FALLING - Into Milestones, Movements and a Possible Cure

Fall in Cape Cod 2019

Fall is upon us, and with the cooler temps come special birthdays and fun events, adding up to a whirlwind of a season.  At this moment, I'm truly grateful for life - for being alive, for the ability to breathe in the crisp air and drink in the smells of my family and nature (can I get a 'whoop' for those yummy fall candles from Bath and Body Works?1?).  This time of year always brings deep emotions, as we are so close to the Eve of Ellie Kate's Month of Reckless Love, coming up in December. The Fall of 2019 is bringing BIG and exciting things - REALLY and TRULY, BIG and possibly life-changing things are happening all around us, unfolding before us.  I yearn deeply for the Lord to allow me to take it all in, to make me a good steward of my time, my giftings, and my resources, where all of these big things are concerned, things I will share with you today.

LUCY BELLE:
Lucy turned EIGHT at the end of September.  She's breathed the earth's air for EIGHT entire years and it's effected both Mike and I in different ways.  Ellie Kate just turned seven, before passing away in December 2012.  Ellie never turned eight years old and yet, here we are with Lucy.  It is MIRACULOUS to me - a wonder and gift beyond comparison - especially since, just two years ago, Lucy was going downhill so quickly, we truly thought it would be her last birthday on earth.  At that time, she was on hospice and we couldn't get her healthy.  We couldn't get her to 40lbs, even at age six, despite our best efforts and medical orders.

Lucy is EIGHT! 

While Lucy is still on Palliative Care, she is no longer considered a "Hospice Patient".  There is new life in her eyes, new energy, a happiness and glee we've never seen in Our Girl before!  She's back to doing what she did before hospice.  For Mike though, it's been incredibly difficult, realizing Lucy is now older than EKM ever was.  It's hard to reason it out, hard to fathom it all - how can you really wrap your mind and heart around the cold truths and heavy loss, even the losses to come?

CONNER MICHAEL:
Conner, our eldest child, is now 16 YEARS OLD!  From the beginning, everyone warned us, "The days are long, but the years are short!" - oh, how true that now rings!   Last weekend, we celebrated Conner with eight of his closest friends.  The boys spent the night (some for a few nights), and I was encouraged by how respectful and helpful they were.  They've come a long way from leaving ice cream cartons and pizza boxes around the house overnight, and we didn't have to remind them to go to bed or be quiet - WOW.  Conner is going through driving school and will soon get his permit.  We are happy to take things a little more slowly with him, making sure he is as safe as possible, while also giving him the independence he needs as a typical teen, as well as a teen who's faced such heaviness at an early age.  Being truthful: it's hard not to be overprotective in every way.

Conner is Sweet 16:)!!

Mike and I beam with joy over Conner's life!  He is so kind and thoughtful, intellectual like his Daddy with tendencies toward mercy and grace, like me.  He loves current events, specifically political ones, and stays abreast of all current affairs.  Conner is a fabulous artist, natural athlete and a fantastic big brother to all of his siblings.  I love how Conner seamlessly pushes Lucy's wheelchair around the mall and quickly gets her out of the car for me in the afternoons, without me even asking.  He dotes on Bowen Jane and stays in awe of all she can do, as a "typical" little girl.  Conner is patient with Henry.  Mike and I have no doubt, Conner's design, his God-given character and spiritual giftings, are matched perfectly with his siblings.  He is patient and kind, doesn't anger easily, does not expect perfection from himself or others, and is quick to listen to his siblings, no matter what they may be focused on.  I'm forever grateful to God for my Precious, First Born.

ELLIE KATE'S WISH:

Each December, we partner with NKH Crusaders by granting "wishes" to fellow families within the NKH Community.  Parents may write letters, explaining their need for medical equipment or specialized toy, not covered by insurance.  They request something the child truly needs or something which would add significant value or safety to their life.  We like to grant as many wishes as possible each year in Ellie Kate's name, but the number of families we help depends upon the funds we are able to raise for this project.  My HOPE is to do an online auction of items donated by you  - pieces from artists, services provided, home-based business products, hand-made jewelry, homemade holiday breads or pies, even donated gift cards!  We like for wishes to be granted in DECEMBER, Ellie Kate's Month of Reckless Love, so we will be working on collecting all funds and items from now through December 4th.

If you have any knowledge or experience with online auctions OR if you have items you would like to donate, please contact me via email at tullyryan@hotmail.com.  If you are a business who would like to support these efforts, providing an important need to a fellow NKH family from around the world, please contact me as well, and we will get a plan in place!  

THE WORLD OF NKH:

NKH is an EVIL disease, and up until recently, we were led to believe NKH would not, could not be cured or lessened in severity, no matter what.  HOWEVER, because of recent strides in research by both doctors at Colorado Children's Hospital AND Notre Dame, we have NEW HOPE that a cure/treatment IS possible, and that it is possible IN OUR LIFETIME!  Stem Cell research has taken off in all arenas and those studying this disease have tirelessly been working on how this can/could/will benefit those diagnosed with NKH.  There are labs with mice and fully, capable staff members working at both locations, as well as a doctor and lab in the UK.

