Back off PWS!!

On this last day of May 2015, I want to share this picture.  It is my most favorite picture ever of Ellie, for lots of reasons.  I look at it and just crack up, because it fits her personality so, so well.  She has a story to tell, and when she does, let's just say she talks with her hands! I also love it because she looks so beautiful in blue.

But it means so much more to me.  I see an inner strength that surpasses anything I could've ever imagined when we received the PWS diagnosis.  She wakes up every single morning happy and eager to start her day.  That's more than I can say for me, most mornings.  She has a zest for life that is contagious, and takes in each new situation carefully and joyfully.  She works so hard at, literally, everything she does, and rarely ever puts up a fight.  She truly is an angel in so many ways, and I know David would agree with me, she is totally our angel, sent to us a just the right time.

I think it is fitting to end the month with this picture because I can almost see Ellie saying the words, "Back off PWS!"  I can see the fight in her, to battle this rare syndrome that we were all slapped in the face with, and most everyone else knows nothing about.  This picture makes me want to fight even harder for her and her friends, because each one that I meet has this fight in them.  They deserve a life not plagued by hunger, anxiety, or hurt.  And when I look in these little eyes, I know she is not giving up, so I never, ever will.  We will fight harder, educate and advocate more, and love deeper.

My prayer for her is that she will remain steadfast and trusting to the wonderful One who made her.  When days are good, we will praise Him.  When days are hard, we will cling to Him.  

David and I, seeking the Lord's will and guidance, will take care of her the best way we know how.

So, Back off PWS!  The Spearmans and all the other wonderful, supportive, amazing members of our community will eliminate you one challenge at a time!!

Ellie smiles...

They will make your heart melt into a puddle, and there's nothing better in the world than an Ellie smile. Ok, ok...maybe an Ellie hug or kiss!!

                                                        

Live Life Full

What does it mean to Live Life Full?

PWS parents take it very literally.  At its most basic level, we simply and literally want our children to be able to eat and feel full...not hungry or starving all.the.time. 

But yes, it also means so much more.

We want them to experience happiness and joy in this life.

We want them to face change and trials confidently and courageously.

We went them to find fulfillment in a career.

We want them to make and keep friends.

We want them to form meaningful relationships, finding their significant other if they choose.

We want them to contribute to society and find their purpose and passion(s).

We want them to have a family of their own, if they so choose.

We want them to impact others and this world we live in.

We want them to make the world better.

We want them to Live Life FULL.

Independence

With each day that passes, Ellie grows older.  She is no longer a baby....even though she likes to pretend she is!  She is a little girl.  Graduations are occurring all over the country...elementary, middle, high school, and college.  

And I bet each parent is thinking the same thing...this happened so fast, my baby grew up right before my eyes, and man, time really flew!  I think about all this in a completely different way.

David and I will be those parents sooner than we know.  Time doesn't and won't slow down.

But we need it to.  I mean need it to.  Each day that passes, is one more day that Ellie has to live with the insatiable hunger that PWS dishes out.  Yes, it's a spectrum disorder, and yes, different individuals experience the insatiable hunger at various levels, ages, and intensities, BUT they will all have to deal with it.  And I want and long for that to CHANGE.  I want to change the course of this syndrome.  Human Growth Hormone (HGH) changed the course of PWS in 2000 thanks to incredible parents, researchers, and medical professionals.  But PWS is complex and it's complicated...the brain is involved, and that's tricky business.  

With each day that passes, Ellie becomes more independent...

I love that, and hate that.  Every parent longs for their child to gain independence, make good choices along their life journey, and ultimately contribute to society....I'm no different. David's no different.  But PWS has thrown us a curve ball, and makes me scared.  Can you see why I need time to slow down.  I need more time and days for Ellie to be able to live independently.  At this point in time, as it stands with the state of the disorder's trajectory she couldn't be left alone around food...she would need help...she would need to live with us or others that could help her.  And while, I fully accept this and will do whatever it takes to keep her safe....I long for her to live life FULL and independent of David and myself.  I have hope, I have fight in me, and I know my God is bigger than PWS.

Normalcy Shorlmalcy

Normal.  What does that word even mean anymore? There was a time where that was all I wanted...to be normal, to live a normal life, and have a normal husband, with normal children.  What was I thinking?! I am so glad our life is anything but normal.  I think if we had a "normal" life, I wouldn't look at this picture and see the sincerity in Ellie's eyes, the innocence in her disposition, or the compassion in her hand.  I would take for granted being grateful,  take for granted  the miracle that is life, and I would miss out on truly celebrating joy and fighting the battles of life.

You see, our life is far from normal.  We are busy, worried, proud parents that want our daughter to reach her full potential just like most every other parent I know.  But, a teeny tiny deletion on her 15th chromosome could potentially hold her back from living that fullest potential.  And, it has caused our life to be anything but normal.  We are regulars at doctor's offices,  and have known nothing but therapy for our child since she was born.  We give her an injection every night, lie awake at night thinking of new ways to raise more funding for PWS research, read every article/post/blog there is on PWS, travel the country to attend research conferences with people that get it, brainstorm ways to talk with friends and educational professionals about a rare disease that, if I'm honest, most people will never be able to fully understand.  We learn to cook differently, spend countless hours packing lunches, snacks, and dinners that are "Ellie safe," inundate news feeds and time lines during May to hopefully reach one more person about PWS, continue to grow and strengthen our marriage because if we neglect it, it could become disastrous.  We translate what our daughter is saying to others because they can't understand her.  And we explain the "allergy" to sugar and carbs when our daughter is eating things that raise eyebrows.  We arrange weight checks and blood-work when our 3 year old seems to be gaining a little bit of extra weight.  We worry about where our daughter is developmentally, if she will continue to make friends.  And try everything in our might not to fear the future.

