Loving Baby Ella

An Enormous Thank You

2010-11-09T19:34:00.010-05:00

Friday night's fundraiser for Ella was a beautiful event. Thank you to the Barefoot Peddler for donating some of their profits. I never in a million years thought I would bring my baby into a bar, but hey there is a first for everything :).

Thank you to my dear friends, Laura and Maureen, for hosting and organizing the fundraiser. We will forever be grateful that you took the time and effort, out of the goodness of your hearts, to help our family and our little angel baby. Thank you for this selfless act. You are very special people who I am blessed to have as friends.

Thank you to all the people who came to support us that night and to all the people who were unable to attend, but still made a donation or sent in raffle prizes. We cannot even begin to express our gratitude and the warmth we felt from family and friends, old and new. The funds raised will provide some relief for the many medical expenses we have accrued since Ella's birth.

This year and a half has been extremely difficult for myself, Erik, and Ella and we still have a long road ahead of us. And while this situation has brought some heartache, it has also allowed much beauty to surface. It has given me new perspective on life and taught me never to take anything for granted. It has brought out the kindness, compassion, and generosity of friends, family, and complete strangers (now friends). The idea that people who don't even know us want to help during a trying time truly demonstrates that there is goodness in humanity. Finally, it has brought me the most precious gift I have ever received - Ella, who despite all that she endures still maintains an amazing spirit.

Ella had an absolute blast at the fundraiser. She was very happy, dancing around, and showing all her tricks. I hope many of you got to see her new dance move, "the monkey"- a real crowd pleaser :). It was almost as if she new it was a party just for her. Ella feels the love from all the people around her and she certainly felt a lot of it that night. I will be so happy to someday tell her about the many people who rallied around her, supported her, and prayed for her life.

Thank you from the bottom of our hearts. We continued to be moved and comforted by all the caring people who have touched our lives.

Love always,

Nina

A Long Time Coming....

2010-10-27T05:09:00.013-04:00

Dear Friends and Family,

I am writing on behalf of my mommy who is ashamed to be considered a total blog slacker. Now I won’t sit here and make excuses for her. But, seriously a lot has happened in the past 6 months. I’m not gonna lie…though I know I’m the light of her life, I’m also nothing short of a handful. Anyway, enough about her, let’s talk about me.

I am doing awesome. I turned one in August!!! This was a huge milestone for me and my family, especially since the doctors were not too sure I would survive long after I was born. Clearly God and I had other plans J. Mommy always says she knew I wanted to be here since I started kicking like a maniac in her belly. BTW, that has not changed much. You should see my legs – they’re enormous, but in a good way. Some might say thunder thighs; I prefer muscular and healthy. Future soccer star…watch out. So yes, though I go through a lot and get poked and prodded on a daily basis, I truly am a happy baby. Someone once told mommy, “Ella doesn’t know she has a syndrome or think that she is medically fragile.” And while that statement is quite obvious given I’m baby, if you met me you would quickly learn that it couldn’t be closer to the truth. Someday I will figure out that I’m different than most people – that I don’t have ten fingers, or breathe through my nose, or eat through my mouth (I could go on and on here). But, right now I am content just being me. I know no different. I smile, laugh, and love just like any other baby and want to be loved just like any other human being.

On the developmental front, I am progressing quite nicely if I do say so myself. I am doing things that mommy and daddy weren’t sure I would ever do. I started commando crawling over the summer, then recently it turned into a 3 point crawl with a right leg scoot. I am very creative (did I not mention strong willed???) and so I decided I should improvise when it comes to crawling as well. My PT said she doesn’t care how I crawl so long as I get from point A to B. My parents are huge motivators for getting me to move and so is the laptop. I really love it when mommy plays elmo on youtube and then hides the laptop under a dishtowel for me to find. That’s when I boogy. I just started cruising around the couch and I am learning how to pull to stand (this is difficult for me since my forearms are underdeveloped, but don’t worry I will figure it out soon). In the language department, I am able to sign “me”, “more”, “yes/no”, “hi/bye”, and “all done”. I respond appropriately when mommy says, “touch your belly”, “stomp your feet”, “clap your hands”, “so big”, “hands up high”. When she says “oh my goodness” I put my hands on my cheeks (very cute). I am taking tastes of baby food by mouth, not for nutrition, but rather just to keep up oral stimulation. I also started to open up my mouth a bit more and now I can even make purposeful noises past my trach. I especially like to pull out new noises at bedtime (like I said I’m just like any other baby, I’ll try anything to delay going to sleep). Some might say manipulative, I prefer smart.

