Everly Update 12/27

Thank you for hanging in there with us as we navigate this new path of hospitals, new medical discussions and rhino virus.

It is a couple days after Christmas and I am sitting in the dark of our hotel hospital room while Everly sleeps. So, all you problem solvers out there probably picked up on the fact that means, yes, we did spend the holidays in here.

While it would have been nice to have our annual Christmas Eve appetizer and dessert buffet at our house, we were and still are glad that we have our Sweet Pea...no matter where we are!

So, since we couldn't go to our Christmas festivities, the Christmas festivities came to her! That actually began with our visit from Santa just a couple days before this present hospitalization.

Yup, THE Santa!

One of Santa's elves contacted me in mid-December because they knew that it was not a possibility for us to take Everly into the mall to visit Santa. Our bubble has to be our home so we arranged a visit to the house. I told the kids he was heading our way and Kendan literally was unable to control his excitement! He paced back and forth at the front door, just waiting.

 Wearing our fancy dresses during Christmas time!

Our visit with Santa was nothing short of pure MAGIC! From his warm disposition with the kids to his bringing and then playing the nose flute with the boys. I think Santa was as smitten with our sweet angel as we are! He held her, held her AND held her! She was just as smitten with him as she continued to stare at him with such intrigue. Santa told stories about various aspects of keeping the reindeer in "off season" and gave Everly an elf name. I thought Santa had to hurry home to finish working in the toy shop but, much to our surprise, he said he wasn't in any hurry. As he sat there just looking at Everly, she stared back at him and then just fell asleep in his arms. He stayed there and held that baby up in his arms while she slept for well over an hour without the slightest movement. The most special part of the visit was when Santa prayed in earnest over Everly.
While still inpatient, my mom single-handedly orchestrated Christmas Eve tradition in the hospital! We had egg nog, cookies and appetizers in the room. (Thanks, Nana, for setting up the whole buffet in the room. I know it was a lot of STUFF to bring in just to turn around and lug it back home.) The boys watched A Christmas Story while we chatted and nibbled the evening away!

The start of the Christmas Eve buffet!

Christmas Day began with the boys and daddy toting all that Santa had left them at our house here to the hospital room. Nana and Papa met over here as well and we all shared in Everly's FIRST Christmas Day! She just sat back in her therapy chair in the center of the room and took in the chaos! She was very good this year because she received exactly what she asked for: Mary Jane style socks, a therapy exercise peanut in PINK and baby nail polish! Good job, E! The boys were also good because they each received the three things they asked for from Santa and a few extras!

Now for the medical update part...

The last update I gave ended with the switch last Sunday from Bipap down to high flow oxygen and then later in the day to room air while awake. (12/21)

That next day it was suggested that a sleep study be completed while inpatient so we could see if she had apnea which would answer the high CO2 retention (part of it anyway). So, that night she had a sleep study. On Monday, the pulmonologist came in early evening to tell us that she had over 100 apneic episodes during the course of the study. She most definitely could use and benefit from the CPAP. It could be life-saving with that number of spells happening!

Trying to find something else to do. You Tube baby stuff to watch.

So, in my naïveté, I thought that the CPAP machine was ordered and then delivered wham bam! Umm. Let me fix that misconception now.

No, it isn't ordered in one day.

No, it certainly isn't delivered it on one day.

The truth is that it is COMPLICATED. The machine is actually considered a ventilator and is treated as such. We won't be having it in that mode as we'll be doing CPT as well.

Because it is considered a life-saving device, it is NOT something that can be acquired through our hospice contract since hospice is comfort care at the end only.

My girl!!

So, that's pretty much where we are now. It has not been ordered as of yet due to a few other issues that will need to be addressed Monday. The approximate time frame we were given from the DME company we'll be using is a week at best to get the machine to us in the hospital. The biggest issue is that the insurance won't pay hospice for care AND also a DME for another piece of equipment since hospice is everything. Therefore, in order to get this machine, we will have to sever ties from hospice. I will, however, check on Monday to see if there's ANY other way around this but I've spoken to two case workers here who have experience in this situation. So, basically what this means is that we would have to give back ALL her machines, tanks of oxygen and who knows what else and depend on an outside company to supply Everly. I am hopeful that the transition that we are now forced to make will be for Everly's benefit.

Everly herself is still sick. Well enough to go home but sick enough still that we will need to be extra vigilant. She is still very congested and has tons of drainage. The drainage is good except that it makes it hard for her to sleep and rest during he day. She is now on CPAP at night and that has given her better rest then. The mask and the pressure input are certainly new sensations that aren't necessarily pleasant and will take some getting use to, especially since the home one will be different anyway. I must say that her sleep the last few nights with the CPAP on her has been definitely helped her. Some hiccups with the mask placement and seal that keep coming up due to head position (babies move) and her NG tube leaving a tiny crack in the seal around her nose.

We are both ready to come home and the boys are ready for us to be there, too. This lack of routine and consistency among other things wears on you. However, again I am reminded that we must stay so that she can get what she needs. I forgot to mention that the company must go through our house first for some inspection. Then when the machine is ready it needs to be brought to the hospital so that we can fix the settings and it can be monitored here for a night of sleep at least. So, those two components also add to our ability to take Everly home.

If you're keeping track, we've spent my birthday (Feb for her birth), Thanksgiving, Christmas Eve, Christmas, and now New Year's Eve and New Year's Day all in the hospital. Thankfully we are surrounded by lots of fun, caring and helpful peeps working here to keep us motivated! We can do this! We're almost there!! Well/non-sick visitors are welcome, meals are also welcome.

We are so reminded all the time about the blessings we seek and how God cares for each of His children. Now is no exception!

With love and appreciation,

Crystal

P.S. Prayers please for our Trisomy 18 friend Elisabeth as she was admitted into the PICU in Texas now as well. This congestion/cold no matter how small is very serious! She is resting comfortably now and we pray that she keeps that up so she can give it her all! Thank you all!

Everly Update 12/21

 This was within minutes of removing the high flow system.

God is so good! Thank you all for praying in earnest for Everly!

She is OFF oxygen!

I repeat: she is OFF oxygen already!

This morning on BIPAP still and tonight she is free!

We most likely will need to give her a tiny amount--.2 or .3 probably--when she hits REM sleep only.

So, let's marvel at this timeline:

Friday morning: EMS to St. Joe's, non-responsive, lethargic
CO2 level is 81 should be less than 45, can easily be deadly, discussion by 4 doctors about the very real possibility of intubating her and that she might not come off of it,
placed on high flow from her regular cannula, later placed on BIPAP for her increasing needs, no feeds all day, IV hydration

Saturday: still no feeds, still on BIPAP with lots of support, retest of CO2 level and it remained okay at 41

Sunday morning: weaned to high flow early morning, then planned to wean to regular cannula but during the transition it was apparent that she no longer needed it, may use oxygen during REM sleep if heart rate sags any, started foods this morning and doing continuous and she's digesting it all.

 

That's my girl!