Recently, I returned from the International NKH Medical Conference in Boston, where I received the latest news on NKH research.  I received a grant to attend the event through the Isaiah Stone Foundation, otherwise, I wouldn't have been able to attend.  I'm incredibly grateful for ALL of those who helped with hotel details and additional funds.  I'm even more grateful for those who cared for my precious kiddos.  It's not an easy gig, ya'll.  It's a tough job, so we can't really hire babysitters because of the situations we face from both NKH (Lucy) and PANDAS (Henry).  Mike and My Parents were valiant warriors, sacrificing all of themselves so that I could go and not only HEAR the newest information shared, but also have the opportunity to meet privately with each set of doctors, going over my family's specific mutations.  I'm still processing it all. 

A few of my Fellow NKH Mommas 

NKH Fundraiser 2019, Weymouth, MA

Did you know? Families are the MAIN resource of NKH research funds, due to the rarity of this disease.  In 2007, at the first NKH Conference Mike and I attended, that fact seemed impossible.  NOW - now, we are closer than ever, on the verge of possible medical trials!  It is a heavy responsibility to bear, raising the hundreds of thousands of dollars needed to "secure the cure" and yet, the ultimate goal will be so worth it.  I'm still trying to process this, as well - trying to wrap my mind around the magnitude of what could be and what could change.

On an official level, Mike and I have never raised funds for NKH research, but that will change in the coming months.  We want to give HOPE and the possibility of a CURE to those who come behind us. Could this research and treatment affect Lucy?  YES!  It absolutely could - we will just have to wait and see for a bit.  Who it will definitely help, are newborns diagnosed with this disease - the hope is to use what is researched, to ultimately STOP NKH progression, from the very beginning.  I WANT to give that hope to someone else.  Who knows?  This research could benefit those in our own family tree, since NKH mutations run on both sides.

If you or your business would like to help us "Secure the Cure" for NKH,
 please contact me anytime.  
We would love to partner with both individuals AND companies, 
especially with our 501c3 status and 
the possibility of your gift being a tax write-off, individually or corporately.  

The Faces of NKH - from all parts of the globe, those we've lost and 

those who are still with us today; we fight for you!

Oh, Friends, 

Thank you for your continued prayers and encouragement.  I often find myself forgetting to respond to messages, or failing to follow up with friends with whom I have the best of intentions.  The only thing I can say is, parenting intense, medical and special needs, as well as extreme behavioral/mental health needs, is most often all-consuming. It takes ALL of me, everything Iam, just to do it, and it taxes me mentally, spiritually, physically, and emotionally in ways I don't fully understand or even recognize. This week, I found out my stomach and intestines are full of ulcers - the stress of life doesn't help, for sure. I am thankful for the JOY, which DOES come along in the journey - a supernatural joy, which God brings every single day, no matter how dark it might be. Even in loss.  Even with stress.  Even with seven stomach ulcers.  

I long for an easier season, a lighter daily routine for my entire family; I'm trusting the Father to orchestrate it as He will most be glorified, while also asking for mercy over my children.  I appreciate your understanding throughout the MANY seasons of our journey.  I look forward to hearing from those who can help with the auction, Ellie Kate's Wish and even fundraising for research!

In His Time,
Ryan

MORE ON NKH: 
Non-ketotic Hyperglycinimia (NKH) or Glycine Ecephalopathy, is the rare genetic disease, both Ellie Kate and Lucy were born with.  Researches have found that these individuals make too much glycine, which collects at the base of their brain, causing extreme damage in every possible area of the body and mind. Most children born with NKH are diagnosed with the "Severe" form of the disease, as were Our Girls.  Most children with Severe NKH do not walk, talk or eat by mouth.  Many of them have had tracheotomies; some cannot breathe on their own.  Our children with NKH are bombarded with severe epilepsy - most NKH kids are on at least 3-4 anti-seizure drugs, but continue to have seizure activity because of the damage done by the extra glycine.  Some children have hundreds of seizures a day, seizing every few minutes, every hour, every single day.  This especially happens when our children fall ill, which also happens often, as NKH seems to somehow lower the immune sytsem.  We know this is true for Ellie and Lucy, as they've faced ecoli, continuous c-diff, MRSA, cellulitis and more.  We also know, NKH causes every body function, to MIS-FUNCTION.  This is seen in Ellie Kate's gut issues and how her gut eventually shut down before she died.  It's seen in Lucy with the need of her vesicostomy, due to recurring UTI's, which occurred because Lucy cannot urinate properly on her own.


