Tuesday, December 3, 2013

Making Great Progress with the NAM!

Happy December, everyone! I hope you all had a wonderful Thanksgiving. We had 2, one for each side of the family, and both were fantastic. We have so much to be thankful for this year, and I'm particularly grateful for the amazing care Will is receiving from the NYU cleft team. I cannot believe Christmas is only a few weeks away... which also means that I only have a few weeks left of my maternity leave before I go back to work on Jan 6 - where has the time gone?!! It's been a little more than a month since I last posted (wow!), so I thought it was about time for me to share an update on how things are going. Will has been wearing the NAM for about 8 weeks now, and I am just amazed at the incredible progress he has made with it. Even his ortho is impressed with how well he's responding to the treatment, to the point where we are actually going to move up his first surgery (which will be to repair his lip) by a few weeks! We don't have an exact date yet, but it looks like it will be mid-February. I am both eager and completely dreading that surgery - I absolutely adore his little face and beautiful smile just the way it is and I am having a hard time picturing what he will look like with his new smile, but I know we will love it just as much. Not to mention I already know I'm going to completely break down and lose it when they take him out of my arms to bring him back into surgery. Tearing up now just writing this, so we'll change the subject! Here's a collage of Will's progress with the NAM after the wearing it for the number of weeks I indicate in the picture. So crazy!

As far as Will's life outside of his cleft, he's had a lot of exciting accomplishments in the past few weeks and it's been so fun to watch him grow and become more interactive with the world. He started "talking" with coo's and ah's around 4 weeks old, smiling at 5 weeks old, gave us his first laughs at 8 weeks old (which is pretty early!), and was even consistently sleeping through the night at 8 weeks old too (he goes to bed at 7:30p just like his sister, and it's SO NICE to have a couple of kid-free hours each night to relax and unwind!!). He absolutely loves mobiles and will lie there watching it go round and round, talking and kicking away at it. On the flip side, he has hated bath time and tummy time from day 1, and still hates those. Can't win 'em all. As far as bottles go, we determined that unfortunately there is no magic answer - he's just a fussy eater sometimes. We tried Zantac for reflux, gas drops (multiple brands), gripe water, probiotics, switched to sensitive formula and nothing made a difference. Then we tried a few different nipples for his bottles and found one that he seems to prefer, and with those he does better (although every once in awhile we still get a meltdown). But it's definitely better than it was before, which I'm sure is a combination of the nipples, him maturing, and us getting better at managing when he's fussy before it turns into a meltdown. Either way, he's a much more content baby than he was a month ago, so we are immensely thankful for that! And with all that stuff going on in his mouth, I guess I can't blame him for being fussy when he eats sometimes!

So that's our update. We're excited for Christmas and are eager to watch Will achieve more firsts!! So until next time - enjoy your December and Happy Holidays!!

Tuesday, October 29, 2013

Cleft schmeft (no, really!)

Wow - time is really flying! Will is now over 5 weeks old, and who would've thought - with all the research, preparation, and endless worrying about Will's cleft while I was pregnant, I have to say that to my great surprise, dealing with his cleft has almost been a non-issue so far! Yes, he has his NAM and we have weekly appointments (aka treks into the city) to get it adjusted, but we really haven't had any issues with that yet. What's been most stressful so far, you ask? The reality is that Will is finicky. Sometimes he fusses and cries through bottles, and the biggest challenge has been trying to figure out why he cries through some bottles while others he eats with no problem whatsoever. At first I suspected gas, but the gas drops and gripe water don't really seem to do anything. Then I suspected silent reflux, but after 5 days on Zantac it doesn't seem to make a difference either. So maybe he's just fussy sometimes?? Then, add on top of that an already feisty toddler who has decided to get a little feistier since that baby arrived (and who now wakes up MORE overnight than her baby brother does), has been the hardest thing for us at this point. If you know me, you know I'm a planner and I like to know what I'm going to do and when it will occur... clearly this conflicts with the reality of caring for a newborn who does not care what my schedule or plan is and this, my friends, has been the most difficult part for me to manage. Luckily, Will is a great sleeper, taking good naps during the day and sleeping 5-7 hour stretches overnight already (what a difference a good night's sleep makes). Super thankful for that!