I continue to be amazed at God! He no doubt heard everyone lifting up Everly's name to him! What a blessing it is for our family to have all of you praying for her and just enveloping our family with love and support!

I am just in shock and awe that this was all done today! But God can do it all and He did! Oh, how He loves His children!

 How perfect is this balloon!!!

Everly Medical Update Part 2 (all you wanted to know AND more!!) (This is the forever promised "medical stuff" update!!)

**PLEASE NOTE: THIS UPDATE WAS ACTUALLY WRITTEN ABOUT OUR LAST VISIT IN NOV (THANKSGIVING) AND NOT THE CURRENT ONE (12/19/2014). JUST GETTING AROUND TO EDITING AND POSTING NOW.**

 Dr. Niebauer!!! Our angel doctor! 

How we love you! It's probably sinful.

Let me first start by saying that when we were initially hospitalized and then released, I was hoping to never have to revisit the hospital. Not because of the staff or St. Joe's in general but because it would mean that she needed to be in the hospital for some reason and that is what I wanted to avoid.

But then I must remember that God's in charge, not me.

Never me, always Him.

Being admitted twice has turned out to be the BEST thing that could have happened to her.

It was His plan all along.

Philippians 4:19 And my God will meet all your needs according to the riches of his glory in Christ Jesus.

Not only has God done that for us since we've been in the hospital, He's gone above and beyond to further my faith in His providence.

I love this description of what providence is in relation to God: Providence, then, is the sovereign, divine superintendence of all things, guiding them toward their divinely predetermined end in a way that is consistent with their created nature, all to the glory and praise of God. This divine, sovereign, and benevolent control of all things by God is the underlying premise of everything that is taught in the Scriptures.

Ever hear about God placing people in your path for His purposes?

I know that every step of our journey has been just that...people placed in our path based on His plan...our us placed in their path for His plan.

Most recently, our paths crossed with a doctor in the PCICU who in no uncertain terms has changed our lives and more importantly, bettered and, I'm positive, prolonged Everly's.

Dr. Julia Niebauer is an angel. A real, doing-works-on-earth, angel.

She is an out-of-the-box-kind of doctor. She didn't just treat Everly for the acute illness. No, ma'am.

She looked over her chart, her labs, her history. Every single detail.

Then she leapt into action. And I mean literally 0-60 in 5 seconds!

To try to give a play by play of the last two weeks, this would turn into a small book. So, instead, I will just cover the highlights.

Everly's Feeds/Weight: Dr. Niebaur inquired about her feeds and the fact that she wasn't consuming enough calories. I was taken aback that she had noticed because I have felt that for the last two months and even went to our GI to address this. She also asked if we had ever tried continuous feeds (feed running continuously for a period of time). I responded no but I was open to any changes, especially when it will help her to gain. So, literally that night she started continuous feeds from 8pm - 8am. Everly's body did well with the change. From there, Dr. Niebaur continued to tweak her feeds, both timing and volume, until currently she is at the optimum schedule. Prior to the changes, Everly would eat every three hours over the course of an hour and fifteen minutes...around the clock. Each time, the process was the same: check placement, residual, heat food, clean food bag if pump feeding, if not, then process was longer because we would feed her with syringes (bolus feed) by hand over that hour time frame. Now she eats at 11 am, 2 pm, 5 pm over a half hour period and then has continuous slow feeds over a longer period of time (12 hours) all via the pump. Plus, this allows her to receive more volume (=more calories=more weight gain) overnight. Yay! I'm normally not one to embrace change but this is one I'm digging! Oh, AND, Everly gained a whole pound while in the hospital over 2 weeks!! Currently 12 lbs 8 oz!

Everly's Meds: Another positive change that was made...ditching the caffeine that Everly has been getting since NICU! She received it because of her central apnea. However, while we were in the hospital, angel Dr. Niebaur asked why she was taking it. After we told her, she said that they hadn't recorded any apneic spells while there since they monitor her 24/7. She stated that it is possible to outgrow this apnea and would we be game to try to titrate her off the caffeine. You betcha! The least amount of meds, the better, I say! So, that we began the process the very next morning. Within days, Everly was completely off of the caffeine...had to go slow due to side effect of possible headaches (you know how you feel during a caffeine withdrawal!). Praise God for this step!

Everly's NG Tube: Another concern, angel Dr. Niebaur had was of Everly's NG tube. She at first was worried that she may have been silently aspirating and that was part of the reason she had pneumonia from continued build up in her lungs. She consulted with Dr. Pham and he came to see us and together we came up with a plan. We would take her to nuclear medicine for a silent aspiration test to see if she was or was not having this issue. If she was, then step one would be to replace her NG tube that runs to her stomach with a longer one that would go lower and not allow the silent aspiration. The problem with this solution for us is that the tube can only be reinserted in the hospital not at home how we'd been doing it. Also, when it came time for a G tube surgery, she would need an additional procedure and a different kind of tube set up which is not what we felt was ideal. However, following the test, we found that she in fact does NOT silently aspirate so the NG can stay as is and there would not be any other kind of G tube needed. Another praise report!

Everly's Immune System: Well, we already knew she was medically fragile and ridiculously susceptible to illness. While inpatient, we also learned just HOW fragile her immune system truly is at this point. Dr. Bash, our hospice doctor, had her system tested to see what her antibody levels were overall. Found that she has something called Hypogammaglobulinemia which means that she has an abnormally low level of immunoglobulins (antibodies also called proteins that help fight infections). The thought is that she has more specifically Transient Hypogammaglobulinemia of Infancy which means she would grow out of this between the ages of 2-5 normally. Basically what this means is that to protect herself she requires an IV infusion of antibodies in the hospital or immunology office every 3-4 weeks. So, the poor baby has to get an IV each time and the infusion lasts between 2 to 6 hours, depending on how she can handle it. She received her first infusion inpatient at St. Joe's the night the blood test came back. They wanted to protect her right away.

What does this mean for us as a family? Well, that means that we are going to be the Germ Gestapo now as if we weren't before. So, in a nutshell, our home will become (like it almost is) our safety shell for her. Our outings with her during this Sept-Mar yuck season will be severely limited when it involves crowds, groups, etc. We will stick to our hikes, park outings, walks and the like. We'll play lots of games, build lots of puzzles and finally finish the last seasons of Little House on the Prairie! We will divide and conquer for errands and such...one will stay home with Everly (like we already do actually) while the other goes with the boys and/or by self. After April, we should be able to open the bubble a little more. I know that some are able to take their fragile T18 babies out and about but after now at this sitting 3 hospital admissions, 2 ambulance rides and upwards of a month sleeping with worries and fear--I'm happy to remain close to home where my girl is safest. Please understand how crucial these precautions are to her ability to live...you see how fragile she is. One common cold has done all of this to her. And the third admission is even AFTER the IV infusion so that's not a magic pill by any means. Friends, you have done an amazing job helping us keep her safe for 10 months and we just respectfully ask that you continue to walk with us on this journey. I've never been around anyone so fragile with their immune system and the doctors have even told us that it is surprising (interpret as a MIRACLE) that she hasn't visited the hospital or at least had one sniffle before 9 months! So, something we are all doing has worked, right??!! **My boys really would like to get out and hang with friends and now that we really can't have friends over to our house, they would love to have some invites. Yes, that was shameless begging maybe. This is a HUGE transition now even more for them as our circle has shrunk EVEN MORE. Thank you for your love for all of us :-)

