One Day at a Time

Hi Everyone,

I'm sorry it has taken me so long to write an update, we have been overwhelmed taking care of Ella these past few days.  Not enough time to eat or sleep, forget about think and type.  She was released from the hospital on Saturday afternoon and since then we've been back at the Ronald McDonald House in Charlotte.  Ella is doing better, Amen.  She is her old self about half the time, while the other half she is irritable and in pain (as expected).  The good thing is that we've been able to wean her off narcotics and just manage her pain with motrin and tylenol.  She is a feisty lady during pin cleaning...takes 3 people to hold this strong girl down.  The first time I turned her pins was on Sunday and I felt like I needed a carafe of wine afterwards.  It seems so barbaric, especially when you hear the clicking noises of the screws turning under the skin.  And of course it just feels horrible inflicting pain on your child.  But, I've been reminded that with each turn of the pins we gain a bigger airway.  I have to keep reciting that in my head :).  Lucky for me this little girl is incredibly forgiving and she recovers quite easily afterwards.  This entire experience has Erik and I feeling absolutely blessed to have this child in our lives.  Seeing her through such a tough time and then watching her spirit come back has been truly uplifting.  She started to show her "Ella" self on Friday, wanting to be pushed around the hospital floor in a wagon, then ordering the nurses to dance and play peek-a-boo.  Saturday as we left the hospital her entire demeanor changed; she was so happy, like she was being freed and allowed to finally grace the outdoors.  Ella is loving the therapy dogs at the Ronald House, she asks for them every single night though they only come twice a week.  She keeps signing to us that she wants a big dog when we get home to NY...and believe me she won't forget.  Persistence is one quality she absorbed from her parents, maybe me :/.  Today we saw Dr. Matthews and other than her pin sites looking red he thought she looked good.  He wants us to increase the pin turning to twice a day and switch the ointment to calendula, just in case the bacitracin is irritating her skin.  We will be back to his office on Friday, when he will administer FSM (Frequency Specific Microcurrent).  FSM is suppose to help in decreasing inflammation and repairing cells.  Other positive news to report is that Ella is already smiling and offering up kisses to Erik and myself.  Those are great signs because it means Dr. Matthews was able to steer clear of her facial nerves.  She even put a spoon in her mouth last night, which is awesome because a few days earlier she was aversive to anything coming even close to her face.  Once again, Ella has shown us just how amazing of a being she truly is and how God continues to walk beside her.         

Love and thanksgiving,

Nina


P.S. For those coming to visit soon dress for warmth and bring your flip flops.  It already feels like summer here in Charlotte.  We thank you again for sacrificing your time to help Ella and our family.  

 Still not at her best, but at least venturing out of the hospital room.

Ella leaving the hospital incognito.  Her first attempt at cracking a smile.

 Ella with Mr. McDonald himself...

Already wanting to eat...she's half italian, what do you expect?   

Chipmunk Face

Ella bella is a little more alert and coherent today.  She continues to need morphine, but we are able to spread it out a bit more.  This morning I got her to play with finger puppets for a little and she seemed to enjoy face timing on the iPad with some friends and family, but as the day grew on she became increasingly unhappy.  As you can see in the pics, she definitely is getting more swollen by the hour --- that combined with the broken jaw certainly doesn't feel good :(.  Everybody keeps calling her a chipmunk, but I think she looks more like a mini sumo wrestler between the hair-do, the eyes, and the chubby face.  Well, she's still an adorable sumo wrestler to me.  

Today she went for a CT scan under anesthesia just to make sure distractor placement is correct and fortunately it is.  I asked them to clean her face and pins while she was sedated and thankfully they were able to.  It's been really difficult to clean her pin and trach sites while she's awake as she won't even let us near her face.  I'm hoping she gets better with this over time because at home in addition to her normal care, I'm going to have to clean the pins 3x/day and turn them too.  Eeek...I am not at all looking forward to that.  Erik has already volunteered me to be the official pin turner...isn't that nice of him?  Dr. Matthews thinks we will start the pin turning tomorrow.  He said we will start out slow with a half a turn working our way up to a full turn.  I've heard that the turning process is quite painful, so I'm just praying that she tolerates it because I certainly won't have morphine back at the Ronald House.  For those of you that know Ella, when she doesn't want something it ain't happening...unless of course an army holds her down.  But, you got to love that about her because it is her feisty personality that has gotten her this far.   

