God's Timing: BIG NEWS

"Lean on, trust, and be confident in Me with all your heart and mind."

This was the message in my devotion this morning. I stand amazed at how just when you need something the most, God is one step ahead of you putting everything into place. Just like this message I needed to see today, he perfectly orchestrated the events that have led up to this post.

We have known all of Everly's life that her pulmonary hypertension was balanced...

...until last week.

Whether the change occurred due to her ongoing fight with rhino then with the bacterial infection, her new medicines or even her age, the fact is that her balance has changed.

And with that change comes ramifications for Evelry's body and quality of life. We have already begun to see the changes: excessive sleepiness, irritability, excessive sweating, lack of energy, color changes, lack of interest in playing and interacting, basic personality changes.

Even when we returned home, she continued to exhibit these new behaviors, or rather lack of in some instances. It is unsettling for us to watch her now. Her bursts of personality are so fleeting now that we knew that action needed to be taken.

So...

Because her biggest issue is the Pulmonary Hypertension, we decided to contact the team at the Pulmonary Hypertension Clinic at the Children's Hospital at Eggleston in Atlanta. They were able to get us into their weekly clinic at the crack of dawn Wednesday. So, myself, my husband and my mom packed up Everly and ALL of her machines, meds, and assorted other accessories which may or may not have included a few headbands and left for GA at 5:00 p.m. Tuesday evening. We arrived at 2 a.m. to the hotel got her set up and settled in by 4 a.m. and up by 6 a.m. to go to the appointment.

We were in clinic all day Wednesday, complete with echo, EKG, blood work, x-ray, and a collaborative meeting with the PH team. We expected, honestly, for them to say the plan of action in Tampa is exactly what they would do; however, that wasn't quite how it played out. The cardiologist recommended at the end of our first meeting that she be admitted into the hospital right then for a heart cath today or tomorrow. To say we were shocked would be an understatement. We said we needed to stop and think about this given all the risks involved but also all this could mean for her. This is a big procedure for her and she would need to be intubated. Of course, the fear is being extubated or even worse during the procedure.

We took a break for lunch and to discuss everything before we committed. We had some pastoral support from our Pastor's wife at that time and just had a family huddle including input from my dad back in Tampa. It was decided and agreed upon by each of us that this was a God-given opportunity, one that we had prayed for and one that we knew was appointed by God. With the information gleaned from the heart cath, the surgeon can determine if closing her VSD would be possible. They are very concerned that the window of opportunity has been missed (typically 4-6 month range) but are optimistic to move forward to see. We have been very encouraged by the aggressiveness of the team and the care they have taken to put everything into place for us. They have spoken to us about an aggressive treatment plan, while scary, that should ensure Everly would be the best surgical candidate she could be in preparation for open heart surgery.

We met with them again in the afternoon and we agreed that we would admit this morning rather than last night because we really wanted to spend a quiet night with Everly. So, today we have been waiting for the "the call" that they have a bed ready for her. This place is packed to capacity! No open beds, rooms or the like here at all.

Part of me is scared because we are not with our "home team" but I know that if we don't follow through with this plan today, right now, we might not get this chance again for her.

I know that we are doing the right thing by her and that this all fell into place literally within 36 hours with no effort on my behalf other than getting her here. In that and in all the arrangements just "falling" into place, we know that today, at this time, it is God's timing. We will be obedient to Him and cast our fears which are MANY onto Him as well. Without our faith and our complete and utter trust in Him, we have nothing outside of empty hope.

At the VERY moment we received the phone call saying there was a bed available and to head over, the PH team nurse called and said that they were able to accommodate getting Everly onto the already full cath schedule for tomorrow. God's timing at its best!

So, friends and family, we ask that you go to bended knee for Everly and for us as we go through this together. Pray for positive results and for successful extubation quickly. Pray that the staff, doctors and her team will be wise in their decisions. Pray that she remains healthy while in the hospital. Pray that she is comfortable and is surrounded by love at al times. Pray for her please.

I have so much more to write and may later but for now we have to take Everly over and admit her. Such a hard thing to do.