Parenting is the hardest job on the planet.  Hands down.  We all have and/or will have our trials in life, and I would argue that we all lead far from normal lives.

What type of life do you want to live?

Look at Ellie.  She's anything but, normal.  She's extraordinary.

ABCs

SO.PROUD.OF.HER

The are the most precious ABCS we have ever heard, and were so, so worth the wait.

Keep singing Ellie Belle ♡♡

Music & Tunes

If there is one way to Ellie's heart, I believe music would be it.  She lights up when we sing songs she knows, or make up silly ones together, and dance to good tunes. Music impacts her, and it makes me estatic to see her so happy.  She will bust into song randomly and as her language continues to improve it is the most precious thing ever to hear her voice.  The first song she really took to was....no surprise here..."Let It Go!"  Frozen became our favorite movie, and heck, David even came around to it!  We watched it enough!

Right now Ellie absolutely loves to sing "The Wheels on the Bus."  She is hysterical and really gets into character with the "babies on the bus saying waaa waaa waaa!" Some other favorites include, " Head, Shoulders, Knees and Toes," "Itsy Bitsy Spider," and "ABCs."

She loves to dance too.  At her recent school musical she was moving and grooving to the beats during the times there were no words or movements.  Watching her, I truly believe she has got some rhythm.. must be her daddy in her.  He would be elated if she wanted to play the drums, or any instrument for that matter.  I would too, but am not the musically gifted one of the family!

I think what I love most about Ellie's interaction with music is the fact that it truly makes her happy.  You can see joy pour forth all over her face and throughout her body.  It calms her, makes her smile, makes her giggle, and truly touches her soul.  We can't wait to see how it continues to impact her life and the lives of others.

Playgrounds and Play Places

Oh, I can remember this picture like it was yesterday...

It was Ellie's first time at the mall Play Place.  I had been nervous to take her to one for a number of reasons, but mainly because I worried it would make my heart hurt.  Playgrounds had already done that.  I had remembered how I felt seeing the other kids Ellie's age run and jump off things.  Even stepping over the beams holding in the mulch was easy for them, while Ellie had to crawl over them and try and get her balance.

At this point in time, we were in Hickory indefinitely because my dad had suffered complications from surgery and he was very, very sick.  So sick we almost lost him.  I wanted Ellie to be able to exercise, and break up trips to the hospital because her world and routine had been turned upside down just like the rest of ours.  We headed to the mall to play...once we got there I remembered that pain and the struggle of PWS.  Ellie had a fantastic time and we were both so, so glad we came.  But a little piece of me remembers feeling just how hard Ellie's muscles work everyday as I helped her climb.  Oh, they worked so hard.  She held my hand and she used everything she had to climb, walk, and scale the play area. In that instant I remember hating PWS for how hard it was making my daughter work, but ultimately I remember being so absolutely proud of her for showing it who was boss.

Oh how she loves thee..

As Ellie grows, it is so fun to see what her interests and loves are.  She has the sweetest disposition and truly greets everything she loves with awe and excitement.  Here are her loves...

• Frozen...Elsa, Anna, Olaf, Sven and Kristof 
• Singing
• Dancing to good beats
• Her teachers
• Her "church house"
• Her grandparents 
• The iPad!!!!
• Playing tee ball with her new buddies
• The pool
• Her friends
• Egg salad
• Her "Special coffee" 
• Barnes and Noble 
• Smoothies
• Books
• Puzzles
• Accessories 
• Stickers

Can't wait to add to her list ♡♡♡♡

Hospital Gowns

We've had to be strong in hospitals more times than I would like to count.  And while it is absolutely nothing compared to some parents, each time we're there I can't help but be catapulted back to the NICU...scary and unknown.

PWS has changed me.  It has made me way more outspoken and assertive than I ever dreamed possible.  All it takes is one medical professional not being educated on my daughter's rare condition to bring out my inner momma bear mode...just ask David.  

I demand what's best for her, expect care and respect, believe in her potential, and appreciate all the people that have and will take care of her and us along this journey.

I fully believe God has placed us in positions to educate others about PWS, impact lives, and share Christ's love through our precious daughter's journey....hospital gowns and all.

Let them eat "fruit"

Can birthdays be fun without cake?  Absolutely!!  Just look at Elmo.

For Ellie or any individual with PWS cake, cookies, treats, and sugar-laden junk food are not a great combination.  That's not to say that they cannot have a treat, or have a small piece of cake at a birthday party, it can just make things more difficult.  Individuals with PWS do not process carbohydrates and sugar properly.  They are not used for energy, but rather are only stored as fat.  Ellie and others with PWS metabolize their food at 60% of how we metabolize food.  Really think about that for a second.  60%.....