Everly's Heart: Her sweet, tiny heart. Well, where to begin really. I have posted before that she has a very large VSD and that her lung pressures have been balanced since birth. She's had pulmonary hypertension since birth and since it's a progressive disease, it will only get worse with age. Once she got this Rhinovirus, it seemingly has altered that balance ever so slightly. The doctors felt like this was the right time to test her reactivity to a vasodilator like nitric oxide. "Vasodilators are medications that open (dilate) blood vessels. They work directly on the muscles in the walls of your arteries, preventing the muscles from tightening and the walls from narrowing. As a result, blood flows more easily through your arteries, your heart doesn't have to pump as hard and your blood pressure is reduced." If her body had not reacted--her SAT's quickly rising to mid-90's--then we would know her body had passed the window of opportunity to use these medications. The pulmonary hypertension would have been considered irreversible at that point. Note: if a person's body is found to be reactive still and they use the vasodilators, it does not mean that the PH will never come back just that it can be managed. Well, the good and exciting news for us it that her body was still reactive to the nitric oxide! And immediately! So, cardio started her on Sildenafil, liquid Viagra, and the oral version of nitric oxide. FYI: Viagra was first used and made for this purpose. The "other" was found to be a side effect and marketed to that population after the fact. This dosing of Sildenafil will help to manage this PH until further plans are made to investigate further the option of heart repair.

One of the most exciting things to happen during our stay was a pow pow that occurred among a small group of physicians regarding Everly's future heart repair plans. I told them as we began our stay that our goal is to repair her heart to the extent that it is a feasible and logical option for Everly, not just because a surgeon can do it. Following that meeting, our cardio--the one we've had since NICU--came in and after a long day for her in clinic and then a heavy, medicalese-laden conversation, told us that she would do the heart catherization needed to see if open-heart surgery (repair) was an option.

Why is this important?

Simply this: Cardio had told us from day 1 that a heart catherization was not an option for patients with Trisomy 18. Bigger than that, without this procedure that measures all sorts of complicated vitals heart-related, heart surgery is not even on the table.

This, my friends, is A BIG DEAL!!!

FYI: The date was Dec.1 so it's now a recorded date and a family holiday! lol

So, here's the part that is important to remember: just because a procedure--like open heart surgery--can be done, doesn't always mean it should. For example: trying to repair her heart with the balance that she's had over her life would most likely kill her. Why? Well, it's complicated but trust me, not an option.

Herein lies the necessity of this heart catherization. "It is a surgical procedure used to evaluate heart function and diagnose cardiovascular conditions. During cardiac catheterization, a long narrow tube called a catheter is inserted in an artery or vein in your groin, neck, or arm. This catheter is threaded through your blood vessel until it reaches your heart. A dye can be injected through the catheter to allow the doctor to look at the vessels and chambers of the heart with the use of a special x-ray machine. Once the catheter is in place, doctors can use it to run diagnostic tests." Basically, this can help the cardiologist to determine the value and benefit of heart surgery.

 

Our nighttime gal pal, Laura! Always a smile and even Everly is amused! 

She's on it and even thinks of things we don't!

The heart is complicated and one change affects something else. So, for example, the concern that we have for Everly still needing O2 is that because of it being a vasodilator, it will hasten the change in balance she currently has. If she is preparing to go into surgery for her heart repair, then this is what is needed and the protocol would be for her to be on continuous 02 and a med like Sildenafil. However, if she is not heading into that scenario, the concern would be altering an otherwise "stable" balance that is allowing her to have the kind of life she has without aggressive intervention.

So the next BIG news...is...are you ready???

There is an amazing surgeon who has agreed to do Everly's heart repair here at St. Joe's if it comes to that!!! God put the right people in place at the right time with the right heart. That is all I can say. This is AMAZING! Even if heart repair is not an option physiology wise, I will always know that someone deemed MY child's life AS VALUABLE AS ANYONE ELSE. I am humbled and so grateful. God is SO good.

NEXT STEPS: As for our next steps, the plan is to schedule the g tube surgery as she's approaching the year mark. Next, depending on we feel with the response her body has to anesthesia and the like, we will then schedule the heart catherization. Following that, we will have to wait and see the results from the heart cath to see what should be the appropriate next steps. By the way, we are not going to have the malrotation of the intestines corrected at this time. People can survive without that fix for quite some time; you just need to be aware of the danger signs. **Note: I believe due to the nasty germ season we are currenly in, we may wait until spring to schedule the surgery. Right now, germies are everywhere and are dangerous, especially in the hospital so if we can avoid it during this time, all the better!

 

Love Kathy! She's always cheerful and wanting to know how she can help you! A doll!!

Other: Everly is just so special to all of us! She has these piercing eyes that look as if she has something prophetic to say. She has clear likes and dislikes. For example, she loves to touch hands, fingers, facial hair, or just about anything else you keep close to her. She enjoys lots of stimulation and can get easily bored when there's not much going on around her. She recognizes faces and people and will smile to let you know. She loves her brothers greeting her in the morning and LOVES to watch them play together. She's extremely content lying next to them just attentive. She is so joyful and rarely cries and then it's for a specific reason, to be held, moved, changed or something like that.

Pediatrician: Most of you know that our beloved pediatrician is moving to another type job right after the new year and we will need to find another. Just to update you: my search is turning up a lot of No's. Meaning they upon learning of her diagnosis have felt like she'd be better cared for by someone else...that's me putting it in a nice way because, honestly, my daughter has every specialist under the sun, all she needs is a ped who can see her for shots, monthly growth charting, yeast infections, whatever. Can't a regular ped do that for ANY child, regardless of special need? Please, please pray for us to find THE one that was called to care for Everly because mama is discouraged. DISCOURAGED. Maybe I should send them a video of her smiling and interacting and being very TYPICAL??!! FYI: Taking names, suggestions and if you HAPPEN to be a pediatrician reading this and you know how deserving she is of quality and equal care like any other kid, pretty please contact me?!?! SHE IS WORTH THE EXTRA TIME A VISIT MIGHT TAKE!!! crystaldmm@earthlink.net

One Last Thing: As you can all see from our experiences as of late, it doesn't take much to take down a baby with Trisomy 18. So, please keep our whole community of kiddos in your prayers this germ season. Our friend, Elizabeth Maxine in Austin, and all our other little friends need your prayers. Please pray that Everly has had her LAST hospital stay until her g tube surgery in the spring. St. Joe peeps--we love you better from afar! lol

 

The Nitric Oxide set up. Dum dum dummm.

See why it's taken me SO long to write this up?? It's A LOT of information and we were slammed with lots over the course of two weeks. But now you are caught up to date with all of Everly's medical stuff for the most part. There's always more but that's another message on another day.

