Stomping Grounds Fun & Ear Trouble?

Today was fun! We had a great play date at Stomping Grounds with some friends, followed by a relaxing afternoon - something that was much needed!

 

We have been really busy this week with Bella's stuff and have had a lot weighing on our minds. we got some pretty disappointing news about Bella's ears. We don't know much yet, we are actually waiting to hear back from her ENT who wont be back until next Tuesday.

 

We found out that Bella has something called Mondini Deformity, this is a inner ear malformation that could be very devastating to us. Bella's type of deformity is characterized by an enlargement of her vestibular aqueduct, bilaterally (both sides). We don't truly know what this means for Bella, in our case, but we do know that this is associated with balance issues (vestibular system) and the majority of children will develop some degree of hearing loss in their childhood years. Bella's physical and occupational therapist have become very interested in this, as Bella has been struggling tremendously with balance issues for a long time now. They have had a lot of trouble trying to perfect her sitting and standing balance and are noticing that one day she has balance then the next she has none. They think this has a lot to do with her Vestibular system.

Her therapist have explained to us that you have to have three components to walk. Your eyes, ears and legs/ feet muscles. We do know that a child's body can overcome vestibular dysfunction, but they have to have at least TWO of the three components. If you are blind, then you need your vestibular system (ears) and legs to function. We already know that Bella has some muscles issues that are predominantly worse in her legs than arms and now we might be dealing with vestibular dysfunction? This is going to be a big hurdle to jump...

We need to either get Bella's balance stabilized soon, or get her muscles stronger in her legs so that she can overcome the balance issues. It really sucks that we are dealing with 2 main hurdles rather than just one now! - when it comes to walking at least..

On the other end of the balance problems is the potential hearing loss, this is something that we have not even remotely been prepared for. We have always said that Bella is going to go through a lot in her life and we are at least blessed to be able to help and talk with her about it. Before having children, one of my biggest fears was to have a child who was deaf. I could not imagine my child not hearing my voice. It was something that literally terrified me.

Is this God's way of proving to me that we will be alright regardless? If so, he can stop testing us now. I think we have been tested enough this past year and I am not sure how much more I can take. God, please spare Bella her hearing, or at least the majority of her hearing.

Once we find out more detail about this and how it may affect Bella's future, I will be sure to share it with you all. It is just something that I needed to get off my chest for now. I have been trying to keep it in the past few weeks until we know more, but that was not working too well.

Therapy Ups & Downs: It's Not About What We CAN'T Do, It's About What We CAN Do!

This morning at PT the dreaded conversation of increasing therapy came up. I have had a feeling that this chat was coming soon.

Ever since we moved from Cypress back to Sugar Land, we cut Bella's PT/ OT down to 2 times per week. She was doing good at the time, progressing, and we really wanted to stay with her current therapist until she was walking. Decreasing therapy was the viable solution that has kept me driving to Cypress every Monday and Wednesday morning at 7 am. (Her therapist were actually the ones who recommended it so that I did not get burned out).

Bella's wonderful therapist have been with her since she was 5 months old and she has really taken to them. Actually, I would say she has a love hate relationship with them. They really are awesome!

For the past several months we have been working VERY hard towards bearing weight on her feet/ legs, sitting balance, standing unassisted, and eventually taking a few steps on her own... Fortunately, Bella has been able to practice some of these tasks, but we are still very far from where we wanted and expected her to be right now.

We know that these "plateau" time periods can happen and do happen, but it is still tough to hear it. We hope that Bella works through this quickly, gets back to improving soon, and shows us that she actually wants to move forward and push herself.

For right now, the best thing we can do it be creative, find new ways to encourage her to move the way we want, pray that God brings her body strength, and be thankful for what she CAN do at the moment.

By the way, I am excited to say that Bella's 2nd tooth popped through today!! Yippee! The next tooth to show is her "special" tooth, I am actually super duper excited to see my beautiful baby with her snaggle tooth ;) . Who can say that they only had one 'big' top tooth? Not many..

 

Madison Steiner: Peach's Neet Feet

"Changing the world is really not as tough as it seems.

Just takes a little effort and a whole lot of happy.
The world will not change with a wish or a wand.

No abra-kadabra or shooting star.
Some may think it takes a miracle or magic but ...

I have something to say that might clear the static.
I have dreamed a dream of something improbable because to me,

 in life, there is nothing but possible.
The answer really is no secret to keep;

you just need to be kind to each soul that you meet.
A task that takes just courage,

you see a gut full of gumption and brain that believes.
It is a heart full of hope and a hand that can help.

To deliver such kindness that may have never been felt.
So do what you can, no matter how small.

\Stand up for what is good and stand 100 feet tall.
Paint a picture, hold a door, give a nickel or give more.
Give a stranger a cup of joe or donate your old winter coat.
Do these acts at random and I bet you’ll find,

that you are making a difference each and every time.
So tell me friend, have I convinced you why,

that making a difference is as easy as pie
Maybe I should remind you this life isn’t all about “me”

There is more to this life, a reason for existing.
So be fearless, be fierce and maybe a little insane.

 Now do something rad and watch the world change."

-Peach

Who wrote this wonderful poem you might ask? Well, that would be Peach (Madison Steiner). If I had to opportunity to meet anyone in the world right now, it would be Madison. She is truly the epitome of an awesome loving person. She is not some celebrity that most people drool over, she is a young soul from Colorado.