So, what does that mean for us?  We substitute high healthy fat in place of carb-loaded, typical toddler diet.  Is it harder, yes....Goldfish, chicken nuggets, macaroni and cheese, cookies, and sugar is EVERYWHERE.  

Doctors are starting to find that PWS kids on low carb, high healthy fat and moderate protein diets are faring better mentally and physically, than individuals on low-calorie diets.  So, bring on the avocado, Greek yogurt, eggs, nuts, and fruit (think berries).  Ellie does well with this diet, and we have learned ways to make occasions special and fun.

If you really stop and think about how many times we "celebrate" something or someone...it is constantly.  Toddlers live at birthday parties, have countless school celebrations, "go out for ice cream," receive food for a job well done, or a task accomplished.  Should we, as parents really be promoting that?  Can't we find other ways to show that we are proud of our kids?  

What if it was taken away from you.....

You adjust, adapt, and overcome.  You sub fruit for cake, veggies for carbs, and activities and small toys for candy.  To be honest, birthday parties scare me, intimidate me, and cause loads of anxiety surface in me.  Each year at Ellie's birthday, friends' birthdays, and school parties, and sports functions, I rack my brain, talk with other PWS parents, and stalk Pinterest for new ideas.  

It can be done.  They can be fun.

 

  

Reality Check

PWS Reality Check Tonight :-(

Bring on the portion tweaking, and exercise.  We got this ♡

Pink

This is how silly and funny our daughter is...there are no words.  The moment Ellie put this wig on, she became a little giggle seeker, laugh maker, pink wig wearing performer.  She tossed her head around like she was 16 years old, and when the pink hair got in her face or mouth, she would take that little cute hand of hers and flip it out of her face.  She loved this wig, and still loves to make people smile and laugh. I hope she never, ever stops doing that, because laughter is good for the soul.

Beauty

Goodness, I look at this picture and am in awe of God's beautiful creation.  I simply cannot imagine life without her, and she is just the most precious little girl.

Her beauty radiates outwardly in this picture.  I just love her sweet face, and her wispy, corn-silk curls.  Those cheeks, I could just squeeze.  That smile makes me melt.

Ellie's beauty is so much deeper than outward appearance as well.  She has a sweetness about her that is infectious, and a personality that draws people in.  She loves to make people laugh, and sing them a tune.  She loves people.  She remembers names.  She is kind.  She is gracious.  She is polite.  She is thankful.  She is brave.

I pray every night for her mind, body and soul...that her mind would reach it's fullest potential, that her body would remain healthy and that she would be able to (with the help of others and herself) discern what is good for her body and what is not, and that her soul and heart would love Jesus completely, and share His great love with others.

She has a story to tell, a life to love, and a journey to travel.  David and I are so blessed she is ours, and are so thankful God entrusted us to be her parents.  We look at her, and then look at each other..."She is perfect."  Thank you, God for this beauty.

Green beans

Green beans...for most moms they don't produce much anxiety.  Some kiddos like them, others are repulsed by them.  From the looks of this picture, Ellie likes them.  

For me though, green beans and other new foods gave me anxiety.  What was difficult was the fact that beginning "big girl food" was hard.  I had just mastered feeding Ellie the bottle, and it was tough.  Now, we were supposed to introduce solids?!  Having a child with Prader-Willi syndrome produces anxiety surrounding food. 

Honestly, little things set me off and cause my heart to hurt along this journey.  Beginning solids was one of those times...I wanted to make sure I did it JUST so, because I had A LOT riding on getting it right.  My child, unlike most, has to eat healthy, and cannot splurge on sips of sweet tea here, and tastes of icing there.  I can remember feeling like all of my "mommy instincts" went out the window, and I felt paralyzed.  I wanted someone to tell me EXACTLY what to do and what to feed Ellie.  I felt guilty for not making her baby food from scratch.  I felt guilty I wasn't a professional chef.  I felt guilty I didn't really enjoy cooking.  We set up consultations with a nutritionist that really helped me through, and I can remember thinking one of the first things she said to try with Ellie...cheesy eggs.  Well, of course, why hadn't I thought of that?!  I can also remember breathing a sigh of relief when Ellie wasn't allergic to eggs, and really liked them. Eggs have become a staple in her diet.  So have green beans.  This picture, was taken after I had gotten past some of my anxiety, and really tried to enjoy feeding Ellie.  She liked green beans, whew!  

Something as minuscule as gaining confidence in what to feed your daughter, was huge along our journey.  Ellie's diet is a major part of her functioning at her best, helping with her mood and behavior, and helping to ward off the thing I fear the most....the unrelenting, insatiable hunger.  She follows a lower carb, high protein, and high good fat diet...try going out to eat and ordering off the kids menu.  Not going to happen...you have to get creative, and sometimes bring your own food.   

Ellie amazes me everyday with her diet, and as we move forward and she becomes older it is my hope that we really help her make healthy decisions and understand why some foods aren't good for her tummy and mind.  We have some great families to help us along the way that are great role models, and couldn't be more thankful.

So, make sure you eat your eggs and green beans, and when you do, think of Ellie.