Thank you everyone for lifting us up in prayer and specifically for lifting Everly's name up for healing. This has been a very difficult time on the whole family. Here's to looking at 2015 with good health for all! Before I forget, please pray for our whole family that we don't get sick because then we would have to stay away and that would be hard, too.

With love and appreciation,

Crystal

Back in PICU

Just a real quick update to let you know that we are back in PICU. I know some of you aren't on Facebook and might not have seen the news about it.

She's been sick since Monday with nasty congestion. Took her to doctor on Wednesday to see what's going on and it sounded like it was all in her upper airway, not lungs. Continued with chest PT and xoponex at home.

But Friday morning she wouldn't wake and when I tried to wake her she was not responsive. Her eyes were open but they were glossy and staring into space. Her numbers looked fine but she did not. Made the decision to call EMS. Upon arrival, they agreed her demeanor was questionable based on what I described as her baseline. So off to St. Joe's we went.

Upon testing, it was determined her co2 level was in the 80's well above the acceptable limit. This retention of gas could be deadly if not addressed. So it manifested itself by causing her heart rate to drop into the 70s then slowly go back up. This, we didn't know at the time was the reason, also happened the night before. But then we thought it was the lead on her pulse ox and so we changed it. Turns out it really was her and not

There's a feeling this retention of gas was what was causing her to be lethargic and have drops in her heart rate. A thought is that the congestion is some virus causing the retention as well.

So, while intubation was tossed around as an option, the decision was made to go from high flow cannula to bipap to allow her time to rest and help her expel the co2.

She's been on it all night now and is doing well with it. Only a couple bradycardia episodes and seemingly holding steady. Waiting for new x rays, doctor rounds and setting adjustments.

I'll touch back base when I'm able and have more information. We would appreciate and thank you for your prayers for Everly. For strength, God's healing hand, peace and calmness in decision making and in this situation.

Thank you to Tiny Superheroes for the cape we have in Everly's PICU room. It came in the mail last week and is the perfect sentiment for her!

With love and appreciation,

Crystal

Everly Medical Update Part 1

Heading into the ER via stretcher

I know many of you know the events of the last few weeks but there are some who aren't on Facebook which is where I update daily. So much has happened this month that I'm going to do a quick timeline for time's sake. We are currently in the Pediatric Intensive Care Unit at St. Joseph's Hospital.

Nov. 10: Everly wasn't feeling or acting great so we took her in to see Dr. Graham. Advised that it was probably a virus and we would watch it during the week.

Nov. 11-12: She acted okay, not great but better than Monday.

Nov. 13: She seemed to be worse so took her back in to Dr. Graham. Pneumonia :-( Shot of Rocephin antibiotic.

Nov. 14: Okay during the day but that evening she began to and she became extremely lethargic with a low-grade temperature. Headed back in to Dr. Graham's office. Double pneumonia. :-( :-( Another shot of Rocephin. Her appearance was concerning to say the least. When asked about Everly's condition, Dr. Graham quietly said that Everly might not come out of this. That jarred us to the core. We went home, spoke as a family and decided we would take her into the emergency room. If you recall, we had always said no hospital and no 911 as well. However, since turning 6 months, we felt that maybe we needed to follow her lead, so we did ultimately decide to take her to the ER. *I did speak to our friend Beth and my best friend's husband to get their take on whether we should proceed with the ER visit.

Nov. 15-18: St. Joe's Hospital Stay

Nov. 18: Released from hospital but Everly's lung was still collapsed and she still had some congestion so we were to continue the chest PT at home.

Nov. 26: Noticeably different with a deep cough, lethargic and marked difference in her appearance. Dr. Bash, the hospice doctor, prescribed breathing treatments and steroids to help clear her up.

Nov. 27 (Thanksgiving): Everly had a rough night the evening before of crying and just being uncomfortable. Early that morning, her heart rate dropped quickly to 50 and remained. Her face was white and her lips cold to the touch. We called 911 and within minutes the EMTs arrived. She was able to recover herself without any intervention from them but it was necessary to still transport her to the ER via ambulance. Once there, she was admitted to the hospital and moved to the Pediatric Cardiac Intensive Care Unit.

Riding in mama's lap to her room in the hospital after admittance

We are currently still at St. Joseph's Hospital with Everly still trying to fully recover. It's been almost one month now since she started acting not herself and exhibiting cold-like symptoms. We are cautiously optimistic for the all clear soon!

In my next, I will address the diagnosis, treatments and plan of action for the future. I really wanted in this one to record the sequence of events, not only for my own preservation, but for anyone who does not follow Facebook, know how we got to where we are now and what is to come.

Thank you for continuing to follow and pray for our Everly! Every prayer is heard by our Heavenly Father, the Great Physician. He knows all things and has plans for us but He also listens to us. If you are so inclined, we would ask that you continue to pray for complete healing of her little body, discernment on our part in decision making and compassion and wise judgement for the physicians.

A visit from the brothers but she is still very sick and sleepy

Please continue to keep in mind our sweet friend Elizabeth Maxine in Texas. She will be 7 months old soon and is also beating the odds in Trisomy 18! Let's lift her name up and and pray for her continued good health and that of her family who embrace and love on her. A special shout out to Aunt Bees who has enough love to cover both sweet babies and more! We love you Scholes Family and Aunt Bees!

Our God has kept us grounded and focused on Him even through this particularly scary time. He is faithful and His love never fails. We are so grateful for His promises and His presence in our lives and especially keeping Everly in His hands.

With love and appreciation,

Crystal

Still very sick after a few days in the hospital

Sleep was all she did the first few days of her stay

Just Read Please

My specially ordered shirt to wear to our first Pediatric Cardiologist visit in November. I was speaking His word over our appointment.

First, let me start by saying how much each of mean to me. Your continued support has, at this point, lasted a bit over a year. Some of you are family. Some of you are personal friends who I’ve known for years. Some of you are “virtual” friends. Some of you are followers who love Everly.

Second, let me also tell you that, though I did not think things could get any harder, they did.

Third, I've thought long and hard about this message I'm sending you and have had it in the queue awaiting publishing since the weekend. I've also spoken with my husband, he has read this and he is fine with this going out.

I have shared all parts of this journey with you and this is an important part. A big part.

I need to make a quick timeline to help refresh your memory of this last year to better understand the latest news.

August 2013. Had Maternity 21 screening which looks for Trisomy 13, 18, and 21. Less than 1 percent risk out of 10,000. No concerns.

October 2013. Atour 20 week ultrasound, choroid plexus cysts were found on her brain. Referred to high risk clinic.

October 2013. High risk OB sees hole in her heart and tells us that she suspects something called Trisomy 18 which we had never heard of before. She tells us that it is a fatal genetic condition. I refuse an amino to confirm the suspicion. They refer us to a fetal cardiologist.

November 2013. At fetal cardiologist's office, he isunable to see any problems or concerns with Everly’s heart or development. He gives the all clear but asks us to come back in a month. We rejoice and celebrate this incredible miracle! Dismisses the suspicion of Trisomy 18.