Will's cleft treatment, on the other hand, is something I CAN plan for and schedule, so maybe that's why it hasn't been as hard as I expected that part to be.  And speaking of, after just 3 weeks with the NAM we are seeing amazing results. Will's premaxilla (the part of his lip and gums that is detached under his nose) started out to the side and is now nearly centered! This first picture shows our progress after just 1 week and 2 days with the NAM:

And here is Will at 1 month old (taken on 10/20) - I just LOVE his sweet face:

Tomorrow, Will gets the nose stents added to the NAM, which will help to shape his nostrils - I hope that goes as smoothly as everything else has gone so far. Wish us luck!! But more importantly, send us some good vibes in the hopes that we either find what's causing him to cry through some of his bottles or that he grows out of it - I'll take either at this point (serenity now)!!

Friday, October 11, 2013

Reality Time: Settling In and Starting NAM Treatment

Believe it or not, Will is 3 weeks old today and I cannot believe how fast time is flying! So much has happened since I last posted, so let me get right to it. Will's time in the NICU lasted for a total of 5 (long) days - once I took charge of his feeding he started doing much better and we were able to bring him home on September 25. He has been an eating machine ever since. To be honest, my reflection back on the whole hospital experience is pretty negative. I was advised to switch hospitals and have Will in Morristown because they were supposedly "more equipped" to deal with any feeding issues, but the reality is that no one knew how to deal with a cleft baby any more than I think our smaller hospital would have dealt with it. The fact that he was swept away to the NICU immediately after being born and given a feeding tube after 2 failed attempts at using one specialty cleft bottle (which, by the way, the nurses were using INCORRECTLY, which is why he wasn't able to eat with it) is really disappointing. He was never given a chance to eat on his own and never should have been given a feeding tube that early on. I'm frustrated that no one from the hospital's cleft team was available until 3 days after he was born, and they didn't do anything to prep the nurses or hospital of Will's arrival even though we had met with them ahead of time. Overall, I see major room for improvement and plan to share my experience and feedback with them in the hopes that other families don't have the same negative experience. Anyway, we were so happy to get him eating well and to get him home!  Here was Will ready to go home:
After getting home, the next hurdle was to get started on Will's treatment. A major part of his plan involves what's called a Nasoalveolar Molding (aka NAM) device - it's like a retainer that he must wear nearly 24/7 and its purpose is to move his lips and gums into closer alignment over the course of several months, thus creating the need for fewer surgeries with better success rates. The NAM is pretty standard for care in bilateral cleft cases like Will's because there is so much work to be done to move everything into place. Here's a generic picture of what one looks like:
Typically, babies are fitted for the NAM within the first week or two after being born. Well, the pediatric dentist from the local cleft team was supposed to visit Will while he was in the hospital and take his impression, but she didn't make it. And after multiple failed attempts to contact her to make an appt to get him fitted, I started to doubt her capabilities and commitment to Will's care, which made me second guess my choice of going with a more local cleft team versus the team at NYU. The NYU team invented the NAM, and is considered one of the best cleft teams out there. They also have a ton of experience working with bilateral clefts and have fantastic success rates. But of course, on the other hand, it requires a trek into the city every week for the next 5-6 months while Will has the NAM, so it's a major commitment. After much deliberation, based on the negative experience I had with the pediatric dentist on the local team, coupled with the fact that NYU is one of the top cleft teams out there and has much more experience with bilateral clefts in particular, we decided to change cleft teams and go with the team out of NYU. I gave them a call last Friday at 5p, and they had Will in for an appt that next Monday afternoon to meet the surgeon and get fitted for his NAM, and had the device expedited so that he could have it placed on Wed. The sense of urgency and focus they gave to Will in just one week has already proven to me that we made the absolute best choice for his care and I could not be happier! Here is a picture of Will just before getting his NAM placed:

And here he is sporting his new look for the next 6 months:
I'm happy to report that 2 days into the NAM, Will seems to be doing great with it. His little fingers do have a tendency to grab at the tape and he has managed to pull it out once (I think inadvertently), but it doesn't seem to bother him at all and he can eat better with it in since it provides more of a palate for him. I know it's early and it will be a long road ahead, but I'm optimistic!! So now we continue with weekly visits to the NYU team to have the NAM adjusted until he's ready for his lip repair around 5-6 months. I will of course post updates of his progress throughout the months leading up to his surgery to document his transformation.
So that's where we are. Having a newborn again has definitely been an adjustment (we're so tired!!), especially with a 2 year old also at home too, but we're starting to get into a groove and I know it will continue to get easier as we all adjust and get used to our new normal. I think Buddy and I definitely blocked out the memories of how hard it is having a newborn - it must be a form of self preservation because if you really remembered how hard it is I don't think anyone would ever have more than 1 kid. LOL. Regardless of how hard it is, Sadie loves her little brother, and so do we!