Our goal

This...Just This...

This is our goal on this journey with PWS.

Miller Movement

This picture.

I remember the feelings of this moment.  Sheer excitement and anticipation.

You see, we had just driven 7 hours to Gainesville, Florida to visit with the leading PWS pediatric endocrinologist...Dr. Jennifer Miller.  I had researched all about her, and we were in awe of her at the Philadelphia FPWR research conference we attended two months prior to this picture.  I can remember we had made a pit stop along the journey and when we made it back to the car I said to David and Ellie, "Let's take a picture to show everyone how excited we are that we are seeing Dr. Miller tomorrow."  David said, "Show your daddy how much you love him for driving you 7 hours, and show your mommy how much you love her for setting this appointment up!"  Literally, it was the FIRST time Ellie had initiated this precious kiss and it was absolutely perfect.  I will treasure the picture forever and ever, because we all look so, so happy.

The appointment was in two words, simply amazing.

Relief, Hope, Encouragement, Joy, Pride, Love, Peace we all feelings we felt upon leaving the appointment.  For the first time, since Ellie's diagnosis we truly felt like things were going to be okay, especially having her a part of our team.

Dr. Miller was everything we anticipated, and so much more.  I cannot put into words the appreciation we have for her.  She is beyond knowledgeable about PWS, works tirelessly for all individuals with PWS, educates other medical professionals about the syndrome, is inundated in research to eliminate the challenges of PWS, challenges families to help their children do their very best, and most of all loves our Ellie Belle and all kiddos with PWS unconditionally.  

I thank God every.single.night. for Dr. Miller.

Present & Future

It starts with one SMALL step...

That sums up our approach to dealing with our "new normal."  We have taken small steps, toward big dreams, goals, hopes, and expectations for Ellie.  First, we navigated the diagnosis.  We researched. We connected with other families.  We became involved with The Foundation for Prader-Willi Research.  We learned to advocate and educate.  We hosted our first event called One SMALL Step.  And, we haven't looked back.  This picture reminds me of so many things.  I love it for how strong Ellie looks sitting up all by herself. Sitting took lots of hard work and determination on her part.  She looks so confident in this picture, and proud of herself.  

I also love the fact that I look at this picture, and it takes me back to the decision to host our first One SMALL Step event.  I remember being able to breathe after we decided to host an event, because for the first time since Ellie's diagnosis, I felt like I got a little control of life back.  I needed to be able to "do" something to feel like I was helping Ellie, and this was absolutely perfect.  These events help me feel like I have a little piece in changing the future for Ellie and her friends.  Mentally, I set lofty goals to fund-raise major dollars and I lose sleep at night thinking of ways to bring in just a little more.  I drive my husband crazy!  But, I truly believe it is my momma bear coming out, and I just want the absolute best for my little girl.  What parent doesn't want that, right?  David and I are in a unique situation because in some ways we have almost been told the future of our child.

And it scares me.  I have been told it will be hard, challenging, uncertain, limited, painful...

Wouldn't you want to change that?  That is what keeps me up at night.  That is why I host events.  That is why I raise awareness.  That is why I shamelessly ask for donations.  That is what I pray everyday for.  I want to be a part of changing the course of my daughter's future for the better.  I want to help eliminate her challenges.  I want to take away her pain.

So, as I look at this picture it fills me with hope.  By taking One SMALL Step, David and I can help make a difference in Ellie's life.  Our 2015 event is less than a month away, and I'm excited, nervous, and joyful that it will be our best yet.  Events like this change lives, change hearts, and change the world....and we will be part of that for Ellie and all her friends fighting PWS every.single.day.

http://onesmallstep.fpwr.org/dw/walking/location/1387

Toes in the sand

I look at this picture, and it takes me back to our Greenwood Genetics visit right before Ellie's diagnosis.  That day was so incredibly hard because even though everyone in the office treated us with the utmost care and respect, we were still told things that rocked us to our core.

Clinically, they believed that Ellie showed all signs that would point to a diagnosis of PWS, and I truly believe David and I knew it in our heart as well, but it still wasn't confirmed...we talked about PWS and Angelman syndrome (both were equally terrifying) that day, but spent more time discussing PWS.  

We heard things like Ellie will most likely never be able to have children, she most likely will not graduate high school (and if she does it will probably be with a certificate and not a diploma), she will have a hard time managing money, she will have to live with you the rest of her life, she will always have to be monitored around food and could be very stubborn and manipulative.  

Naturally, David and I were crushed and tried to remain strong, but we broke completely down.  I remember two things we specifically asked, "Will we ever be able to go out to eat as a family?  Will we be able to go on vacations as a family?"  Two very small things that we took for granted before now.  The diet, the food, the behavior, the tantrums, the food seeking, the manipulation, and the safety...could it happen?  And then, the geneticist showed us a picture in a textbook, and I feel like some of our hope was restored.  We saw a thin, happy little boy playing at the beach in the book.  Dr. Geer looked at us, and said, "With a lot of discipline and hard work, this could be Ellie, because this little boy has PWS."

