fallopian tubes are “tied,” our family is complete, and maybe writing things down will help me to not forget things.

Despite all of these challenges, they are 1000000000000000000% worth it. They are undoubtedly the absolute best thing that has ever happened to me. I can’t imagine my life without them at this point. We literally thank our lucky stars almost every day. We talk about what life was like when we were praying for our BFP, and the hardships that this summer brought to us. Gosh, we were incredibly fortunate that we have two healthy, happy babies with us. I hope everyone who wants children will experience this unbelievable happiness. I can hardly wait to see what the future has in store for us.