With love and appreciation,

Crystal and Family

P.S. We, as everyone knows, were thrown another curveball last Friday with the Lasix overdose and subsequent overnight hospitalization. I don't plan to do an entire update on just this right now because of my lack of time, so I'll do the best I can now. The update since then: I went to the pharmacy Monday not knowing they were already aware because of the cardio's call to them Friday. They had filed an internal incident report and told me that a district pharmacy manager would contact me. On Monday afternoon, the actual pharmacist responsible phoned me and apologized and asked how Everly was doing. Today (Thursday) the district pharmacy manager did call me and told me that someone would follow up with me. So, that's where we are with that.

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

Just the Facts

 Dr. Graham speaking sweetly to Everly who is all ears

It feels like so much--and it has--has changed in the past month. I thought I would take this journal entry to update everyone on where Everly is today.


1. STATS


62 days old 6 lbs 9 oz 19 inches long 50 ml of milk (with extra fortification) every 3 hrs around the clock


2. MEDICAL


GI: They are taking care of maintaining her feedings, extra calories and overall nutrition. Right now we are trying to help her become more "regular" so her tummy doesn't hurt. We are also waiting for the go ahead to have the G Tube placed in her abdomen for feeding in place of the NG tube (nasal tube). Not happy about the procedure just because it is a procedure but it is the best decision for her based on her GI and Pediatrician's recommendation and we agree. Many factors played a role in our decision to do it but a few of them include: reduced risk of milk getting into her lungs, reduction (hopefully) in her reflux which causes desat's and aspiration, increased ability to learn to oral feed with nasal tube removed. Those are only 3 reasons but tremendously important ones.


PEDIATRICIAN: We have a new one! We are thrilled to now be seeing one consistent doctor who adores Everly! I must give a shout out to her, Dr. Graham at Healthpoint Medical in Westchase, because she was literally an answer to a prayer. As of late, my prayers have been for clarity in decision making and discernment and compassion on the part of the doctors caring for Everly. And just like that, in a true moment of clarity, it occurred to me that I should contact Dr. Graham who I had met prenatally in January. At that time, I thought we were only dealing with Congenital Heart defects and she agreed to see her even still. But with our new diagnosis, I was not sure so I put a call to her office and within a couple of hours she (yes, the dr!!) actually called me personally. After explaining our situation, she told me that she had cared for babies up north with T18 prior and that she "would love to care for our Everly." Oh, what I would have given to record that statement! This past Monday was our first visit with her and our sweet pea received her first head to toe full and complete newborn check up. She finally was able to receive her vaccinations as well as get lots (and I do mean lots) of questions answered. She was in no rush to move us along and allowed us ample time to ask questions and fully answered them. The best part (really!) was that at the end of the exam she gave me and Everly a collective hug and whispered in my ear that "she is glad to be on this journey with us." I'm not kidding! God really orchestrated this one perfectly because she was exactly what the doctor ordered (pun intended)!

 

Her 2nd month birthday dress and her Easter dress as they were the same day

CARDIO: Everly's last monthly Echo and EKG were both "good" according to the doctor. She is due for another repeat test of both May 12th. We are currently in prayer for either the doctor to open her mind and her heart in caring for Everly or for another cardio group who will. We invite you to be in prayer for this as well.


PULMONOLOGIST: Left the St. Joe's area for this one and headed across the Bay on a recommendation to see Dr. Kriesman at All Children's Hospital. Good call! Not too much at the moment that he can do for her, but he did recommend a sleep study for her to determine if she has central apnea alone or obstructive apnea in addition. We would also learn if she's holding CO2 in as well (remember that time I picked her up limp shortly after we got home...we suspect that to be the culprit). So, we'll see about this study.

 

Nana with her little baby girl

THERAPY: The best part of the week! Everly began speech therapy (aka feed therapy in babies) last week at All Children's Outpatient in Palm Harbor. Love, love! Ms. Kay is our therapist and works so well with Everly. We are just beginning so we have a bit of a road ahead of us but keep in mind that Everly doesn't take any milk by mouth yet. She only has taken literally a few drops over the past couple of weeks. The reason it's important for us to do this is so that when the time comes to introduce any baby food, she will have already experienced the sensation of swallowing "food" and the varying tastes, temperatures and textures to some extent. Without that, introducing anything else will be more challenging. At the same complex, Everly also received a physical therapy evaluation last week. However, she did so well that the evaluation turned into an impromptu therapy session. Go girl! That was so fun to watch! I wish the family could have been there because I learned so much during that hour. So, she will now be receiving speech therapy twice a week for 1/2 hour and physical therapy once a week for an hour. Those will be fun appointments!