After that, I began to research and read (David stayed as far away from Google as possible) about PWS.  I connected with others on Facebook, we became involved with The Foundation for Prader-Willi Research and Prader-Willi Syndrome Association (USA). We hosted a One SMALL Step for PWS 5K.  We attended a FPWR research conference. We never looked back.  But, I truly believe that we both remembered that picture, because in our darkest moments it gave us hope, and motivated us.  

Yes, we can go out to eat as a family.  
Yes, we can go on vacations as a family.  
Yes, we all can be happy.

This picture above was taken on Ellie's first family vacation to the beach.  It cracks me up, makes me tear up, and makes me so happy all at once.  She reminds me exactly how I feel when I'm at the beach.  Relaxed, slightly hot and bothered (due to the sand, sunscreen and my fair skin), chill and happy.  All emotions I had longed for when thinking about taking my child to the beach.

As time has passed, David and I have realized that even though our journey has been hard, it has also come with huge blessings.  One of those precious blessings is the friendships we have made along the way.  PWS will bring you best friends you never knew you would have, nor wanted!  Vacation has morphed into amazing times, now spending it with dear friends that truly get our life.  This past March we vacationed with the Peden family, friends we have made through PWS.  It was so much more than vacation to us, and I think about that picture our geneticist showed us, and am in awe of the ways God has blessed us since that day. Spending time with this family was wonderful, and yes, we spent time talking about PWS, but we also spent time getting to know one another outside of PWS as well, and had so, so much fun.

Could vacations change a little for us as time passes?  Yes, possibly.  Could we have to make accommodations as Ellie gets older?  Probably.  Will we enjoy time together as a family and with friends?  Absolutely.

Strong girl

This is one of my most favorite pictures of Ellie.  It not only shows her physical strength, but those eyes show emotional strength too.  Looking into them gives me such peace. Sometimes I think back to when she was a baby and wish I hadn't worried so much about every little detail of her life, and wish I just enjoyed it.  That's not to say I didn't, but worry and anxiety provoke fear a lot of times, and rob you of joy.  I constantly had to (and still have to) work on this...living day to day, and in the moment.  I can't get too far ahead, or that awful anxiety creeps in.

When you are given a diagnosis of PWS, you are also given an huge laundry list of things your child may not accomplish, and are told milestones will be delayed, and some possibly not met.  We spent a large part of Ellie's infancy trying to prove she would defy those odds. We, along with her, spent hours upon hours working on tasks that many parents don't ever have to think twice about.  We worked tirelessly on Ellie holding her head up, tracking objects side to side and across mid-line, rolling over, supporting herself at tummy time, sitting by herself, pulling up, standing, and walking to name a few.  

It's interesting becoming a parent, because much of your time is spent talking with other parents about milestones and asking, "How old is your child?"  Oh, and when did they start rolling over?  When did they start walking?"  As parents we pride ourselves in telling others, that our children completed milestones well before they were developmentally "supposed to."  Why?  Because it helps us know are children are developmentally appropriate and "normal."  What happens when you are told your child is developmentally delayed, and "different?"

Milestones become that much more sweeter.  It's our nature to compare, and I know regardless of how hard we try not to, we always will.  But think for a second if you weren't able to say, "My child began walking at 9 months, way ahead of when they were supposed to."  Or think if you spent your time explaining to others why your child doesn't talk yet, or why when they do, no one can understand them.  It changes your perspective.  It changes your outlook.  It changes your heart.

When this picture of Ellie was taken, it was the first time she had supported herself fully during tummy time.  She was delayed.  It wasn't when she was "supposed to" be able to.  It was later.  It wasn't typical.

But just look at her...Confident, poised, determined, beautiful, and strong.  Our strong girl. And those eyes, those beautiful, peaceful eyes staring at me, letting me know it's okay and these things will come...on Ellie's time.

Knock at the door

I can remember the day the oxygen tanks were delivered to our house like it was yesterday. Ellie was 4 months old, and I couldn't believe this was happening to our family.  We had been to see the endocrinologist and sleep medicine doctor almost simultaneously.  I had been researching treatment options, and educating myself on PWS, and we both knew we had to get Ellie started on Human Growth Hormone injections (GH) immediately.  GH would help her with her low muscle tone, lean muscle mass, height, and small hands and feet. Endocrinology had ordered a sleep study right away, and it was a terrifying night.  They hooked Ellie up to all kinds of wires and probes, and expected us both to sleep.  I was a nervous wreck because we were on a strict feeding schedule and my poorly little baby was hooked up to all these tubes and wires again.  How on earth would I get her to eat? Somehow, we made it through the night.  But life was about to change again.

So, in a matter of months we had two things added to our lives that I never saw coming...nightly shots and oxygen.