December 2013 Return visit to fetal cardio’s office and this time he finds a large hole in her heart (VSD). Suspects Double Outlet Right Ventricle. We are shocked but thankful this is repairable and not fatal. Still no additional concern of any genetic anomalies.

January 2014 Return visit to fetal cardio’s office and this time he tells us that he also suspects coarctation of the aorta in addition to the other congenital heart defects. Weare crushed but still again feeling glad that it is repairable and not fatal. Still no concern of any genetic anomalies.

October 2013 – February 2014 Continue to also be seen by OB doctors twice weekly, one visit for biophysical (ultrasound) and one for non-stress test. No mention or concern still at this point of genetic anomalies, even though my mom and I had done research and knew to ask about clenched hands as markers.

At birth, there was suspicion of some chromosomal abnormality and a FISH test was ordered. Results came back on 2/25 that she was Full Trisomy 18.

Fast forward to October 6, 2014. We went in to the geneticist for the first time since birth so we could help them to see the “other” side of Trisomy18. While there, we found out that her original test at birth stated that she was borderline Full Trisomy. Basically what that means is that she was close to being considered Mosaic Trisomy 18, which has a much longer and better-fairing prognosis. Mosaicism occurs when the extra 18th chromosome is only found on some cells inthe body, not in all like in Full Trisomy. Many children with Mosaicism can walk, sit up, feed themselves, eat bymouth and SO much more. But the biggest thing is their life expectancy is much, much longer and can be into the 30’sand 40’s. In addition, the medical community typically offers those children surgical interventions like heart repairs which drastically improves their quality of life as well as their lifespan.

Jimmy and I at the baby event days after her "clear heart" report in early November.

In hearing her borderline status, which was news to us, we requested her to be retested and so she was. I didn’t think too much about it honestly because at birth they said it was close enough to be considered full (though they hadn’t told us this information). About two weeks later, I received a phone call from the counselor that stated Everly’s test came back that she was indeed MOSAIC Trisomy 18!!!! I was in shock and utterly speechless. We decided to schedule an appointment with the geneticist to get more details and more about what this diagnosis this means from a genetics standpoint before sharing with everyone. In the meantime, I was on cloud nine knowing how this would change her life and ours drastically! I would not lose my daughter so soon, she could live life with us. I felt the blessing of God and that He had answered our prayers from so long ago. Though she wouldn’t be whole, she would be here for a much longer time! What a cause to celebrate!

In the meantime, we decided right away that we needed to get her G tube surgery with this new info. So we met with GI who then ordered the Upper GI which found her malrotation of the stomach. We also told our cardio at our appointment last Monday (November 3) and she was completely caught off guard by the new diagnosis. She then said she would need to think about it but given the new prognosis, heart repair or banding could possibly be a consideration!!! I was jubilant!

Her first smile at about 33 weeks.

That follow up appointment with the geneticist was also last Monday (November3) in the afternoon. My intent for going was to try to get some information about Mosaicism. However, my husband had a doubt about the validity of this new test because Everly had mouthcare (breastmilk) about two hours before. The test was a cheek swab. He was concerned the test picked up some of my DNA instead tainting the test. So, he requested that this test be redone. They redid the test then—she had mouthcare again with breastmilk about 5 hours prior this time as I was not anticipating having her retested. Results were due in on Friday, November 7.

As you can surmise, I was devasted, distraught being a better word, at the thought of retesting. However, I had faith that God would show up in this new test as well. I knew my husband, coming from an atheist viewpoint, could not just believe that the result was what God had wanted. He needed to question it. I also knew that that I was to honor my husband and this included going through with the retesting.

Baby Everly at 20 weeks. Love this one.

Last week was almost unbearable. I finally had a breakdown last Tuesday. It all came crashing down at once and it was just too much. The days couldn’t pass quick enough. My heart was racing, my prayers more determined. On top of the waiting, his sister and his brother-in-law came into town on Thursday so I needed to put on a happy face. Though I was looking forward to their visit, I just was having a hard time coping with it all. The year seemed to come to ahead this exact week.

Then Friday morning came.

I knew when the caller-ID said “Caller Blocked” who itwas. I also knew the minute she spokethe first word what the news would be. I can’t quite remember what she said but I do recall hearing “this rollercoaster” and “Full Trisomy” somewhere in the call. It was like February 25 all over again. The same darkness reappeared. The same abyss. I couldn’t speak. And I couldn’t understand it in my human, earthly existence. Why, God? It didn’t make any sense to me at all. Jimmy was beside me and I had the phone on speaker and I just handed him the receiver and walked away. My baby, who just got a second lease on life, would not be with me like promised just four short weeks ago.

I just did not understand.

This “roller coaster” the counselor spoke of was a ride that I didn’t seem to be able to stop, let alone get off of at all. It’s like the cruelest of jokes. From way back last year with our first Maternity 21 test all the way to that moment.

It was all a cruel joke.

Our first of many circles for Everly outside our church.

I had to make the call on Monday to tell the surgeon with whom we had met the Thursday prior (remember that scare? Yes, that was the very same week as all ofthis!) of the new results. I received a phone call message later that day from him stating that “given the new Full Trisomy diagnosis the benefits of repairing her malrotated intestines would not outweigh the risks of surgery seeing that the prognosis in Trisomy 18 children is that they live less than 2 years.” **Please know that I still think he is a great surgeon from all I have heard and I honestly believe that this sentiment is not exclusive to him alone. Many use what they've been taught, statistics, neonatologist and geneticist recommendations, etc.**

Another cruel joke.

My daughter was offered this same repair not days before…what was so different about her now? She still has malrotation of her intestines and complications of which could turn into an emergency at some point just as cautioned us at the appointment.

Forget about repairing it and heart repair…ha!

Now, of course, I'm not going to just leave these issues like this and I have messages waiting to be returned and doctors to now visit, but I want you to know what it is like to have a child considered "not worthy" (my words, yes) of an operation, a repair, an intervention that would be offered to ANY other child without Trisomy 18. I now have to fight.

I’m defeated. I’m not going to lie.

I pray this feeling is temporary and that I will just pick myself right back up, dust myself off and get moving. I’m really starting to get Job and how he must have felt to some small extent. Setback after setback and still you must move. In the face of severe adversity,you must seek God because without Him, there is no hope.

I know all of this in my head, but my heart is in about a million pieces again.

Like I said, I will pick myself up but right now…

I. Am. Just. Here.

It all boils down to the fact that I will still praise Him while this storm rages. I know the blessings that He has provided and that He will continue to provide. I know that I will continue to ask for healing in her body and that I would ask for intercessory prayer for her. I have my arms around my little miracle whoI’ve been immeasurably blessed to hold for going on 9 months. I can’t discount the hugeness of this fact alone. We are SO blessed that she is still with us this many months later. I have two boys who believe in the power of our God and who in the face of what our family endures daily still hold Him in reverence. Our family has an amazing support system surrounding us that has kept us fed, uplifted and overall just wrapped in love. For that, we are blessed beyond measure.