Madison Peach Steiner founded Peach's Neet Feet (PNF), which is a non-profit organization that donates hand painted shoes to children, or fighters, as PNF refers to them, battling cancer and other serious lifelong illnesses. Madison hand paints all of her shoes, along with a few guests artists, and has "shoe fairies" present the personalized pair shoes to each child.

Madison was also awarded the 2012 Good Samaritan Real Heroes Award for NW New Mexico for her unselfish character! What a great accomplishment.

PNF is trying to supply "fighters" with a little "heArt in their Sole". - What a wonderful way to help these children get through their daily struggles!

Go support this amazing organization by sponsoring a child's pair of shoes! You can contact Madison at madisonsteiner@gmail.com or visit PNF to find out how else you can help spread the love :-)

Special Needs Or Not, Bella Is Just Bella

I get a lot of questions about how Bella's life is. What does she do everyday, what is it like to have a child with so many different needs?

Yes, Bella does have different needs than a normal 13 month old child, but Bella is still a typical baby. She does all of the same things that any other baby would do. Sometimes she just needs more assistance.

She loves to play with her big sister, Kallie. She laughs, she cries, she hates strangers, she is sweet, she gets mad, she gets into things, she takes baths and she even eats. Can you believe that?

Two peas in a pod

Yes, we go to several long and exhausting doctors appointments a month and to therapy 3 times a week but that does not mean she is not like other kids.

Every morning Bella wakes up and eats breakfast. We are still working really hard on trying to eat 'table foods' so I always start her out with that. Usually mini pancakes that I tear up really small. After we try a few bites of those I can tell if it is going to be a successful table food day or not. Lately it has not been, so we revert back to baby food for the rest of that meal and try again at lunch.

We know that Bella has swallowing difficulties, but we are not sure why she is having so much trouble transitioning from baby foods to table foods. She chokes and gags on almost anything besides cheese. She will choke so much that she actually makes herself throw up. Its lovely and yes, everyone stares at her when we are out to eat.

Bella also has not transitioned to a sippy cup. We still try everyday, but I only do it for so long because I don't want her to end up rejecting it or associating fear with it. She chokes on anything that comes out of a sippy cup. We have tried every sippy cup known to man, and she still has issues with it. She is still only comfortable with a bottle and a stage 1 nipple. A stage 1 nipple is usually used until 3 months of age, Bella is over 13 months old.

Bella has had two swallow tests, one before her surgery in December and one after. Both did not have great outcomes, but were not terrible. She did penetrate the lungs (not aspirate) and has some odd pooling in the back of her throat before she actually swallows.

After Bella's second swallow test, her pulmonologist suggested using a thickener. She has been on Simply Thick for a while now. We are supposed to use it in ALL liquids- milk, water, juice, anything that goes in her mouth. It makes it very thick, it's actually called nectar consistency, but she still can not use the sippy cup even with the thickener. We have resorted to using the bottle for her milk and no thickener since she does good with the level 1 nipple. In the sippy cups we add the thickener and let her try a little bit everyday.

Although Bella has some muscle spasticity and high tone, she still plays and crawls like other babies. We just have certain ways that we encourage her to do things. We place toys in strategic places and encourage normal movements, pushing her body to learn to do the movements correctly.

Play Time!

Bella is a very smart girl and she has found alternative ways to go about doing things. If she does not like to stand and scoot along furniture because it is more difficult for her, then she will get down, crawl, and come back up where she wants to be. That would be fine if she could actually perform the task and was just doing it differently as a personal preference, but she is doing that because it is a strain to her. If she does not learn the right way now, it will become more and more difficult the older she gets.

She might have a few things that hold her back but we still go play with friends, go to the park, and she loves to be in the pool!

Kallie loves to be in charge of little sis!

Having a baby with special needs might make our daily life different than an average families, but it does not mean that Bella can't interact normally like other children. She is still a child that loves the same things any other child would. She has no idea that she is different from the kid next to her, and I hope that she never feels that way in her future...

Bella's Birth Story Part Three: The Medical Center

It was a Friday afternoon when we were visiting Bella and talking with the neonatologist when we found out that Bella was going to need to be transferred to the Medical Center. Kallie was with us that day so we had about 45 minutes to make arrangements for her, or else we were going to have to let Bella go on her own and us meet them at the other hospital, and that was NOT happening.



Sweet Sleeping Angel

I put Mike in charge of figuring out where Kallie was going, and getting her there, while I stayed with Bella to get all of the ridiculous amounts of paper work filled out. It was overwhelming being in Bella's room with the current NICU team, the transport team and many nurses all telling you a ton of information. I also had no idea what to expect at the new NICU. One of the nurses told me that she will be with an awesome medical team once we get there, but to be prepared as it is a culture shock compared to Memorial Hermann Memorial City.

Waiting for the transport team to get everything ready

I was also panicking because I knew that the medical center was about an hour away from our house and that was 30 minutes farther than where we already were. I knew that I was not going to be able to be right by her side ALL THE TIME because we had Kallie who was 21 months at the time and Mike had to work too.