In 2007Mike and I traveled with a baby Ellie Kate at that time, not knowing just how much our world would change upon meeting families like ours.  Being surrounded by families with older children at that conference was HUGE for us; it gave us hope, when the medical community told us not to "expect much" from Ellie Kate, warning us her life would likely not exceed three years.  While that is still a likely outcome for many, because of medical advances, more children with NKH are living longer , which means more children NEED A CURE AND A HOPE.  We hope you'll join our cause! If you want to jump in early and help, please donate at the following: https://www.nkhcrusaders.com/donate

To The Rescue

Have you ever cried out for help?  I mean, have you felt the searing need to be rescued from someone or something?  Maybe you were lost, caught in a life or death circumstance; maybe you've needed "rescuing" on something as trivial as a chemistry final or help in your flower beds. No matter how smart, how strong, how wealthy you might be - no matter how many accolades you've received, how many rewards you've won or how well you work a room. . . 

At some time in your life, you'll find yourself in a true place of desperation, depression or oppression - in a pit, where something is taking everything away from who you are, what you have or from who you want to be.  I'm not talking about a trivial kind of need, but rather one where you are so desperate, you can't catch your breath, or can barely see beyond the horrific sights which lie before you.  

Psalm 6:1-4

Be gracious to me, O Lord, for I am languishing

heal me, O Lord, for my bones are troubled.

My soul also greatly troubled.

But you, O Lord - how long?

Turn, O Lord, deliver my life;

save me for the sake of your steadfast love.

It's a GOOD place to be, needing help and rescue from those outside of yourself.  We need each other, as friends, as strangers, as humans.  Being "needy" makes one vulnerable, which is a most beautiful place to be.  As a Believer, being vulnerable is important because we are called to live in a vulnerable stance; we are called to give our lives away to God and to others.  We're called to humble ourselves, first at Salvation and then continuously, giving our lives to God for HIS glory, HIS plans, HIS purpose, and not our own. 

Just imagine with me, for a moment- 

What happens when it's YOU who's in need of help and rescue?  

What happens when it's your family, in a constant state of need? 

What if the oppression, loss, heartache, physical pain literally won't. ever. stop?

Even when you are living a "good" life or, as the Bible tells us, an "obedient" life, surrendered to the Lord, you'll find yourself there, in need of rescue.  Even when we consistently lay ourselves before Him, asking for HE alone to be glorified - EVEN THEN, heartache, loss, sickness, desperation, depression, death, and oppression of all shapes and sizes will, at some point, show up in your life. God promises it, and I can tell you from experience, it's a lonely, isolating, exhausting place to be. 

How often my breath is taken away, weak in energy and faith, 
My Spirit just a whisper, where there used to be a strong and healthy cry, and so I will continuously say,
"God, I believe; help my unbelief".  

Mark 9:24
Immediately after, the father of the child cried out, 
"I believe! Help my unbelief!"

Think back with me now, to the most desperate time in your own life, when YOU were the most vulnerable and the most in need, as in flat- on-your-face, crying out, with moans too deep for words, sick-at your-stomach, kind of need. Remember, for a moment, those feelings, those thoughts, that agony you likely felt, the desperation in your heart and soul. . . 

Now, think of a time when someone swept in and rescued YOU, comforted you or provided, even in at your most needy state; your very lowest point.  How did it feel, knowing someone cared enough to notice where you might be emotionally, or cared enough to hear your cries of brokenness? Someone saw the horridity of the heartbreak and mess you were in, yet dove into the flames to rescue you, with no thought of themselves! 

It happened - you were saved! 

It wasn't Wonder Woman or Superman who've come to your rescue, but a regular human being.  Maybe your Rescuer was  spurred-on by the Holy Spirit.  Maybe "life-saving" is physically in their job description. Maybe they just have a heart for others.  Bottom line - SOMEONE came to your rescue.  SOMEONE stepped up and helped.  SOMEONE decided to give to you, to love you, even in the darkest of times.  

Right at this moment, there is someone in a dire situation; 

They need YOU to rescue them.  

There's a call on your life to be a, "Rescuer", a helper, someone who brings life back to another human being.  

It's YOU, Friend. 

You are the One - the one to step up and step in; the one to bring life with encouraging words, a purposeful gift, a listening ear; 
the one to bless financially, 
The one to offer physical labor or sacrifice.  

IT IS YOU. 

Your giftings, your background, 
your current position or place, 
have prepared you to RESCUE, 
at this very moment in time. 

Don't hesitate.  Don't miss out on an opportunity to feel the freedom which comes from recklessly giving of yourself.  Don't miss out on something you were created to do.  Don't let fear, distance, or awkwardness, stop you.  Just jump in.  You may bring light to someone's deep darkness.  You could be freeing someone from ruin.  Be a vessel for something good. 

****************************************************
Thank you for your continued prayers for our family!  There are so many things coming up, including Lucy's 8th birthday, Conner's 16th birthday and the NKH International Conference and fundraiser, located in Boston, MA.  In the coming days, I hope to update on all of these things and more.

Right now, we covet your prayers for our Nurse E, from Cameroon, Africa.  Their country is at war and violence is very common, especially for those who have family in the United States.  Nurse E's sister was kidnapped a few days ago, and although they've recieved a ransom request, the details have still not been worked out.  