Oxygen, could my baby really need oxygen?  Oh, the day the tanks arrived to our house, it was a kick to my soul.  Ellie's sleep medicine doctor had called us immediately after the sleep study, and I knew that couldn't be too great.  She had pretty severe to moderate central and obstructive apnea. We threw ourselves into learning the best ways to keep the oxygen on while she slept, made sure we carried tanks with us everywhere, because you didn't know when Ellie might fall asleep. When she was asleep, that was when the oxygen had to be on...you see, her brain doesn't tell her precious body to breathe in her sleep.  So that results in central sleep apnea.  Also, her airway is floppy (low tone again...see, I told you it is everywhere), and causes obstructive breathing/apnea as well.  The oxygen allowed her to get good, restful, deep REM sleep.  Sleep is so important for our whole body to function effectively.  Individuals with PWS tend to suffer from sleep issues because the hypothalamus (the part of the brain that has a defect in those with PWS), so we knew Ellie needed to get good sleep.  Our doctor also mentioned that by treating sleep apnea aggressively, it could even boost one's IQ by 25 points.  He didn't have to say anything else to us....that nasal cannula was in Ellie's nose anytime those pretty blue eyes of hers were closed.  We vowed that we would do all in our power to see that she was able to live the best life possible...even if that meant oxygen and shots.

They both have become our new normal.  Oxygen has since turned into a CPAP or "nose hose" machine, and we constantly hear, "Oh, my grandpa has one of those."  We just grin and nod our head yes, "so does our 3 year old...who would've thought?"  The shots are just a new nightly routine, and Ellie has been so strong throughout.  They haven't come without struggle, but looking back I am no longer scared of these things like I once was.  We adapt, we move forward, we work through the difficulties, celebrate the accomplishments, and look forward to the knock at the door because we know that GH and oxygen/CPAP are the best treatment options for Ellie right now, and we will need refills!

My girl

Motherhood has changed me, challenged me, inspired me, and filled me with more love than I could ever imagine.  This girl has my heart and I couldn't be more blessed to be her mommy.

Oh, that lip...

Oh, that lip...Ellie can stick it out in a hot minute.  What's interesting is that it took a little while for us to even see her get upset.  Laid back, quiet, good, easy-going were all words used to describe our Ellie Belle.  I am being serious when I say we could count on both hands the number of times we heard her cry her first year of life.

Sounds like every parent's dream doesn't it...

As glorious as it sounds, we longed for Ellie to cry, make sounds, get upset, show us a little attitude.  Her low tone caused her to be super quiet, and not cry like a typical baby.  She never, ever cried to tell us she was hungry, wet, hurting, or upset.  Ellie was fed every three hours, and we set an alarm.  I can remember the sheer panic that set in the night we slept through the alarm.  We could have been the family every parent envies, because our baby would have slept through the night literally from her first day of birth.  It hurts when I think about the times we've been told "you're so lucky she never cries," and "it must be so nice to have such a quiet, good baby."  It's hard to explain to others, and quite frankly, until you're in a situation like it, you just really won't understand.

So, as pitiful as this face is, we were happy to see it!  There was a battle...we wanted her to show various emotions, but when she did we almost panicked and overreacted, mainly because it was new and uncharted territory.  Children with PWS can struggle with "meltdowns," or have extreme up and down emotions, and not do well with moving on past issues.  David and I have come to the realization that our entire life, we will ask, "is that due to PWS or is that typical behavior?"  We've decided that even though we know these questions will arise, we will just take them one day at a time, and just go with, "It's just Ellie, and we love her all the way!"

Daddy's Love

Talk about melting your heart, this picture will do it.  It was one of the first pictures of David and Ellie taken at home.  I can just look at it and tell, his heart is bursting with pride, filled with love, hurting for his baby girl, and scared senseless...all at the same time.

From the moment David met Ellie, he fell in love and I could see his demeanor change; he had become a dad, and it was the most precious moment ever.  Her birth was traumatic for all of us, but I can remember looking at David when they showed Ellie to him, and he just said, "She's just the prettiest little thing."  His strength in the NICU was unshakable and he was my rock.  We became the true definition of team, and began our life as a family...a little differently than we had imagined.  There wasn't a day or night David missed seeing his little girl in the NICU.

But he was scared and hurting for his precious baby girl too.  I know he would have traded places with Ellie in a heartbeat.  This situation was like nothing either of us had ever battled, and David has battled some incredibly hard and trying situations.  I saw him cling to his faith like never before, and fervently pray for our family.  There were lots of unknowns for our family then, and still are today, and still will be in the future.  David's unwavering peace helps me see that no matter what challenges we face, we will be OK.

His love for Ellie is unconditional, indescribable, and is totally a daddy's love.

This Look

This look...it will forever be ingrained in my mind.  I waited, and waited, and prayed, and cried.  A deep, dark place within me wondered if Ellie would truly bond, connect, and know I was her mommy.  Her low tone and laid back nature caused her to be a "good" baby in many people's opinion, but I remember a time early on, and after we brought her home, where I would stare in her eyes.  I remember painfully questioning if she was there and seeing her mommy because I couldn't see it.  I tried to, begged her to, plead with God, cried to my mom and David, and still nothing.

And it killed me.  I longed for her to give me looks, cues, expressions, emotion.  

Anything.

And then this look came, and it was more perfect than I could have ever imagined.  It may not have been on the "typical" schedule, but it was on Ellie's time.  She is fully there, and definitely knows and sees her mommy, and everyone else around her.  It took time, but she heard every word I spoke, every song I sang, every kiss I gave, every pray I prayed. Prader-Willi syndrome will rock you to your very core, will make you question God, will make you angry at our food driven society, will make you grieve the loss of the child you thought you would have, will make you ache to see your child struggle, but it will give you so much more. It will give you patience to wait for accomplishments, trust in a God that is good and sovereign, perspective on the intricacies of life, bravery to fight, educate and advocate, a love that knows no boundaries.  Finally, it will give you a child like no other that will truly inspire you and utterly amaze you every single day.