On the medical front, I’ve had a few bumps along the road. Last Spring, I had a bad staph infection at my trach stoma site. Trust me, “impressive” and “I’ve never seen this before” are two comments you don’t want to hear from a doctor…highly reassuring. I was hospitalized at CHOP last June when going for a routine consult. I had a nasty case of Rhinovirus, which is like the common cold, but when you’re dealing with a trached kid it doesn’t seem so common. So far this Fall I’ve had another cold and then croup without being hospitalized (thank God). In September I had major hand surgery to move my right index finger into an opposable thumb position. I was in a full arm cast until I managed to remove it 4 days post-op. This little stunt sent my mom and nurse in a frenzy down to CHOP’s ER in the middle of the night. My parents were happy when I went into a hand splint (until of course I figured out how to remove this too…hourly). Today I went to CHOP to see the surgeon for a post-op visit and he said my new thumb looks excellent. It even started to move a little yesterday. But, now the real work begins – I have to exercise my thumb daily and I go to outpatient hand therapy 2x/week (this added to my already busy schedule of 11 other therapy sessions per week plus Dr. visits). I should be moving it more in the next two weeks and if all goes well I should be pinching with my new thumb 6 months from now. Later this year, I will undergo the same surgery on my left hand. As far as my jaw and cleft palate surgeries, mommy and daddy are taking me across country to meet with some of the top experts in their field. It will be a difficult decision to make since many docs have differing opinions on what methods to use for the jaw distraction. Because there aren’t many kids out there with Nager there isn’t much research on which is the most effective/least risky. Most of their opinions are based on personal experience with patients. I will likely have eye surgery this year as well. My eyes tend to wander outward from time to time and so to fix this they have to weaken my outer muscle, which will force the inner muscle to get stronger. Hopefully, they can combine at least two of these surgeries so that I won’t have to go under anesthesia so many times.

Mommy and daddy are hanging in here. They are doing the best they can to take great care of me. They are often sleep deprived and extremely busy - not only handling my medical care, but also dealing with issues involving insurance, Medicaid, medical supplies and equipment, nursing coverage, therapies, and doctors visits. Some days are more chaotic than others and they are still trying to take it one day at time. People say there is no book that teaches you how to be a good parent; well there is certainly no book on how to be a good special needs parent. You got to go with the flow and just hope and pray to God that you are doing right by your child. At the end of the day (or even at the beginning) my parents may be tired, stressed, and overwhelmed, but they say I am worth it all. They tell me that I bring them more joy in life than anything they have ever experienced. And hey – I feel pretty good about thatJ.

Thank you everyone for your help this past year and especially for all your prayers and good wishes. I know you can’t truly understand unless you are going through it yourself, but many of you through words and actions have shown us that you are trying to understand and that means the world to us. Please continue to keep our miracle baby, Ella, in your prayers. We ask for prayers that He continue to make her healthier and stronger in body, mind, and spirit each and every day. Please pray for guidance for Erik and I that we make the best medical decisions for Ella, particularly concerning her upcoming surgeries.

I also want to thank my dear friends Laura Leonardi and Maureen Alchermes who out of the kindness of their hearts are hosting a fundraiser for Baby Ella. This is a beautiful gift to Ella, Erik, and I and will help greatly with the medical expenses we’ve been accruing for Ella. The fundraiser will be Friday, November 5^th from 5:30-9:30pm at the Barefoot Peddler in Greenvale. For more info, you can email lleonardi3@yahoo.com or alchermesm@yahoo.com

Much love always,

Ella Rose

P.S. Apologies for poor grammar/spelling…I did write this at 3 in the morning while staying up as Ella’s night nurse.

Home Sweet Home

2010-03-15T00:50:00.008-04:00

Two months. Nearly two months since we’ve been home with Ella and can spend each night as a “somewhat normal” family. By that I mean all of us sleeping under one roof, not separated by time, work, highways, or bridges. It was surreal bringing our little angel home, leaving the controlled environment of the hospital and entering a world of unknowns. After 6 months of living in a NICU, we were finally home… finally able to watch her sleep in the middle of the night, kiss her in every single room of the house, bathe her in her own tubby, and eat a meal with us all around the kitchen table. It took a long time to get to this place…so let me rewind a bit.

The week of November 16^th Ella was to be transferred from CHOP to LIJ/Schneider Children’s Hospital in New York. This was the most stressful and grey hair-inducing week of my life. To make a very long story short, within a span of 5 days Ella ended up in the operating room twice and coded once. It was the second time in her short life that we almost lost her. Blood started coming up from her trach and quickly obstructed her airway. She stopped breathing, turned blue, and her heart rate went down to 44 (a number I will never forget). Again, God was with her. After giving Ella breaths with an ambu bag, a nurse was able to get her to come back up. Clearly, Ella didn’t want to leave CHOP and was pulling out all the stops to avoid it. So when the day finally came to transfer her to LIJ we made sure to keep it a secret.