Saturday, September 21, 2013

The Big Day!

Well, the big day has finally come - William Gary Zierer was born yesterday, 9/20/2013 (right on his due date) at 5:54am. He weighed in at 7lbs 12oz and is 20 3/4". We are so in love! This labor and delivery was totally different from my first one - labor progressed slowly and seemed to feel way more painful, but the actual delivery was done in 15 min with 3 pushes - whew! Here we are right after he was born:

And now the real journey begins. As we suspected, along with Will's bilateral cleft lip, Will's palate is also involved. That means that feeding can be tough because it is very hard for him to get the suction he needs to eat from a bottle. So we have found ourselves at our first test - feeding. After spending about an hour and a half with us after he was born, he was then taken up to the NICU to get fully checked out and to see how he does with feeding. Luckily, he has received a clean bill of health so that is fantastic! And we were encouraged to find that his first feeding went pretty well - he took a good amount from the Haberman bottle (which is a special bottle for babies with clefts). His second feeding also went fairly well, but it took a long time for him to eat, and then he ended up spitting up some of it. The third feeding he just really wasn't getting anything from the bottle, so the decision was made to give him a feeding tube to ensure that he gets the nutrients he needs to stay healthy and keep his weight up. Since then, we continue to try to feed him with the bottle but usually he only is able to get a small amount and it takes him a long time to get it, so we finish the remainder of his feedings with the feeding tube. And so, the reality is that Will needs to stay in the NICU until he is able to eat on his own without the feeding tube - which could be a few extra days but could be longer. I had really hoped that we would be one of the lucky ones where our baby would be able to eat well and we could go home after the normal hospital stay just like everyone else, and once the feeding tube came into the picture, I knew this would not be the case for us, so that's been a little hard for me to accept but I know in the long run we'll figure it out.

Right now, I am feeling so many mixed emotions. Of course, the biggest is that I am overjoyed and completely smitten with my sweet baby boy. He's more handsome than I could have ever imagined and he's such a complete sweetheart. But I'm also feeling other emotions too. I feel guilty and sad that he is having a hard time eating and as his mommy, I can't help him right now. Little Will is a fighter - he tries so hard to eat from the bottle, but the poor little guy just can't seem to get anything from it. I am also feeling bad since we can't be like everyone else and just go home on time and get started into our new routine as a family. Plus I miss Sadie and feel guilty that I'm not able to be with her right now. Tomorrow I get discharged and will have to make the heartwrenching decision of whether I stay or leave the hospital without my baby... I don't even want to think about that yet. But I know Will is in good hands, and that we will get through this by staying strong and taking it one step at a time. We meet with the feeding specialist on Monday, so I am hopeful that they will have some different techniques for us to try and will help us find the best way to help Will eat on his own. We will also meet with the Plastic Surgeon on Monday so he can do an initial review of Will's cleft and give us an idea of what treatment plan he'd like us to follow, so that is exciting. I know it will all work out, and I am grateful that Will's NICU stay is not something life threatening. I just have to remember to stay strong and positive for my little fighter. And by the way, he sure does have the sweetest little smile if I do say so myself... don't you agree?

Sunday, September 8, 2013

Clefts 101: Arming Ourselves with Information

As we all know, there is certainly no shortage of information accessible through the internet. We have a plethora of information available at our fingertips at any given time... so much that it can be hard to weed through it all to find the most valuable/useful/accurate information out there. Proceed at your own risk, right? I kept all of this in the back of my mind as I set out to scour the internet and educate myself with as much information about clefts as I could find. Causes, treatments and surgeries, timing, other associated issues linked with having a cleft, how to find a doctor, really anything and everything I could find. For those of you that know me, I'm a bit of a control freak and I'm a planner - I feel the need to know everything about, well, everything, so my near-obsessive search for information and subsequent analyzing and organizing of said information shouldn't come as much of a surprise to you.