 

It's hard work being that cute!

In addition, we were notified yesterday that we have been assigned an Interventionist Therapist to work with Everly. As it turns out, she lives in our neighborhood and is a pediatric RN. The best part of the whole thing is that she's been following Everly's updates and progress and praying for her already prior to her being "assigned" Everly as a patient. God knows what He's doing, doesn't he?


ODDS & ENDS: We continue to be advised by each of her doctors to keep her from enclosed places or large close gatherings. As hard as that continues to be, we do so knowing it's in the best interest of Everly. A simple cold her for her can turn serious quickly. Everly herself is so much fun to be with everyday. She has the cutest facial expressions and while she can't speak, her face can!She has a pretty consistent daily routine that I can tell she recognizes. Ala (our dog) continues to help me mother Everly even killing a bird and bringing it to the foot of the crib on day. Spent her 2nd birthday at Philipe Park in Safety Harbor with family. Celebrated with a cake and a gift. As for the rest of the family, I'll give you a bit of an update on us as well.


SPIRITUAL: The boys have been able to attend church on Sundays thanks to my parents coming by and picking them up. I am able to watch the same service live online from the safety of the house with the baby. I've also DVR'd a gazillion Joyce Meyers Ministry and Joel Osteen episodes so I catch one of those an evening or two a week while feeding Everly (takes 45 min every 3 hours).


PHYSICAL: Well, since our YMCA days are on hold, I knew we needed some movement. I have heard about the power of P90X for years now and decided it would probably be the best thing for us. So after a bit of research settled on P90X3 and figured we all could get something out of it. I knew it would be hard (only to find out after we started that hard is putting it mildly) but also knew there would be modification options on screen. We are now on Day 7 and we love it! It's such a great physical release not to mention a morale booster. Even Garren is excited and I no longer have to prod him to get dressed for a workout. We continue to do our nightly walks with sweet pea and our 2.2 mile hikes nearby but those don't count for our exercise really.


SOCIAL: The boys have both been fortunate to have opportunities most weeks to fellowship with friends both at our homeschool co-op and on outings. Garren even had his two closest friends spend the night recently for his birthday. I have also just begun feeling able to entertain the thought of getting together with friends (with Everly in tow, of course). Thankful for friends who have been patient with me over the past 8 weeks. It's been nice to get spend time away from the house, outside and with the boys and Everly AND with friends! It's a win-win all the way around. Feel free to get a hold of me if you'd like to get together :-) Jimmy isn't as social as the boys and I are so he's content to have free time at home while I'm out visiting with the kiddos. I guess it's a win-win for him, too. :-)


SCHOOL: All I can say is thank God I had all my lesson plans complete prior to giving birth! That has made everything SO much easier and I'm not sure we would be doing school had I not done that ahead of time. I planned out Garren's year last July and Kendan's shortly after that. Then spent the summer and last fall copying and filing lapbooks, papers and units for the school year. So, I'm glad to say that some progress has been made in the past 8 weeks. However, we will be spending this summer catching up on a few things. With all her appointments, the mere worry of time the first 4 weeks and just the lost momentum with mom/teacher busy some, it's expected that we would need a bit of make up time. Glad we had that freedom to spend unlimited time with her at the beginning when we thought we needed it right then!

 

A morning hike with the Edgar family

I think that about wraps up all the facts and details that I know have been updated and changed since the last time I wrote about them. Thank you for all continuing to follow her story and her journey. I can't wait to see where God takes her and I'm thrilled you are along for the ride!


Please continue to share her story, her FB page and her website. So many do not know of Trisomy 18 and it's important to educate. Why? So that eventually medical providers won't discriminate or withhold treatment just because a label has been stamped on their medical record. Not everyone fits in a mold and only God knows His plans for her, not even the best doctor can tell us that. So, how can you help? By sharing this page and sharing her FB page. Let the world...everyone...see the face of Trisomy 18 and let them see that these sweet babies can thrive. Help Everly get the care she needs by transforming the hearts of the medical community and enlightening those (me included at one point) who do not know about Trisomy 18.


Please also pray for Everly's two friends Melanie and Elizabeth. Melanie who also has Trisomy 18 will be having a procedure done this Friday. Elizabeth was born 2 1/2 weeks ago and has Trisomy 18. I haven't heard how she's doing this week but last week she was home and doing well.


With love and appreciation,

Crystal Hopkins