Also, a precious family prepares to say their, "earthly" goodbyes to the daughter, wife, mother, friend, sister, and (recent) grandmother. This family has been hit with cancer THREE TIMES; they've all lived through the first two fights and now, C's fight has become overwhelmingly strong.  Her physical presence with her family will soon be at an end.  Pray for PEACE and COMFORT. 

Jesus, we take so much for granted.  The world is hurting.  Our people, our friends, hurt even now.  You continually equip us to RESCUE the people you place in our paths!  There is NO hope without you, Lord.  May my life bring your HOPE to those around me.  All belongs to you; nothing belongs to me. 


Ryan

    

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glish Standard Version
Immediately the father of the child cried out and said, “I believe; help my unbelief!”

King James Bible
Return, O LORD, deliver my soul: oh save me for thy mercies' sake.
Darby Bible Translation
Return, Jehovah, free my soul; save me for thy loving-kindness' sake.
World English Bible
Return, Yahweh. Deliver my soul, and save me for your loving kindness' sake.
Young's Literal Translation
Turn back, O Jehovah, draw out my soul, Save me for Thy kindness' sake.

Morning Has Broken!

My heart is overcome with gratefulness, spilling over with joy!  No, we did not win the lottery or fall into some lofty inheritance.   Mercy who has brought me to this place, allowing heavy, dark concrete to fall off my body, mind and spirit at this moment in time.  This joy isn't because of anything I've done, but I am more grateful for these moments than years of "thank you's" and praises could ever express!  

 

The Spirit speaks to me so often through song. Some may remember the old ballad of Cat Stevens' about a breathtaking morning, the peace so tangible, it dances like a mist over the meadow; the delicate birds begin to sing the songs God placed inside them. 

"Morning has broken like the first morning

Blackbird has spoken like the first bird

Praise for the singing

Praise for the morning

Praise for them springing fresh from the world" 

- Cat Stevens 

For the last thirteen years of our roller-coaster Journey, one desperate song has continually played in my mind, body and spirit.  God placed words in my mouth at the beginning, when Ellie Kate was in the PICU as a newborn, as the doctors told us she would likely die that very night.  I had no words - groanings too deep for words.  Some of you know exactly what I mean.   I only remember the chorus, but maybe that's all I'm supposed to have stamped upon my heart . . .  

"Oh, Lord Jesus, Come quickly to me!" 

 

 

But if we hope for what we do not see, we wait for it with patience. Likewise the Spirit helps us in our weakness. For we do not know what to pray for as we ought, but the Spirit himself intercedes for us with groanings too deep for words. And he who searches hearts knows what is the mind of the Spirit, because the Spirit intercedes for the saints according to the will of God.

Romans 8:25-27

At times in recent years, I've felt empty and broken, as if a piece of me were missing - a piece I needed to keep going.  During the trials and heartaches of this life, every single one of those feelings is normal.  Every feeling is okay to have, to FEEL.  Don't be ashamed of those feelings - there is nothing to be ashamed of, Dear One.  You are human; God made you with feelings and created you in a way to express them. 

 

There are no magic words, no specific Christian book to read or Study to go through; your heart and mind won't change when you hope or "wish" it away.  No, it comes in the Lord's timing.  He may give you this joy and freedom DURING a struggle, or He may do so in between the seemingly constant trials.  All we can do, all we MUST do as Believers, is SURRENDER - surrender over and over, our hearts always bent before God. 

 

Your Father WILL sweep in and show Himself in your life, even in the midst of the heaviest trial.  He is, "Mighty to save", continually moving mountains on behalf of His beloved Children (even if we don't see them).  

 

 "The Lord your God is in your midst, a mighty one who will save; 

he will rejoice over you with gladness; he will quiet you by his love; 

he will exult over you with loud singing".

Zephaniah 3:17

 

Our God continually PROMISES to make ALL things work out for YOUR GOOD (and the good of your children, spouse, family), and for HIS GLORY.  As followers of Christ, what more could we want in this life than for God to be glorified through our lives and the lives of our children, AND for Christ to get the glory for everything?!? 

 For I know the plans I have for you, declares the Lord, plans for welfare 

and not for evil, to give you a future and a hope.

Jeremiah 29:11 (ESV)

 

This is God’s Word on the subject: “As soon as Babylon’s seventy years are up and not a day before, 

I’ll show up and take care of you as I promised and bring you back home. 

I know what I’m doing. 

I have it all planned out—plans to take care of you, not abandon you, 

plans to give you the future you hope for.

Jeremiah 29:11 (MSG)

 
Oh, Father!  Thank you for this glimpse of happiness, joy and sweet expectation!  Thank you for relieving my heavy burdens, even if only for a moment!  How grateful I am to breathe so lightly, to rest so deeply and freely, knowing I can trust YOU with everything.  Life will always be difficult.  I will continue to live in survival mode because of our circumstances, however, YOU are there.  I find hope and peace, knowing you are using all of this for the good of my children, my family.  How humbled I am that YOU would be glorified in me, a broken and sinful human.  When I'm next in the darkness; if the heaviness of these trials once again dims my spirit, may I remember the feather-weight of these days.  