P.S.  To all the parents out there who have already gone through cranio surgery with their children, I give you so much credit.  Just as your children are warriors, you are too.  Though we've seen pictures, there is nothing that could have prepared Erik and I for this.  It is awful watching your child in pain and look so uncomfortable.  The only solace I have is knowing that this is the first step towards helping Ella someday eat and breathe on her own.  Two things that we all take for granted.

Love,

Nina  

Finally Clean!!!  Although it didn't last long. 

We are starting to take profile pics, that way we can see how much her jaw has grown by the end of the distraction.  

It's over...thank God

Ella's surgery was long and difficult, taking nearly 5 and a half hours today.  It was completely agonizing for Erik and I.  The surgeon said that since her opening is very tiny it made it all the more difficult placing the distractors.  We had a good surprise though...after breaking her jaw, he was able to get into her mouth with enough room to close her cleft palate.  We were extremely happy with this as it will make for one less surgery down the road.  He said some of her muscles were strong, some were weak, and one didn't move at all...so we'll just have to see what that means for swallowing in the future. 

What we are not so happy about is how much pain this little one is in.  She is on morphine and dilaudid around the clock, but is still very uncomfortable.  She keeps crying, wimpering, and signing to me she has an owie on her mouth and where the IV is placed.  Her face is swollen and the doctor said we can expect the swelling to increase until day 3, then it usually starts to come down.  It is breaking our hearts to see her in this much pain so I just ask you all to pray for her comfort and that this all becomes a distant memory to her.  Erik and I feel horrible though we know it was the best decision for her.  I just hope she forgives us :(.  She already is giving me these awful stares, but not to Erik of course...Daddies always get a pass from their little girls.  

Hoping and praying for a better tomorrow...

Thank you from the bottom of our hearts for all your love, prayers, and support. 

xoxo,

Nina

Surgery Tomorrow

We are down in Charlotte, North Carolina where Ella will be having craniofacial surgery Wednesday morning.  She will be admitted to Levine Children's Hospital at 5:30am tomorrow and she is scheduled to be in surgery from 6:45am to 10:30am.  This will be Ella's first jaw distraction surgery; it is the first step towards helping lengthen her jaw and open up her airway.  We are very nervous, yet we know she is in excellent care with Dr. Matthews and that God continues to have His hands on her.  If you have a moment we just ask that you say a prayer for our little Ella.  We are praying for the surgery to be successful, for there to be no complications, and for her pain to be controlled post-op.  Not sure how long we will be in the hospital, but definitely for several days.  Then we will be back at the Ronald McDonald house for 3-4 weeks during which we will have to turn the pins in her jaw. After that Ella will go back into the OR to get the arms of the distractor removed.  Many of you have asked for email updates so I thought it would be easier to just put updates on Ella's blog.  I will start doing that tomorrow, once she is stable.  

Thank you to all the people who have given us support and encouragement throughout these past few weeks.  Ella is so very blessed to have many real life angels around her.

Love,

Nina

A Prayer for Surgery
Heavenly Father, who created Ella in Your image and likeness. You know every fiber of her being and have willed that she be born. We ask You, Lord, to guide the minds and hands of those who will operate on her so that she may be restored to health and well-being. Help us, Lord, to pray with all our hearts and allow the burden of concern and anxiety to be lifted up with the knowledge that You are with her and her family now and always. And Lord, when she awakes from surgery, take her by Your hand and guide her through her recovery and towards a life of thanksgiving and peace. Amen

An Enormous Thank You

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....

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

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.