Kallie brought her favorite "George" to watch over baby sister

Mike was able to follow in the car and I rode with Bella in the ambulance. I had never been in an ambulance before that day and I hope to never be in one again. Bella was hooked up to all of her equipment and safely strapped inside the incubator, but it just didn't feel right. Once we got to the medical center we were directed upstairs to the 7th floor level III NICU. We were really hoping to be able to go to level II NICU instead so that we could have our own room and be able to have more visitors and so forth. Unfortunately, Bella needed a little bit more attention than level II was able to offer so level III it was. We were not allowed to go back with Bella while they admitted her, we had to wait in the waiting room where we were instructed on the long visiting rules/regulations, informed on the process to visit your child, and given a tour of the Ronald McDonald House (I will explain that in a different post). After about 1 1/2 hours we were finally able to go see Bella again. We were escorted back the long hallway by Bella's transport nurse who was GREAT. She was so sweet to us and understanding. She made jokes to lighten the day and prayed with us before she left. She told us about her son who had growth deficiencies and was on growth hormones and explained to us that we are not alone, we had all the other families that were there, as well as God.

We already had some culture shock just being in the waiting room, but when we walked into where Bella was going to be until she was able to come home, we were more than shocked. There were 8 other babies in her room. There were tons of nurses watching over the babies and visitors for other babies. There was no designated area to sit, they would pull you up a chair if you were going to try and nurse and give you a curtain to try and block off the area. The nurses were different, they did not seem as compassionate. That could have been just me too, as I was already upset at having to bring Bella here.

Bella's note to all the Nurses and Doctors.
"Please Do NOT suction my nares" Thanks, Bella

It was a Friday evening and we REALLY wanted the ENT team to make it to see Bella that day so we did not have to wait through the weekend. (We were under the impression that we would only be there a few days) I remember waiting for several hours with nurses telling us that they were not sure if or when the ENT's were coming and that we could leave and they would call us if they came. Mike and I finally decided to wander down to the cafeteria to see if we could get something to put in our stomachs after this long day. When were walking downstairs we walked right past a group of doctors that were walking briskly with a brief case in their hands. I heard one guy with an accent say that the patient had severe nasal blockage and it was not thought to be Choanal Atresia. At that point I KNEW that was our team of ENT's. We turned right around and followed them upstairs.

The volunteers made Bella a hat :-)

When we got back to Bella's pod, we were instructed to please wait outside in the waiting area as parents/visitors are not allowed to be in the room when any procedures are going on and that they would come get us when it was all over with. (Yes, this is for every child, no parents or visitors are allowed. You are constantly being asked to leave the room for other children's procedures, or when a specialist is coming in to examine a child). We were not able to talk with Bella's ENT's that evening but they left us some information. They were able to scope one side of Bella's nasal passages but were not able to make it down the other and there was severe nasal narrowing. They scheduled an emergency CT scan and would discuss the results with us the next day. That evening we went home and they had not done her CT scan yet. She ended up having the scan early the next morning, and by that afternoon Dr. Yuksel had called us with Bella's diagnosis of Pyriform Aperture Stenosis (CNPAS).

Bella's lips were always chapped because
 she having to use her mouth to breathe.

I don't remember what I was thinking at that moment, but I do remember replying with, "What?" It was different to actually hear a diagnosis rather than a whole bunch of speculations from other nurses and doctors. I remember Dr. Yuksel spelling it for us and saying that we could look it up online, but to be careful about what we read since it can be associated with other issues and that her CT Scan looked pretty good otherwise. (Her adjusted age was still only 37 weeks, so she was not even full term yet.) The first thing we starting doing was research of course. I remember reading about all these horrible things that are associated with CNPAS, such as, brain anomalies, a central incisor (which totally freaked me out), and other serious syndromes and mid line anomalies.

The plan was to watch Bella over the next few days and determine if she needed surgery while she was still in the hospital. Her doctors really wanted to wait on surgery if at all possible, as it would put a BIG strain on her body and could be difficult for her to recover. Bella needed to do several things before she would be allowed to go home regardless of surgery or not. She needed to gain weight, (she was still loosing weight, she lost a total of a 1 1/2 lbs!) she needed to hold her temperature better, she needed to stop having A's (apnea) and B's (bradycardia) and she had to learn to eat! She still had a
little ways to go..

Cuddles were always the BEST

Over the next several days we were taught A LOT about a baby with upper respiratory problems and what it entails when we go home. Scary to say the least. We were taught how to 'pace' Bella's feeds. We would have to put the bottle in her mouth let her suck 3 or 4 times and then pull it out so she could breathe. We also had to hold her a different way with our hand holding her head/ neck while she is in a reclined sitting position on our knee. Yes, we became very talented.. No cuddling our sweet girl while feeding, unless we wanted her to turn blue. Feedings were the time that I totally dreaded, it was the most stressful experience ever. She would constantly stop breathing during EVERY feed, we would have to stimulate her feet or blow hard in her face to startle her to start breathing again. She would also choke a lot too.Who wants to feed a baby like that? It is definitely not relaxing or comforting.

Since the day Bella was born (actually before that) I was determined to breast feed her. I had some trouble with Kallie so I was a little bit nervous. I was a ritual pumper while Bella was in the hospital, I would pump every 2 hours, put it in the specified bottles from the hospital, stuck Bella's label on it, wrote the date and time and then pack it up to bring to the hospital the next morning. When we were at Memorial City it was nice because they supplied me a pump in Bella's room so I could relax in there, watch TV and be close to Bella. At the medical center they had what they called a "pumping room" you walked into this stale room, sit in a chair behind a curtain, pump for 20 minutes, get up wash your hands, transport your milk and then delivered it to the lady at the front desk so she could put it in the freezer room for you. It was terrible, I hated it. My milk was slowly drying up because I was not around Bella enough to be stimulated and she could not breastfeed because she had CNPAS. She had no latching abilities and also had a terrible sucking reflex. It was to the point that Mike was recording snip its of Bella crying while we would visit her and then he would play it over and over and over again while I was trying to pump at night.. Oh the things we do for our children.