 

I Surrender, 

Ryan

 

CONNER is enjoying his freshman year at Westmoore and is working hard, playing on the soccer team.  High school is a whole new world, but we are incredibly proud of the young man Conner continues to be - tender-hearted, affectionate, witty, helpful with his sisters and patient with his brother. His life brings peace, balance and LAUGHTER to our Home.  I'm so grateful for Conner (or, 'Boy' as Bowen calls him). 

BIG grin from Henry - looking up to Big Bub

 

HENRY has been initiating his schoolwork and chores, showing more and more of the "Real Henry" we know and have missed.  He's growing more responsible and even more affectionate, hanging on to every verbal affirmation that comes his way.  Henry has changed for the BETTER since his IVIG treatment last fall, and we've seen a HUGE difference in his life.  His life is more calm and steady, without as many highs and lows.  He is able to eat and has gained back the weight lost last summer due to PANDAS symptoms.  Henry's playing soccer again this year and has played every game in it's entirety (without taking a mental break , quick walk to calm his mind, not even a break for reassurance from me!  This is HUGE.  I hope to update more on Henry soon.  In the meantime, THANK YOU for praying for our Sweet Boy.  Thank you for giving, helping to provide a way for him to go to D.C., paying for his IVIG treatment - a treatment which has clearly changed his little life for the better! 

Look at the smiles on those faces!

 

BOWEN is a light and complete JOY!  Speaking in sentences now, we take great delight in hearing her speak, which happens to be quite often.  Bowie is athletic and is now obsessed with our "mamboleen" (trampoline).  She's small for her age, which just adds to the fun for me, as I want her to stay a baby as long as possible:).  Bowie Jane is just as strong-willed as our other kids, and has recently said, "Mommy, YOU MUST obey me!", "Mommy, PLEASE, no talk, no sing (I like to hum and sing around the house)".  She's graduated from "Bubba" to "Enry" (Henry), although she still goes back and forth on those.  My parent's dog is named, "Duggah" (Sugar) and Lucy's name is not only "Sissy", but also, "Juicy" (Lucy).  She enjoys singing, "Lucy, My Belle" with me - a favorite of "Juicy's", which PawPaw Stan would sing to her often.  Mike and I can't imagine life without Bow, and the boys often say the same.  

Joyous Bowen!

 

LUCY is growing, which is wonderful!  A year ago at this time, Lucy was so still ill and thin; she barely had energy to move at all on her own, something she loves to do.  Now, she's weighing heavier than ever, which is a MIGHTY blessing!!  Her energy level amazes us, although she still literally falls asleep anytime, in any place, in any position (she's a flexible pretzel due to the affect NKH has on her muscle tone).  She's moving more and more on her own, pulling herself up to her knees.  We haven't seen Lucy this happy and have not heard her giggle this much, since she was an infant.  It is a GIFT we don't take for granted.  

Happy Day at the Park!

 

Mike and I have counted approximately 30 urinary tract infections, which Lucy has had in her seven short years on earth.  This is heartbreaking to us and, through many tests over the last several years, we know Lucy retains her urine and the retaining is only worsening, instead of getting better.  The medications we've tried have not worked.  Lucy's still often in pain and at times, in her own way, can show/tell us what's hurting.  I know how excruciatingly painful my Intercystial Cystitis can be, and I fully believe that is how Lucy feels, likely even worse.  I can't imagine a child in such pain.

 

Even though Lucy is on Palliative Care, we have decided, with her specialists, it would be best to place a permanent catheter.  Most of the time, surgery wouldn't be done on someone on Hospice or Palliative Care, but we all agree this step is a MUST for keeping Lucy as pain-free and happy as possible. The catheter will allow us to drain Lucy's urine as needed, preventing retention, which in turn, helps to prevent bacteria growth, which in turn, eliminates or limits the amount of infections, which in turn, makes a happy and healthy Lucy Belle!  She will have to go "under" and be placed on a ventilator during the surgery, something we are extremely nervous about, as there is no assurance Lucy will wake up from that state.  We've never chartered this territory before; EK never had bladder issues, so it will be a new learning experience for us all.  Surgery is set for the end of April.  

Thank you for praying.  Thank you for loving.  Thank you for supporting us, in so many ways. We'll keep you posted.

Sometimes, It Goes Downhill So Quickly (Lucy Update and More) . . .

Psalm 34:18 (ESV)

The Lord is near to the brokenhearted
    and saves the crushed in spirit.

There's much to update, especially since I haven't blogged in a while and haven't been very consistent in updating these last many months.  This has been quite intentional, as the Lord made it blatantly clear that my family needed my attention more than ever, which has recently proven itself to be true many times over.  I won't lie - today has been incredibly difficult for us all, and while I've felt a pull to post recently, I know after today's events, it is important for me to do so tonight (err - this morning).