Our stay at CHOP was entirely due to medical necessity; our stay at LIJ on the other hand was entirely due to the bureaucracy of New York State’s Medicaid system. In order to take Ella home we needed to have nursing care at home, something our private insurance doesn’t cover. This was the worst part. We knew Ella was stable enough to come home, yet because the Medicaid paperwork was taking forever to get to and through Albany Ella had to remain hospitalized. And each day we thought we were one step closer to taking her home, there’d be a setback with the paperwork damaging our hopes of ever being together as a family. Thanksgiving passed, Christmas passed, New Year’s passed, my birthday passed and we were still there. I tried to remind myself that though it was disappointing to celebrate such events in a hospital, away from home, it would have been more devastating to celebrate those days without my beautiful baby girl. Finally, the day came when we were approved for the Care at Home program through Medicaid. We were all on cloud 9, but still cautious given our past history. We had to tell Ella a little fib – that she was going to Disney. After all, we didn’t want her pulling any of the same funny business like when we were due to leave CHOP. Erik and I both kept saying we won’t believe it’s real until we walk through our front door with Ella in our arms. On January 18^th it happened.

We are home now and there are moments Erik and I still can’t believe we made it to this point. Though being home has brought an entirely new set of challenges. We struggled to get the Medicaid and now that we have it we’re struggling to get qualified nurses. Taking care of a person with a trach can be intimidating to some; taking care of a BABY with a trach can be outright scary to even the most seasoned nurse. So until we have a full and competent staff life will be a bit hairy in the Monaco household. I am indebted to my mother who is staying up nights with Ella. Without her we would have never been able to take Ella home. The days are long and the nights are longer. She gets continuous care throughout the day and in order to avoid a code situation like at CHOP her cardiac and respiratory status must be monitored 24/7. However overwhelmed, stressed, and sleep deprived we all are, we wouldn’t change having her home for the world.

Ella is doing fantastic. She is a whopping 14lbs 11oz and growing longer by the day. She is extremely active. In fact, her nickname is Little Zilla (short for Godzilla) because is not unusual for her to make her entire crib shake. Ella smiles, chuckles (this takes major entertainment on our part), makes her version of a raspberry, shakes her head NO, slaps her legs when excited, rolls to her side and to her back, and is now learning how to sit unassisted. She has a hearing aide that she loves to take off (a major challenge for us) and is constantly popping off her HME (the little sponge on the end of her trach). She plays with toys and is even learning to compensate for her lack of the thumbs by grasping things between her index and middle fingers. She was recently evaluated for Early Intervention Services and will begin receiving therapy in two weeks. She’ll get occupational therapy 3x/week, physical therapy 3x/week, speech/feeding therapy 2-3x/week, and special education 1x/week. Phew…she’ll be one busy lady and I’ll of course be her secretaryJ.

From a medical standpoint: she is going to have the first of two pollicization surgeries around 1 year of age. This is when docs will move her index finger into a thumb position so that she’ll be able to grasp things without limitation and eventually do things like button a shirt and use scissors. Oh how we take for granted all that we can do with thumbs! Around one year of age she will also be reevaluated for jaw distraction surgery and a repair of her cleft palate. Right now her jaw is too tiny for the device used for the jaw distraction and she doesn’t open wide enough for the surgeon to get into her mouth to do the cleft repair. Ella’s heart problem is still under control, with no obstruction presenting at this point. Because it is such a rare finding, she will have to be closely monitored and get an echocardiogram every two months. There is more, but I’m drained just writing about it so I will it leave there for now.

So we’ve had a bit of bumpy road this past year and a half and I’m not sure when it will get easier. People often ask me “how are you doing it?” Honestly, never in a million years did I think I could do this and sometimes I still think how in the world am I going to do this and get it all right for Ella. I’m never going to make Ella totally healthy, give her new thumbs, or help her brain grow faster. I’m doing the best I can and by that I mean giving her the love and support necessary to meet her greatest potential in life – whatever that potential may be. I will never give up on her; she hasn’t given up on us. For many months I’ve been underestimating this baby and frankly it was out of self-preservation and protection. I’m learning that no child should be doubted or low-balled for such selfish reasons. Ella is still a child of God and I’ve decided to let her show me the way. And so you wonder how am I doing it? I just do. Life is funny like that…we surprise ourselves and somehow rise to the occasion when least expected, especially when Love is involved.

I want to say that many of you have brought countless smiles to our faces. To those of you who have sent gifts, brought over hot meals, went to the grocery store, pulled all nighters (you know who you are): thank you from the bottom of our hearts. People have taken their time, money, and energy to help make are situation a bit easier. And please know that you have. Many of these people have never even met Erik, Ella, or I, yet have acted as family and friends would. These are true acts of kindness and generosity for which we will be forever grateful. Thank you to those of you who continue to pray for my dear Ella and for her health, for our strength and perseverance, and for the hands that care for her. Prayer is the most important gift of all, for without it I don’t believe my little angel would be with us today. Lastly, in such a bitter situation thank you for helping us see the sweetness in people.

To Ella: Thank you for surprising me everyday with the incredible amount of strength that flows out of tiny you. Ti amo.