As we were trying to get Bella to a good place to be able to come home, she continued to have severe apnea's and bradycardia's. She would still stop breathing, but there were several times that the nurses would have to intervene with oxygen and so forth. It was to the point that doctors and nurses thought they were not solely coming from the CNPAS. They decided to call in a pediatric pulmonologist to monitor Bella. Her name was Dr. Jon, we LOVE her. She is still taking care of Bella today and she is awesome. Bella needed to have a sleep study done to figure out the source of her apnea's. Apnea's are common in premature babies, but when they are associated with bradycardia's they become very dangerous. It is the body's way of trying to reserve "life" when the baby stops breathing. Once the baby stops breathing for long enough, the heart and pulse start to drop drastically to try and compensate. Most of the time these types of apnea's resolve themselves. Bella's were not resolving on their own. Dr. Jon informed us that a sleep study is very difficult to do on a baby that is "in patient" in the hospital and advised us to wait until she was released. She told us that regardless, Bella was going home on an apnea monitor so we were going to be alarmed if she stopped breathing for more than 20 seconds.

It took almost 2 weeks for Bella to show that she is "thriving". We had learned to pace, she was controlling her temperature, they taught us how to stimulate Bella if she was to stop breathing, she only needed to gain some weight, or else we were staying much longer so she could have surgery. They were not going to give her much longer, but she finally gained an ounce! That was a GREAT day :) They started to schedule things such as the apnea monitor company to come train us and we both had to take an infant CPR class. Her doctors all needed to sign off on Bella going home as well. It was a long 2 day process, but Bella was finally allowed to come home!!





Are we really leaving?

We knew we were nowhere near the end of Bella's adventure, we were leaving the hospital with 3 follow up appointments for that week, a list of emergency phone numbers, and packets of information. It sure did feel like a huge relief to finally have Bella in her car seat looking excited to be leaving!!





First time being home!

 

Children's Craniofacial Association Volunteer

I am so excited to be a part of the Children's Craniofacial Association! I have been following CCA Kids, as well as several of the children affected by craniofacial abnormalities, since Bella was born and recently applied to become a volunteer.

This weekend I recieved a box of goodies that will help me begin to raise awareness for craniofacial diseases, such as SMMCI Syndrome.

The Children's Craniofacial Association is a non profit organization located in Dallas, Texas that is commited to helping children, and their families, affected by craniofacial abnormalities. CCA addresses several concerns relating to children with craniofacial abnormalities, such as, medical, financial, pyschosocial, emotional and educational support.

To find out more information about the Children's Craniofacial Association, or to find out how you can help too, visit CCA Kids.

By the way, September is craniofacial acceptance month! Let's make sure these kids feel ACCEPTED, you can purchase bracletes, t-shirts, mugs and much more HERE to show your support throughout September, and the rest of the year :)

Promising News: The Carter Centers & How They Can Help Bella

Over the past several months, I have met a few people through Facebook that have children with SMMCI Syndrome, the same syndrome as Bella.

Mike and I have searched for almost a year looking for other families that have been going through the same thing or similar things as us. Before we found our SMMCI families, we were frustrated, we felt alone and even our doctors did not have any information for us regarding Bella and her issues. They were treating every issue that Bella had separately. I kept feeling like there was a reason that all of these random things were going on with her. Yes, she has SMMCI syndrome, but that is usually accompanied by something "bigger" and if not, then it is just an isolated anomaly. We knew that Bella was having feeding and swallowing issues, muscles spasticity, high tone, breathing difficulties, central sleep apnea, and recently P5P deficiency, which is associated with epilepsy. We also knew, from the very beginning, that SMMCI syndrome is highly associated with Holoprosencephaly (HPE) and often debated on if it is a micro form of HPE.

Here is a brief overview of what HPE is. HPE is a condition when the brain does not grow forward and fully split into two separate hemispheres. The malformations of the brain can range from severe to mild. In a child with mild HPE, the brain is split but  the child has some very mild abnormalities that a lot of times are over looked as HPE. HPE occurs in about 1 in 5,000 to 10,000 live births and current studies indicate that only about 3% of all fetus's with HPE survive the delivery and the majority of those die within the first 6 months of life. With that said, VERY little is understood about the most mild cases of HPE. We do know that children with HPE can also have secondary disorders, such as epilepsy, gastroesphageal reflux, hormone disorders, movement disorders and spasticity, respiratory disorders, and a few others.

At 10 months of age, Bella had an MRI to check for Cerebral Palsy. They were looking for any major damage in the brain, and luckily did not find anything.

One of our wonderful families that we met through Facebook opened my eyes to The Carter Centers.  The Carter Centers are a brain research company mainly focusing on HPE. They are trying to update the statistics and information known about HPE. Since HPE is usually considered such a major deformity, there is not much information in the medical field for children suffering from the most mild forms of HPE.