I couldn't be more grateful for this look.  It is what makes me fight to eliminate the challenges of PWS, it gives me purpose, and it makes me proud each and every day.  She is most definitely there, sees her mommy and knows I love her dearly.

Pink Room

What a pretty pink room, for a precious little girl.  I can remember telling David I wanted to paint Ellie's room pink, and for any of you that know David, know that this was WAY out of his comfort zone!  We picked the perfect pale, dainty shade...Pink Air.  Even daddy-to-be was excited about it!  He did such a good job painting the room, and installing the ceiling fan.  It was so wonderful to see him put so much care and time into her room...he wanted everything to be perfect for her.

Together, we put her crib together, and had to snap this picture afterwards.  Her room looks so serene, peaceful, calm, yet girly too.  I can see the excitement in our eyes and can tell how proud of it we were.  I also look back at this picture and see how clueless we were to the journey ahead of us.  We have both changed.  Hopefully for the better, but I can't help but see how a little piece of us, our innocence, was taken away when Ellie was born.  We were thrown into parenting head on, like every new parent.  But, we were forced to learn things most parents don't ever have to, nor want to think about or learn.  Of course, David and I both would take away PWS from this world if we could, but that would change our whole course, path, and way of parenting.  Ellie is exactly who she was intended to be, and we're so thankful for her and what she has taught us.  Hands down, she has made us stronger, better parents.  

And her little pink room suits her perfectly...

Welcome Home

Oh, what a joyous day!  After 26 long, hard, days in the NICU we were able to bring our baby girl home.  Our dear neighbors, and friends made it extra special when we arrived with these precious decorations.

I can remember feeling a sense of ease that we were home, and at this time we still didn't know what (if anything) was going on with Ellie.  No diagnosis, no tubes, no real clarity....just home.  

I honestly felt at this point, we were out of the woods, so to speak.  After all, we were home right?  Ellie was safe, and exactly where she needed to be, and where we wanted her to be. We started to build our new life together.  We introduced her to her "brother" basset hound, Hemi, got her acclimated to her new room, thought about putting her in her crib right off the bat, and then opted for our room, gave her the first bath in her pretty new pink tub, and loved on her every second.  Home....it provided peace, security, and normalcy to our rocky start as a family.

But, David and I have talked about this time, and we both agree that as joyous as it was, it also stung a little bit too.  We felt robbed the experience of leaving the hospital with our baby, together.  To come home that first time without Ellie was like a punch to the gut.  The house seemed eerily empty and sad.  We cried a lot.  To bring her home, almost a month after she was born, hurt too.  It wasn't how we envisioned it to be, nor wanted it to be.  Even though it might hurt, and wasn't our plan...it was His plan.  God is so much bigger than our situation, and He knew we would go through this.  The hurt may not go away, but the joy will always remain...we are thankful for dear friends and family to celebrate with us, and we were so grateful to finally be able to whisper in Ellie's ear, "Welcome Home baby girl."

Small Victories

This picture has such significance to our time in the NICU. Immediately upon looking at it, I feel relieved and think 42 mLs!!! The memories surrounding this picture are bittersweet  because we captured the sheer joy of Ellie meeting her goal feeds and drinking her bottle, but we also captured her utter exhaustion and struggle.

The joy was like nothing we've ever experienced.  We were so proud of her, and so relieved that we finally felt like something was working in our favor.  At this point, our doctors had taken so much blood from Ellie to run all sorts of tests, she had undergone a cranial MRI, abdominal ultrasounds, eye exams, and doctors we not really telling us what they suspected...and that made us extremely nervous.  It turned out, Ellie really stumped them, and they didn't know what might be going on with her...they had lots of balls in the air of possible diagnoses, and they consulted with numerous pediatric specialists.  Spartanburg Regional treated us with the utmost respect and care, and we couldn't be more thankful for the doctors and nurses...many of whom we still keep in touch with!  

So, the day she drank her whole bottle, we celebrated, and were so happy.  We stopped at Krispy Kreme on the way home from the hospital for doughnuts (I later felt so unbelievably guilty for doing that upon receiving the diagnosis of PWS, and so many emotions come racing back anytime I drive by Krispy Kreme).

But, I also look at this picture, and am filled with sadness.  My precious baby girl looks so, so tired and she just drank 42 mLs (just under 2 ounces) of milk.  She used every ounce of energy she had to do it, and it kills me.  We would spend 30 minutes working with her, and I can remember, David and I would both be sweating by the time it was over.  We were nervous and scared, and just wanted our baby home.  There were ups and downs, good days, bad days, good nights, bad nights.  She was exhausted, but she kept working, kept pushing, and ultimately showed us, "Mom, Dad, I got this!" And that wouldn't be the first, or the last time she has done that.  