Much love and gratitude,

Nina, Erik, and Baby Ella Rose

P.S. Apologies for not responding to the many emails I’ve received over the last 7 months. While I did not always get an opportunity to write back, please know I did read them and they did comfort me.

Not Without a Hitch

2009-10-30T17:39:00.005-04:00

Ella is still recuperating and so I thought I’d give her a little break from writing the blog. So I know I’m not as funny as the little one (not as cute either), but for now I will have to suffice. I apologize for not posting sooner; it’s been two weeks and we’ve hit a few bumps along the road. Ella’s surgery went as planned on the 16^th. She got her G-tube (feeding tube) put in and her rectum rerouted to its appropriate spot. You can see in the picture the large appliance on her belly that is holding the feeding tube in place. Thankfully, she won’t have that forever. In 2-4 weeks, when she is a bit bigger and her stomach is more healed, they will replace the appliance with what is called a mickey button. Basically it is a flap (flush to her stomach) that we will be able to open and close when she needs to be fed. Her new bottom looks amazing. In fact, the most exciting thing that happened two days after the surgery was that she pooped out of her new hiney! This little event that us healthy folks do every single day and take for granted was a milestone for my baby Ella, and one that we celebrated. So all in all her surgeries went well. She came out of the OR looking great, until we noticed her foot. Before surgery an IV was placed in her left foot. She couldn’t eat for 6 hours prior to surgery and this was making her quite mad. She was kicking (again with the bicycle), screaming (not literally of course), and turning beet red. When they got up to the OR, before they even put her to sleep, they realized that her IV had infiltrated. When this happens the IV fluids leak into the surrounding tissue causing a chemical burn. This was upsetting for us and certainly painful for Ella, but it has since improved and looks a lot better than it did a week ago. And as if this little girl needed another thing to cause her discomfort, her trach site (called the stoma) became inflamed last week and began oozing discharge. While in the OR the anesthesiologist recommended they change her trach to a cuffed one. This kind of trach allows for minimal air leaks. On Friday, when Erik and I performed her trach change, we noticed that her stoma looked very irritated and swollen. The ENT thinks it is inflamed because of her skin not being used to the different type of trach. They’re giving her steroid drops down the trach, which hopefully will help the swelling and redness come down. So with good reason, Ella has had days when she’s been extremely agitated and fussy. On her bad days, she cries often and is inconsolable. She is off the morphine, but they give her Tylenol from time to time to take the edge off. Yesterday was our first good day, thank God. She took long naps, but also had many calm awake periods. She played with the occupational therapist and even practiced sucking on her passy.

As for Erik and I, we too have our good days and bad days. For Erik, it absolutely kills him to leave Ella and I on Monday mornings. We keep in close contact during the week, but of course he wants to be here and see for himself what’s going on with the little one. For myself, it is very difficult to be without my husband and family. There are days when I could really lean on them physically and emotionally, days when the stress of it all starts to drain you. There are still times when we mourn the loss of a typical baby experience. After three months of living our lives in a NICU, we long for that day when we can take Ella home and finally be a family. Never in a million years would we change having Ella. Sure, we wish she was healthy - we definitely would change that if we could. And certainly if we could, we would take away all of her pain in a heartbeat. But, despite all the heartache that we are going through she remains the best thing that has ever happened to us. She has brought a joy to our lives unlike anything we have ever experienced. On days when I am down, I try to remember that it is not about me, but about Ella. She is our mission, the reason we wake up each day, continue to put one foot in front of the other and keep going. And every morning when I arrive at the hospital and see her beautiful eyes and sweet lips, a smile comes to my face reminding me of how blessed I am to have her in my life.

Thank you again for all your prayers for Ella and our family. Please continue to pray for her healing and our strength.

Love always,

Nina

Another Lesson

2009-10-12T23:41:00.006-04:00

This past week was bittersweet. I lost my roommate, John. He was brought here after his mommy noticed that his tummy was getting severely distended. It turned out he had a neuroblastoma. Mommy was sitting with me on the sleep chair and the nurses were preparing to transport John to radiation when all of a sudden nearly 30 doctors and nurses blast through the doors attempting to revive this helpless baby. Mommy was shaken up and after a few minutes they asked her to leave with the social worker. When she returned an hour later she learned that John did not survive. She heard his parents behind the screen sobbing, devastated by this horrible and completely unexpected news. Mommy wanted so badly to pick me up and hold me in her arms, but she didn’t. She wanted to be sensitive to John’s parents. As they walked out of the room, she didn’t want to remind them of the thing they will no longer be able to do – hold their precious child. It was a sad day, but mommy’s eyes were opened from it. Her and daddy have been getting upset at the fact that it has been nearly three months since Mommy has been away from home and her family. They have been upset that they can’t bring me home yet and that they’re not even sure when they will be able to bring me home. After John didn’t make it, Mommy realized that they have to be grateful that I am here, that I am strong, and for the amazing progress I have shown. The events of the day brought her back to August 7, 2009 when the docs told her I might not survive. It reminded her of how far I’ve come and just how thankful she should be because of that.