The Carter Centers will read your child's MRI, review your child's blood work, preform genetic testing and do patient evaluations (if you live close to one of their centers) - ALL FOR FREE; if you have a child that meets their requirements of course. They will the provide you with their findings and tell you what other children are like that have similar defects.

Our dear friend who brought The Carter Centers to my attention, has a daughter who is 2. She also has SMMCI and a lot of the same symptoms/ issues as Bella. She had an MRI and hers came back "normal" as well. After feeling like something was just not right and spending lots of time doing research, her mother found The Carter Centers. She sent her daughters MRI to be reviewed and it came back as Septopreoptic HPE, the most mild form of HPE... To most people, this would be devastating news. It is TERRIBLE to read about and something that you would never wish upon your worst enemy, BUT this is answers for parents like us!

We have already talked with The Carter Centers regarding Bella's case and they want to review Bella's MRI. If they come back with anything, this could mean a lot to us. This could give us the answers that we have been looking for. We wont know about Bella's future, but we would have a diagnosis, something that we feel we are so far away from right now. We have a lot of random diagnosis for Bella right now, but nothing really makes sense. This would pull them all together and ultimately, not seem so scary for us. This could be ONE big diagnosis that houses all of her other issues.

It will be a while before we get any response from The Carter Centers (8 - 12 weeks), but it is worth it!

Meeting Claudia- Bella's Newest Therapist

Bella was in rolled into ECI (Early Childhood Intervention) close to a month ago and yesterday we met our first in home therapist. The therapist, Claudia, is working on Developmental Therapies with Bella. This is an all around therapy. Working with her body and mind as a whole rather than just focusing on a specific area like they do in Physical and Occupational Therapy.

Bella has been really shy and has a hard time in new places so it was really nice to have someone come to our house and work with Bella in her own environment! Bella really seemed to enjoy it and took well to her, plus Kallie got to be involved too. It was much more like play therapy, we had an hour long supervised play session.

Claudia would place toys in certain areas of the living room to make it a challenge for Bella. She asked a lot of questions regarding Bella's Physical and Occupational Therapies and feeding / speech issues. Claudia was extremely pleased with Bella's curiosity, focus and "smarts". She said that is the best thing going for Bella. A lot of times you see kids that are behind but they are also severely cognitively behind as well. Bella is not too far from where she should be cognitively, but physically she is still several months behind and has a hard time doing things "correctly".

A struggle in therapy these days, with Bella, is that she has actually figured out how to do things in different ways. If it is a strain on her body, then she has another way to do it and get it done. This sounds like it would actually be a good thing right? The issue is, if she adapts and learns other ways to do things, such as getting around, then she will never learn the correct way. Her therapist strongly believe that if we can teach Bella's body and mind the correct way to do things (crawling, sitting, walking, running etc.), then she will flourish and be able to do them for the rest of her life, but is it going to take work!



Trying to hide from us!

It takes a long time to break bad habits but once they are broken, they usually stay that way. This is true as long as Bella does not have a big growth spurt where her muscle tightness causes her to develop slight deformities, such as walking on her tipy toes, that need to be worked through again.  Claudia told us, much like all of her other therapist have mentioned, that Bella's inability to do certain things seems to be coming from her high tone and spasticity, not her brains inability to function correctly. Her brain knows what to do, her muscles a forbidding it.

Of course, this is all speculation in the therapy world, but it sure does give a lot of reassurance. As a parent all you want is the best for your child and if Bella requires therapy for the rest of her life, then that's what we will do! We will make it fun, we will make it a daily routine and the whole family will do daily stretching and exercises with her to make sure she doesn't feel alone :-)

Bella's Birth Story Part Two: Welcome to the NICU

Bella was here after a rough two days and I was stuck in the recovery room because I was having a bad reaction to the anesthesia. I had lock jaw, terrible shivering, low heart rate and low blood sugar (I am type 1 diabetic). Unfortunately after surgery you can't eat of drink anything for a while and I was totally freaking out with out being able to explain in full sentences that I NEEDED something with sugar or else they were going to have a much more serious problem on their hands than just a shivering woman, I was going to pass out!! After about 30 minutes they were finally able to get glucose for my IV and get my blood sugar to come up some.

Mike was asked to keep away from Bella while in the operating room, they finally thought that they had her stable enough and he was able to make a quick run with her to the waiting room to see family. It did not last long and they then whisked Bella away to the NICU. No one really explained anything except that she was not going to the nursery, but instead up to the NICU because she was not breathing correctly.

Daddy showing off Bella for the 5 seconds he had her.

Finally, Mike was escorted upstairs to the NICU to be by Bella's side. She was already hooked up to oxygen, monitors, and IVs etc. He said it was very overwhelming and scary to see all of that. At Memorial Hermann Memorial City they have every NICU child in their own closed off room. It is visible by a big glass window and sliding door for the nurses to get in fast, and believe me they are FAST! When their little phones/pagers start beeping (which is constantly) they are running to the room to check on that baby, and each nurse has their own station directly out side of the babies room. An awesome NICU might I add!



Ou sweet, swollen baby girl.