Well friends, with all that data searching, information is what I got - lot's of it - and I thought I'd share some of my key learnings here for your benefit as well:
  1. One of the first things I learned, which surprised me, is that a cleft lip and/or a cleft palate is one of the most common birth defects in the US - about 1 in 700 babies are born with it. We always see those commercials for the children in other countries who have clefts, but we really don't ever hear about its true prevalence here in our own country. Interesting.
  2. The causes of clefts don't seem to be very well understood yet. There is a general agreement that it could be genetic/hereditary, but can also be caused by environmental factors such as lack of folic acid, smoking, drinking, and exposure to certain chemicals during the early stages of fetal development. For many of us, though, none of these circumstances apply, as is my case, so obviously there are still some causes unknown, or I guess it just can happen by chance.
  3. Cleft lip surgery generally takes place between 3-6 months of age, and cleft palate around 9-12 months, but the exact timing all depends on each individual child's circumstances.
  4. There are a number of approaches and/or devices that can be used in preparation for the various surgeries, and each doctor seems to have their own philosophy on what is best. The devices range from something that is surgically implanted into the baby's mouth, to another that fits like a retainer and is taped into place, while some teams just use taping on its own without the aid of a device, and others don't use anything. The purpose of any of these methods is to close the gap of the cleft as much as possible prior to surgery, which is believed by many doctors to allow for better results and reduces the need for additional touch-up surgeries.
    • Our surgeon uses the retainer-type device, which is called Nasoalveolar molding (NAM). It sort of looks like head-gear for a baby, and from what I have read from other parents who have had to use it on their babies, it is a bitch to use but totally worth it in the long run. Usually a baby will get fitted for it within their first few weeks of life, and will then wear it up until the gap is closed sufficiently enough for surgery. Be warned: I'm sure you will see many posts from me about the NAM once we get started with it.
  5. A cleft lip and/or palate can thankfully be cosmetically repaired, but there are other medical issues that stem from this and treatment can last well into the child's late teenage years. Most people's initial reaction upon hearing that a baby will have a cleft lip or palate is to reassure the parent by stating something along the lines of how medical technology is fantastic nowadays and you can barely even tell when a child has had a repair. This is often true, but it doesn't tell the whole story - children with a cleft often also face issues with: feeding from the time they are born (most cannot suck, so they will usually require special bottles for feeding); ear drainage issues which can impact hearing as well as impede speech development if not treated properly; dental issues as teeth may not come in where the cleft was located, or they may get teeth in unusual places (think roof of the mouth); not to mention the need for multiple surgeries as they get older and their faces grow since they may not always develop the same as children who do not have clefts (including nose, lip, jaw line, bone grafts for their teeth/gum line, etc). So while yes, it is certainly a treatable condition, and other children have it far worse, it is not as easy as just having a surgery or two and then getting on with life... this will likely be a way of life that the child and the family will need to deal with for many, many years.
  6. For the reasons above, it is usually recommended that children with a cleft are treated by a cleft or craniofacial team, which consists of 10 or more multi-disciplinary specialists who work together to come up with a coordinated treatment plan for the child, based on his/her specific needs. A team? I had no idea this was the approach, but I'm so glad it is because after better understanding all of the complex medical needs the baby will have, I couldn't imagine trying to find and work with all of these specialists one by one.
We are very fortunate to live in an area of the country that has multiple cleft teams within an hour to 2 hour drive from our home, which meant we had a choice of who we wanted to use for our baby's care. It was a tough choice, but we've decided to go with the cleft team out of Morristown's Goryeb Children's Hospital here in NJ. We met with them back in July (yes, ALL of them at the same time - it was quite intimidating) where we got to ask any questions we had about their approach and how they work together, and we walked away with a great feeling from the team. The plastic surgeon on the team who will do our baby's lip and palate surgeries really impressed us with how approachable he was and how patient he was in answering all of our questions. Not to mention he regularly volunteers and goes on mission trips with Operation Smile, where he will perform around 15 cleft repair surgeries A DAY while he's there!! I just find that to be a huge testament to his character and dedication to improving children's lives all around the world.

So there you have it - I just dumped a TON of information on you, but hopefully you will find it informative, especially for those families who have or are expecting a child with a cleft and are looking for clarity on all of the information out there. To that end, for those looking for further info I highly recommend checking out www.cleftline.org, which contains plenty of educational material as well as a listing of cleft teams across the country.

Saturday, September 7, 2013

The Start of Our Cleft Journey

Two more weeks. That's how long I have until my due date arrives, and I am so excited and anxious to meet our little peanut, who will have the sweetest little smile. I'm pregnant with my second child, and I cannot believe how fast the time has flown! In general, this pregnancy was much like my first: I was blessed to be spared many of the uglier side effects of pregnancy both times around - no morning sickness, no swelling or bloating, practically all of my weight gain isolated to my belly, and overall just feeling great throughout the whole pregnancy. However, in another aspect this pregnancy has been the polar opposite from my first: during our 20 week ultrasound, we found out that this baby has a bilateral cleft lip and likely palate.