As for me, I am feeling much better than when I last wrote. I am getting back to my normal self, no longer lethargic and breathing fast. I am now eating 60mls of fortified breast milk every three hours. I am packing on the pounds, weighing in at around 7lbs 6oz. Do you believe one of the nurses even called me thunder thighs! Unreal…damned if you do, damned if you don’tJ. I am also back to sprinting now. Today, I went off the ventilator for 8 hours straight with no problems. My surgery is set for this Friday, October 16^thand I don’t think I’m going to get out of it this time. I just ask that you pray for God to continue watch over me and touch me with His healing hands. My mom asks for prayers that He helps me to recover quickly and that He guides the hands and hearts of the doctors and nurses taking care of me. I think my feistiness along with your prayers will help me to live up to my nickname here at CHOP, “NICU Rockstar.”

Love and God Bless,

Ella Rose

Surgery Postponed

2009-10-04T19:06:00.008-04:00

The stars didn’t quite align for me to have my surgery on Friday. It all started this past Monday. The docs did their rounds in the morning and because they thought I had a great weekend they not only bumped up my sprints to 6 hours twice a day, but they also increased my feeds to 55mls per feeding. The latter I was psyched about because I don’t know if you noticed, but I’m a hungry little girl. Plus, I’m growing so I need all the calories I can get. Believe it or not, I am now 6lb 12oz and proud of my chubby legs and adorable rolls (a little scared though, cuz sometimes my mom and dad say they want to eat them…not too sure what to make of that). Anyway, little did the docs know that I was brewing something. On Monday, I had to end my sprint an hour early because I was retracting (using accessory muscles to breathe). This means I was getting tired and thus having difficulty breathing. Then on Tuesday, not even an hour after I started my sprint I began getting tachypneic. Fancy word, right? My mom’s medical vocabulary is becoming pretty extensive. In fact, it’s funny because sometimes the docs go to her for updates rather than the nurses. So that big word, tachypneic , just means that my respiratory rate was high, in other words I was breathing fast. This coupled with me looking lethargic and having a high CRP level (measures inflammation in the body), made them suspicious that I am under the weather. The docs felt that they rather not send me to the OR when I’m not at my best; when I’m not 100 percent. So I wiggled my way out of it for now, but I’m sure I won’t be able to put it off forever.

In other news, I am starting to make some progress in the area of sucking. Not only is my jaw small, but my mouth is not so spacious either. I like sucking on my mom’s finger, but not so much on that thing they call a passy. The passy is not nearly as soft and malleable as my mom’s finger. Plus, I get frustrated with the passy because I’m not able to really control it in my mouth. Oral stimulation is important for me because trached kids sometimes develop oral aversions, especially since they are not used to eating through their mouths and tasting food. Also, my mom and dad each completed their first trach change. This must be done once per week so that bacteria don’t develop in the trach and get me sick. Mom and dad cleaned my stoma (the opening where the trach goes in), and then pulled out the old trach and the respiratory therapist put the new trach in. Mom said her stomach dropped the second she pulled the trach out. Each time they change my trach, the worry is that they won’t be able to get the new trach in and that I won’t have an airway. But, thankfully that didn’t happen and they know what to do in such an emergency situation like that. The final good news of the day is that from a developmental standpoint I am developing appropriately so far, yeah!!! Friday mom met with the developmental psychologist, who meets with parents of all the “old timers” in the unit. To qualify as an “old timer” in the NICU you must be 2 months or older. I feel bad for my 7 1/2 month old roommate, Max. He must be considered ancient! So I am doing things like focusing on faces and bright colors, following people as they move about me, grabbing at toys, my face (the scratches prove it) and any other items I can get my hands on, adjusting to new positions and experiences (the swing, papisan, etc). Speaking of, that vibrating chair is calling my name right about now. Gotta run, but I will update you when I get a new date for my surgery.

Love always,

Ella Rose

Introducing Ella Rose Monaco

2009-09-25T01:01:00.006-04:00

Micrognathia (severely small jaw), obstructed airway, tracheostomy, cleft palate, absent thumb, underdeveloped thumb, underdeveloped radii, fused elbows, small lungs, heart anomalies, asymmetrical ventricles of the brain, imperforated anus, conductive hearing loss, and eyes that make your heart melt. Phew…that was a mouthful. My name is Ella Rose Monaco and my mommy and daddy tell me that I am the best thing that has ever happened to them.

Though I’ve come a long way, my entrance into this world was a rocky one. I was heavily sedated, catheterized, trached, and had many tubes and wires coming out of me my first few days of life so I will try my best to recall the chain of events J. When I was born I was breathing on my side, but whenever they turned me on my back my tongue would fall back, obstruct my airway and prevent me from getting the oxygen I needed. They tried to intubate me, but that didn’t work so they had to put a hole in my neck, straight to my trachea, so that I could get air through a tube. Still my pulmonary veins were so tiny that they weren’t doing a good job of getting oxygen to the rest of my body. The docs weren’t too sure I would survive, but I’ve been proving them wrong ever since.