Bella was placed in the NICU for breathing difficulties, low blood sugar and a few other things that, what we thought, were related to her prematurity. I was still downstairs and was not able to see her until the next day. Yes, I cried most of the night because I was not allowed out of the bed!! I still had not even met her yet! I only got to see a few pictures that Mike was able to get while upstairs with her :( I was SAD! Luckily, we had some wonderful nurses, and doctors taking care of Bella and they called many times throughout the night to let us know how she was doing and Mike was constantly running up and downstairs to check on his ladies :). Another thing that was really nice about this NICU is that every 12 hours you are assigned a nurse to your baby and you are given their direct phone number, they carry around these phones and the monitor beeper everywhere they go in their pocket. You can call and talk to YOUR nurse anytime of day. They also would place your same nurse with your baby each day depending on the level of care your child was needing. The nurses are ranked so that if you had a "high level" nurse and a baby came into the NICU that was more sick than yours, then you would get a different nurse the next day so that the other nurse could care for the sicker child.

The next morning I was finally able to be wheeled upstairs to see the new princess. It was sad, I was not able to hold her yet and her monitors were constantly beeping. She had a feeding tube and was VERY swollen from all of the fluid I had during my pregnancy.

Meeting my hero for the first time!

The next day the nurses where making their rounds, downstairs, with me, when they noticed that my blood pressure had been steadily rising. The doctors at first rubbed it off as a reaction to the c-section and my body was just trying to find normal again. I was to be monitored a little bit more closely.

Bella really had no changes over these next few days besides having Jaundice (which is pretty common with preemies) and was still on and off of oxygen, on the feeding tube but was having desaturations (dsats) with her oxygen. She would struggle to breath, then stop breathing and then her heart rate and pulse would drop. The nurses would have to intervene with "blow by" oxygen or even put her back onto the oxygen for a few hours at a time and try to diminish it little by little. On day three after Bella was born, our favorite nurse, Pam starting saying things like, I don't think her breathing issues are preemie related and that there may be more issues . They described her as a "stuffy" baby earlier and said she had swelling from being suctioned at birth and that was contributing to her breathing issues as well.

Bella sun tanning :) - Jaundice Treatment.

Bella's day 2 of Jaundice treatment, stylish glasses.

Bella had her feeding tube removed one morning so that she could have a "better try" at feeding from a bottle. She ended up having some really bad dsats and bradys (heart rate dropping) during her bottle feed and needed a feeding tube again. Nurse Pam decided to pass it through her nose so that we could practice bottle feeding with out having to remove the tube. The tube would not go down either side. We were downstairs and got a phone call to come up and talk to her as soon as possible. We got up there and she said we think Bella might have something called Choanal Atresia. Three of the nurses had been trying to pass a feeding tube and the smallest catheter down her nose for the past hour with no success. Then she wrote the name "Choanal Atresia" on a piece of paper and said that they have an ENT coming to see her first thing in the morning. When we looked up CA we were devastated. Our daughter might not have an opening in the back of her nasal passages? She might have to have surgery tomorrow? What are all of these other associated anomalies about that we are seeing in these Google searches? We were nowhere near prepared for this type of news.

Daddy trying to teach Bella how to bottle feed.

The next morning the ENT arrived and was not able to make it down either side of her nose and said that we needed to start neosynephrine drops (which had been used on and off for the past few days) to keep the swelling down and see if it gets better. That afternoon, nothing was working so they started her on steroids. ENT returned the next morning and was able to partially get down one side, he knew it was not totally closed off, but was not sure about the other.

Meanwhile, downstairs in my room my blood pressure continued to rise and I also started to swell pretty bad. I looked like a balloon quite frankly. I remember when the nurse came into check my blood pressure and other vitals I was telling her that I was having a terrible headache and needed some Tylenol, or something to relieve the pain. She checked my blood pressure and ran out of the room, not saying anything, I mean ran. Doctors and nurses started pouring in the room and told me to lay down we are going up stairs. They were on their phones and running me through the halls. My blood pressure was reading 205/128. They told Mike that they had to get me to the ICU immediately as I was past "Stroke" phase and could have a stroke and start seizing at any moment. Mike panicked. I was wheeled into a room and started IVs and medications at lightening speed. Mike said people in the halls were moving faster than he had ever seen. All of our bags, flowers, and my first solid meal since the baby was born was still downstairs in our other room.

I was having what they call Postpartum Preeclampsia. A very serious treatable condition that can happen anywhere from 48 hours to 6 weeks after delivery. Luckily I did not seize or have a stroke. I was seen by many specialist to try and figure out why my blood pressure was rising so much more lying down than standing up. It is usually the other way around. The doctors were baffled. The treatment for Postpartum Preeclampsia is magnesium. The have to give it to you through an IV for 24-48hrs. You have a catheter in and are not allowed to move, so no seeing princess Bella for me. This was even harder now that I new something was terribly wrong with her. I could not comfort her or be there by her side. I was breastfeeding every 2 hours to try and get her the best nutrition I could as a mother. Mike would take what I pumped upstairs every time to get it into her fridge. He would go be with her, then come stay with me. He was awesome at this time when we both needed him so badly! He even found a moment to run out and grab me a subway, since my first real meal was left downstairs. Yes, I got a foot long and ate probably close to all of it!

First "real" meal after Bella's birth.