I know in my broken and hurting heart that we will overcome this hurdle (though that word seems so insignificant in light of theenormity of this situation) but it’s going to take some time. This year has taken its toll and this pastweek was most certainly a pivotal point for me.

Your prayers, messages, texts, calls and emails are so treasured by me and I thank you for those over the past year.

This whole “prolonged grief” combined with the incredible joy of being Everly’s mom mixed together is an emotional and sometimes difficult journey.

I know it’s been hard with lots of twists and turns.

I know that it still continues.

I thank you for going alongside us.

With much love and appreciation,

Crystal

Medical Update and Urgent Prayer Request

This past Monday, Everly wasn't acting herself and was quite congested in her throat and chest. After hearing that she'd had a rough night, Nana convinced me to take her in to see Dr. Graham, her pediatrician. So we did.

Everly was found to have just a slight possibly viral infection that we hoped would run its course. No antibiotics yet just keep a close eye and let her rest.

She seemed to perk up on Tuesday and Wednesday and then Wednesday night have a miserable time trying to rest. She whimpered and was hoarse most of the evening and couldn't get comfortable no matter where I placed her. So put a call in to the doctor Thursday morning.

Dr. Graham advised us to head back in at 4:45 if she didn't improve throughout the day. Indeed, she did not. We spent the day cradling Everly and trying to make her as comfortable as we could. However, she continuously coughed and sneezed all day which would cause her some considerable discomfort. We ran a humidifier wherever she was and gave her nothing but rest.

The most alarming thing for me was the color change I witnessed throughout the day. Her fingertips and forehead were bluish/dusky...a color you do NOT want to see on your child. EVER. I felt that she definitely was lacking oxygen, probably because she was working so hard, and put her back on the pulse ox machine to get a read. My instincts and vision were correct because she was Sating in the mid 70s. Not good. Flipped on the O2 and gave her some blow by air. Her numbers really never rose past 85 when normally they range 94-96 with no air.

Once in the office, Dr. Graham quickly found that Everly had the crackly gunk in her right lung indicative of pneumonia. Ugh. Not what we expected to hear at all. Thought things had gotten worse but not that bad.

She decided that an injection there of Rocephin would be best and then also one the next day. It was a considerably thick medicine so it stung going in Everly's leg. She also would need continuous O2 while she fights off the yuck that was trying to bring her down.

We pray for a quiet night for her with much rest and peaceful sleep so she can have the energy to fight this. Praying for her doctors to make wise choices concerning her care and treatment. Praying for the pneumonia to clear up without any further incident. We pray that we will not have to take her to the ER or the hospital that she can handle this fight here at the house safe from more stuff aiming for her.

We thank you and would appreciate you joining us in prayer.

With love and appreciation,

Crystal

Everly's Bucket List

From the moment we were told the devastating news of Everly's diagnosis of Trisomy 18, we knew we wanted her to experience "life." Our goal from that very day was to get her out of the confines of the hospital and home with her family.

While we waited for all the necessary requirements before being able to leave, we began to think about all that we would not get to do with her. The thoughts were overwhelming. Debilitating. Depressing.

As the days went by, however, our mindset changed to suddenly what things we wanted her to experience and not what she could not do. We knew we could give her a taste of our life...the one she was born into and we wanted her to experience as much of it as we could. Everly's Bucket List was born.

See, we have been lovers of the "bucket list" for a long time now. For us, it's something to look forward to, work towards, embrace with joy and aspire to complete. It is not nor has ever been synonymous with death or anything of the like. We had a bucket list, and still do, for our marriage. For example, we'd like to hike the Grand Canyon together. We have a bucket list for what we would like the boys to experience or be taught before they leave home. We have a family bucket list of experiences we would like to complete together, like getting scuba certified. So, while we may be loosening the constraints of the title, bucket list, we have used and loved them for years. Several years ago we found tiny silver Dollar Store buckets that we have put slips of paper filled with the list items that we use to actually symbolize the proverbial bucket.

Now, I know what some of you about right now are thinking...how can a newborn or infant have a bucket list? Am I right?

Well, it's simply like this: part of Everly's Bucket List is the experience she would have doing various activities, like feeling the sunshine on her face or hearing the sound of the waves. But other parts of her list, like being in the midst of her brother's nerf fight or hiking on the AT, is important to us because it's a important to someone in our family. And in those cases, we get joy from having those moments with her.

At the end of the day, this Bucket List is about creating memories of our life with Everly. It's joyful. It's life-affirming. It's anything but sad.

When we left the hospital we had a very short list, maybe 6 things. One at a time, we checked them off the list. Feel the sunshine. Check. Go for a walk. Check.

Until those things were all completed.

We added a few more things. As it was now Easter, we added some new Bucket List items like get an Easter basket and go to church. Check and check.

And so it continues to go.

We discuss as a family what will be placed on her list and then we proceed with checking them off.

I still remember the giddiness I had late summer when I realized I was actually thinking about her Halloween costume, then Thanksgiving and unbelievably, Christmas!

So, more list items were added for each of those holidays and seasons. I never thought in a million years based on what the doctors predicted (congestive heart failure at 2-3 months with no option of a heart repair due to her diagnosis) that I would be looking ahead to these holidays!

So, Everly's Bucket List continues to evolve. And for that we are grateful.

Everly's Bucket List: Completed Items

Ride in the van to the house

Feeling sunshine on her face

Walking neighborhood

Go on a bike ride

Walk on the beach

Get an Easter basket

Go to Adventure Island

Hike Lake Rogers

Lay in each boys bed (they added this one)

Go Nana & Papa’s house

Look at a Mylar balloon (Daddy's wish)

Participate in boys nerf war

Taste whipped cream

Daddy/daughterdance (at her party)

Go“swimming” (in her bathtub at the pool with our water)

Go the mountains

Hike the Appalachian Trail

Hear rushing of a mountain stream

Have her nails painted

Play dress up (check, check AND check!!)

See Halloween decorations at our house

Go to family’s favorite restaurant

Attend church

Go to the zoo

Visit beach and dip toes in sand & water

Have a birthday party

Listen & watch the rain

Ride a ferris wheel

See fall colors

Everly's Bucket List: Not Yet Completed

Visit Atlantic Ocean

Go camping (may have to cabin camp or camp on our back patio this time) (Camping has always been a family favorite and one we do monthly between Oct-Mar.)

Have brother drive van with us in it (our newest driver!)

Go for a train ride

Have a tea party

Celebrate all family members birthdays (only two left on Nov.8 & Nov. 10!!)

Celebrate1st birthday!!!!

Ring in the New Year

Taste Nana’s Thanksgiving turkey gravy

Watch Christmas movies

Watch Macy’s Day Parade (It officially begins the holiday season in our house. No football here. Just the parade in all its glory! We are always glued to the TV.)

Pick a Christmas tree together (We buy real and it’s a very big deal when we go pick it out…we call Lowe’s days ahead and arrive when the truck from NC is due to pull in. One year, we were there when the doors opened and SNOW was still on some of the trees! So, it’s AN event to get our tree.)