You see, I’m a fighter; I don’t give up that easily. I’m also a feisty little one. Small, but feisty - just like my mom. Even though my mom thinks it is the saddest thing to see me cry, but not hear sounds coming out of my mouth, I have plenty of other ways of showing her I’m angry. I kick (mainly the bicycle), I turn bright red, tears roll down my cheeks, oh yeah and my heart rate goes above 200. Like any other baby, this usually resolves when either a) I get picked up (which my mom is a sucker for) b) I get fed or c) I get patted firmly on my hiney (I know it is a little odd, but I like it, what can I say).

I have overcome many obstacles for being less than 2 months old. I no longer need to be sedated (only when I have a big test or procedure), my catheter was removed (I do an excellent job of peeing and pooping on my own in case you were wondering), and I don’t need any IVs or special lights anymore. In the beginning I was not able to eat for a couple weeks. Now, they feed me through an OG tube that goes from my mouth down my esophagus to my belly. That silly tape that covers my upper lip (or white moustache as mommy likes to call it) secures my feeding tube in place so I don’t pull it out. I take 50mls of my mom’s breast milk every three hours. I don’t know what she puts in that stuff, but whatever it is it’s certainly working. When I came out I was a peanut, weighing only 4lbs 5oz. Now I’m filling out quite nicely; in fact I just broke the 6lb mark.

When I was first born, I relied heavily on the ventilator. Right now I am getting minimal support from the ventilator. They are allowing me to “sprint”, meaning for four hours in the morning and four hours in the evening I go off the vent and breathe on my own. Sometimes I do incredible and impress everybody, and other times I start to get a little tired. They can sense this because I start to breathe fast and my nostrils begin to flare. When that happens they put me back on the vent to let me rest. I’m still very tiny and they don’t want me to overwork my lungs.

As for my diagnosis, the docs think I am showing characteristics of a very rare genetic disorder, Nager’s Syndrome. There have only been 90 documented cases in the whole world, so I am very special. Thankfully, they ruled out Fanconi’s Anemia and my chromosome tests all came back normal. Unfortunately, there is no test yet to confirm that I have Nager’s and that is because they haven’t found the gene for it yet. Mommy and daddy donated a little bit of my blood to a research study with the hope that we can help them find the gene that causes Nager’s. Frankly, what they call my diagnosis means less to me than the fact that I am getting bigger and better each and everyday and that I will hopefully have a good quality life.

As for my mom and dad, they are doing well. My mom spends her days and nights at my bedside, holding me (she cherishes this since she wasn’t allowed to pick me up until a few weeks after I was born), changing my diaper, pumping breast milk, setting up my feeds, and taking care of my trach. My dad has to work during the week, but always comes down to spend time with me on the weekends. He even comes to say goodbye to me at 5:30 in the morning on Mondays before he leaves for work. He likes to do all my care on the weekends since he doesn’t get to see me during the week. Mommy and daddy often argue about who gets to hold me, while I’m just sitting there thinking, “someone, anyone, just pick me up already!” Mommy and Daddy are also taking a trach class on the weekends. They are learning all about how to suction my trach, change my trach gauze and strings, and change the trach itself. They seem a little scared about all the things that can go wrong, but I am confident that they will rise to the occasion.

So all in all I’ve made amazing progress (you can see this from the pictures-top being most recent). Your prayers and messages of love and support have definitely been working…so we thank you from the bottom of our hearts. God is listening and is working a miracle through my little body one day at a time. Mommy truly believes He has a parade of angels watching over me and that I myself am a little angel sent to touch the lives of many. Please continue to ask Him to heal me. I have made great strides, but I still have a long journey and many challenging roads ahead of me. I especially ask for your prayers next Friday, October 2^nd, when I will be undergoing 2-3 surgeries. They will be repairing my rectum, basically rerouting it to where it is suppose to go. The docs will also be putting in a G-tube, a feeding tube that goes directly into my belly so that I can’t pull it out like the one in my mouth. So the next few weeks will be a little scary for my mom and dad and likely uncomfortable for me, but hopefully worth it in the long run.