 Please bear with me as I try to briefly but sufficiently cover several things, including Lucy's declining health, Henry's IVIG update, DC trip info and more . . . 

Lucy Belle:  Our Girl turns SIX next month, and it's so hard to believe that much time has passed since we were preparing for her arrival and even MORE hard to believe that it's been so long since Ellie left for Heaven.  I vividly remember thinking then, "if Lucy follows Ellie Kate, I at LEAST have SIX, FULL more YEARS of life with her and I will drink in every moment!".  I know that sounds twisted for some, but it is an honest picture of my mind at that moment.  Lucy has been a life-line for me in Ellie Kate's earthly death and she has saved me as I long to be a good mommy to her and to the boys, especially through all of the seasons we face.  

One of our Favorite Summer pics of LuLu - 

feminine and sweet, with her hair growing so long!

Our Sweet LuLu yesterday, snuggling with Mommy

Miss Lu Lu (aka, 'Lullie') has taken a turn for the worse, although she is stable at home tonight.  As many of you know, Lucy went on hospice a few months ago and while we knew she was declining, the main reason for choosing hospice was for palliative care, meaning we could better address Lucy's immediate issues (most definitely including her intense pain) while receiving the attention and other various benefits hospice provides.  It was at that time a few months ago, several of Lucy's doctors at OU told us they thought she was following "her sister's life-pattern" and because of that, they encouraged us to leave the regular Sooner Pediatric Clinic and thus, we are now seen outpatient at The Children's Center.  This was a HUGE decision for us, but we have not doubted it one bit (other than missing our favorite nurses and folks in the SP Clinic).  

Our new pediatrician specializes in medically-complex children and cares for many of our Hope Link friends, and he is on the Board for our current hospice service.  We've been SO blessed by Russell Murray Hospice and never could have prepare nor guessed that this same hospice, these same precious nurses, would soon be caring for FIVE of our Hope Link friends at the same time - walking with two of our dear families as their babes recently left this earth.  

In recent months, weeks and even days, we've noticed Lucy's seizure activity increasing, which we had addressed accordingly.  Right now though, her neurological state and seizure activity is changing SO quickly that we can't really keep up with it.  All along, especially in this decline and in light of what we went through with Ellie Kate (or rather, what Ellie Kate endured in her pain and suffering), our main concern has been that Lucy NOT be drugged and "out of it", but that GREAT efforts be made so that she may be at peace, her body and mind at ease and without pain. 

This week, despite some rough recent days, Lucy started school.  She even made it an entire FULL day this week and YA'LL, that's HUGE!  We are SO incredibly proud of Lucy starting kindergarten and we are forever grateful for her teacher, Jana Neisent and the staff in her classroom, who could NOT be MORE precious, sincere, intentional, kind, and even prayerful.  Though Lucy was able to attend some school, she still has experienced great pain, the source really unknown, and she's needed pain medications to keep her calm and happy.

This morning after an unusually good night with Nurse Emma, Lucy rolled over and vomited and let out a strange cry - all unusual for her.  Emma yelled out for me and once I got to the room, Lucy was completely and absolutely unresponsive in every way.  NOTHING was waking her and we noticed her breathing was labored.  She started taking some deep, strange and struggled breaths so we immediately called hospice, who joined us shortly after.  Lucy's BP has been low, her heart rate extremely high, her temp normal, but shes's chilling and shaking then sweating (unlike her completely).  Even in doing vitals, she didn't wake or stir for many hours this morning, so Mike came home and MiMi (Mike's Mom) ran over to get Henry and Bowie (Conner was at school).  Lucy stirred a couple of times throughout the day but never has been back to herself, back to "baseline".  Tonight, when she has been awake, she's been screaming hysterically with no way to comfort her, and we aren't sure if it's pain or something neurological.  


We know this much about Lucy: 

• Lucy's issues today aren't bc of an overdose of pain meds or meds in general 
• Lucy's gut hasn't worked well for a while; absorption and processing has been slow AND
• We know the gut issues seem to be getting worse by the day. 
• We know Lucy is no longer having bowel movements, even with "help".   

Our Fears for Lucy:  By far, the gut issues are the MOST frightening, as Ellie Kate's life ended when her gut eventually completely shut down, and it looked much like this.  Holding her listless body today as she was taking those labored breaths, so reminded us of Ellie Kate and her last days. Unfortunately, it does look like NKH is progressing rapidly in Little Lucy but we should soon know if new meds may play some sort of part, esp because of her metabolic issues.  Our hearts hurt and although we've known from even before we ever held our blond-haired girl, this would one day happen, it doesn't make the sting any less harsh or the thoughts any gut-wrenching.  

The Future for Lucy:  We don't know what will happen with Lucy; she could wake up tomorrow completely back to her normal, and if that's the case, we will dance up and down the streets in praise to God (I will even take video)!  But if she doesn't bounce back quickly, or if she doesn't bounce back at all, we will continue to huddle in as a family and drink in every moment.  