By the time my treatment was over, I had drained almost 10 pounds of fluid! I no longer looked like a balloon :)

I was released from the hospital with 3 different blood pressure medications. Although Bella was in great hands, the LAST thing I wanted to do was leave our sweet princess there. That was a heartbreaking moment. I felt empty, like I was forgetting something. I cried the whole way home. As soon as we got home I called to check on Bella and laid on the couch. Bella must have wanted me back there because a headache started approaching again. We checked my blood pressure and sure enough I was back at the hospital within the next hour. We were instructed to go straight up to the labor and delivery floor to begin a second treatment of magnesium. Although I was not able to go sit with Bella, I was in the same place. I felt safer being there just minutes from her rather than at home where it would take us almost an hour to get to her side.

One more 24 hour treatment and I was good to go. This time we were sent home for good. The next afternoon we were visiting with Bella, talking with her neonatologists, ENT's and nurses when we found out the Bella was going to have to be transferred to the medical center. I started crying. We knew that was going to be a WHOLE different ball game. No personal rooms, you are actually in what they call a POD with 8 other babies. You don't get a personal nurse and you can't stay with your baby at all times if you wanted too. BUT she was going to get awesome medical care. She was going to be seen by a team of pediatric ENT's that could truly help her and figure out what was wrong with our baby. A team that we are still with today, that have been so special to us this past year. We thank God for them everyday.

Sweet Angel :)

{Bella's ambulance transport and Medical Center NICU stay coming soon...}

Breaking It Down: What Is Congenital Nasal Pyriform Aperture Stenosis?

Most of the world has never heard of CNPAS, or know what it entails. I am going to write a, hopefully, easy to uderstand post focusing only on CNPAS. Not on how Bella is affected by it, but how it affects any child who is diagnosed.

CNPAS is a life-threatening form of nasal obstruction in the front of the nasal passages located at the pyriform aperture and was first described in 1989. The nasal passages are severely narrowed due to bony overgrowth in the mid line of the nose and can cause significant respiratory distress in a newborn. For the first 6 to 12 weeks of a newborns life, they are obligate nasal breathers and unable to use their mouth as us adults would if our nasal passages were impaired.

The first symptom of CNPAS is usually respiratory distress. Most of the time it is believed that the newborn is having "swelling" of the nasal passages from being suctioned during the birth, or considered a stuffy baby and dismissed as something that will pass with time. Unfortunately the babies nose will continue to be traumatized with suctioning, which will actually cause the passages to become more swollen and cause greater distress in the child. Symptoms are typically more pronounced during feedings and relieved by crying and supplemental oxygen, when needed.

Most diagnosis of CNPAS occur when a medical professional tries to pass a small catheter into either side of the nose. Alot of times when the catheter is not able to be passed, it is thought that the child may actually have Choanal Atresia, which is much more common than CNPAS. It is not until a CT scan is preformed that a professional can confirm CNPAS. To be considered a diagnosis, the pyriform aperture must be less than 8mm wide.

{CT image of a newborn with normal nasal passages}





{CT image of a newborn with CNPAS}





CNPAS is sometimes an isolated anomaly, but usually it is associated with other craniofacial anomalies, or a much bigger condition.

CNPAS is frequently associated with Holoprosencephaly (when the brain does not separate completely), hypotelorism (eyes close together), SMMCI, pituitary deficiencies, flat nasal bridge, chromosome abnormalities, narrowed tear ducts and abnormal sinuses.

Some cases of CNPAS are mild enough to just be monitored until the face can grow and hopefully provide more room in the nasal passages. CNPAS repair is needed when the child has cyanosis, apnea, persistent feeding difficulties and inappropriate weight gain. The most common approach to repair the nasal passages is through a sub labial approach. The doctor will make two small incisions under the lip and enter the pyriform aperture area of the nose. He will then drill the bone on either side to open up the nasal passages as much as they can. They can only go so far before they would damage the sinus cavities located next to the nose. Nasal stents are also placed for 4-6 weeks to prevent swelling and re-stenosis (re-narrowing). Re-stenosis is always a possibility, but it is uncommon.

{Bella's Birth Story: Part Two, coming soon...}

Bella's Birth Story Part One: A Barbecue Or A Baby?

Where do I even begin?

July 3rd was a totally normal day; in pregnancy standards that is. It was hot, I was tired and I'm sure I was cranky. I was 34 weeks into my "high risk" pregnancy. I had just seen my OBGYN, Dr. Levin, on July 1st when she informed me that she was leaving for the holiday and that I would definitely make it till the following Tuesday to see her in her office. (I saw her every 2 days for fetal stress tests and ultrasounds) and our first, Kallie, was induced at 36 weeks. I was NERVOUS considering she was never out of town and I was 34 almost 35 weeks pregnant! We were at my in-laws getting ready to have an early July 4th BBQ with the family and I was having contractions. This was nothing new as I had been having them for almost a month by now and didn't think twice about them until I went to the bathroom... and there was blood. I calmly walked outside and Mike (my husband) said, "what's wrong?" I'm sure my face was white. I was not thinking and announced to everyone (including my father-in-law) that I was bleeding. If I remember correctly, the response was where? C'mon people, you are all aware that the pregnant woman was just using the restroom- and are fully aware I had been contracting all day long! Needless to say, I called the doctor on call and she said "since your high risk and 34 weeks, lets get you to the hospital to get checked out, I will call them and let you know that you are on your way." So off we go with no bags, leaving big sis behind and our yummy steaks...