Love and gratitude,

Ella Rose

Counting Down

2009-07-24T22:46:00.004-04:00

It has been nearly 5 days since I've landed in Philly (really Camden, NJ since that is where I'm boarding). As usual, it has been a whirlwind of a week. One would think I would get used to this rollercoaster ride experience of carrying a child with an unknown syndrome, but I am not numb to it yet and they certainly catch me by surprise when something new or unexpected is thrown my way. Let me explain... My sister, Gina, and I went to CHOP for an appointment with the maternal fetal medicine (MFM) Dr. on Tuesday. This was just for a routine growth sonogram to check the baby's development and amniotic fluid levels. The Dr. said my ears should have been ringing lately because Ella has been the talk of the hospital. There has been great debate over whether or not to do the Exit procedure as a mode of delivery. Though they initially thought it was not necessary, the airway team seemed to have second thoughts. Their argument is that it is the most controlled situation, since the baby will be oxygenated through me while they are trying to access the airway. They also are worried that if I go into spontaneous labor that they can't guarantee that the right docs will be available to help her. On the other side, are the OBs/MFMs and the hospital's chief of surgery who feel they will have sufficient time to access the airway and that an Exit is not necessary. Each team is coming from different perspectives, with the airway team considering doing what's best for the baby and the OBs considering what's least risky for the mother. And of course, my husband and family were adamantly opposed to the Exit since it is a rare procedure with higher chances of blood loss, hysterectomy, and morbidity. This put me in a most difficult position because I want to do everything I can to protect Ella, but I also have to consider the feelings of my husband and family.

I would be lying if I said I wasn't scared...if we don't do the Exit there's a chance that the baby will suffer the effects of oxygen loss and if we do it there's a chance that I will suffer the effects of blood loss. After our meeting on Tuesday, I had this completely unsettling feeling. Nothing had changed with the baby in the last 4 weeks for them to turn the tables on me. Ella grew significantly (now weighing approx. 4lbs) and the amniotic fluids were normal, suggesting that she's still swallowing. So we met with the airway and OB docs again yesterday. I expressed my concerns much more clearly this time, as I was no longer caught off guard and could gather my thoughts and articulate myself in a better fashion. It was obvious that the two teams were on different pages. It was also obvious that one of the major reasons that this was even being discussed was that the ENTs were not able to guarantee that their airway experts would be available upon normal delivery. We came to a compromise - if an Exit is the most controlled situation and a vaginal delivery the least, a cesarean was somewhere in the middle.

On Thursday, August 6th, I will be having my baby via c-section. I am praying to God that they will get her breathing right away and that the worst case scenario is that they will have to trach her. There are so many unknowns with this child, however, one thing we do know is that we already love her dearly and we cannot wait for her arrival. In less than two weeks, we will meet her for the first time. The waiting and anticipation will be over with and we will just begin to know better what her issues are. Her birthday will be the first day of a very long journey, equally beautiful and challenging I am sure. I just ask that you continue to keep this little girl in your heart, praying to whomever your God that her delivery goes smoothly and that she has a chance at having a good quality life. Also, Erik, myself, and my family are not nearly as strong as everyone thinks we are. In fact, there are many of days that I feel like buckling, like I cannot handle one more stitch of bad news. So we just ask that you pray for our strength and courage to get through this adversity. We still have faith that a miracle can happen, that God can heal baby Ella. If that is not His will we will cherish her and love her with all of our hearts just the same.

Reality

2009-07-08T14:05:00.000-04:00

My mother and I just returned from our second trip to Philly. It was a mentally exhausting two days, meeting with doctors, midwives, social workers, and digesting the reality of what our lives will be like for the next year or so. We first met with the maternal fetal OB and it looks like things are status quo with the baby. Fluid was seen in her stomach, which is a good sign because it suggests that she is able to swallow. Though she is still extremely small (below the 10^th percentile), she did grow from our last visit and is now weighing in at 3lb 2oz. Docs will continue to monitor the fluid levels and her growth, as it will determine whether or not we do an Exit procedure and when she will be delivered. If her growth plateaus then they may deliver her early because she’ll have a better chance of growing outside the womb.

We also met with the craniofacial specialist. Without a normal size jaw, Ella would never have a chance of breathing on her own. They have several ways to correct the jaw, however it completely depends on how much bone they have to work with. They do what is called a jaw distraction: Under anesthesia, the surgeon cuts the jawbone on both sides of the face. He then anchors an appliance to the bone and turning a screw on the distractor gradually increases the distance between them daily. New bone is formed in the gap as the gap widens. Once the desired length is achieved, the turning stops and the device is usually left in until the bone is stabilized. This is usually 6-8 weeks, but it varies depending on the child. If there is enough existing bone, the distraction surgery will take place a 1-2 weeks after birth.

If there is little to no bone to work with, then the surgeon must wait until there is some growth or they have to borrow bone from another part of her body. In that case, she would have to be trached, because intibation is not sustainable over a long period of time.

The lovely people at CHOP gave us a tour of the Special Delivery Unit as well as the NICU. It was definitely a reality check for us. The room where I will deliver has a window through which they will quickly pass Ella once she is born. Waiting on the other side will be an airway team made up of ENTs and other docs, who will first try to intubate her. If the intubation is unsuccessful, they will then try to trach her. The number one priority, of course, is getting this baby oxygen. Unfortunately, we learned that we will not be able to hold her for some time, at least until she is off the ventilator. This is extremely upsetting to us because as parents-to-be you long for those first moments when you can look into your baby’s eyes, cradle her in your arms, and caress her. However, we know it is for her own good and, luckily, we will still be able to look at her at touch her.