Our Current Feelings on Lucy's state:  Sincerely, we feel grateful in that we get a SECOND chance to walk the road of child-loss, and what I mean by that is, we already well-know what we WISH we would or could have done towards the end with Ellie Kate, but we GET the chance to do it better and accomplish all of those wishes and regrets as Lucy's NKH progresses.  Truly, Friends - we see this as a comfort and gift from God and once again, Ellie Kate's life has made Lucy's sweeter, easier, less-complicated and SO much more!  
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Adoption Finalization . . . On the last Monday in June, we officially and legally welcome our youngest into the family before the State of Oklahoma.  Bowen Jane is now a McLaughlin and we cheerfully have added, "Bowie Mac" to her list of nick-names.  We are BEYOND grateful for this unplanned, life-changing GIFT from God (which is one meaning for her name) and although the timing has been mysterious at times, we see Bowie as SUCH a healing balm to us ALL.  Watching the boys, Lucy and even both sets of grandparents enjoy such a joyful, happy, delightfully growing baby girl within our home brings us endless laughter, even during uncertain times. God's timing is ALWAYS best, even when (and especially when) we don't understand or expect it. 

Bowie Jane Mac and MiMi awaiting the finalization

It's Official!  Professional photos coming soon.

I couldn't hug him tight enough that morning, waiting for the judge to call us in.  

My heart is SO grateful for our New Baby Girl!  

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Henry's IVIG . . .The Saturday after Bowen's finalization, we flew to D.C., much to the courtesy of the Isaiah Stone Foundation here in OKC.  There was NO way we could have financially made the trip, even with Mike's excellent, steady job and paycheck (which we are beyond grateful for).  Our family spends over 20% income on healthcare each year, so a trip like this would have been difficult, especially after trying to settle and pay adoption fees.  GOD provided a way for both boys, Mike AND myself to go and we even toured the Capital a bit before and around Henry's appointments for his PANDAS.  What a GIFT!  

It's "Darryl" from The Office!  

Conner was thrilled - 

 my boys love them some Craig Robinson!  

Did you know that Albert Einstien never learned how to tie his shoes?  That's a familiar thing to Henry, so he made a cool connection!  

Henry's legs were hurting too badly to walk the museums 

(PANDAS can do this, much like Lyme Disease), 

so we used the wheelchair while "adventuring" together. 

The Air and Space Museum was a HUGE hit! 

      Tour Buses were our friend!

Sweet Boy, Taking it all in

We met with Dr. Latimer, one of the three PANS/PANDAS specialists in the U.S. (her words), and we were blown-away.  Dr. Latimer confirmed everything we felt and thought about Henry as parents and supported all of the test and blood results taken over this almost four-year adventure.  She collected ALL of Henry's info and studied each part, getting to really know him and US.  Dr. Latimer told us more about WHY Henry acts the way he does - ex: she noted Henry's chorea movements (which we barely notice), and some tics which also had not jumped out to us much.  She also physically pointed out Henry's reflexes aren't working in the normal way (he is hyper-tonic).  She read through the EEG's, as she's also a renowned Neurologist, and was able to give us insight there.  Dr. Latimer also told us more about those with PANDAS, who only get approx 3hrs of REM sleep every 24 hrs, so they are always tired in every possible way.  This gives way to extra crankiness and confusion, which can trigger the already heightened "fight or flight" response.  It's a recipe for disaster without sleep but there is no cure for that part of it, or for any of it, at this time.  

After visiting with us for several hours, Dr. Latimer suggested IVIG treatment, which is what we expected and hoped she would do.  Our doctors here in OKC have recommended it but it's rarely done here for PANDAS - that isn't the case out of state and it's an especially normal thing to do in that part of the country.  We hoped she would invite us back for the treatment sometime very soon, but to our SURPRISE, Dr. Latimer suggested we come in immediately for IVIG treatment.  Mike and I already knew this was what Henry needed.  It's the last-effort treatment, the best and most-effective treatment, for severe versions of this disorder.  It's not the quick or easy decision you hope it to be, as the cost is due in full upfront and in the clinic, was $12,500 without ANY assurance it would be covered immediately (or soon) by insurance (in hospitals it runs anywhere from 40-60K). Mike and I spoke in closed doors before leaving the doctor that day, and although we KNEW Henry needed this, there was NO way we could pay (We are still paying off some of Ellie's bills and of course the all-expensive law school (seriously ya'll, think about it before you go- tongue in cheek)).  

That night, we walked around D.C, staying out late (I admit to being somewhat down, wondering when and how God would provide, why He had given us this opportunity to just send us home, and more).  We literally, physically ran into Paul Ryan (surrounded by secret service but at least we got a smile and nod!) - of course I couldn't say a word except his name (why couldn't I have given him some friendly, special-needs momma advice the night before the big healthcare vote:)?).  Before bed, we received a text from some very dear and generous friends whom Mike had randomly filled-in about the treatment for Henry.  Ya'll, these friends offered to PAY FOR HENRY'S TREATMENT IN FREAKING FULL, with no pressure on us to pay them back in a certain amount of time, which means we can work hard with insurance and fight for coverage if needed (although we haven't yet heard from insurance).  WHAT?! WHO DOES THIS type of thing?!  I CRIED in JOY, thanksgiving and relief, amazed at God's provision yet again for our family.  He ALWAYS provides what we NEED!  I excitedly called the doctor early the next morning and made plans for the IVIG treatment to take place over the next two days, just in time for us to leave straight from the office to catch our flight home (yay for Uber!).   