The whole way to the hospital I was in total denial. I was mad about having to leave, I was hungry and most certainly freaking out that if something needed to happen, like have a baby, our doctor was not there! I had a pact with her that we would not be having any babies with out her. When we got to the hospital everything became a blur. The nurse checked me and said I was 2 cm dilated, no different than I was the prior Friday. She then began checking my vitals and hooking me up to the fetal monitors. This is when things began to go sour. My blood pressure was high and the baby's pulse was low. Next thing I knew, they were rushing me to the other side of the floor (one side is for monitoring, the other is delivery / labor) and began preparing me for an emergency c-section. I have NO IDEA what happened next but some how everyone began to calm down and said we are going to wait and watch. This made me happy! I was having a difficult time imagining some other doctor cutting open my stomach and delivering my baby with no idea about anything that has gone on in my pregnancy. I am sure he was a great Dr., but seriously, I like mine better...

So we watched and waited. They gave me tocolytic medicines to prevent labor and gave me blood pressure medicine to try and keep me stable. I really wanted to hold off as long as I could for the baby's sake and for Dr. Levin to return. Tuesday morning they had me scheduled for an ultrasound in the same hospital with the Maternal Fetal Medicine group. They had seen me prior in my pregnancy for a few "big" ultrasounds and wanted to get one done to truly see how well the baby was doing and help decide if it was time to deliver. We agreed and had the ultrasound, although I always had my guard up and really just wanted to see MY doctor. I knew Dr. Levin was coming to see me that afternoon as soon as she was back in Houston, she had already called. I just quietly listened to these other doctors tell me that the baby seems OK and that I needed to stay in the hospital on bed rest to prevent labor and wait to see what my doctor says, blah, blah blah.

When Dr. Levin finally arrived I was totally relived! I was beyond excited to see her and she knew it! She told me "I told you I would be here when this baby arrives, and I will be." Those were some awesome words to hear. She had not looked at the monitor feed yet and said that she thinks we will be waiting until at least the morning for a baby. She then noticed that I was having constant, big contractions and decided to check for her self. Sure enough, I was 5 cm dilated, 90% effaced, and in active labor. There was no time to think, we were having a baby! She warned us about the baby
possibly having to go into the NICU if she was not breathing on her own and also said that the NICU team would be assisting with the delivery. I didn't think much about this and just thought, OK at worst she will need a little bit of oxygen for the first few days, no biggie. Boy was I wrong... Our sweet Bella Michelle arrived at 7:02 pm on July 5th, 2011. I knew something was wrong when they asked Mike to give them a "few moments" and they would let him know when he was allowed to go by her side. Disheartening words to hear when you are laying on a table, stomach wide open and shivering tremendously from a reaction to the anesthesia. This was supposed to be one of the most memorable, happiest days of our lives and it was slowly being filled by grey clouds.

{Stay tuned for a peek into Bella's first 72 hours}

A Peek Into Bella's Life

The past year and a half has been something that I can barely even begin to explain. It has been sad, terrifying, happy, exciting, confusing, overwhelming and most definitely heartbreaking. I have been contemplating starting this blog for a LONG time and have finally decided that it is time for me to let all of this information that is locked up in my brain, flow freely. This is for our friends, family and the rest of the world to learn about our Bella. Her past, future and present. I want future mothers of children like Bella to learn from my blog. Learn how to cope, learn what to ask doctors and learn how to open up and share their child's story as well. This will be a place for me to cry, a place for me to express our frustrations and excitements, and a place to share :)

Bella's birth story will be for another day. For now, I will start with Bella's struggles and diagnoses.

Bella was born on July 5th, 2011 with SMMCI Syndrome and Pyriform Aperture Stenosis. She has one single large top tooth instead of two (her two top teeth are basically fused together). She has narrowed nasal passages, a low palate, feeding difficulties, weight issues, central sleep apnea, spasticity and hypertonia (increased muscle tone). Bella's recent lumbar puncture showed that she had a deficiency in Pyridoxil 5 Phosphate concentrate which can relate to Epilepsy, but we do not know what this means for Bella yet as we do not believe that she has been having any seizures. It has been recommended by a few specialists to test for PNPO gene.

We do not know much about Bella's current diagnoses, but we do know that she has the midline syndrome (SMMCI) and some sort or muscle issues. We do not think that these two issues are related, and that in-fact she will end up with two "separate" main diagnoses.

SMMCI is an extremely rare diagnosis, and very little is known about it. It is estimated to occur in 1:50,000 live births. Nasal obstructions occur in about 1:8,000 live births, and out of those, about 1/3 are Pyriform Aperture Stenosis. To give you an example of how rare this is, Autism occurs in 1:150 births and Cleft lip/ Palate occurs in 1:600 births.

Bella has been in PT and OT for 7 months now. She is starting developmental therapy tomorrow, and speech therapy later this month.

Bella underwent surgery in December, just shy of 5 months old, to try and repair her nasal obstruction. It has relieved some symptoms, but she is still not where we would like her to be. Daily life is a struggle for the whole family and we are uncertain what Bella's future will look like. We do know that she is LOVED by so many, and that her biggest cheerleader is her big sis, Kallie! This has been a long journey for Bella. She has gone through so many tests and procedures to try and give us, and her doctors some insight into our sweet girl. We know that we are nowhere near the end of unraveling God's gift, but he sure did bless us with an angel :)

Over the next several weeks, be prepared as I am going to pull all of this jumbled information off of my brain and share Bella's birth story as well as her 1st year of life!