Put up Christmas Decorations

Go see Christmas lights

See snow (maybe a white Christmas)

Go golfing or at least ride in the cart (for Papa)

Go to Disney World and be where everything is wonderful!

Have a meal with the princesses at Cinderella’s Royal Table at Disney (Very cliché but Disney IS the happiest place on earth.

**List subject to change as needed :-)

With love and appreciation,

Crystal, Jimmy, Garren, Kendan, Everly and Ala

Not So Organized

Bundled up for the cold. Heading out to hike but was completey snug in Mommy's Moby wrap.
  
"Sometimes our lives are so organized that God can't get into them at all." Joyce Meyers

I love this quote by Joyce Meyers because it has my name all over it. I am a proud, card-carrying member of this club. I love, thrive and enjoy things--all things--laid out all nice and neat. Predictable, you could say.Needless to say, my life as of late is as far from organized as one could probably be. But you know what?

God is MORE present now in my disorganized, jumbled up life than ever before! I see Him working in ways that I most surely would have missed before when everything was "just so." Let me tell you about our weekend.

On a bit of a whim, we decided to take a whirlwind trip this weekend to Atlanta to check "seeing fall colors" off of Everly's Bucket List. We planned to cram a lot into a short amount of time. God really showed His goodness and grace while we were there, let me tell you! I'm so glad He's been--er, shall I say, helping me over the last 8 months--to be less organized and more ready for a moment when it happens.
 

Our surprise by the Coca-Cola Family

Starting with our hike. It just so happens that our ranger, Don, who took us on a guided hike was a God-fearing man who appreciates our beliefs and faith. He understood our purpose for coming and made the experience top notch, telling us all the fascinating details of the history of this Civil War park that homeschoolers just devour. Don taught us about the many varieties of trees on property and showed us how to identify leaves, nuts and vines. We learned so much and were thankful to be able to share our praise with what the Lord has been doing in Everly's life with him at the same time. Cool fact: Scenes from the new Hunger Games movie (Mockingjay) were filmed here. Didn't know that before we arrived. A HUGE shout out and THANK YOU to park ranger Kelly at Sweetwater Creek State Park in Lithia Springs, GA for making our tour with Don a reality!

In the true fashion of a homeschool family on a vacation, even a mini one, we made the most of the short time we had and went to the World of Coca-Cola for what we were hoping would be a great lesson in business. What. An. Experience. For real. The lessons learned in this one visit reached far beyond the pages of a school text.

While we wanted to take the boys on this tour, it was still an indoor activity in the midst of cold and flu season. Our plan was to take them on a small tour so that we could bypass as much of the crowd as we could, thus keeping Everly as safe as we could. Because God is watching out for His children, He provided in a way that only He could have. The entire Coca-Cola staff must have "known" about Everly because we were given star treatment, including a stellar tour guide, Steve, to weave us in and out of crowds, bypassing nearly everyone who could have breathed on her. Staff members would randomly stop and say hello and ask how Everly was, using her name, as if we were old family friends. We felt so welcomed and safe there. Then towards the end of our tour, the Coca-Cola bear and some of his buddies found us at a display with Steve and came bearing (yes, pun intended!) a gift for the family. The boys loved the tour as much if not more than we did, not only because of all the cool stuff there, including a plant line AND a tasting room with 100 different Coca-Cola products, but because for one three hour period, one afternoon, one part of a day, we just were a family of five touring an attraction. Thanks Steve and the Coca-Cola family for giving our family a day of memories and a wonderful visit!

God moments just appeared in SO many ways for us on this trip! I'm so thankful and grateful to him for giving me the EYES I needed to SEE what He wanted us to experience.

THE Carl Azuz and for us, a true treat to meet!

As we made our way back to Tampa on this quick trip, we decided to swing by quickly to see the CNN Center where our favorite show of 6 years is filmed, CNN Student News. As we entered the atrium area, we found a spot to wait while Jimmy ran back out to the van to grab something. I happened to glance to my left and I see Carl Azuz, the Student News anchor, writer and pun-writer extraordinaire, walking by. My knee jerk reaction was to call out his name and at that, he turned, smiled and walked over to our table. He chatted with us for a good bit, took a few photos with the boys and graciously ignored my over-exuberant excitement at meeting him! What a treat it was for us just by "happenstance" (insert God's doing here) that the ONE person we hoped to see walked right by us! And, please, let me tell you that he was so incredibly amenable to visiting with us, answering questions and was not in any apparent hurry to move along. Carl Azuz...what a way to end our trip! Thank you!

See, while some may consider all this "good luck" or something of the sort, I know that God knew just what our family needed right then. We had just gotten the news about the malrotation of Everly's stomach and a 5 day minimum hospital stay to have surgery for it and the g tube. Of course, this was quite emotional to think about. Cue music. Just like God did for us one year prior, a quick trip to completely take our minds off the fresh news was in the works. He threw in all sorts of little God winks (the faithful ranger, the special treatment at Coca-Cola, and the surprise sighting of Carl Azuz) to top it all off! You have to understand that we've had emotional highs and lows for the past year so these moments like the trip, big ones like Go Shout Love this month and the everyday ones that come in the form of texts, calls and prayers ARE what helps our family keep putting one foot in front of the other.
 

Hiking by the river on a crisp fall morning.

May God continue to bless Everly and our family and those who love her. That we will continue to have experiences such as those from this past weekend. Traveling and doing "typical" vacation activities as a family of five is not lost on us. We are grateful for the opportunity and will continue to work to make those things happen as long as we are able.

With love and appreciation,

Crystal

GoShoutLove!!

 

My beautiful girl...inside AND out! Oh, how I love her

 

Literally just PUMPED with EXCITEMENT!!!

GoShoutLove is an organization that works to help raise funds, awareness, and just shout LOVE for families who have a child with a rare illness. Each month a different family is selected and the whole month is spent showering the child and family with love!

Guess who was chosen for November?? YES! Our very own EVERLY!!

Loving a child with a rare illness is EASY. Dealing with some of the facets of what it entails is not and even more the gravity of the diagnosis. So, it means the world for those of us who do live this day in and day out to be showered with love and to be given the assistance that will allow us to just focus on making every moment count. We are touched, honored and appreciative of this truly special opportunity.

Go check it out using any of the following:

Instagram: @goshoutlove
Web: www.goshoutlove.com
Love For Everly’s FB Page

Friends and Family, can you please share this post to get the word out?

This is such a worthy organization! The families they support are incredibly blessed by their involvement. Saying thank you is just NOT enough but we DO thank you!

We would LOVE to see everyone sporting a new shirt for Everly! Go get yours today. Thank you :-)

*Thanks to Jaimee Ponce Photography for the photo!

To God Be the Glory

 

Boy, oh, boy! God is good and God's timing IS perfect!! Oh, I praise you!

So, yesterday was Everly's upper GI procedure. It went fine and she actually enjoyed it as you see from the pictures.

Received a phone call within ...a couple of hours from her GI. Everly does indeed have malrotation of her stomach. Since it's fresh, I haven't had much of a chance to research it.