Equally upsetting was seeing all these sick, tiny, helpless babies in the NICU and knowing that my baby will soon be one of them. You can’t help but want to pick up each and every one of these children and whisper in their ears that everything is going to be all right. Though I know my baby will be in the hands of expert doctors and compassionate nurses (you have to be to work in a NICU), I still wish I could hold her in my arms and take her home like most other parents can.

And so the decision was made that I am due to move down to Philly on July 20^th, when I’ll be 35 weeks. It seems I will be there for quite a while, roughly a month before she is born and likely several months or more after her birth. Before birth, I will be staying at the Ronald McDonald House in Camden, NJ. Once the baby comes I will be moving to the Ronald McDonald House in Philly, which is only six blocks from the hospital. I will have some time on my hands and rather than letting all this worry consume me, I would like to find things to occupy myself. If anyone has any suggestions (a good book, movie, hobby) or would like to come visit I would be absolutely welcome to that.

Finally, let me end by saying thank you for the outpouring of love and support. Thank you for your many phone calls, emails, comments, prayers, and offers to help. If I haven’t responded to you, I apologize. It is still very difficult for me to talk about without breaking down. I thought with each passing day that it would get easier, but with the reality of our future setting in it remains tough. I know this will eventually change and that once this child arrives we will not only accept our circumstance, but truly embrace it. Please know that hearing that so many people care about Erik and I and about this little one, whom you have yet to even meet, brings us great comfort.

Love and God Bless…

Nina

P.S. A few people had mentioned they are having problems posting a comment on the blog site. I amended the settings to allow all people to post, regardless if they have a Google account.

33 Weeks

2009-07-06T10:17:00.000-04:00

I decided to follow my gut instinct and go down to Children’s Hospital of Philadelphia (CHOP) to get a second opinion for baby Ella. We originally decided to go down to find out whether an Exit Procedure was necessary as a mode of delivery. An Exit Procedure is performed mostly in cases where the baby’s airway is obstructed. In our case, it is the micrognathia (severely small chin) that made Dr.s in NY think that the baby would have a hard time breathing on her own. It is similar to a C-section, however the mother is put completely out, an incision is made, and while the baby is still partially inside the uterus and connected to the umbilical cord they try to intibate or trach her. So if all goes well the baby is not without oxygen for even a second. However, the mother is given a specific anesthesia to keep her uterus big and relaxed - which in turn increases the chances of heavy bleeding. This is an uncommon procedure, with the risk of morbidity mostly to the mother due to possible hemorrhaging. CHOP has done over 80 of these surgeries, whereas hospitals in NY have only done a handful.

So the good news (at least for now) is that CHOP docs did not think that I needed an Exit. The sonograms and fetal MRI showed that the baby is swallowing amniotic fluid, indicating that she may be able to breathe on her own. They will have to reassess the need for an Exit if the amniotic fluid in my uterus starts to rise. In a few weeks, I will be relocating to Philly so they can monitor this situation more closely and so that if I go into labor early I will be close to the hospital.

The bad news is that this baby likely has one of three syndromes – all of which are extremely rare and serious. They mostly involve facial and limb defects. However, one of them, Fanconi Anemia, can be fatal as it is associated with blood and other types of cancers. Right now they think that her right thumb is underdeveloped and they don’t see a thumb on her left hand. Her ulnae are also way behind in growth and she has a few vertebral anomalies. They also are concerned about part of the back of her brain. They are not sure if the black on the scan is an indication of fluid or underdevelopment.

As you can imagine, Erik and I are shocked and devastated by this completely unexpected news. Our feelings are so volatile right now, one moment feeling proactive, the next angry, and the next sobbing. And while I know that this baby is still blessing and will serve a very special purpose in this world, I cannot help but feel pain for her already. I am her mother and it absolutely kills me to think that she will suffer or that we will lose her. We are trying to take it day by day and think positive, but this is hard to do when all we read or hear about these syndromes is terrible.

This is the most difficult thing I have ever had to endure in my life. I know that God has a plan in all of this, though I am not yet sure what it is. Perhaps it is to bring us closer to Him, to each other, or to show His love through this little spirit. I truly feel he is giving us this baby because he knows we will not forsake her. He knows that through all the trials and tribulations we will face we will continue to love and care for her, showering her with compassion and affection every step of the way.

So we thank you all from the bottom of our hearts for your continued love, support, encouragement, and most of all for your prayers. Erik and I are extremely grateful to have one another and to have such loving family members and friends. There is no way we could get through all of this on our own. While the road ahead of us will not be easy, I know in my heart we will get through this and that we will eventually smile and laugh again. No matter what illness plagues this baby, she is still ours and she is still a precious human being, a beautiful blessing sent to us by God. We already love her so much and will continue to love her unconditionally.