Feeding, drinking, nursing...it's critical for a babies survival and development.  We have learned more than we ever thought possible about it, and more than we ever wanted to know, honestly.  Ultimately, we are thankful the knowledge we have gained, and the people that were placed in our lives to help us on this journey to help our daughter safely get the nutrition her body needed.  In the midst of trials and tribulation, we must celebrate the small victories, because they will ultimately sustain you when moments become tough again.  

The small victory

The feeding struggle

A range of emotion comes over me as I see this picture.  I feel nervous, sad, and exhausted.  A child with Prader-Willi syndrome does not eat like a typical baby does.  The low muscle tone wreaks havoc on their tiny bodies, and they work so hard for every single milliliter.  If they spend too much time feeding by mouth (our nurses and therapists told us not to go beyond 30 minutes with Ellie) then they will actually start to burn calories, instead of gain them.  Ellie worked so, so hard, and would be absolutely exhausted after eating. Being an only child, and having her as my first child, I was a nervous wreck to do anything...change her diaper, hold her, and feed her.  It took everything in me to feel confident to feed her, and quite frankly I felt like I did a terrible job of it most of the time.  We had to remember to do so many things:  prop her up because of reflux, hold her head and neck, support her cheeks, support her jaw, spin the bottle, tap the bottle, give her breaks, burp her, make sure her sats didn't drop below 90, massage her cheeks, thicken the milk, and crank her arm (when all else failed...David discovered this one).  I just wanted to cry most of the time, but apparently the baby can pick up on your emotions while feeding, so I had to be strong.  It was mentally and physically exhausting.  When we weren't feeding Ellie, I was pumping and then David would clean everything for the next shift.  Totally exhaustion.

I become mad thinking about this cruel disorder, Prader-Willi syndrome.  How could a child struggle so much to eat in the beginning of their life, and then develop a ravenous, insatiable hunger, what many describe as starvation 24/7?  It is heartbreaking, terrible and as parents, we would do anything to trade places with her.

Ellie made progress.  I can still remember...the doctors and nurses set a goal of 42 mLs every feeding (3 hours) for her.  She could barely take 5 mLs when we started, and by the end of 26 days in the NICU, she was taking 42 mLs every feeding.  It was nerve wracking going home and to the pediatrician, because what if she didn't reach her 42 mL goal when we got home?  What if she lost weight?  Would we be back in the hospital and headed for surgery?  Ellie showed me through all this how strong, resilient, and brave she is...it also shows the grace and goodness of God.  David and I fervently prayed throughout this time, and God took care of Ellie in ways too numerous to count.  I prayed He would take away her PWS.  I prayed that the geneticists got the diagnosis wrong.  I prayed for a miracle.  When I stopped trying to pray for God to take away things, and make things "normal," I still prayed for a miracle, and I prayed for God's will to be done in Ellie's life.  His will is clearly evident each day we spend with her.  She is perfect, and exactly who she was intended to be.  She is a miracle.

And what was the feeding struggle, became the feeding triumphs.

Wires & Tubes

Oh, to see Ellie like this makes me sad and grateful.  Sad because she looks so sick. Sad because we didn't get the typical post birth experience.  But, truly grateful that modern medicine is able to help babies grow and become stronger in a Neonatal Intensive Care Unit (NICU).  Grateful because Ellie was exactly where she needed to be in that moment.  She needed all those wires, and that tube.

Those wires monitored her heart, and her oxygen saturation levels, or "sats" as as we came to know them.  Normal blood oxygen levels are considered 95-100 percent.  If the levels are below 90 percent they are considered low and can cause respiratory distress, breathlessness, and increased rate of breathing.  If the levels are below 80 percent, it can compromise organ functions and should be addressed right away.  Continued low levels can lead to respiratory or cardiac arrest, and oxygen will be used to aid an individual.  David and I watched those monitors like hawks, and I would panic anytime they alarmed.  Ellie's "sats" would hover anywhere from 90-100 most of the time.  When we began feeding by mouth, I remember them at 79-82, and that was NOT good.  We would immediately have to stop if that happened because her little body was working too hard, and her organs could have been compromised...  

Thus the need for the "ng" (nasogastric) tube.  This was a tube inserted through her nose, past her throat and into her stomach.  The ng tube gave her nutrition without her body having to work so hard (due to her low muscle tone, this was the challenge).  It is amazing how little I knew about "muscle tone" in infants and people, before Ellie.  It.is.everything.  I will never take feeding a baby for granted anymore, because now I know how hard it is, and what a miracle it is.  Babies with low muscle tone struggle and work harder to do the simplest of things, like drink a bottle or hold their head up.  Many children with PWS must come home with an ng tube or a gtube (they have to have surgery to place this, and it aids them in getting the nutrition they need, while working on feeding by mouth.

David and I vowed that we would do everything possible for Ellie not to have surgery, or come home with an ng tube.  Once we made that vow, our strict, dedicated, methodical way of feeding Ellie began.  I look back and remember how incredibly hard and exhausting that was, but then I could kick myself for thinking that.  Ellie works hard every single day of her life, and always will.  I have two thoughts about our ng tube experience...not wanting to come home with one made us all work harder, and learn to capitalize on Ellie's feedings strengths, but I absolutely, positively think there is nothing wrong with coming home with an ng tube or a g tube.  Both are amazingly strong ways to help your baby, and I will forever be thankful for those wires and tubes.