But I do know this much: if not caught and allowed to stay unrepaired, the bowel can stop functioning and intestinal tissue can die from lack of blood supply if not treated..meaning potentially life-threatening.

As you all know, we've been on the fence about getting a g tube. However, with her increased reflux/choking, we felt like we must see the doctor to discuss it. Once decided, she sent us for this upper GI procedure that ultimately found this problem.

Was that luck, fate, chance?

No way!  That was our loving God orchestrating this sequence of events...in the right time, how He ordained it to be.

I am 110% on board with having the surgery for her as I know Jehovah Rapha (our Great Physician) planned it so.

*As for the tube, it will have to be placed surgically and will most probably be down alongside the repair she will need. Looking at about a week but surgeon and her recovery will better determine that.

FYI: that would be dr appointment #4 for next week. Pray I can take good notes to share the info with hubby but mom's going with me to all again like she did yesterday.  

Please be encouraged that God plans things for good. While we can't explain the "bad" in the world, we can be sure that God will work it for our good as difficult as certain situations appear (ie. Everly's Trisomy 18 diagnosis for example).

Finding out my daughter now has to have a surgery for something completely different isn't good to me. Finding out that God is saving her life from an unseen danger strengthens my faith.

Okay, sermon complete but I HAD to share this!

We will be meeting with the surgeon next Thursday to discuss plans to repair her malrotated stomach, how it's affecting her now and moving forward with G-tube insertion.  I'll update more when we know more.  Cardio appointment on Monday as well and occupational therapy starting for Everly on Tuesday so a very busy week coming up! 

We thank you for your continued prayers.  They really mean so much and your messages to us are read by each member of our family and just taken to heart.  Thank you!

With love and appreciation,

Crystal

It's Time...

Just love this one. One of the 9,873 times she's pulled out the tube. lol

It's time.

It's finally time to make a change to a more permanent feeding solution. As much as I'd like to admit it's not necessary, it truly is.

She has figured out the ever so delicate skill of removing her NG tube in the blink of an eye. With each and every reinsertion we run the risk of nasal ulceration, laryngeal injury, and pharyngeal discomfort. The NG tube may also precipitate gastroesophageal reflux, which can lead to aspiration; a major concern for us with Everly as it will turn into pneumonia.

As we continue to work on her oral motor skills and speech therapy, her swallowing of milk via mouth and solids are most assuredly negatively impacted by the presence of the NG tube. It is thought that she will react more positively to oral stimulation once this noxious piece of equipment is removed once and for all.

In meeting with her GI doctor yesterday, it was mutually decided that removing the NG and moving forward with the PEG (percutaneous endoscopic gastrostomy) is the absolute best option for Everly.

For any other "typical" child or even adult who is in need of a feeding tube (non-NG), the procedure is in and out really. However, in Everly's case, it's not as cut and dry.

The biggest concern was, is and will be for us how she handles the intubation and extubation. Again, "typical" children with "typical" anatomy, breathing patterns, brain connections will handle breathing on their own again just fine. She, however, may do well but there is a risk that she may not. As much as I DO NOT want to take this risk, we know we are also taking a risk by keeping the NG tube. It's not meant to be a long term solution.

Love. Her. My goodness!

When we first spoke about getting a more permanent tube, we really thought she would not make it, following the experience of our doctors at the time, but a short amount of time. However, at this point, we need to respect how well her body has done, God's obvious plan for her life and the advice of our trusted medical professionals. We have spent 8 months researching this topic, time discussing the procedure and plenty of time praying for God's guidance in making the final decision. We are at peace with the decision.

So, to briefly explain how this will work, what we know and what we don't know. I won't bore you with too many medical terms that will just go through one ear and out the other unless you're personally invested in this topic. Tomorrow is Step 2: have an Upper GI completed which consists of x-rays of the pharynx, esophagus, stomach, and upper small intestine. The series shows how your digestive tract is functioning and is used to diagnose ulcers, tumors, hiatal hernias, reflux disorders, and many other conditions of the upper digestive tract. Once completed, the radiologist will write her report of the findings and send it off to the GI doctor.

Step 3 is to meet with our cardiologist since she is a heart patient. They have their own team of anesthesiologists that the cardiologists prefer to use from what we were told. So in meeting with our cardio, we can address our concerns related to putting her under anesthesia and how it will affect her heart.

Step 4 is waiting to hear back from the GI who will have read the upper GI findings AND consulted with our cardiologist about the best plan of action for her.

Step 5 is scheduling us to come in for pre-op (not day of or even near) to go over what the upper GI showed her. If it showed her that Everly's stomach is malrotated then doing full surgery to place a low profile button will be the choice. That is a week stay and is much more of a major procedure just in that it is not endoscopically. We will also meet with the pre-op anesthesiologist and he/she will discuss how the procedure will go specifically the protocol for Every. If there is no malrotation present or any other causes for concern, the procedure can be done endoscopically which is less of a hospital stay.

Love my three kids' costumes!

The biggest question will be what kind of G tube can they place in Everly's stomach. There are a few main types. The low-profile would be placed if she must have the procedure surgically. While the longer tube selection would be inserted through her mouth and into her stomach endoscopically. Our hope is that the surgeon can bypass placing the longer tube choice as we would have to take her in again when she weighed 20 pounds to have it changed to a low profile one. If her anatomy is correct and all is well when he gets in there now, he may can just proceed surgically mid-procedure and place the low profile tube which we are hoping for. This means that it would be all done and over in one procedure with only 2 additional hospital days as opposed to coming back next year (if that's when she hits 20 lbs) and going through this again.

Step 6 is to schedule the procedure while the GI and our cardiologist work behind the scenes to find the right people to work in Everly's situation.

Step 7 is surgery day when we meet with the actual anesthesiologist who will be in the operating room and the GI doctor. We will be allowed to hold her following and also to stay with her overnight (love to see them tell me NO...lol...#justkiddingnotkidding) for the time she's healing.

Talk about dressed up!

There are many questions still left to work out based on what they find in the Upper GI tomorrow and even when they have her the day of surgery. For me, not being able to have it all planned to a T and know each step in full detail...unsettling to say the least. I know God has, is and will be watching over her and helping guide the surgeons as they work on her. Faith can take you far, that's for sure!

So for prayer requests: that her upper GI finds nothing out of the ordinary, they are able to complete surgery with no complications and that she is extubated easily, and that she is able to have the low profile G tube placed at this time.

Sleeping beauty

A shout out to the Scholes family in Texas who recently celebrated Elisabeth Maxine's 6 month birthday! She's moving just like Everly...up, up. up! We are in awe of how well she's doing, too. I watch the strength of her family, including their Aunt Bees (shout out to you!!!) who is ever present, and am just amazed at them and the joy that they are living. Their E also has Trisomy 18 and is moving mountains! She's also on Caring Bridge so check her out if you'd like to see the cutie!

Joshua 1:9

"Haven't I commanded you? Strength! Courage! Don't be timid; don't get discouraged. God, your God, is with you every step you take."

With love and appreciation,

Crystal (will keep you updated about this)