Happy Henry during IVIG

Pals receiving treatment the same day!

Henry took it all on like a champ.  We didn't know what to expect, but there was another little guy receiving his second day of treatment our first day (it's a 5/6 hr treatment over two days), which provided Henry a true playmate for the entire day of treatment - we couldn't have planned it better.  It also provided solace and peace for us as parents, as we could connect and share stories and in sharing, there is HOPE.  We saw NO signs illness or reaction to the IVIG (reactions can look like that of chemo), although they loaded us with the proper meds and made sure Henry received plenty of fluids for the flight home.  It wasn't until we got back that Henry suffered set-backs and vomited with severe headaches, for over a day or so.  We know it could have been so much worse and are sincerely just grateful we could even have the chance to do it!  

The ultimate test will be in four months, about six months out from the treatment, when we should see the height of the IVIG success.  Folks have asked, but until recently, we had not seen many changes in Henry.  Now though, he is doing SO much better - making better decisions and increasingly choosing to do what he needs to each day, many times even on his OWN. It's still a daily struggle and I continue to believe this journey is much harder than dealing with feeding tubes, pumps, suction machines, seizures, and wheelchairs. We hope the benefits will be even MORE clear this coming week as Henry starts at Epic Charter schools, with a special-education teacher and principal.  OUR God has provided this trip AND this treatment so clearly, so neatly, to timely, I have NO doubt that He's actively at work on behalf of Our Son!

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Oh, Friends - thank you for staying with me throughout this long update.  I'm sure you get tired of my words and the length of the blog, but you will never know how much your endurance and friendship means to us.  

While we do face struggles 

in other areas at this time, 

our current prayer requests are listed below.  

Thank you in advance for championing 

and interceding on our behalf!  

• Please pray for my Little Sister Rachael, who gets married later this morning (it's Sept 1st now!).  Ellie Kate died on Rach's bday and although Bowie was born on that same, precious and ordained day, it's still bittersweet for her.  While we do not believe Lucy will go to Heaven this weekend, I hate the thought that tragedy once again mars my Baby Sister's life and on one of her biggest days yet - her wedding day.  Prayers for peace, understanding, hope, and JOY are much appreciated!  
• Please pray for provision for the IVIG medical bills so that we can reimburse our friends, who so willingly GAVE that Henry could RECEIVE (they love him dearly).  Please pray too for those insurance issues to settle easily, quickly and peacefully, all in a Divine Way. 

• Please pray for Lucy's complete comfort and healing.  We pray she would be healed here on earth, but we want what is best for HER, always and forever, no matter what that may look like.  

• Please pray for all of those involved in Lucy's care - hospice, home health, doctors, specialists, and more; that God would open eyes and hearts and that He would guide every decision on Lucy's behalf, especially during this time of uncertainty.

• Please pray for us as Lucy's parents - that the Lord would UNITE me and Mike together, binding us tightly, and that He would draw us to Himself as well as to each other.  Please pray healing for us in ways God understands and we ask that He too will give us the Divine wisdom and direction we need to make every move.

• Please pray for Henry as his IVIG still "sinks in", so to speak.  Pray for HEALING here on earth for Henry - body, soul, spirit, and mind and that God would be preparing Henry for school starting next week.  Pray that things would fall into place with school, despite what's happening at home, and that Henry will once again be able to thrive and gain confidence in that.

• Please pray for Conner as he's started the BIG 8th grade - and we all know how difficult and scary that age can be. Please pray for Conner's heart to be engulfed by the Spirit and that the Lord would capture him even now and amidst the hardship surrounding our family.  He so often gets understandably angry and frustrated with it all and yet he carries it in ways I can't begin to understand or imagine.  We only have about four more years with Conner at home and we want those to be HAPPY, HEALING years. 

• Please pray for our families - our parents, in particular, as they struggle and hurt when their children AND grandchildren hurt.  Pray for strength, provision and availability for them to be with us as we need them and as they want to be.  

• As always, please pray for great peace and comfort as we face unknown days ahead - like so many of those dear to us are walking through now.  

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We BLESS you tonight, and as a united family, 

thank you and pray God's peace be upon you and your family and that He alone would meet your needs in the above ways as well.  

Psalm 34:1-4

I will extol the Lord at all times;    his praise will always be on my lips.
² I will glory in the Lord;
    let the afflicted hear and rejoice.
³ Glorify the Lord with me;
    let us exalt his name together.

⁴ I sought the Lord, and he answered me;
    he delivered me from all my fears.

Ryan Elizabeth