I had gone to this appointment alone, and was so excited to see pictures of the baby that the thought never even crossed my mind that there could be anything wrong. The visit itself was pretty normal, and although I noticed the sonographer spent a bit of time taking various face and head shots, I really didn't think much of it and she didn't say anything to me otherwise. Then she went to get the doctor to review the pictures, and when the doctor came in to speak with me, I knew right away something was wrong (the doctors never come in if everything looks good). She told me they could see a bilateral cleft lip on the ultrasound, meaning a cleft below both nostrils where the lip failed to fuse together during development, and that it looked like the palate was involved as well. She stressed, though, that everything else looked perfect, so she suspected that this was an isolated issue (sometimes clefts can be associated with other birth defects and syndromes). She told me we needed to make an appointment with the Genetic Counselor (who luckily is in the same office) to discuss the circumstances in more detail and determine the appropriate course of treatment. I was stunned. A cleft? A genetic counselor? How can this be? I called Buddy, my husband, from the exam room because I knew I wouldn't be able to wait until I could see him in person, and after I shared the news we both cried together. The news devastated me and immediately started a flow of emotions and questions about how this could happen - I wondered "why me; why my baby?" and even hoped that maybe they were wrong.

The rest of the week was a roller coaster of emotions and doctors visits. We made an appointment to see the genetic counselor the next day, and at that appointment we were asked tons of questions about our family history (we have zero history of clefts on either side), what medications I had taken (just something for hypothyroidism), whether I smoked or drank while pregnant (no and no), whether I had been taking folic acid before and during my pregnancy (yes), etc. Basically nothing to point to the cause of the cleft. Then the counselor offered us the option to have an amniocentesis to check for any other genetic disorders (since as I mentioned earlier sometimes clefts can be associated with bigger issues or syndromes), to which my immediate response was no. However, Buddy and I discussed it more that night and decided to go through with the testing after all - we knew that it would not change whether or not we kept the baby (believe it or not, we were told we had until 24 weeks to terminate a pregnancy - really??!), but we wanted to know if there was anything else wrong so we could properly prepare for what was to come. I had my amnio the next day (it was actually very uneventful, thank goodness). During the amnio, the sonographer did some 3D images of the baby's face, and that was when we first saw the baby's cleft lip - it was clear as day right in front of us, no denying it. So it was real. Here's the picture we got from that visit (they added little hand arrows to point out the clefts):

The first week after receiving the news, I had a very hard time coping and mourned the loss of the "perfect" baby that everyone hopes for. Thank god for Buddy - of course he was upset too, but he was also much more positive and optimistic, encouraging me to be strong and reassuring me that we would get through this together. After that week, especially with the support of Buddy, I knew being sad was not going to do anyone any good, nor would it change anything, so I decided to turn my energy to educating myself on what was to come so that I could be as prepared as possible before the baby's arrival. Even though I was warned not to Google it, I did anyway. Yes, I came across those heart-wrenching images of cleft-affected children who don't get treatment right away, as well as many images of some more severe cleft cases, and those were hard to see. But really, I was able to navigate through all of the information and within a week or so had found the key cleft organization websites that provide very useful information about clefts and their treatment (I'll dedicate some future posts to sharing some of this info), and more importantly I had found some amazing support groups made up of other families who have children with clefts. Through these forums, I was able to connect with other families in similar situations, ask all of my questions and vent about my fears, and see so many encouraging pictures of beautiful babies and children both before and after their surgeries. The amount of support and advice I've found has been incredible - in fact, I don't know where I'd be without it, so I'm immensely grateful.

Fast forward to now, 18 weeks after that ultrasound where we received the diagnosis, and I feel like I am in a completely different place. Yes, I'm nervous about the reality of actually seeing and dealing with the cleft when the baby is born (now that it's only 2 weeks away), but mostly I'm just super excited to meet my baby and enjoy everything that goes along with having a newborn! Sometimes there are difficulties feeding babies with cleft lip/palate, so we are preparing ourselves for a possible NICU stay, but hoping that's not necessary. And thankfully, all of the results from the amnio came back normal, so we are hopeful that the cleft is isolated, but are prepared just in case there is anything else wrong. Now all we can do is wait and see. And so I've decided to start a blog about our journey for a variety of reasons - to document it all so we can reflect years later on what it was like, to help educate others on this very common birth defect, and most importantly, to share our experiences so that it might help other families who are going through the same thing.

So here's to the start of our family's new journey, documenting the story of our beautiful bilateral cleft baby, who I already know will have "the sweetest little smile". I cannot wait to welcome our peanut into this world as part of our family!